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10 9 8 7 6 5 4 3 2 1
DEDICATION
To Mary Norman Jones, my beloved wife

Mary has been a steadfast and loyal supporter of my various academic pursuits. Their beginnings
can be traced to my first major clinical research project during my neurology residency at Mayo.
There are not enough words to begin to appropriately thank Mary for her love, dedicated
parenting of our four wonderful children, companionship, and encouragement in these various
projects, particularly including my two Netter volumes. Mary also became a friend and admirer
of Frank Netter’s many talents during our frequent sojourns to Palm Beach from 1982 to 1985
while Frank and I were composing the Nervous System Part II.

v
ABOUT THE EDITOR
H. Royden Jones, Jr, MD, the Jaime Ortiz-Patino Chair of Neurology at the Lahey Clinic,
was born in central New Jersey and grew up in North Plainfield and Point Pleasant Beach. He
graduated from Tufts College with a Bachelor of Science degree and, on the advice of his dean
at Tufts, ”went west” to obtain his MD at Northwestern University. While there, he met and
married Mary Elizabeth Norman. Dr Jones served as a rotating intern at the Philadelphia
General Hospital. An internist at Northwestern encouraged him to take his internal medicine
residency at Mayo. During this period, a neurology rotation rekindled his neuroscience inter-
ests, and Dr Jones transferred to Mayo’s neurology program. This experience was interrupted
by a commitment to the United States Army Medical Corps, where he became Chief of
Neurology at the 5th General Hospital, Bad Cannstatt, Germany.
After Dr Jones completed his residency, he moved to Boston to join the Lahey Clinic, where
he has been a member of the neurologic staff since 1972. He has served there in a variety of
administrative roles, including chair of the departments of education and neurology, the per-
sonnel committee, and the divisions of medicine and medical specialties, as well as being a
member of the board of governors.
Neurologic education has always been one of Dr Jones’ major interests. After coming to
Lahey, he was also appointed to the teaching staff of the neurologic unit at Boston City
Hospital, led by a superb educator, Norman Geschwind, MD. Here he received his initial
Harvard appointment, as a clinical instructor. In 1978, based on his training with Ed Lambert,
MD, at Mayo, Dr Jones was asked to develop the pediatric electromyographic laboratory at
Children’s Hospital Boston, where he maintained his Harvard Medical School academic affilia-
tion, leading to his later becoming a Clinical Professor of Neurology.
His initial clinical research interests were in cerebrovascular diseases. However, as his expe-
rience at Children’s Hospital Boston increased, Dr Jones focused his attention on neuromuscu-
lar disorders of children and concomitantly on Lambert-Eaton myasthenic syndrome, stiff man
syndrome, and variants of immune-mediated motor neuropathies in adults.
Dr Jones edited the original Part II, Neurologic and Neuromuscular Disorders, of Netter’s
Nervous System, Volume 1, as well as two other monographs as a primary editor, including
Clinical Pediatric Electromyography with Drs Bolton and Harper and Neurologic Disorders of
Infants, Childhood, and Adolescence with his child-neurology colleagues Drs De Vivo and
Darras. He has served on the American Board of Psychiatry and Neurology in a number of
capacities, most recently completing an 8-year term as an elected director, has been a member
of the Residency Review Committee of the Accreditation Council of Graduate Medical
Education and previously represented the American Academy of Neurology at the American
Association of Medical Colleges.
At the conclusion of 2004, Dr Jones will have completed all of his local and national admin-
istrative responsibilities and plans to return to his primary neurologic interests, seeing patients
in the clinic and electromyography laboratory, while hoping to do more teaching and clinical
research once again. Equally importantly, he plans to spend more time with his family, includ-
ing Mary; his four children, Roy, Kate, Fred, and David; and his grandchildren, Erik, Kristin,
Kendall, and Sam.

H. Royden Jones, Jr, MD


Jaime Ortiz-Patino Chair in Neurology
Lahey Clinic
Burlington, Massachusetts

vii
PREFACE
The spectacular developments within clinical neurology during my lifetime often have been
beyond any reasonable prediction. Consider just a few: One of the most dramatic has been
the successful introduction of new pharmacologic modalities as well as deep brain stimulation
for Parkinson disease; truly miraculous changes occurred for some individuals as portrayed by
Oliver Sachs, MD, in Awakenings. Another is the discovery of the role of autoimmune patho-
physiology in various peripheral and central nervous system disorders, including myasthenia
gravis, and the acquired demyelinating polyneuropathies. This has led to very successful novel
therapeutic intervention that have literally put many patients back on their feet. More in-depth
neurophysiologic techniques have provided for an increased role of surgery for patients with
previously socially devastating seizure disorders, especially in those uncommon scenarios
where the many new and usually effective anticonvulsants have failed to control these events.
In the past decade, the multiple sclerosis enigma has finally been opened with some effective
prophylactic therapies; however, we need more innovative measures that will be effective early
in the disease. Our psychiatric colleagues now possess specific and effective new medications
targeted for depression and the affective disorders as their neurochemistry is being refined.
In neuroradiology, the replacement of direct-puncture carotid angiography and/or pneu-
moencephalography first by computed tomography imaging and now by finely tuned magnet-
ic resonance imaging has led to Nobel recognition for 2 different teams of basic physical scien-
tists. New classes of fine catheters now provide access to the almost microscopic cerebral
arteries, providing the potential for direct infusion of thrombolytic agents or placement of coils
directly into a berry aneurysm. Positron emission tomography scanning now offers another
adjunctive role in clinical research and particularly our understanding of various seizure and
neurodegenerative disorders.
Although spongiform encephalopathy has been recognized for many years, Prusiner’s novel
concept that a prion is the responsible mechanism has led to yet another Nobel award to a
neurologic colleague. However, many challenges remain as there are still no effective therapies
for a number of devastating neurodegenerative and biochemical disorders of the nervous sys-
tem, including these rare prion disorders as well as the much more prevalent dementing disor-
der of Alzheimer disease. Many eagerly await further pathophysiologic elucidation that will
lead to effective therapy. The emergence of the human immunodeficiency virus (HIV) pandem-
ic, with its potential to involve so much of the entire nervous system, has caught us all by sur-
prise; not since syphilis was conquered by penicillin 60 years ago have we witnessed a neuro-
logic disorder with such an overwhelming and devastating predilection for both the central and
the peripheral nervous system. This terrible epidemic has also emphasized the vast capabilities
of our bench researchers at various medical industrial complexes. They have developed novel
therapies, some proving so effective that some of us have not evaluated a new case of neuro-
logic acquired immunodeficiency syndrome (AIDS) in more than 10 years.
Lou Gehrig disease, ie, amyotrophic lateral sclerosis, remains one of our greatest challenges.
This and other neurodegenerative disorders, such as the spinocerebellar ataxias, deprive
patients of their strength and equilibrium, respectively. All neurologists eagerly await resolution
of these complex problems; we hope that these means will be expedited in the near future. To
be successful, our colleagues will undoubtedly be aided by the many discoveries accruing
within the framework of the DNA revolution; these findings may provide great help for our
many patients with genetic disorders, particularly within the framework of child neurology.
My own romance with neurology began as a result of 2 interrelated events during my first
year of medical school at Northwestern University in Chicago. The first was a wonderful

ix
PREFACE
teacher, Ray Snider, PhD, who was a very wise neuroanatomist. He had the pedagogic gift to
make this complex subject intellectually stimulating and clinically relevant. My second was our
introduction to the magnificent artwork of Frank H. Netter, MD, through his initial collection of
Nervous System Illustrations. Dr Netter’s skillful interpretations allowed me to more easily and
clearly conceptualize this most complicated of body systems. Frank Netter soon became one
of my first medical heroes. At that time, it would have never entered my imagination that I
would one day have the opportunity to work closely with Dr Netter, let alone that this would
eventuate in our becoming dear friends. His was a special career that few physicians have suc-
cessfully emulated.
Colleagues have often asked how I came to work with Dr Netter. In 1978, at the American
Medical Association meeting in San Francisco, I serendipitously visited the CIBA (now
Novartis) pharmaceutical booth when I noted in passing that it was promoting Dr Netter’s art-
work. By this time, Frank was the world’s most prestigious medical artist. I naively inquired of
the CIBA representative whether Dr Netter had considered doing a Clinical Symposia on the
mononeuropathies. A few months later, I received a letter from Frank Netter asking me to elab-
orate on my ideas, and soon after, I was sitting in Frank’s Palm Beach studio smoking cigars
with this icon of medical artistry. During these sessions, Frank would have me discuss the vari-
ous clinical entities. Then he would ask for my suggestions as to how he might conceive plates
for the subject. Dr Netter always demanded that he himself fully understand the specific med-
ical issues appropriate to the subject at hand. Together we would decide on the most suitable
venue to illustrate each entity. On an average day, we would get through 12 to 15 potential
subjects, with Frank taking notes for his planned rough drafts that he would later send on to
me. Even though I would have loved to watch him paint, I never asked to do so; from my own
amateurish experience, I knew that this was a very private moment for an artist trying to con-
ceptualize something.
On a return visit, Frank first mentioned to me that he was giving serious consideration to
updating his original nervous system volume; he asked me to join with him in bringing this proj-
ect to completion and asked that I propose a plan to complete this venture by utilizing his pre-
viously completed plates and helping him consider what new art was necessary. Thus was born
the 2-volume Netter Nervous System published in 1986. We hoped to make the clinical vol-
ume relevant for a long time to come by illustrating the basic anatomy, pathophysiology, and
clinical symptomatology of the most common of the many neurologic disorders. My subse-
quent long weekend visits with Dr Netter became a cherished time for both my wife Mary and
me. Frank was a gentleman in the classic definition, a dedicated and passionate artist, and a
superb golfer. It was on the links that he frequently took challenging projects to finalize their
conceptualization.
We usually started our days together at about 7 AM. Although most of the original Netter
plates were designed for ongoing relevance, sometimes new information made them obsolete.
For example, in the mid 1950s, Frank had painted some 20 plates on the thoracic outlet syn-
drome. Then it was thought that this entity was the underlying mechanism for that most com-
mon of all neurologic complaints, ie, intermittent, usually nocturnal, numbness and tingling of
the hands. When electromyography came into early use at the Mayo Clinic and Queens
Square later in that decade, it was then recognized that the primary lesion related to median
nerve entrapment at the wrist, ie, carpal tunnel syndrome; this had nothing to do with cervical
ribs. Frank was very proud of his thoracic outlet syndrome plates and it took a lot of gentle per-

x
PREFACE
suasion to get him to agree not to publish even one of his artistically accomplished but now
scientifically rarely relevant plates.
The resultant text, Nervous System Part II, Neurologic and Neuromuscular Disorders,
became very dated over the past 10 years. This related to the tremendous surge in neuro-
science advances particularly in the “decade of the brain.” Some of these milestones are high-
lighted in an earlier paragraph. When Icon Learning Systems had the good judgment to gain
publication access to the Netter plates and asked me to help with this neurology volume, I
could not have been more thrilled.
As neurology is the finest tuned of the clinical sciences, I wished to infuse life within this vol-
ume by including clinical vignettes in each chapter; many were or are patients of mine and the
remaining are from my Lahey coauthors. In so doing, my colleagues and I wanted to bring spe-
cial relevance to the student of neurology, whether someone very early on in their medical
education, or the resident or fellow, or even the practitioner. We think that this case method
approach, with emphasis on the history and clinical examination, will enlighten the student as
well as even stimulate the most experienced clinician’s interest. Probably a number of the
“patients” presented here will be old friends to our neurologically savvy readers.
Because neurology and neuroscience have been constantly advancing, we needed to
update some of the original classic Netter illustrations as well as to develop a number of new
ones. In this all-important task, my new artistic collaborators John A. Craig, MD, an ophthalmol-
ogist, and Carlos A. G. Machado, MD, a cardiologist, have been superb colleagues who have
followed the pathway that Frank Netter, MD, so spectacularly blazed while still being true to
their own artistic expressions. It has been an honor to get to know these wonderful and
superbly talented artists and physicians.
My colleagues and I hope the readers of this volume will find in it a resource that will be
useful to many neurologic “students” throughout all stages of their professional lives.
Furthermore, we particularly feel that the artwork of Frank Netter will once again come full cir-
cle to inspire yet another generation of medical students to enter the forever stimulating fields
of neuroscience.

H. Royden Jones, Jr, MD


Burlington and Boston, Massachusetts

xi
Frank H. Netter, MD

Frank H. Netter was born in 1906 in New York City. He studied art at the Art
Student’s League and the National Academy of Design before entering
medical school at New York University, where he received his MD degree
in 1931. During his student years, Dr Netter’s notebook sketches attracted
the attention of the medical faculty and other physicians, allowing him to
augment his income by illustrating articles and textbooks. He continued
illustrating as a sideline after establishing a surgical practice in 1933, but he
ultimately opted to give up his practice in favor of a full-time commitment
to art. After service in the United States Army during World War II,
Dr Netter began his long collaboration with the CIBA Pharmaceutical
Company (now Novartis Pharmaceuticals). This 45-year partnership resulted
in the production of the extraordinary collection of medical art so familiar
to physicians and other medical professionals worldwide.

Icon Learning Systems acquired the Netter Collection in July 2000 and
continues to update Dr Netter’s original paintings and to add newly
commissioned paintings by artists trained in the style of Dr Netter.

Dr Netter’s works are among the finest examples of the use of illustration in
the teaching of medical concepts. The 13-book Netter Collection of
Medical Illustrations, which includes the greater part of the more than
20,000 paintings created by Dr Netter, became and remains one of the
most famous medical works ever published. The Atlas of Human Anatomy,
first published in 1989, presents the anatomical paintings from the Netter
Collection. Now translated into 11 languages, it is the anatomy atlas of
choice among medical and health professions students the world over.

The Netter illustrations are appreciated not only for their aesthetic qualities
but, more importantly, for their intellectual content. As Dr Netter wrote in
1949, “. . . clarification of a subject is the aim and goal of illustration. No
matter how beautifully painted, how delicately and subtly rendered a
subject may be, it is of little value as a medical illustration if it does not
serve to make clear some medical point.” Dr Netter’s planning, conception,
point of view, and approach are what inform his paintings and what makes
them so intellectually valuable.

Frank H. Netter, MD, physician and artist, died in 1991.


AABCKOUT
CKNOWLEDGMENTS
THE EDITORS

It is very important for me to be able to pay tribute to some of my teachers, first and fore-
most my loving Mom and Dad, who always encouraged my academic interests. In high school,
I found that one special teacher, Albert A. Surina; it was his chemistry course that stimulated
me to seek a career in science and not the law. My family internist Leonard Williams, MD, also
took a special interest in my career when I entered Tufts as an undergraduate; “Doc Willie”
gave me the necessary confidence to consider a career in medicine. Our organic chemistry
professor at Tufts, “Pop” Dolman, turned this subject into the most intellectually stimulating of
my college career; in a way, his approach to teaching, this most important of the premed
courses, set the stage for the reasoning process so essential to the differential diagnosis a
neurologist entertains each time she or he takes a history. Clifton Emery, PhD, Tufts’ Dean,
encouraged me to consider Northwestern. Instead of “becoming a provincial New Englander,”
he told me to expand my horizons.
At Northwestern University medical school, Ray Snider, PhD, our teacher of neuroanatomy,
was the initial preclinical scientist to bring clinical medicine into the first-year classroom. His
challenging and thoughtful lectures and examinations made the nervous system take a life of
its own for me, and I began to consider a career in this field. Later, the opportunity to study
with Martin Brandfonbrenner, MD, a brilliant young cardiologist with whom I was privileged to
spend almost an entire year, introduced me to clinical research and didactic teaching rounds at
their best. Neurology took a back seat in my career plans. Lastly, a clinical internist at Evanston
Hospital, Dr Franklin Kiser, was the one who encouraged me to go to Mayo, and there my
medical career became truly blessed.
Mayo was endowed with so many fine clinicians and clinical researchers I will certainly be
remiss when I mention just a few. At the beginnings were Peter J. Dyck, MD, and Kendall
Corbin, MD, PhD, who were my first neurology attendings, or visits, as they say here in Boston.
In January 1965, having had almost 3 years of internal medicine, I was fully committed to
becoming a cardiologist. However, a 6-week rotation on the hospital neurology service was so
stimulating with Dr Corbin that he encouraged me to speak with Robert G. Siekert, MD, about
a neurologic career. Thus, my initial medical school interests were vitally reactivated.
Once this decision was reached, I had the good fortune to work with many other masters of
the neurology profession. Some of the foremost being Drs Donald Mulder, Norman Goldstein,
and Bill Karnes as well as Don Klass and Ed Lambert in their renowned neurophysiologic labo-
ratories. Most influential was Dr Bob Siekert, who has continued to be a dear friend as well as
an early collaborator. Peter Dyck is still available for phone consultations in addition to reading
all our sural nerve biopsies some 40 years later. One could never have dreamed of having such
wonderful role models.
I wish to pay special thanks to my Lahey colleagues who have been so supportive over the
years. This includes Steve Kott, MD, our chair for more than 2 decades; Steve Freidberg, MD,
chair of neurosurgery; his predecessor, Charles Fager, MD; and Dick Baker, MD, Lahey’s first
neuroradiologist, who has been a major help as a contributor of all neuroradiographic images
for both the Nervous System Part II and this volume. Our former chief executive officer Robert
Wise, MD, supported an all-too-spirited young neurologist through thick and thin, in particular
making it possible for me to found the electromyography laboratory at Children’s Hospital
Boston, when I was recruited there in 1979, while maintaining a full time appointment at
Lahey. It has been a great pleasure to see our neuroscience efforts grow so strongly here over
these 3-plus decades. The continued support and friendship of our outstanding current chief
executive officer at Lahey, David Barrett, MD, is also greatly appreciated. Many others deserve

xiii
ACKNOWLEDGMENTS
mention, but space is short, and so to those dear colleagues all I can say is many thanks, the
trip has been a ball!
Very importantly, I will forever be indebted to my dear friend, Jaime Ortiz-Patino, who
underwrote the Jaime Ortiz-Patino Chair in Neurology at Lahey for me. This Medici-like gift has
made it possible for me to have time for this second venture with Frank Netter, albeit in absen-
tia this time since Frank’s death in 1991. No one could be more fortunate. I feel truly blessed to
have had the tremendous collaboration of my many Lahey Clinic neuroscience colleagues to
bring this latest Netter venue to fruition.
Additionally, I wish to acknowledge Susan Gay, Jennifer Surich, Carolyn Kruse, Tamara
Myers, Jennifer Withers, and Greg Otis at Icon Learning Systems who have so faithfully worked
on the nuts and bolts of this venture. It has been a pleasure to have the opportunity to be col-
leagues with my new artist heroes, John Craig, MD, and Carlos Machado, MD, to whom I owe
many, many thanks. It has been an honor to work with them as well as much fun; I hope that
we can collaborate in the future.
Lastly, I wish to thank Carol Spencer for her special help with this project, as well as her
ongoing dedication to all of the physicians at Lahey. Carol has continually made it easier for us
to accomplish our clinical research projects by creating a wonderfully accessible medical
library for many years. In addition, I wish to acknowledge the many contributions of Joyce
Royston, Carol’s able assistant in our medical library.

H. Royden Jones, Jr, MD


Burlington and Boston, Massachusetts

xiv
CONTRIBUTORS
All of the contributors are associated with the Lahey Clinic Medical Center unless otherwise noted.

Editor Monique M. Ryan, MB BS, MMed, FRACP


Former Fellow in Clinical
H. Royden Jones, Jr, MD
Neurophysiology/Electromyography and
Emeritus Chairman, Department of Neurology
Neuromuscular Disorders
and Division of Medical Specialties
Senior Lecturer
Clinical Professor of Neurology, Harvard
Discipline of Pediatrics and Child Health
Medical School
University of Sydney
Director EMG Lab, Children’s Hospital Boston
Jayashri Srinivasan, MBBS, PhD, MRCP
Associate Editor Department of Neurology
Richard A. Baker, MD Yuval Zabar, MD
Diagnostic Radiologist Director, Center for Memory and Cognitive
Lecturer on Radiology, Harvard Medical Disorders
School Department of Neurology
Associate Clinical Professor of Radiology, Tufts
University School of Medicine Contributing Authors
Assistant Editors Lloyd M. Alderson, MD, DSc
Department of Neurosurgery
Gregory Allam, MD
Department of Neurology Timothy D. Anderson, MD
Ted M. Burns, MD Senior Staff
Assistant Professor Department of Otolaryngology
Department of Neurology Diana Apetauerova, MD
University of Virginia Department of Neurology
Ann Camac, MD Jeffrey E. Arle, MD, PhD
Department of Neurology Director, Functional Neurosurgery
Claudia J. Chaves, MD Department of Neurosurgery
Director of Multiple Sclerosis Clinic
Peter J. Catalano, MD, FACS
Department of Neurology
Chairman, Department of Otolaryngology
Stephen R. Freidberg, MD Associate Professor of Otolaryngology, Boston
Chair, Department of Neurosurgery University
Kinan K. Hreib, MD, PhD Ellen Choi, BS
Director Stroke Service Senior Medical Student, Tufts University
Assistant Clinical Professor, Tufts University Medical School
James A. Russell, DO Department of Otolaryngology,
Director of Muscular Dystrophy Head and Neck Surgery
Association Clinic
Vice Chairman, Department of Neurology

xv
CONTRIBUTORS
Donald E. Craven, MD N. George Kasparyan, MD, PhD
Chair, Department of Infectious Diseases Director of Hand Surgery
Professor of Medicine, Tufts University Assistant Professor of Orthopedics, Boston
School of Medicine University, School of Medicine

Carlos A. David, MD Kenneth Lakritz, MD


Director Cerebrovascular Surgery Department of Psychiatry and Behavioral
Co-Director Skull Base Surgery Medicine
Department of Neurology Marie C. Lucey, BS, PT
Clinical Assistant Professor, Tufts University Physical Therapist
School of Medicine Center for Balance and Mobility, Gordon
College
Peter K. Dempsey, MD
Department of Neurosurgery Subu N. Magge, MD
Department of Neurosurgery
Francesco G. De Rosa, MD
Assistant Clinical Professor Steven W. Margles, MD
Infectious Diseases, University of Turin Department of Orthopaedic Surgery, Section
of Hand Surgery
Robert A. Duncan, MD, MPM Assistant Clinical Professor, Department of
Senior Staff Physician Orthopaedic Surgery, Boston University
Center for Infections Diseases
John Markman, MD
Jennifer A. Grillo, MD Department of Anesthesiology and
Former Fellow in Clinical Neurology
Neurophysiology/Electromyography and
Neuromuscular Disorders Ippolit C. A. Matjucha, MD
Staff Neurologist, Holy Family Hospital Neuro-ophthalmologist
Department of Ophthalmology
Paul T. Gross, MD
Chair, Department of Neurology Daniel P. McQuillen, MD
Clinical Professor of Neurology, Tufts Department of Infectious Disease
University Assistant Professor, Tufts University School of
Medicine
Jose A. Gutrecht, MD, MSc
Department of Neurology Michael P. McQuillen, MD, MA
Professor of Neurology and of the Medical
Aaron C. Heide, MD Humanities
Director of Stroke Services
University of Rochester, School of Medicine
Department of Neurology
and Dentistry
Valley Medical Center
Eva M. Michalakis, MS CCC-SLP
Alice A. Hunter, MD
Clinical Director of Speech, Voice,
Department of Orthopaedic Surgery
Swallowing
Allison Gudis Jackson, MS CCC-SLP Department of Otolaryngology
Speech-Language Pathologist
Mary Anne Muriello, MD
Department of Otolaryngology
Department of Neurology
Edward R. Jewell, MD Associate Professor of Clinical Neurology,
Chair, Department of Vascular Surgery Tufts University School of Medicine

xvi
CONTRIBUTORS
Winnie Ooi, MD, MPH, DMD Robert J. Updaw II
Travel and Tropical Medicine Clinic Senior Medical Student, Medical School of
Department of Infectious Diseases Ohio
Joel M. Oster, MD Michal Vytopil, MD
Director of Epilepsy and EEG
Fellow in Clinical Neurophysiology/
Department of Neurology
Electromyography and Neuromuscular
E. Prather Palmer, MD Disorders
Staff Physician
Department of Neurology
Department of Neurology
Kevin B. Raftery, MD Harold J. Welch, MD
Department of Vascular Surgery Senior Vascular Surgeon
Department of Vascular Surgery
Edward Tarlov, MD
Department of Neurosurgery Judith White, MD, PhD
Eric T. Tolo, MD Head, Section of Vestibular and Balance
Orthopaedic Surgeon Disorders
Division of Hand and Upper Extremity Surgery Head and Neck Institute, The Cleveland
Department of Orthopaedic Surgery Clinic

xvii
Chapter 1

Clinical Neurologic Evaluation


Kinan K. Hreib and H. Royden Jones, Jr

Neurology is a rewarding, intellectually demanding clinical discipline involving careful history tak-
ing, detailed patient examination, formulation of diagnostic evaluations, and development of treat-
ment and supportive plans for patients. The most important encounter with a patient is the initial ex-
amination, during which the physician should endeavor to build trust and encourage the patient to
communicate openly. This chapter provides the information that forms the basis of the performance
of detailed neurologic evaluations.

NEUROLOGIC HISTORY consuming, the history is the most important fac-


An accurate history requires attention to de- tor leading to accurate diagnoses.
tail, reading the patient’s body language, and in- Unfortunately, the economics of modern
terviewing family members and, sometimes, wit- health care has forced primary care physicians to
nesses to patient’s difficulties. History taking is shorten encounters with patients and their fami-
an art even more time consuming than a com- lies. Many physicians use tests as substitutes for
plete, careful neurologic examination. Some pa- careful clinical evaluation. The combination of
tients have seen one neurologist before they the detailed medical information available on
seek another’s opinion. To prevent bias, a neu- the Internet and the adversarial medical-legal en-
rologist should not read others’ notes or look at vironment have enhanced patients’ knowledge
previous neuroimages before taking the history bases, although not always in a balanced way.
and performing the examination. Patient expectations sometimes affect the diag-
One of the most important attributes of a skill- nostic approach of physicians. In this environ-
ful neurologist is the ability to be a good listener ment, it is not surprising that imaging techniques
so as not to miss crucial historic points. It is im- such as MRI and CT have replaced or supple-
portant to begin the initial meeting by asking pa- mented a significant portion of clinical judg-
tients why they have come; this offers them the ment. However, even the most dramatic test
opportunity to express concerns in their own findings may prove irrelevant without appropri-
words. If possible, the neurologist should not in- ate clinical correlation. To have patients unnec-
terrupt, so that the patient may provide the in- essarily undergo surgery because of MRI find-
formation they deem of greatest importance. ings that have no relation to their complaints
Rarely, anxious or compulsive patients may may lead to a tragic outcome. Therein lies the im-
speak of their concerns at great length; with ex- portance of gaining complete understanding of
perience, physicians learn to use interjections to the clinical issues.
maintain control of the evaluation and draw the Although neurology may seem in danger of
patient back from tangents. being subsumed by overreliance on highly
When the patient’s primary concerns have sophisticated tests, these studies have improved
been established, specific issues can be ex- diagnostic skills and choice of therapy. For ex-
plored. Additionally, making careful observa- ample, much knowledge regarding the early
tions during the history review allows better fo- recognition, progression, and response to treat-
cus for subsequent questions. An accurate ment of multiple sclerosis (MS) depends on care-
assessment of mental status and language can ful MRI imaging.
be obtained from listening to the patient and ob- It is essential to make patients feel com-
serving responses to questions. Although time- fortable in the office, particularly by fostering a

NEUROLOGIC EXAMINATION
2
CLINICAL NEUROLOGIC EVALUATION

positive interpersonal relationship. Taking time failure to undress a patient with numb hands
to ask about patients’ lives, education, and social may deny the examining physician the opportu-
habits often provides useful clues. A careful set nity to elicit an unexpected positive Babinski re-
of questions providing a general review of sys- sponse or even a spinal cord level indicative of a
tems may lead to the key diagnostic clue that fo- myelopathy and not a peripheral neuropathy.
cuses the evaluation. When the patient develops This omission may delay recognition that the
a sense of confidence and rapport with an em- problem lies at the level of the CNS, in the spinal
pathetic physician, he or she is more willing to re- cord or brain.
turn for follow-up, even if a diagnosis is not made As clinicians mature, they learn to eliminate or
at the initial evaluation. Sometimes a careful sec- put into perspective information and findings ir-
ond or third examination reveals a crucial differ- relevant to current problems. Although a full
ence that leads to a specific diagnosis. If patients neurologic examination may not be necessary in
feel rushed on their first visit, they may not return every patient, a thorough examination can be
for follow-up, thus denying the neurologist a used to establish a future baseline for each pa-
chance at crucial diagnostic observations. The tient. Noting a patient’s slightly asymmetric
physician-patient relationship must always be smile, somewhat irregular pupils, or hint of ptosis
carefully nourished and highly respected. is important. Even though these findings may
have no bearing on the issue, their notation may
prove helpful to the patient later. This is espe-
AN APPROACH TO THE
cially true if another neurologist calls for patient
NEUROLOGIC EVALUATION
records for comparison or if the patient returns
Throughout training, examination skills are
with another clinical concern.
continually being amplified, based on ever-
evolving clinical experience and observation of
varied approaches of academic physicians to dif- Basic Tenets
ferent types of patients. It is essential to interpre- The examination begins the moment the pa-
tation of the neurologic examination to under- tient walks down the hall to enter the neurolo-
stand what is normal at different ages as well as gist’s office; it continues during the conversa-
learning how to elicit important, sometimes sub- tions requisite to history taking. By the end of the
tle clues to diagnosis. examination, the findings must be able to be cat-
One of the most intellectually challenging egorized and organized for easy interpretation
aspects of neurology relates to the multiple and, eventually, diagnostic formulation. Many of
potential neuroanatomical sources for many the patient’s cognitive abilities are readily as-
complaints. Therefore, although carpal tunnel sessed during the history as well as the formal
syndrome is the most common cause for a numb neurologic examination. Concurrently, the neu-
hand, be certain not to overlook other anatomi- rologist is always looking for abnormal clinical
cal sites, such as at the level of the brachial signs. Some are overt movements (tremors, rest-
plexus, cervical nerve root, spinal cord, or brain. lessness, dystonia or dyskinesia); others are sub-
Essential to the practice of neurology is gain- tler, eg, vitiligo, implying a potential for a neuro-
ing as much information as possible in patients logic autoimmune disorder. Equally important
presenting with unusual or unfamiliar neurologic may be the lack of normal movements, as seen
complaints. A carefully obtained history and ex- in patients with Parkinson disease.
amination is essential to discerning the eventual The examination is divided into several sec-
diagnosis and exploring treatment options. A tions. Speech and language are assessed during
hasty history and examination can be mislead- the history taking. The cognitive part of the
ing. For example, a diagnosis of early MS may be examination is the most time consuming and
missed by not asking about such things as previ- complicated (chapter 2). However, unless a cog-
ous problems with visual function, shooting elec- nitive or language dysfunction is suspected, the
tric paresthesiae when bending the neck (Lher- multisystem neurologic examination provides a
mitte sign), or sphincter problems. Alternatively, careful basis for the essential clinical evaluation.

NEUROLOGIC EXAMINATION
3
CLINICAL NEUROLOGIC EVALUATION

Neurologists in training and their colleagues in mechanical function of speech. Last, CN-XI, the
practice cannot expect to test all possible cogni- accessory, contains both cranial and spinal
tive elements in each patient evaluated. Certain nerve roots that provide motor innervation to
basic elements are required, most of which are the large muscles of the neck and shoulder.
readily observable or elicited during initial clini- Disorders of the CNs can be confined to a sin-
cal evaluation. These include documentation of gle nerve such as the olfactory (from a closed
language function, affect, concentration, orien- head injury or meningioma), trigeminal (tic
tation and memory. When concerned about the douloureux), facial (Bell palsy), acoustic
patient’s cognitive abilities, the neurologist must (schwannoma), and hypoglossal (carotid dissec-
elicit evidence of an apraxia or agnosia and test tion). However, a single lesion, such as within the
organizational skills, constructional ability, calcu- median longitudinal fasciculus, as seen with MS,
lation abilities, and any tendency to neglect or, less commonly, a small brainstem stroke can
anatomical structures, particularly nondominant lead to involvement confined to the nerves sup-
limbs or visual fields. plying multiple extraocular muscles.
When language and cognitive functions have Additionally, a subset of systemic disorders has
been assessed, gait and equilibrium, visual fields, the potential to infiltrate or seed the base of the
cranial nerves (CNs) (Figure 1-1), coordination, brain and the brainstem at the points of exit of the
muscle strength, muscle stretch reflexes (MSRs), various CNs from their intraaxial origins. These
plantar stimulation, and sensory modalities processes lead to a clinical picture of multiple,
should be examined in an organized fashion. sometimes disparate cranial neuropathies, includ-
The patient’s general health, nutritional status, ing leptomeningeal seeding of metastatic malig-
and cardiac function, including the presence or nancies, such as in the lung, breast, and stomach,
absence of significant arrhythmia, heart murmur, as well as various lymphomas. In these instances,
or signs of congestive failure, should be noted. If a stuttering onset of various symptoms related to
the patient is encephalopathic, it is important to each CN may develop within a short time. Com-
search for subtle signs of infectious, hepatic, re- mon problems that bring patients to medical at-
nal, or pulmonary disease. tention include diminished visual acuity (optic
nerve), diplopia (oculomotor, abducens), facial
THE CRANIAL NERVES: AN pain (trigeminal), an evolving facial weakness (fa-
INTRODUCTION cial), difficulty swallowing (glossopharyngeal and
The 12 CNs subserve multiple types of neuro- vagus), and slurred speech (hypoglossal).
logic function (Figure 1-2). The special senses are Similarly, some chronic granulomatous infec-
represented by all or part of the function of 5 tions, such as tuberculosis and Lyme disease, or
CNs: olfaction, the olfactory (I); vision, the optic infectiouslike processes, including sarcoidosis,
(II); taste, the facial (VII) and the glossopharyn- also require diagnostic consideration in patients
geal (IX); and hearing as well as vestibular func- with multiple or isolated cranial neuropathies.
tion, the cochlear and vestibular (VIII). Another 3 The latter is particularly relevant to patients pre-
CNs are directly responsible for the coordinated, senting with Bell palsy because sarcoidosis and
synchronous, and complex movements of both Lyme disease have a predilection for CN-VII.
eyes: III (oculomotor), IV (trochlear), and VI (ab- When a facial paresis has an atypical nonacute
ducens). The primary CN responsible for facial and progressive temporal profile, a search for fo-
expression is CN-VII, which is important for set- cally invasive malignancies, particularly of the
ting the outward signs of immediate emotional parotid gland, is essential.
bearing. Facial sensation is subserved primarily
by the trigeminal nerve (V); however, it is a CRANIAL NERVE TESTING
mixed nerve also having a primary motor contri- I: Olfactory Nerve
butions to mastication. The ability to eat and In a few specific circumstances, olfactory nerve
drink also depends on CNs IX, X (vagus), and XII function testing is relevant despite its only occa-
(hypoglossal). The hypoglossal and recurrent sional clinical indications. Olfactory function may
laryngeal nerves are also important to the be impaired after head trauma and in individuals

NEUROLOGIC EXAMINATION
4
CLINICAL NEUROLOGIC EVALUATION

Figure 1-1 Cranial Nerves: Distribution of


Motor and Sensory Fibers
Motor fibers
Sensory fibers

III
Oculomotor
(all eye muscles except
I II those below; also ciliary,
Olfactory Optic iris, sphincter) .
hth
Op
IV
Trochlear Motor to
Superior oblique muscles of
.
ax mastication
M
VI V
Abducent Trigeminal nd.
Ma
Lateral rectus Sensory to face
sinuses, teeth, etc

Intermediate nerve
VII Motor—submaxillary,
Facial sublingual, lacrimal glands
Muscles of face Sensory—anterior 2⁄3 of
tongue, soft palate

VIII
Vestibulocochlear
Cochlear Vestibular

IX
Glossopharyngeal
Sensory—posterior 1⁄3 of tongue,
tonsil, pharynx, middle ear
Motor—stylopharyngeus, pharyn-
geal musculature

X
Vagus
XI Motor—pharynx, heart, lungs,
XII Accessory bronchi, GI tract
Hypoglossal Sternocleido- Sensory—heart, lungs,
Tongue mastoid, trapezius bronchi, trachea, larynx,
muscles pharynx, GI tract,
external ear

Strap
muscles

NEUROLOGIC EXAMINATION
5
CLINICAL NEUROLOGIC EVALUATION

Figure 1-2A Cranial Nerves: Nerves and Nuclei Viewed in Phantom From Behind
Superior (cranial) colliculus Oculomotor (III) n.
Relay centers for Red nucleus
fibers in optic tract Accessory oculomotor
Lateral geniculate body (Edinger-Westphal) nucleus
Oculomotor nucleus
Mesencephalic nucleus
of trigeminal n. Trochlear nucleus
Trochlear (IV) n.
Trigeminal (V) n. and ganglion
Principal (pontine) sensory Trigeminal (V) n.
nucleus of trigeminal n. and ganglion
Facial (VII) n. Motor nucleus of
trigeminal n.
Vestibulocochlear (VIII) n.
Abducent nucleus
Geniculate ganglion
Ventral cochlear of facial n.
nucleus
Facial nucleus
Dorsal cochlear nucleus Superior and inferior
Vestibular nuclei salivatory nuclei
Glossopharyngeal (IX) n. Nucleus ambiguus
Vagus (X) n. Dorsal vagal nucleus
Glossopharyngeal (IX) n.
Spinal tract and spinal
nucleus of trigeminal n. Hypoglossal nucleus
Solitary tract nucleus Accessory (XI) n. Vagus (X) n.
Dorsal vagal nucleus Spinal nucleus
Gracile nucleus of accessory n.

Figure 1-2B Cranial Nerves: Schema of Nerves and Nuclei in Lateral Dissection
Red nucleus Accessory oculomotor
(Edinger-Westphal) nucleus
Oculomotor (III) n.
Oculomotor nucleus
Mesencephalic nucleus
of trigeminal n. Trochlear nucleus
Trigeminal (V) n. Trochlear (IV) n.
and ganglion
Abducent nucleus
Principal (pontine) sensory
nucleus of trigeminal n. Facial nerve loop
Motor nucleus of trigeminal n.
Facial nucleus
Spinal tract and spinal
nucleus of trigeminal n. Vestibular nuclei

Facial (VII) n. Ventral and dorsal


cochlear nuclei
Vestibulocochlear (VIII) n.
Superior and inferior
Abducent (VI) n. salivatory nuclei
Glossopharyngeal (IX) n. Solitary tract nucleus
Hypoglossal (XII) n.
Dorsal vagal nucleus
Vagus (X) n.
Efferent fibers Hypoglossal nucleus
Accessory (XI) n.
Afferent fibers Nucleus ambiguus
Spinal nucleus of accessory n.
Mixed fibers

NEUROLOGIC EXAMINATION
6
CLINICAL NEUROLOGIC EVALUATION

with various causes of frontal lobe dysfunction, entiate between 1 or 2 fingers. Most patients in-
especially an olfactory groove meningioma. advertently look laterally at the fingers. The pa-
Clinical evaluation of olfactory functions is tient’s attention must be refocused, and the test
straightforward. The examiner has patients sniff a is repeated. Each quadrant of vision is tested sep-
substance of familiar odor (coffee beans, leaves of arately. After individual testing, both eyes are
peppermint, lemon). Ideally, patients keep the tested simultaneously for visual neglect, as may
eyes closed, and each nostril is tested separately occur with right hemispheric lesions (Figure 1-4).
while the other is occluded with a finger. Subse- Progressively complex perimetric devices can
quently, patients are asked whether they detect an produce rich data on the health of the visual sys-
odor and, if so, to identify it. Important conclusions tem. In kinetic perimetry, a stimulus is moved
are drawn from this simple test: inability or re- from a nonseeing area (far periphery or physio-
duced ability to detect an odor suggests anosmia logic blind spot) to a seeing area, with patients in-
or hyposmia, respectively; inability to identify an dicating at what point the stimulus is first noticed.
odor correctly implies smell distortion (parosmia Testing is repeated from different directions until
or dysosmia); and whether an olfactory distur- a curve can be drawn connecting the points at
bance is bilateral or unilateral, ie, whether the pa- which a given stimulus is seen from all directions.
tient has a total loss of smell or only loss on 1 side. This curve is the isopter for that stimulus for that
Olfactory nerve damage can have significant eye. The isopter plot has been likened to a con-
consequences. When individuals lose their tour map, showing “the island of vision in a sea of
sense of smell, their important ability to smell darkness.” The Goldmann perimeter, a half-
fires or burning food is compromised. sphere onto which spot stimuli are projected, is
the premiere device for this mapping. The normal
II: Optic Nerve visual field extends approximately 90° tempo-
Blurred vision, a relatively nonspecific symp- rally, 45° superiorly, 55° nasally, and 65° inferi-
tom, is the most common problem resulting orly, resembling the oblique teardrop shape of
from optic nerve dysfunction (Figure 1-3). When aviator-style sunglass lenses.
examining optic nerve function, one needs to In static perimetry, the test point is not
look first for concomitant ocular abnormalities moved, but turned on in a specific location. Typ-
that may affect vision, including proptosis, pto- ically automated, computer testing preselects lo-
sis, scleral injection (congestion), tenderness, cations within the central 30° of field. Stimuli are
bruits, and pupillary changes. dimmed until they are detected only intermit-
Visual acuity is screened using a standard tently on repetitive presentation—this intensity
Snellen vision chart held 14 inches from the eye. level is called the threshold. The computer then
Screening must be performed in light to the pa- generates a map of numeric values of the illumi-
tient’s refractive advantage using corrective nation level required at every test spot, or the in-
lenses or a pinhole when indicated. verse of this level, often called a sensitivity value.
A careful visual field evaluation is the other im- Values may also be displayed as a grayscale
portant means to assess visual function. These map, and statistical calculations can be per-
tests are complementary, one testing central res- formed—by comparing to adjacent spots or pre-
olution at the retinal level and the other an eval- calculated normal values or noting sudden
uation of peripheral visual field detection sec- changes in sensitivity—to detect abnormal areas.
ondary to lesions at the levels of the optic Most visual field changes have localizing
chiasm, optic tracts, and occipital cortex. Visual value: specific location of the loss, its shape, bor-
fields are evaluated most simply by having the der sharpness (ie, how quickly across the field
patient sit comfortably facing the examiner, who the values change from abnormal to normal),
faces the patient at a similar height. First, each and its concordance with the visual field of the
eye is tested independently. The patient is asked other eye tend to implicate specific areas of the
to look straight at the examiner’s nose. The ex- visual system. Localization is possible because
aminer extends an arm laterally, equidistance details of anatomical organization at any level
from the patient, and asks the patient to differ- predispose to particular types of loss.

NEUROLOGIC EXAMINATION
7
CLINICAL NEUROLOGIC EVALUATION

Figure 1-3 Autonomic Innervation of Eye


Sphincter of pupil Oculomotor n. root of Accessory oculomotor
ciliary ganglion (motor) (Edinger-Westphal)
Dilator of pupil nucleus (autonomic)

Ciliary m. Oculomotor
Short ciliary nn. (III) n.

Ciliary
ganglion

Nasociliary n.
Middle
Optic (II) n. Ophthalmic n. ear

Long ciliary n. Trigeminal Thoracic


ganglion part of Tecto-
Nasociliary n. root spinal
of ciliary ganglion spinal
cord tract
Internal
Sympathetic root of ciliary ganglion carotid plexus

Ophthalmic a.
Internal carotid a.

Superior cervical sympathetic trunk ganglion

1st thoracic sympathetic trunk ganglion

White ramus communicans


T1
Gray ramus communicans

Presynaptic
Sympathetic fibers
Postsynaptic

Presynaptic
Postsynaptic Parasympathetic fibers T2

Afferent fibers

Visual pathway
T3
Descending pathway

NEUROLOGIC EXAMINATION
8
CLINICAL NEUROLOGIC EVALUATION

Figure 1-4 Visual Pathways: Retina to Occipital Cortex

Central darker circle


represents macular zone.
Overlapping Lightest shades represent
visual fields monocular fields.
Each quadrant is
a different color.

Projection on Projection on
left retina right retina

Optic (II)
nerves
Optic
chiasm
Projection on left Projection on right
dorsal lateral dorsal lateral
geniculate nucleus Meyer Optic Meyer geniculate nucleus
Ipsilateral Ipsilateral
loop tracts loop
6 6
5 5
4 Lateral 4
3 geniculate 3
2 bodies 2
1 1

Contralateral Contralateral

Projection on left
occipital Projection on right
lobe occipital
lobe

Calcarine
fissure

In examining the pupils, one should note the convergence of the eyes, as when patients are
shape and size. A 1-mm size difference in other- asked to look at their nose.
wise round pupils is acceptable as a normal vari- The ciliospinal reflex is useful for evaluating
ant. Pupillary responses are tested with a bright comatose patients. When the examiner pinches
flashlight. A normal pupil reacts to light by con- the neck, the ipsilateral pupil should transiently
stricting. Normally, the contralateral pupil also dilate. This provides a means to test the integrity
constricts. These responses are called the direct of neuropathways to midbrain structures.
and consensual reactions, respectively. The The short ciliary nerve, supplying parasym-
pupils also constrict when shifting focus from a pathetic inputs to the pupil, may be damaged
far to a near object (accommodation) and during by various forms of trauma. This results in a

NEUROLOGIC EXAMINATION
9
CLINICAL NEUROLOGIC EVALUATION

unilateral dilated pupil with preservation of other lodilator musculature of the eye. Typically, pa-
third nerve function. Significant unilateral pupil- tients with Horner syndrome have an ipsilateral
lary abnormalities are usually related to innerva- loss of sweating in the face, a sympathetic paralysis
tion changes in pupillary muscles. with subsequent miosis (constricted pupil), and
A number of pathophysiologic mechanisms ptosis from loss of smooth muscle innervation. The
lead to mydriasis (pupillary dilatation) (Table levator palpebra, a striated muscle innervated by
1-1). Atropinelike eye drops, often used for their the oculomotor nerve CN-III, is not affected.
ability to produce mydriasis, are important (See Horner syndrome image in Figure 20-6B.)
mechanisms that are occasionally overlooked as
potential causes of otherwise asymptomatic, di- Optic Fundus
lated, poorly reactive pupils. Other medications A careful optic funduscopic examination is
may cause certain atypical reactions to light. Bi- essential. This evaluation is best performed in a
laterally dilated pupils, in otherwise neurologi- relatively dark environment to increase pupil-
cally intact patients, likely do not reflect signifi- lary size and improve contrast of the posterior
cant pathology and are probably related to chamber structures. Findings that should be
medications. Parasympathomimetic drugs, such documented include margins of the optic
as those typically used to treat glaucoma, cause nerve, venous pulsations, hemorrhages, exudates,
prominent pupillary constriction. any obvious obstruction to flow by embolic mate-
rial (such as cholesterol plaque in patients com-
Horner Syndrome plaining of transient visual obscuration), and
In Horner syndrome, the affected pupil is typ- pallor of retinal fields that may reflect ischemia.
ically constricted from interference with the sym- Papilledema is characterized by elevation
pathetic nerves at one of many levels. The sym- and blurring of the optic disk, absence of venous
pathetic efferents originate within the pulsations, and hemorrhages adjacent to and on
hypothalamus and traverse the brainstem and the disk (Figure 1-5). The finding of papilledema
cervical spinal cord to reach the superior cervi- may be consistent with anything from increased
cal ganglia, where they subsequently accom- intracranial pressure, including brain tumors,
pany the carotid artery in the neck, proceeding subarachnoid hemorrhage, and metabolic
intracranially to eventually innervate the pupil- processes, to pseudotumor cerebri.

Table 1-1
Pupillary Abnormalities*
Eponym

Argyll Robertson Horner Hutchinson Holmes Adie

Response to light None Yes None None

Other responses Converge Yes NA Accommodate

Margins Irregular Regular Regular Regular

Associated changes Iris depigmented Ptosis/anhydrosis Ptosis Loss of MSR

Causes Tabes dorsalis Carotid dissect III nerve Ciliary ganglion


Carotid aneurysm compression
Pancoast tumor
Syringomyelia

Anatomy Unknown Loss of sympathetic Loss of Loss of


parasympathetic parasympathetic
*MSR indicates muscle stretch reflex; NA, not applicable.

NEUROLOGIC EXAMINATION
10
CLINICAL NEUROLOGIC EVALUATION

Figure 1-5 Effects of Increased Intracranial Pressure on Optic


Disk and Visual Fields

Optic fundus with papilledema

Visual field changes with enlarged blind


spots secondary to chronic papilledema

III, IV, VI: Oculomotor, Trochlear, and horizontal conjugate gaze circuits. The medial
Abducens Nerves longitudinal fasciculus connects CN-III on one
Testing each eye individually is less confusing side and CN-VI on the opposite side. Under-
and helps to identify isolated ocular dysfunction. standing the circuit of horizontal conjugate gaze
It is best to describe the observed loss of specific helps clinicians to appreciate the relation be-
function, ie, an abduction paresis as opposed to tween the frontal eye fields and the influence it
CN-VI palsy, because it is possible that the dys- exerts on horizontal conjugate gaze as well the
function is partial or is not related to CN abnor- reflex relation between the ocular and vestibular
malities. In testing extraocular muscle function, systems (Figure 1-6).
the patient is instructed to follow the examiner’s The connection of the vestibular system to the
finger without allowing head movement. Gently medial longitudinal fasciculus can be tested by
touching the patient’s forehead may remind the cold-water calorics or by the doll’s eye maneu-
patient to keep the head still. ver, wherein the head is rotated side to side
The medial longitudinal fasciculus is responsi- while the examiner watches for rotation of the
ble for controlling extraocular muscle function eyes. Rotation of the head to the left normally
because it provides a means to modify central moves the eyes in the opposite direction, with

NEUROLOGIC EXAMINATION
11
CLINICAL NEUROLOGIC EVALUATION

Figure 1-6 Control of Eye Movements

Excittory endings
Inhibitory endings Fiontal eye fields
Indeterminate endings (Brodmann area 8)

Interstitial
nucleus of
Cajal

Occipital eye
fields (Brodmann
areas 17, 18, 19)

Superior
Superior rectus m.
colliculus
Superior
oblique m.
Oculomotor
nucleus Medial
rectus m.

Abducens Oculomotor (III) n.


internuclear
neuron Trochlear (VI) n.

Cortico-
Trochlear reticular
nucleus fibers

Medial Ascending
longitudinal tract of
fasciculi Deiters
Abducens
nucleus

Superior
Medial Vestibular
Lateral nuclei
Inferior

Inferior oblique m.
Inferior rectus m.
Lateral rectus m.
Vestibular n.

Abducens (VI) n.

Pontine reticular formation

NEUROLOGIC EXAMINATION
12
Figure 1-7 Nystagmus

Slow phase Rapid phase (saccadic movement)


Direction of Direction of
maintained head acceleration maintained head acceleration

Horizontal Horizontal Horizontal Horizontal


semicircular semicircular semicircular semicircular
canal excited canal depressed canal input canal depressed
continues,
but is

NEUROLOGIC EXAMINATION
opposed by
Medial rectus inhibition Medial rectus motor
Medial rectus motor neurons excited
motor neurons from saccadic
neurons depressed
excited center
Abducens internuclear neuron Medial
Ascending Abducens
rectus motor nucleus
tract of Inhibitory neurons depressed
Deiters interneurons depressed
Vestibular Inhibitory
Medial
Abducens nuclei burst
and lateral
nucleus depressed interneuron
vestibular
nuclei, excited excited by saccadic
center Excitatory
burst
Abducens Abducens interneuron
nucleus Abducens (VI) nucleus
depressed Parapontine nerve excited by Saccadic center (para-
Oculomotor
reticular formation saccadic center pontine reticular for-
(III) nerve
Oculomotor (PPRF) mation [PPRF])
(III) nerve Abducens Lateral
Medial Lateral (VI) nerve Medial
rectus rectus rectus
rectus
muscles muscles muscle
Lateral muscle Lateral
rectus rectus
muscle muscle
Eyes move in opposite direction to head; tend to preserve visual Eyes snap back in same direction as head
fixation: rate determined by degree of horizontal canal excitation

13
CLINICAL NEUROLOGIC EVALUATION
CLINICAL NEUROLOGIC EVALUATION

the left eye adducting and the right eye abduct- ate the broad spectrum of facial sensation, the
ing. The opposite occurs when the head is ro- examiner uses a cotton wisp and the tip of a new,
tated to the right. The cold caloric test evaluates previously unused safety pin, the cold handle of
the vestibular-ocular reflex function on each a tuning fork, or both. In a symmetric fashion, the
side. This test generally is used for the examina- physician asks whether the patient can perceive
tion of comatose patients and will rouse a each stimulus in the 3 major divisions of trigemi-
patient in a nonorganic or feigned coma. The nal nerve supplying the face. Additionally,
study is performed by filling a 50-mL syringe with trigeminal motor function may be assessed by
ice water. Patients are placed at an elevation of having the patient bite down and try to open the
approximately 45°. The tympanic membranes mouth against resistance.
are checked for intactness, and then the ice wa-
ter is gradually infused into each ear. In a normal VII: Facial Nerve (Figure 1-8B)
response, left ear stimulation in the awake pa- The motor functions of CN-VII are tested by
tient leads to slow deviation of the eyes to the asking patients to wrinkle their forehead, close
left followed by rapid movement to the right. Co- their eyes, and smile. Whistling and puffing up
matose patients who retain intact brainstems the cheeks are other techniques to test for subtle
have a persistent ipsilateral deviation of the eyes weakness. When unilateral peripheral weakness
to the site of stimulation; however, the rapid eye affects the facial nerve after it leaves the brain-
movement component is lost. stem, the face may look “ironed out,” and when
The center for vertical conjugate gaze and the patient smiles, the contralateral healthy facial
convergence is located in the midbrain, al- muscle pulls up the opposite half of the mouth.
though the underlying circuit is not delineated Patients often cannot keep water in their
(Figure 1-7). The vertical conjugate gaze centers mouths, and saliva may constantly drip from the
can be tested by flexion of the neck while hold- paralyzed side. With peripheral CN-VII palsies,
ing the eyelids open and watching the eye move- patients are also unable to close their ipsilateral
ments. In central processes affecting conjugate eye or wrinkle their foreheads on the affected
gaze, eg, MS, there is often prominent side. However, although the lid cannot close, the
nystagmus. The nystagmus results from an at- eyeball rolls up into the head, removing the pupil
tempt to maintain conjugate function of the eyes from observation. This is known as the Bell phe-
and minimize double images. nomena.
In addition, another motor branch of the facial
V: Trigeminal Nerve (Figure 1-8A) nerve innervates the stapedius muscle. This
The larger part of the nerve is classified into helps to modulate vibration of the tympanic
3 sensory divisions, supplying the face and ante- membrane and dampens sounds. When this part
rior aspects of the scalp. The angle of the jaw is of the facial nerve is affected, the patient notes
spared within the trigeminal sensory territory, hyperacusis, an increased perception of sound,
providing an important landmark to differentiate when primarily listening with the ipsilateral ear.
patients with conversion disorders or obvious
secondary gain. VIII: Cochlear and Vestibular Nerves (Figure
The clinical testing of nerve functions is gener- 1-8C)
ally performed by gently using a sharp object Clinical evaluation of CN-VIII dysfunction is of-
and a wisp of cotton. The corneal reflex de- ten challenging. Bedside hearing tests sometimes
pends on afferents from the first division of the help to demonstrate diagnostically useful asym-
trigeminal nerve and efferents from the facial metries. Using a tuning fork, it is possible to dif-
nerve. This is tested using a wisp of cotton. The ferentiate between nerve deafness caused by
examiner approaches the patient from the side cochlear damage and that caused by conduction
or has the patient look away when stimulating deafness with 2 different applications of the stan-
the cornea. When the cornea on one side is stim- dard tuning fork. With the Rinne test, a vibrating
ulated, both eyelids close because of multisy- tuning fork is placed on the mastoid. As soon as
naptic pathways within the brainstem. To evalu- the patient is unable to appreciate the sound, the

NEUROLOGIC EXAMINATION
14
CLINICAL NEUROLOGIC EVALUATION

Territories
Figure 1-8A
Trigeminal nerve (V) ganglion and nuclei
nerve (V1)
Motor nucleus
branch Mesencephalic nucleus
rve
Principal sensory nucleus
Spinal tract and nucleus

Facial nerve (VII)


Chorda
tympani nerve

Superficial
temporal branches
Articular branch
and anterior
auricular nerves
Auriculotemporal nerve
Inferior
alveolar nerve Parotid branches
Otic ganglion Meningeal branch
(V3) Tensor tympani Lesser petrosal nerve (from
nerve glossopharyngeal nerve [IX])

NEUROLOGIC EXAMINATION
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CLINICAL NEUROLOGIC EVALUATION

Figure 1-8B Facial Nerve Muscle Innervation

Greater petrosal nerve Facial nerve (VII)


Deep petrosal nerve Internal acoustic meatus
Geniculate ganglion
(from internal carotid plexus) Intermediate nerve
Internal carotid plexus Motor nucleus of facial nerve
Lesser petrosal nerve (on internal carotid artery)
Nerve (Vidian) of pterygoid canal Superior salivatory nucleus
Otic ganglion Solitary tract nucleus
Pterygopalatine ganglion

Facial muscles
Frontal belly (frontalis)
of occipitofrontalis
Orbicularis oculi
Corrugator supercilii
Zygomaticus major
Zygomaticus minor
Temporal branc

Procerus
Levator labii
superioris
Levator labii
superioris
hes

alaeque nasi
Levator
anguli oris
Nasalis

Zygomati
Depressor c
septi nasi Occipital
Tas tongue belly
Orbicularis of Buccal
oris branches (occipitalis) of
Occipital occipitofrontalis
Depressor muscle
branch of
ch

anguli oris
bran

posterior
Depressor labii auricular
l
Cervica

inferioris nerve
Mentalis Branches to auricular muscles
(Risorius) Posterior auricular nerve
al
(not shown) Marginibular Nerve to stapedius muscle
mand h
Buccinator branc Stylomastoid foramen
Platysma Tympanic plexus
Tympanic nerve (Jacobson)
Sublingual gland (from glossopharyngeal nerve)
Submandibular gland Glossopharyngeal nerve (IX)
Submandibular ganglion Digastric muscle (posterior belly)
Lingual nerve (from trigeminal nerve) Stylohyoid muscle
Efferent fibers Chorda tympani nerve Caroticotympanic nerve (from internal carotid plexus)
Afferent fibers
Parasympathetic fibers
Sympathetic fibers

NEUROLOGIC EXAMINATION
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CLINICAL NEUROLOGIC EVALUATION

Figure 1-8C Auditory Nerve Testing: Weber and Rinne Testing


Weber Test

Tone referred Tone referred


to poorer ear Poorer Better
ear to better ear
indicates ear indicated
conductive perceptive
impairment. impairment.

Rinne Test
Stage 1
Stage 2

Tone heard longer by air conduction = Rinne positive: indicates perceptive loss
Tone heard longer by bone conduction = Rinne negative: indicates conductive loss

NEUROLOGIC EXAMINATION
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CLINICAL NEUROLOGIC EVALUATION

Figure 1-9 Test for Positional Vertigo

With patient seated on table and head turned to


right, quickly lower him to supine position with
head over edge 30˚ below horizontal. Observe
eyes for appearance of nystagmus.

Repeat test with head


turned to left.

Repeat test with patient


facing straight ahead.

NEUROLOGIC EXAMINATION
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CLINICAL NEUROLOGIC EVALUATION

instrument is moved close to the opening of the nystagmus. This is described as clockwise or
external ear canal to evaluate air conduction. If counterclockwise, according to the fast phase.
the individual has normal hearing, air conduc- In most individuals, a few beats of horizontal
tion is better than bone conduction. If the patient nystagmus occurring with extreme horizontal
has nerve deafness, bone and air conductions gaze is normal. The most common cause of
are diminished, but air conduction is still better bilateral horizontal nystagmus occurs secondary
than bone conduction. In contrast, this finding is to toxic levels of alcohol ingestion or some med-
reversed in patients with middle ear pathology, ications, ie, phenytoin and barbiturates.
ie, conduction deafness. Here, when the pa-
tient’s bony conduction has ceased, air conduc- IX, X, XI: Glossopharyngeal, Vagus, and
tion is limited by the intrinsic disorder within the Accessory Nerves
middle ear. Therefore, the sound can no longer Swallowing difficulties (dysphagia) and
be heard. In the Weber test, the tuning fork base changes in voice (dysphonia) are the most com-
is placed in the middle of the skull, and the pa- mon complaints related to the dysfunction of the
tient is asked to decide whether one ear per- vagal system. A patient with a glossopharyngeal
ceives the vibration better. In conduction deaf- nerve paresis presents with flattening of the
ness, the vibrations are better appreciated in the palate on the affected side. When the patient is
abnormal ear. In nerve deafness, the sound is asked to produce a sound, the uvula is drawn to
best appreciated in the normal ear. the unaffected side (Figure 1-10). Indirect mirror
The vestibular system can be tested indirectly examination of the vocal cords may demon-
by evaluating for nystagmus during testing of oc- strate paralysis of the ipsilateral cord. The tradi-
ular movements or by positional techniques, tional test for gag reflex, placing a tongue de-
such as the Barany maneuver, that induce nys- pressor on the posterior pharynx, is of equivocal
tagmus. At the bedside, with the patient in a significance at best, because the gag response
seated position, the examiner turns the head and varies significantly. Preservation of swallowing
lays the patient back, with the head slightly ex- reflexes is best tested by giving the patient 30 mL
tended to provoke the characteristic rotary, de- fluid to drink through a straw while seated at
layed, fatiguing nystagmus (Figure 1-9). 90°. Patients with compromised swallowing re-
Eye movements depend on 2 primary compo- flexes develop a “wet cough” and regurgitate flu-
nents, the induced voluntary frontal eye field ids through their nose. Accessory nerve damage
and the reflexive vestibular-ocular movement limits the ability to turn the head to the opposite
controlled by the connections. The ability to side.
maintain conjugate eye movements and a visual
perspective on the surrounding world is an im- XII: Hypoglossal Nerve
portant brainstem function. It requires inputs Damage to the hypoglossal nucleus or its
from other receptors in muscles, joints, and the nerve produces tongue atrophy and fascicula-
cupulae of the inner ear. Therefore, in vestibular- tions (Figure 1-10). The fasciculations usually are
ocular or cerebellar dysfunction, the main- seen best on the lateral aspects of the tongue. If
tenance of basic visual orientation becomes the nerve is affected on one side, the tongue of-
more challenged. Nystagmus is a compensatory ten deviates to that side. Two means to test for
process that attempts to help maintain visual subtle weakness include asking the patient to
fixation. push against a tongue depressor held by the ex-
Traditionally, when one describes nystagmus, aminer and having the patient push the tongue
the fast phase is described. For example, left into the cheek.
semicircular canal stimulation produces a slow
nystagmus to the left, with a fast component to CRANIAL NEUROPATHIES AND
the right. As a result, the nystagmus is referred to SYSTEMIC DISEASE
as right beating nystagmus. Direct stimulation of In evaluating patients for any cranial neuropa-
the semicircular canals or its direct connections, thy, other neurologic and systemic complaints
ie, the vestibular nuclei, often induces a torsional

NEUROLOGIC EXAMINATION
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CLINICAL NEUROLOGIC EVALUATION

Figure 1-10 Evaluation

Uvular paralysis: uvula drawn


to nonparalyzed side
when patient says “A-AH”

Hypoglossal nerve paralysis:


tongue deviates toward
paralyzed side when protruded

Vagus nerve paralysis:


accumulation of saliva
in piriform fossa on
affected side due to
cricopharyngeal muscle paralysis
and inability to swallow

NEUROLOGIC EXAMINATION
20
CLINICAL NEUROLOGIC EVALUATION

and findings must always be considered. This is Other important cerebellar signs include
especially true in unusual cranial neuropathies. tremor, nystagmus, and hypotonia. Tremors may
develop from any lesion that affects the cerebel-
CEREBELLAR DYSFUNCTION lar efferent fibers via the superior cerebellar pe-
Evaluation of posture and gait provide the op- duncle. This is most obvious with coarse, irregu-
portunity to observe the most dramatic clinical lar movement. Nystagmus may also occur with a
manifestations of cerebellar dysfunction. With cerebellar lesion. This is often seen with unilat-
midline cerebellar lesions affecting the vermis, eral cerebellar disease; the nystagmus is most
the patient characteristically assumes a broad- prominent on the affected side. Hypotonia may
based stance when walking, not unlike an inebri- be present but difficult to document. Best ob-
ated individual. At the extreme, these individuals served when testing MSR, the most common
are unable to maintain a stance. In contrast, finding is seen on the quadriceps knee jerk.
when there is a cerebellar hemisphere problem, Here, the normal “check” does not occur after
the patient has a tendency to veer to the affected the initial movement, so the leg on the affected
side. With midline lesions, gait is usually un- side swings back and forth a few times after the
changed whether the eyes are open or closed, initial patellar tendon percussion.
suggesting that this is not the result of disruption
of proprioceptive inputs. Patients with unilateral GAIT EVALUATION
lesions are often able to compensate with their When meeting the patient, the neurologist al-
eyes open but deteriorate when they loose vi- ways observes the gait as the patient walks into
sual inputs. the office. A smooth gait requires multiple inputs
Loss of limb coordination is the result of cere- from the cerebellum and primary motor and sen-
bellar inability to calculate inputs from different sory systems. Gait disorders may be caused by
joints and muscles and coordinate them into frontal lobe processes leading to apraxia or weak-
smooth movements. This abnormality is best ob- ness (Figure 1-11E) and or spasticity, neurodegen-
served by testing finger-to-nose and heel-to-shin erative conditions (particularly those affecting the
movements and comparing the responses on basal ganglia), posterior column dysfunction
both sides. When performing the finger-to-nose within the spinal cord, peripheral neuropathies,
test, the examiner’s finger is the target; it is se- myopathies, and changes in vision. These disor-
quentially moved to different locations. The pa- ders are particularly common among the elderly.
tient in turn keeps the arm extended and tries to It is important to record the temporal profile of the
touch the examiner’s finger at each location. gait decline and the circumstances or situations
When unilateral cerebellar dysfunction is pre- that lead to falls, such as catching the toe on a rug
sent, the patient overshoots the target, so-called as one circumducts a spastic leg or having a leg
past pointing. In patients with focal motor or sen- give out going down stairs secondary to weak-
sory cerebral cortex lesions leading to mild arm ness of the quadriceps femoris muscle. Therefore,
weakness or even more so with proprioceptive when testing gait function, one must assess all as-
sensory loss affecting that limb, some dysmetric pects of the neurologic examination.
movements may occur that are difficult to distin- Gait is tested under several circumstances, in-
guish from cerebellar dysfunction. However, in cluding walking straight, walking at least 10 yd in
cerebellar dysfunction, the movement improves open space, making turns, and maneuvering
after a few trials, but with cortical dysmetria, the through a tight corridor. The examiner should
movement continues to deteriorate. note arm swing and the ease of gait initiation. Pa-
Dysdiadochokinesia is a sign of cerebellar dys- tients with Parkinson disease have difficulty get-
function that occurs when the patient is asked to ting their feet started when they stand and tend
rapidly change hand or finger movements, ie, al- to take small steps (Figure 1-11A). There is an in-
ternating between palms up and palm down. Pa- nate, almost waxlike rigidity to their stooped
tients with cerebellar dysfunction typically have body carriage, including the frozen posture of
difficulties switching and maintaining smooth, one or both arms, which lack the normal arm
rapid, alternating movements. swing. The normal degree of foot separation (the

NEUROLOGIC EXAMINATION
21
CLINICAL NEUROLOGIC EVALUATION

Figure 1-11 Various Gait Disorders

C
A B

Stage 2: bilateral involvement


Stage 1: unilateral with early postural changes; Left hemiparesis with
involvement; slow, shuffling gait with decreased arm swing
Typical wide-based
blank facies; decreased excursion of legs sometimes associated with
gait of drug intoxication limited sensation secondary
affected arm in
Stage 3: pronounced gait to a corticospinal tract
semiflexed position
disturbances and moderate lesion
with tremor; patient
leans to unaffected generalized disability; postural
side instability with tEendency to fall

D E F

Characteristic posture in
Wide-based gait of midline Apraxic gait of normal-
left-sided lower lumbar
cerebellar tumor or other lesion pressure hydrocephalus
disc herniation

base) is widened when proprioception or mid- as the opposite, ie, circumducting (Figure 1-11J)
line cerebellar vermis function is compromised the leg, moving the leg through an arc that scuffs
(Figures 1-11B and D). The examiner should note the toe instead of pursuing a normal straight
the ability to maintain a relatively straight line, course (Figure 1-11C). The patient is asked to
any loss of balance when the patient turns, and walk in tandem, with one foot in front of the
any tendency of one side not to function as well other. This is the well-known DWI (driving while

NEUROLOGIC EXAMINATION
22
CLINICAL NEUROLOGIC EVALUATION

Figure 1-11 Various Gait Disorders (continued)

G H I

Typical spastic gait,


scuffing toe of
affected leg

Patient with peripheral


Patient walks gingerly due neuropathy and loss of
to loss of position sense Patient with lumbar proprioception
and/or painful dysesthesia spinal stenosis with
forward flexion gait

K L M

Severe myopathy or NMJ Sudden occurrence of Sudden buckling of knee


lesion with proximal foot drop while walking while going down stairs Muscle cramps from defection
weakness (peroneal nerve) (femoral nerve) energy metabolism, ie,
McArdle disease

intoxicated) test and is an effective means to elicit longer distances and even asking him or her to
a subtle dysequilibrium often related to midline walk several blocks and return to the clinic. Rarely,
cerebellar dysfunction such as with simple enti- this uncovers an unsuspected corticospinal tract
ties including alcohol intoxication. Occasionally, lesion (Figures 1-11C and J; see Figure 12-1).
having the patient climb stairs reveals a subtle de-
gree of iliopsoas weakness as found in various ABNORMAL MOVEMENTS
peripheral motor unit disorders (particularly my- During the examination, the neurologist is al-
opathies) (Figure 1-11K) and, less commonly, ways alert to any adventitious movements, in-
neuromuscular junction or proximal peripheral cluding tremors, chorea, dyskinesias, and ballis-
neuropathies. Finally, the appearance of spastic- mus. The most common movement disorder
ity may be enhanced by having the patient walk encountered in the office is “essential tremor,” a

NEUROLOGIC EXAMINATION
23
CLINICAL NEUROLOGIC EVALUATION

Figure 1-12 Pyramidal System


Hip
Trunk
Hip
Arm

Trunk

er
Elbow
Should
Primary motor Knee t
cortex (area 4) ris Hand
W
s
Ankle ger Face
Fin
b
Thum
Toes Neck

Brow
Eyelid
Nares
Lateral aspect of cerebral
Posterior Lips cortex to show topographic
limb Tongue projection of motor centers
Larynx on precentral gyrus

Internal
capsule
Posterior

Visual and auditory


Anterior
limb Temporopontine
Le
Tr g Sensory
u
Ar nk
Midbrain m
Face Corticospinal (pyramidal)
III, IV, and VI
Frontopontine

Frontothalamic

Anterior
Horizontal section through
internal capsule to show
V location of principal pathways
Pons
VII

IX
X
XI Decussation
Lateral
(crossed) XII of pyramids
corticospinal
tract Decussation

Spinal
cord
Anterior
(direct)
corticospinal Ventral aspect of brainstem
tract showing decussation of pyramids

NEUROLOGIC EXAMINATION
24
CLINICAL NEUROLOGIC EVALUATION

usually benign hereditary condition. These pa- Partial limb weakness is referred to as mono-
tients often seek medical attention because they paresis. Total limb paralysis is referred to as
are concerned that their tremors are a sign of monoplegia. Unilateral weakness of the limbs is
Parkinson disease. Therefore, differentiating be- referred to as hemiparesis or hemiplegia. Para-
tween different types of tremors is a common paresis is used when both legs are affected; if no
and important concern. An essential tremor motor function remains, this is considered para-
characteristically occurs during certain voluntary plegia. Similarly, quadriplegia relates to total
actions, such as when bringing a cup of coffee to paralysis of all 4 extremities.
the mouth. In contrast, with classic Parkinson dis- When analyzing the complaint of weakness,
ease, the pill-rolling tremor is primarily evident at the physician must consider the presence or ab-
rest, when the patient is seated or walking, and sence of associated neurologic complaints or dif-
disappears with use of the extremity. ficulties, such as language, speech, and visual
changes; gait dysfunction; difficulty with rising
MUSCLE STRENGTH EVALUATION from chairs and associated movements; and al-
Weakness is one of the most common com- teration in sensation. The neurologist testing for
plaints of patients seeking neurologic care. The strength must search for atrophy and fascicula-
motor pathways encompass multiple anatomical tions, note the degree of patient effort and coop-
areas within the CNS, including the cerebral cor- eration, and consider associated problems that
tex and important subcortical structures such as may compromise the testing, such as pain and
the basal ganglia, the brainstem, the cerebellum, skin or orthopedic lesions. Formal strength test-
and the spinal cord (Figure 1-12). Although gen- ing must be conducted in a systematic manner.
eralized weakness, fatigue, or both often are not Most neurologic physicians proceed from the
caused by a neurologic disorder, the possibility of CN to the neck, the upper extremities, and finally
a CNS disorder should always be considered, in- the lower extremities, usually proceeding proxi-
cluding MS in younger individuals and Parkinson mal to distal. Initial focus is on the major muscle
disease in older patients. Less commonly, even groups, such as the flexors and extensors, to seek
sleep apnea may come into consideration when out any areas of weakness. More specific muscle
the patient is significantly overweight. Lesions of testing is particularly useful when distinguishing
the peripheral motor unit warrant consideration between lesions of the nerve root and plexus or
in the evaluation of a patient with generalized mononeuropathies (Table 1-2).
weakness (Figure 1-13). These may include When individual muscle testing does not
processes affecting the anterior horn cell (ie, demonstrate specific weakness, other tech-
amyotrophic lateral sclerosis), peripheral nerve niques sometimes uncover more subtle function
(ie, Guillain-Barré syndrome), neuromuscular loss. If the patient is instructed to extend the arms
junction (including Lambert-Eaton myasthenic with the palms up and the eyes closed, subtle arm
syndrome), or muscle cells (various myopathies). weakness may manifest as downward or lateral
Focal weakness often has a subtle character drift of the affected extremity. Similarly, moving
that frequently is not recognized by the patient the fingers as if playing piano or rapidly tapping
as loss of motor strength. Dropping objects or may demonstrate a subtle incoordination. Subtle
clumsy handwriting may represent a single pe- proximal lower extremity weakness may not be
ripheral nerve lesion such as a radial neuropathy appreciated with individual muscle testing.
leading to a wrist drop, or tripping on rugs or Watching the patient rise from a chair may
steps may be the expression of a peroneal nerve demonstrate use of furniture arms to “push off”
lesion causing a foot drop (Figure 1-11L). In con- and is a good means to identify proximal leg
trast, a dramatic whole limb weakness is obvi- weakness. Particularly effective means to un-
ous and of greater patient concern, often lead- cover proximal strength loss include having the
ing to immediate seeking of medical opinion. patient climb stairs or squat and attempt to rise
Bilateral motor loss is most commonly due to le- without arm use. Also asking the patient to walk
sions affecting the spinal cord or peripheral mo- on the heels or the tips of the toes is helpful in un-
tor unit. covering distal leg weakness.

NEUROLOGIC EXAMINATION
25
CLINICAL NEUROLOGIC EVALUATION

Figure 1-13 Primary Sites of Motor Disorders


Anatomical site Disorder Neurological
of lesion findings

1. Cortex 1. Stroke,
tumor Brisk muscle stretch
reflexes, positive
Babinski sign
2. Basal ganglia

2. Parkinson Tremor, rigidity,


disease cogwheeling, petit
3. Cranial motor pas gait
nerve nuclei 3. Multiple Facial paralysis etc
cranial
neuropathies
4. Cerebellar Gait or
4. Cerebellum ataxia extremity
ataxia

5. Spinal Sensory loss, increased


5. Spinal cord injury, deep tendon reflexes,
auto positive Babinski sign
accident
MS,
6. Anterior abscess,
horn cell tumor
6. Motor Increased muscle
neuron stretch reflexes,
disease no sensory loss,
7. Spinal nerve variable body and
tongue fasciculations

7. Neuropathies Diminished muscle


8. Motor end plate stretch reflexes,
decreased nerve
conduction velocity
8. Myasthenia Positive edrophonium
9. Muscle gravis, chloride test,
botulism facial weakness,
pupillary abnormality

10. Connective 9. Myopathies Muscle stretch reflexes


tissue normal or decreased,
muscle enzynes
increased

10. Congenital Muscle stretch reflexes


hypotonia, normal or slightly
Marfan decreased
syndrome,
Ehlers-Danlos
syndrome

NEUROLOGIC EXAMINATION
26
CLINICAL NEUROLOGIC EVALUATION

Table 1-2
Muscles to Test in a Routine Neurologic Examination*
Muscle Action Nerve Root

Infraspinatus External rotation of arm Suprascapular C5

Biceps Flexion of forearm Musculocutaneous C5-6

Deltoid Abduction of arm Axillary C5

Triceps Extension of forearm Radial C7

Extensor digitorum Extension of fingers Posterior interosseous of radial C7

Flexor digitorum Grip Median C7-8

APB/opponens pollicis Abducting thumb and Median T1


touching tip to fifth
finger

Dorsal interossei Spread fingers apart Ulnar C8

Iliopsoas Flexion of thigh Femoral L2-3

Quadriceps Extension of leg Femoral L3-4

Hamstring Flexion of knee Sciatic S1

Gluteus medius Abduction of thigh Superior gluteal L5

Gluteus maximus Extension of thigh Inferior gluteal S1

Tibialis anterior Dorsiflexion of foot Deep peroneal L5

Tibialis posterior Inversion of foot Tibial L5

Peroneus longus Eversion of foot Superficial peroneal L5, S1

Gastrocnemius Plantar flexion of foot Tibial S1-2


*APB indicates abductor pollicis brevis.

Grading Weakness The examiner assesses the symmetry of func-


The traditional, most widely used system for tion and coexisting changes in tone to formulate
quantifying degrees of weakness is based on a appropriate conclusions regarding the signifi-
scoring range of 0 to 5, with 5 being normal. The cance of subtle changes. The patient’s degree of
extremes of grading are easy to understand, al- effort must be assessed to distinguish organic dis-
though the subtle grading between 4 and 5 (ie, orders from feigned weakness in those with so-
4⫺, 4, 4⫹, 5⫺) may be slightly different de- matoform disorders or individuals with potential
pending on the examiner’s own strength (Table for secondary gain, as may occur with work-
1-3). The examiner must recognize that this is not man’s compensation or other litigation. This is an
an athletic match but rather a determination of important and occasionally difficult differentia-
whether the patient has normal strength. There is tion. Always listen carefully to the patient, con-
a significant range of normal, and a sense of that duct reexaminations, and use available imaging
latitude can only be gained by examining multi- and neurophysiologic modalities before reaching
ple individuals. a diagnosis of a functional nonorganic disorder.

NEUROLOGIC EXAMINATION
27
CLINICAL NEUROLOGIC EVALUATION

Table 1-3
Grading System for Clinical Documentation of Degree of Weakness*
Grade Interpretation
0 No movement (complete paralysis)
1 Able to move a muscle but no movement of limb
2 Minor movement of limb but inability to overcome gravity
3 Moderate weakness; movement of limb against gravity
4 Mild weakness; some resistance against mild pressure
5 Normal; resistance against moderate pressure
*Adapted from Brain. Aids to the Examination of the Peripheral Nervous System. 4th ed. Philadelphia, Pa: WB Saunders; 2000.

When evaluating patients with focal weakness, deficits usually point to a left hemispheric
one should document the evolution of symp- processes. Visual field deficits may also de-
toms and any associated changes in sensation or velop, depending on whether there is concomi-
pain. Sudden onset of weakness, without pre- tant involvement of the optic nerve, chiasm,
ceding trauma or associated pain, suggests is- tract, radiation, or optic cortex.
chemic or hemorrhagic nervous system damage. It is also important to differentiate weakness
Pain with localized weakness and sensory caused by brain processes from weakness
changes may imply nerve root involvement or caused by spinal cord or peripheral nerve
peripheral nerve damage. Most myopathic processes. The most common lesions affecting
processes cause symmetric proximal weakness, the spinal cord are compressive lesions from pro-
although such can occur with other motor unit gressive spondylosis (thickening of the bony
disorders, particularly chronic inflammatory de- spinal canal), metastases, trauma, and inflamma-
myelinating polyneuropathy or rare neuro- tory processes, particularly MS or transverse
muscular transmission defects, such as Lambert- myelitis. Depending on the location and tempo-
Eaton myasthenic syndrome. In contrast, CNS ral profile, spinal cord lesions often begin with
processes cause preferential weakness of the subtle symptoms of gait disturbance, weakness,
arm extensors and leg flexors. The strength of or both. Concomitantly, spinal cord lesions are
muscles that are not usually examined, such as usually associated with sensory findings and uri-
neck flexors and extensors, must always be as- nary bladder difficulties. A careful examination is
sessed when a myopathic process is suspected. important to elicit a sensory level; this is often
Certain illnesses, particularly myasthenia gravis best documented by using pin and temperature
and inflammatory myopathies, may lead to weak- modalities even with uncooperative patients.
ness of these muscles. In the most extreme state, Looking for a sweat level is also sometimes help-
some patients may present with a floppy head. ful because the skin below the level of a signifi-
Other lesions are often localized to specific cant spinal cord lesion will be noticeably drier.
parts of the nervous system by analyzing the pat- Pain is often an associated symptom when le-
tern of weakness, the onset of symptoms, and the sions of peripheral nerves, including nerve roots
associated findings. For example, neglect of the and plexuses, cause weakness. Sensory changes
affected arm or hand, in association with variable are frequently identified, but occasionally, they
degrees of left-sided weakness, often occurs with are subtle in character and not yet recognized by
pathologic processes in the right hemisphere. the patient. Because sensory examination is the
Pure motor weakness of the arm and leg, with most subjective part of the neurologic examina-
slurring of speech, is the hallmark of a stroke in tion, its reliability sometimes must be called into
posterior limb of the internal capsule. Deeper question. Asking the patient to roughly outline
strokes involving the brainstem can often be as- the area in question using a finger can often be
sociated with cranial nerve findings. Language very useful, particularly when the pattern of loss

NEUROLOGIC EXAMINATION
28
CLINICAL NEUROLOGIC EVALUATION

Figure 1-14 Dermal Segmentation

C2

C3
C4
C5 C2
T1
T2
T3 C3
T4 C6
C4
T5 C5 C5 C7
T6 C6 C6 C8
T1
T7
C8 C7
T8 T1
T9 T2
C8 T3
T10 T4
T11 T5
C6 T6
T12 T7
C8 T8
L1 T9
T10
S2, 3 T11
L2 T12 L1
C7 L2
L3
L4
L3 L5
C6
S1 S2
C8 S3
L4
C7 S4
S5
L1

L2
L5
L3

S1
S1 S2
L5
L4 L4

S1
Despite schematic demarcation, in actuality L5
there is considerable overlap between nerve L4
supply of adjacent segments

NEUROLOGIC EXAMINATION
29
CLINICAL NEUROLOGIC EVALUATION

fits the distribution of a particular peripheral glia, cerebellum, brainstem, and spinal cord. Pro-
nerve or nerve root dermatome. Detailed knowl- jections from the pontine reticular formation and
edge of the specific sensory territories of the reticulospinal tract also have direct connections
nerve root dermatomes (Figure 1-14) and the with α and γ motor neurons in the proximal and
specific peripheral nerves is essential to per- axial musculature of the body. These fibers origi-
forming an accurate and useful clinical sensory nate from the cerebrum and cerebellum and are
examination. inhibitory, functioning to decrease motor tone.
Some peripheral mononeuropathies present- Subsequent to damage of structures above the
ing with unilateral weakness, particularly the pontine reticular formation, this circuit loses its
wrist drop of radial nerve lesions, are mistaken inhibitory input from the cerebrum and cerebel-
for processes above the foramen magnum, often lum, leading to excessive stimulation of a motor
mimicking a stroke. Understanding the motor neurons, especially in the antigravity muscles,
distribution of the major peripheral nerves ulti- the arm flexors, and the leg extensors. This in-
mately aids in the correct diagnosis. Although a crease in tone is referred to as spasticity. Its most
peroneal nerve lesion similarly causes a foot extreme clinical manifestation is found in se-
drop, an L5 nerve root lesion also presents with verely ill patients who have decerebrate rigidity
a foot drop. However, the L5 lesion also pro- (Figure 1-15) or posturing, analogous to spastic-
duces weakness of the posterior tibial muscle, ity.
providing the means to make a clinical distinc- Three primary types of changes in tone are
tion from a common peroneal nerve lesion. found in patients with primary CNS disease: spas-
Typically, concomitant sensory involvement ticity, flaccidity, and rigidity. It is important to
manifests as back pain. Interspinal disc hernia- consider perceived changes in motor tone in the
tion and spinal stenosis are the most common context of the complete neurologic examination
processes affecting individual nerve roots. The rather than in isolation. The patient’s body tone is
symptom onset and progression can help in the best evaluated when the individual is fully re-
diagnosis. laxed. Sometimes it is useful to check tone more
Peripheral nervous system involvement often than once during the examination. Tone is de-
leads to atrophy of muscles innervated by the scribed as the patient’s primary level of muscular
involved nerve. Measuring extremity circum- tension. To become comfortable with this part of
ference may document significant side-to-side the examination, students should perform this
asymmetries and, by inference, muscle atrophy test as frequently as possible.
secondary to anterior horn cell, nerve root, or Hypotonia is occasionally demonstrable in pa-
peripheral nerve damage. tients with cerebellar hemispheric lesions. For ex-
Fasciculations, spontaneous firing of small ample, the distal part of the ipsilateral extremity
groups of muscle fibers, commonly accompany may not be able to perform rapid alternate move-
lower motor neuron weakness, particularly with ments (called dysdiadochokinesia) because of the
lesions at the motor neuron or nerve root level. inability to maintain a stable posture. Similarly,
Although often perceived by the patient as the smooth, straight pursuit seen when one elicits
twitching or jumping, they may not be easily the knee MSR loses the out-and-back motion that
seen with the naked eye. Sometimes it may be typically has an inhibitory cerebellar check. In-
necessary to watch a specific muscle for several stead, on return, there is overshoot with no check,
minutes to see these signs. Placing a hand on the leading to a repetitive pendular response. This
muscle when the patient is at rest may allow fas- classic hypotonic cerebellar tone is a relatively un-
ciculations to be felt. Additionally, with lower common finding. A more generalized loss of nor-
motor nerve lesions, there is a concomitant mal tone is most commonly seen among infants
diminution or absence of specific MSRs. with either central or peripheral motor unit disor-
ders, classically spinal muscular atrophy (Werd-
MOTOR TONE nig-Hoffmann disease). Although a similar exam-
In its most basic form, the motor system de- ple is not seen in adults, rarely, floppy head
pends on inputs from the cerebrum, basal gan- syndrome develops in an older patient.

NEUROLOGIC EXAMINATION
30
CLINICAL NEUROLOGIC EVALUATION

Figure 1-15 Motor Tone Abnormalities

Rigid limbs

Extensor
response⫽2

Decerebrate
rigidity

NEUROLOGIC EXAMINATION
31
CLINICAL NEUROLOGIC EVALUATION

Flaccidity, a total loss of tone, is seen in vari- cerebellum through the inferior cerebellar pe-
ous disease processes affecting the upper motor duncle (Figure 1-16). In isolation, it is difficult to
neurons. Most commonly, this occurs with a re- assess the contribution to motor control of each
cent stroke or an acute spinal cord injury, ie, tract. Experimental posterior spinocerebellar
spinal shock. However, with both of these, the tract lesions result in muscular movement inco-
flaccidity is temporary, and tone increases later ordination or asynergia.
to present with significant spasticity. With simple passive stretching, such as occurs
Spasticity is defined by extreme muscle tone with tapping the patellar tendon at the knee, the
that is maximal at the initiation of the physician’s intrafusal muscle spindle is activated, leading to
attempt to move the limb and then suddenly re- a direct stimulus to the large a motor neurons.
leases partway through the movement (a clasp- These in turn stimulate the extrafusal skeletal
knife, spastic release). Significant degrees of muscle fibers leading to the clinically observed
spasticity are easily elicited with any reasonable muscle contraction (Figure 1-17). If the afferent
stimulation of muscles that induces the stretch sensory or efferent motor limb of this nerve sup-
reflex. More subtle spasticity may be obvious ply is damaged, the MSR is affected and may be
only with stretching the muscle in a specific di- diminished or lost, as occurs with many periph-
rection and at a specific rate. Increased tone, eral neuropathies. These reflexes are sometimes
such as may occur with stroke or spinal cord in- inappropriately referred to as deep tendon re-
jury, evolves from flaccidity to spasticity over a flexes when in fact their physiologic basis pri-
matter of days to weeks subsequent to the initial marily depends on the intrafusal muscle spindle
neurologic injury. fibers, not the Golgi tendon organs. MSR is a
When there is total loss of a motor neuron in- more accurate term.
hibition, as may occur with an upper brainstem During the neurologic examination, MSRs
injury, one sees the syndrome of decerebrate (named for the muscle stretched) are usually
rigidity. Here, a simple noxious stimulus leads to readily elicited by tapping lightly over the mus-
bilateral extension in unison of all 4 extremities, cle insertion tendon while palpating the tendon
with the arms rotated inward. Most commonly, and then percussing the palpating digit. Occa-
one sees this in the setting of cardiac arrest or sionally, it is difficult to obtain MSRs in healthy
from shear injuries to the brain from automobile individuals. It is sometimes useful to distract the
accidents. When such patients survive 1 to 3 patient or apply techniques that reinforce the re-
months and are totally otherwise unresponsive, flex to potentiate MSRs. The most common
they are said to be in a persistent vegetative state. method is the Jendrassik maneuver, wherein pa-
Increasing tone from basal ganglia disorders, as tients flex their fingers, interlocking one hand
may occur with Parkinson disease, is known as with the other and pulling on the count of 3
rigidity. Rigidity creates a continuous sense of while the clinician percusses the appropriate
tightness in the attempt to move the joint through tendon at the knee or ankle. For the upper ex-
a full excursion from extension to flexion. tremities, the patient may be asked to clench the
contralateral fist as the neurologist percusses
MUSCLE STRETCH REFLEXES over the arm tendons, activating the intrafusal
The Ia and Ib afferents join the fibers of the muscle spindle.
spinal cord posterior columns, whose primary When grading MSRs, the extremes are easy to
function is to convey information from touch appreciate and range from 0 to 4. A reflex grad-
and pressure receptors. Therefore, although the ing of 0 is indicative of complete lack of MSR.
muscle spindles and Golgi tendon organs can- A generalized loss of reflexes is pathologic and
not be specifically tested, some of their spinal is known as areflexia; this typically occurs in
cord connections can be clinically evaluated by Guillain-Barré syndrome. Briskly responding
testing position and vibration sensory modali- MSRs are graded as 4. In brisk MSRs, a single
ties. Additionally, the Ia and Ib afferents convey Achilles tendon percussion sometimes elicits a
similar information to the cerebellum via the pos- repetitive series of dorsi movements, plantar
terior spinocerebellar tract that travels into the movements, or both in the foot. This is known as

NEUROLOGIC EXAMINATION
32
CLINICAL NEUROLOGIC EVALUATION

Figure 1-16 Cerebral Afferent Pathways

Superior cerebellar peduncle


Middle cerebellar peduncle

To contralateral cerebellar cortex


Cortical input
Leg
Nucleus Arm
reticularis Fac Primary fissure
tegmenti e
pontis

Pontine nuclei
(contralateral)

Spinal input

Inferior olive

Upper part
of medulla To nodule and flocculus
oblongata
Vestibular Inferior cerebellar peduncle
nuclei
Spinal input

Reticulocerebellar
tract
Vestibular nerve Cuneocerebellar
and ganglion tract
Lower part Gracile nucleus
of medulla
oblongata Main cuneate
nucleus (relay
Cortical input for cutaneous
information)
Lateral reticular
nucleus External cuneate nucleus
(relay for proprioceptive
Spinal input information)
Cervical part From skin (touch
of spinal cord and pressure)
Motor interneuron From muscle (spindles
and Golgi tendon organs)
Rostral
spinocerebellar From skin and
tract deep tissues
(pain and Golgi
Spinal border cells tendon organs)
Motor interneuron From skin (touch
and pressure)
Lumbar part
and from muscle
of spinal cord
(spindles and
Clarke column Golgi tendon
organs)
Ventral spino-
cerebellar tract Dorsal spinocerebellar tract

NEUROLOGIC EXAMINATION
33
CLINICAL NEUROLOGIC EVALUATION

Figure 1-17 Muscle and Joint Receptors and Muscle Spindles

IV (unmyelinated) fibers from free


nerve endings (pain)

Detail of
muscle spindle
Extrafusal muscle
fiber
Intrafusal muscle fibers

Sheath
Lymph space Efferent fibers
Afferent fibers
Nuclear chain fiber

Nuclear bag fiber


Ib fibers

Ia fibers ++++
Baseline firing: +

Extrafusal muscle fiber

Intrafusal
muscle fiber

Golgi tendon
organ

NEUROLOGIC EXAMINATION
34
CLINICAL NEUROLOGIC EVALUATION

Figure 1-18 Elicitation of the Babinski Sign

Babinski sign (neurologic examination)

clonus. This does not commonly occur sponta- as stroke, tumor, encephalitis, and spinal cord
neously, but clonus may be elicited by giving a trauma, or demyelinating disorders affecting the
quick snap to the dorsiflexed foot as it is held in spinal cord, the brain, or both. Additionally,
the palm of the hand. This also occurs, rarely, at these signs of upper motor neuron lesions are
the quadriceps tendon. The remainder of the observed in patients during the postictal period
grading is easier to understand. A reflex of 1 is a after a seizure or in patients who have toxic or
mere contraction of the muscle, a 2 is a con- metabolic encephalopathies. Therefore, al-
traction that leads to actual limb movement, though brisk MSRs and a Babinski sign are non-
and a 3 is a reflection of brisker and more force- specific regarding the anatomical setting of the
ful movement. CNS abnormality, their presence provides un-
The Babinski sign is an important pathologic equivocal evidence of upper motor neuron
reflex that is elicited at the lateral, plantar surface pathology.
of the foot using subtle, careful stroking with a
tongue depressor or the base of a key. The great SENSORY EXAMINATION
toe extends, and the remaining toes fan out (Fig- A carefully designed sensory system evalua-
ure 1-18). A more exaggerated response, known tion serves to define the presence or absence of
as triple flexion, includes flexion of the hip, normal sensation and, if abnormal, to define spe-
knee, and foot, often with a Babinski response. cific anatomical patterns of loss for the affected
Because this reflex primarily depends on sensory modalities. Because part of the sensory exami-
stimulation of the foot, a kind, gentle, nonirritat- nation is fairly subjective, the examiner should
ing stimulus is best to obtain an accurate re- analyze the consistency of responses. Addition-
sponse. It absolutely does not require excessive ally, the relevance of sensory changes to the pa-
or painful pressure. With sensitive or ticklish pa- tient’s complaints and other findings needs to be
tients, appropriate responses can usually be ob- evaluated. Initially, the examination should focus
tained from a careful stimulation of the lateral on defining the presence or loss of sensation.
outside foot surface. However, some patients One needs to avoid having the patient be overly
have a withdrawal response wherein the foot cooperative, trying to define the subtlest differ-
and entire set of toes dorsiflex. This is often over- ences in sensory appreciation. This often leads
come by separately pulling down on the middle to an exhausted patient and a frustrated clini-
toe while carefully stimulating the sole in tradi- cian. In most clinical settings, it is best to sepa-
tional fashion. rate the sensory examination into 2 major cate-
A clinical combination of brisk MSRs, clonus, gories derived from superficial skin receptors or
and a Babinski sign indicates an upper motor deeper mechanoreceptors. The former are
neuron lesion. These abnormalities result from small, unmyelinated, slowly conducting type C
various pathophysiologic mechanisms originat- fibers or larger, slightly-myelinated, somewhat
ing in the brain or spinal cord. The many possi- more rapidly conducting type A-δ fibers. These
bilities include destructive cerebral lesions, such small fibers primarily subserve pain (tested using

NEUROLOGIC EXAMINATION
35
CLINICAL NEUROLOGIC EVALUATION

Figure 1-19 ing-glove distribution (Figure 1-19). These then


Documentation of Various spread proximally past the ankles and wrists into
Types of Sensory Modalities the legs and forearms. On examination with a
in a Peripheral Neuropathy cold object, a pin (for small fiber function), a tun-
ing fork, and position sense (if large fibers are
also involved), the examiner notes a distal loss
that is maximal in the periphery and gradually
reaches normal at a more proximal site.
Individual mononeuropathies are typified by
symptoms and findings specific to a peripheral
nerve. For example, the patient notes numbness
in the thumb, index, middle fingers, and adjacent
lateral aspect of the fourth finger if the median
nerve is involved. In carpal tunnel syndrome
with entrapment of the median nerve at the
Graduated glove-and-stocking wrist, the examination results are often normal.
hypesthesia to pain and/or However, using a reflex hammer to tap over the
temperature
entrapment site commonly elicits brief paresthe-
siae in the classic distribution of the median sen-
sory fibers. This maneuver is known as the Tinel
sign; the name applies to instances wherein a
simple provocative test is used to define the le-
sion site with a mononeuropathy
Plexopathies are usually unilateral in distribu-
tion, affecting the brachial or lumbosacral groups
of nerves. Typically, they have combined sensory
loss involving multiple peripheral nerves within
the affected limbs. These have a broader distribu-
Impaired vibration sense tion of motor and sensory loss than a single nerve
root or mononeuropathy. Therefore, when the
examination reveals findings not exclusively del-
a pinpoint), temperature (tested using a cold ob- egated to the defined distribution, the possibility
ject such as the handle of a tuning fork), deep of a plexus lesion must be considered.
pressure, and gross touch modalities. The large, Radiculopathies are frequently more subjec-
well-myelinated type A-α and A-β fibers carry the tive, with intermittent or persistent symptoms
kinesthetic modalities of position studied by the confined to the dermatomal patterns of one spe-
examiner’s passively moving the digit in the ver- cific nerve root (Figure 1-14). Pain is the most
tical plane and asking the patient whether the common symptom, starting in the neck, shoul-
movement was up or down. Additionally, fine der, and low back, often radiating down the pos-
tactile discrimination can be evaluated by using terior thigh. The classic examples include the C7
a pair of calipers to check their ability to recog- nerve root in the neck with paresthesiae primarily
nize whether 1 or 2 points are applied to the in the index and middle fingers and the L5 nerve
digit. Some vibratory modalities are probably root in the low back, where the patient reports
also subserved by type A-δ fibers. numbness in the first and second toes and the
lateral calf. Concomitant MSR loss often occurs,
Classic Syndromes of Sensory Dysfunction ie, the triceps reflex with a C7 root and the
Peripheral Nervous System Achilles reflex with an S1 root. If the lesion is se-
Generalized polyneuropathies typically pre- vere, the examiner often notes concomitant
sent with symptoms of numbness and tingling at weakness in a specific radicular distribution,
the tips of the toes and, later, fingers, ie, a stock- such as the triceps muscle with a C7 radiculopa-

NEUROLOGIC EXAMINATION
36
CLINICAL NEUROLOGIC EVALUATION

Figure 1-20 Somesthetic System: Body

Cerebral cortex:
postcentral gyrus

Posterior limb of
internal capsule

Ventral posterolateral
(VPL) nucleus of
thalamus

Mesencephalon
(cerebral peduncles)

Medial lemniscus

Gracile nucleus
Spinothalamic tract
Cuneate nucleus

Lower part of
medulla oblongata
Fasciculus gracilis

Fasciculus cuneatus
Reticular formation
Dorsal (posterior) spinal
root ganglion

Proprioception,
position Large
myelinated
Cervical part Touch, fibers
of spinal cord pressure,
vibration
Small
Lateral Pain, myelinated
spinothalamic tract: temperature and unmyelin-
pain, temperature ated fibers

Lateral cervical nucleus


Ventral (anterior)
spinothalamic tract: Spinocervical tract
touch, pressure

Lumbar part
of spinal cord

NEUROLOGIC EXAMINATION
37
CLINICAL NEUROLOGIC EVALUATION

thy and the tibialis anterior and tibialis posterior Brown-Séquard syndrome occurs. This syn-
in the case of an L5 root involvement. drome is characterized by contralateral loss of
pain and temperature sensation, ipsilateral loss
Spinal Cord Syndromes of position and vibration sensation, and ipsilat-
The site of a spinal cord lesion is defined by eral upper motor neuron weakness.
identifying the exact distribution of specific mo- Syringomyelia or a central hemorrhage leads
tor and sensory deficits of various modalities to another anatomically specific spinal cord le-
(Figure 1-20). A complete lesion of the spinal sion. The pathology occurs at the center of the
cord leads to total loss of function distal to the cord, destroying fibers carrying pain and temper-
site of the abnormality. A distinct level of sensory ature sensation from both sides as they cross in
loss can be discerned with tests for loss of pain the anterior commissure. Because the fibers car-
and/or temperature sensations, associated with rying vibration and position sense do not cross in
loss of sweating below the lesion level. Con- the spinal cord and ascend posteriorly, a small le-
comitantly, all muscles subserved by anterior sion in the center of the cord spares those path-
horn cells distal to the site of the lesion experi- ways. This causes a dissociated sensory loss with
ence paralysis (Figure 60-3). isolated loss of pain and temperature sensation,
A lesion in the anterior lateral aspect of the usually in a “cape” distribution, while concomi-
spinal cord causes contralateral loss of pain and tantly, position sense is preserved.
temperature sensation. If the lesion is more ex- A patient with an anterior spinal artery infarc-
tensive, leading to damage of the anterior and tion presents another classic sensory picture.
posterior aspects of the cord on one side, Here, the bilateral damage to the spinothalamic

Figure 1-21 Thalamus and Its Multiple Nuclei

3rd
Interthalamic ventricle
adhesion
Internal medullary
n ) lamina
edia Pulvinar
ina

e (m
lin
lam

d l
Mi dia a Intralaminar
Me llary lamin MD LP nuclei
d u
l me
Inte
rna LD VA
LP M
3rd ventricle VL VPL
VP VI CM
Reticular
M

M
VP

nucleus of
VP thalamus
L
VP
External
Pulvinar medullary
Thalamic nuclei
Lateral geniculate body lamina
CM Centromedian Midline
LD Lateral dorsal Medial geniculate body
(median) nuclei
LP Lateral posterior
M Medial group Schematic representation of thalamus
MD Medial dorsal (external medullary lamina and
VA Ventral anterior reticular nuclei removed)
VI Ventral intermedial Schematic section
VL Ventral lateral Lateral cell mass through thalamus
VP Ventral posterior (ventrodorsal) Medial cell mass (at level of broken
VPL Ventral posterolateral line shown in figure
VPM Ventral posteromedial Anterior cell mass at left)

NEUROLOGIC EXAMINATION
38
CLINICAL NEUROLOGIC EVALUATION

and corticospinal tracts spares the posterior col- 1 cm (2-point discrimination). They are unable
umn function. The patient is paralyzed and has to recognize numbers traced on the palm
total loss of pain and temperature sensation, but (graphesthesia).
position and often vibratory sensory modalities Many other sensory abnormalities occur, in-
are preserved. cluding complete loss of sensation on the con-
tralateral side of large strokes involving the pari-
Thalamic Involvement etal area and a neglect with right parietal lesions,
The ventral posterior lateral and ventral poste- wherein the patient is unaware of paralysis or
rior medial thalamic nuclei are the 2 major sen- sensory loss of the contralateral limbs. These are
sory relay nuclei (Figure 1-21). Lesions in these especially obvious with double simultaneous
areas can cause loss of sensation to all modalities stimulation (extinction). One or two sides of the
involving the entire contralateral half of the body are variably stimulated, and the patient is
body. This most commonly occurs in patients asked to identify the stimulus location. Individu-
with bland or hemorrhagic infarcts. Initially pre- als with a more subtle parietal sensory loss can-
senting with a relatively tolerable numbness, not identify the contralateral stimulus when bi-
eventually the damage incurred from the stroke lateral stimuli are applied.
may produce an unpleasant, sometimes dis-
abling, hyperpathic sensory alteration known as REFERENCES
Bates B. A Guide to Physical Examination and History Taking.
the thalamic pain syndrome. Rarely, this loss of 4th ed. Baltimore, Md: JB Lippincott Co; 1987.
sensation can lead to limb sensory choreoa- Brain. Aids to the Examination of the Peripheral Nervous Sys-
thetosis. Lesions within the corona radiata, un- tem. 4th ed. Philadelphia, Pa: WB Saunders; 2000.
dercutting the parietal cortex, can cause similar, Brazis P, Masdeau J, Biller J. Localization in Clinical Neurol-
ogy. 3rd ed. Baltimore, Md: Lippincott Williams & Wilkins;
although often less extensive, findings. 1996.
Carpenter MB, Sutin J. Human Neuroanatomy. 8th ed. Balti-
Cortical Sensory Involvement more, Md: Williams & Wilkins; 1983.
The parietal lobe receives topographically or- Kandel, ER, Schwartz JH, Jessell TM. Principles of Neural Sci-
ganized sensory inputs from the thalamic nuclei ence. 4th ed. New York, NY: McGraw-Hill, Health Profes-
sions Division; 2000.
brainstem, spinal cord, plexi, and peripheral Luria AR. Higher Cortical Functions in Man. 2nd ed. Basic
nerves (Figure 1-20). The important function of Books, Inc; 1980.
the parietal lobe is the integration of that infor- Mayo Clinic Department of Neurology. Mayo Clinic Exami-
mation with other sensory and motor informa- nations in Neurology. 7th ed. St. Louis, Mo: Mosby; 1998.
tion to formulate body awareness. In the purest Miller N. Clinical signs and symptoms. In: Walsh and Hoyt’s
Clinical Neuro-Ophthalmology. 5th ed. Baltimore, Md: Lip-
form of cortical sensory dysfunctions, patients pincott Williams & Wilkins; 1998.
are unable to differentiate the location of their Peters A, Jones EG, eds. Cerebral Cortex: Vol 4. Association
toes or fingers in space, between 1 point and and Auditory Cortices. New York, NY: Plenum Press; 1985.
2 points of contact, and points separated by

NEUROLOGIC EXAMINATION
39
Chapter 2

Cognitive and Language


Evaluation
Kinan K. Hreib

Clinical Vignette
A 70-year-old previously healthy man was referred for evaluation of an inability to ambulate. According to the
family, during the past year, the patient had had difficulties standing up and getting out of the car and often sat in
the car for hours until someone actively encouraged him by either gently pushing or pulling him out of the car. On
further questioning, it became evident that he had similar problems under other circumstances. He tended to sit
on the toilet seat for hours. His wife reported that often in public places, she had to ask security to get him out of
the bathroom. In the morning, the patient stayed in bed until physically coaxed to get up.
The family commented that he had experienced a concomitant change in behavior. He might lie in urine while
in bed, often seemingly unaware of the problem. On a regular basis, he was incontinent of stool, often while stand-
ing up. His wife would urge him to clean up, but to her amazement when she returned a few hours later, she found
him still standing in the same spot.
He ate if reminded and shown the food but usually did not ask for or make his own meals. At dinner, he typically
took a piece of bread, eating it slowly but often not proceeding to the rest of the food unless helped by the family.
He read the newspaper regularly, usually comprehending and remembering its content. However, when he fin-
ished reading, he folded and unfolded the newspaper for hours. When the patient was asked about these issues,
his answer was brief: “I do not know.” His wife did not notice any problems with his memory but emphasized that
his level of interaction with the family had significantly decreased.
During his neurologic examination, he often smiled and looked around the room as if searching for something.
He was always pleasant but not conversational. When asked a question, his responses were brief and usually mono-
syllabic but appropriate, without speech or language dysfunction. He did not seem to have prominent memory dif-
ficulties, nor did he have cranial nerve involvement or motor or sensory deficits. His gait was normal. His reflexes
were 1+ throughout, and his toes were down.
Subsequently, his symptoms have progressed. He is now less interactive and continues to be unable to partici-
pate in his own hygiene or to eat on regular basis. MRI demonstrated significant frontotemporal atrophy (Figure 2-
1). This clinical picture is compatible with the diagnosis of frontal-type dementia. Pick disease may have a similar
presentation but often with significant language difficulties (chapter 34).

The initial clinical evaluation affords the oppor- nitive examination is needed in addition to the
tunity to assess whether a neurologic patient has standard (chapter 1) neurologic examination.
overt cognitive or language difficulties. The abil- This part of the neurologic evaluation strives
ity to give a well-organized history provides the to determine the precise level of various higher
experienced neurologist with insight into the pa- cortical functions. The human cerebral cortex,
tient’s general language and cognitive function. It with its multiple gyri and network of many mil-
is usually clear that intellect and speech are ap- lion interconnections, is the most complex part
propriate to the setting, so a more formal set of of the brain. Anatomically, the cortex is classified
mental status tests is unnecessary. However, in into 4 major functional areas: frontal, temporal,
instances of overt intellectual function changes, parietal, and occipital lobes (Figure 2-2). These
when the patient’s demeanor suggests that pos- anatomic substrates are carefully intercon-
sibility or the family suggests an additional prob- nected in a complex network. For the sake of dis-
lem to the primary complaint of the patient (as in cussion, these cortical areas are described in iso-
the vignette in this chapter), a more detailed cog- lation, but in reality, interconnections with other

NEUROLOGIC EXAMINATION
40
COGNITIVE AND LANGUAGE EVALUATION

Figure 2-1 orientation, concentration, constructional abil-


Frontal Temporal Atrophy ity, apraxia, any language deficit, and recall (Fig-
ure 2-4). The Mini-Mental Status Examination, of-
ten used to quantitate cognitive function, is
limited by its lack of a true language element and
inability to test for most frontal lobe dysfunc-
tions. Language deficits provide another major
obstacle in a detailed cognitive examination.
Similarly, the delirious patient with poor concen-
tration, poor attention, or both has a limited ca-
pacity to respond to the examination.

APHASIA
Language dysfunction is manifested by im-
pairment in oral or written communication or
both. Deficits vary in quality and severity de-
pending on the extent of anatomic involvement
within the language areas. Aphasias result from
involvement of the dominant hemisphere, which
is the left hemisphere in all right-handed individ-
Coronal FLAIR MR image demonstrates ventricular en-
largement especially of the right temporal horn, atrophy uals and approximately 50% of native left-
of superior and middle temporal gyri (arrow), and promi- handed individuals. In the remaining left-handed
nence of frontal sulci. Notice prominent widening population, the language center is located in the
of the interhemispheric fissure (arrowheads).
Courtesy of Richard Caselli, MD. right hemisphere. The aphasia may not be ac-
companied by other neurologic deficits; there-
cortical and subcortical areas are critical for fore, such patients are often initially mistaken as
brain function (Figure 2-3). Because some pa- being confused. A good history from the family
tients are not able to give a history or cooperate regarding symptom onset or other important
with the examiner, the history from reliable fam- medical history often helps in the diagnosis.
ily and friends is the most important part of the In the acute clinical setting, the classification
evaluation. Interactions with the patient also of aphasia is challenging. The different types of
help to determine the level of cognitive neuro- language deficits depend on the brain area in-
logic function. The patient’s attitude, affect, level volved (Table 2-1). The easiest to understand are
of cooperation, and distractibility are notewor- those involving the cortex (Figure 2-5). Although
thy. For example, patients with dementia may aphasias are often part of a large hemispheric
seem pleasant and jovial, often finding excuses stroke, many devastating aphasias can result
for their inability to answer questions, rarely with from small processes affecting the temporal or
any insight into their deficit. In contrast, patients frontal lobes, or even some subcortical struc-
with severe posterior aphasia may seem agitated tures, such as the thalamus.
and uncooperative, and often respond intermit- The degree of language fluency is the earliest
tently to certain commands but not to others. manifestation of aphasia that can help to deter-
Many fascinating and complex syndromes af- mine its specific characteristics. For example, a
fect higher cortical function. Often they occur in patient with a posterior temporal infarct of the
association with other significant neurologic dominant hemisphere presents with poor com-
deficits, particularly with a stroke, the most fre- prehension but fluent speech, ie, the patient is
quent clinical cause for these lesions. often talkative, albeit his or her language is not
normal. In contrast, fluency is significantly dimin-
COGNITIVE TESTING ished in patients with a frontal lobe lesion, partic-
Factors to be documented in an elementary ularly with Broca aphasia. Thalamic aphasias are
cognitive examination include level of alertness, often characterized by apathy, hypophonia, and

NEUROLOGIC EXAMINATION
41
COGNITIVE AND LANGUAGE EVALUATION

Figure 2-2 Superolateral Surface of Brain


Central (rolandic) sulcus Superior (superomedial) margin of cerebrum
Precentral gyrus Postcentral gyrus
Precentral sulcus Postcentral sulcus
Frontal (F), frontoparietal (FP), Supramarginal gyrus
and temporal (T) opercula
Superior parietal lobule
Superior frontal gyrus Intraparietal sulcus

Superior frontal sulcus Inferior parietal


lobule
Angular gyrus
Middle frontal
gyrus Parieto-
occipital
Inferior sulcus
frontal
sulcus

Inferior
frontal
gyrus FP

F
T
Frontal Occipital
pole pole
Calcarine
Lateral sulcus Anterior ramus sulcus
Inferior
(Sylvius) Ascending ramus (infero-
Posterior ramus Lunate sulcus
lateral) (inconstant)
margin of
Superior temporal gyrus cerebrum Transverse
Temporal pole
Superior temporal sulcus occipital sulcus
Inferior
Middle temporal gyrus temporal Preoccipital notch
Parietal lobe
Inferior temporal sulcus gyrus

Frontal lobe Occipital


lobe

Temporal lobe Central


sulcus
of insula
Circular
sulcus
of insula
Short gyri
Insula Limen
Long gyrus

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COGNITIVE AND LANGUAGE EVALUATION

Figure 2-3
Cerebral Cortex: Localization of Function and Association Pathways
Ms I
Motor-sensory
Ms II Sm I
Sensory-motor
Sm II

Premotor; orientation; (to Ms II)


eye and head movements Sensory
analysis
Visual I
Visual II
Prefrontal;
inhibitory control
Visual III
of behavior;
higher intelligence

Language; reading;
speech
Motor control
of speech Auditory I

Auditory II

Ms I Sm I
Motor-sensory Sensory-motor
Ms II Sm II
Premotor Temporocingulate and
Prefrontal; parietocingulate pathway
inhibitory control
of behavior; Visual III
higher intelligence
Visual II
Visual I

Fronto-
cingulate
pathway

Cingulate gyrus Corpus callosum


(emotional behavior) Hippocampal commissure
and cingulum Olfactory Anterior commissure

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COGNITIVE AND LANGUAGE EVALUATION

Figure 2-4 Testing for Defects of Higher Cortical Function


A. Appearance and interpersonal behavior

Pleasant, neatly Depressed, Belligerent


dressed, sloppily dressed,
good careless
spirits

B. Language Good Defective

Doctor: “Write me
a brief paragraph
about your work.”

C. Memory 5 minutes later:


Patient: “I’m sorry,
Doctor: “Here are 3 I can’t remember.
objects: a pipe, a pen, and Did you show me
a picture of Abraham Lincoln. something?”
I want you to remember them
and in 5 minutes, I will ask
you what they were.”

D. Constructional praxis and visual-spatial function

Doctor: “Draw me “Draw a


a simple picture clock face
of a house.” for me.”

Good Abnormal
Good Abnormal

E. Reverse counting

Doctor: “Count backward Doctor: “Spell the word worlds


from 5 to 1 for me.” backward for me.”
Patient: “5...3...4..., Patient: “W..L..R..D..S.”
sorry, I can’t do it.”

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Table 2-1
Characteristics of Aphasias
Parameter Anterior Posterior Conduction Subcortical Transcortical

Location Frontal/ Temporal/ L.-inf.-parietal Basalganglia/ Thalamus/


Broca Wernicke white matter anterior-lateral Motor Sensory
Fluency Poor Good Good Variable Poor Poor Good
Naming Poor Poor Poor-fair Variable Poor Poor Good
Repetition Poor Poor Poor Good Good Good Good
Paraphasia Common Common Common Variable Variable Common Common
Comprehension Good Poor Good Good Poor Good Poor

echolalia. Most often, lesions in the anterior-lat- from the adjacent motor and premotor areas,
eral thalamus cause the most profound aphasia. particularly in their connections with other corti-
Transcortical aphasia refers to aphasias near cal areas and the thalamus. Most thalamic con-
the classic language areas. These include the nections are with the dorsal medial nucleus, a
posterosuperior temporal lobe (Brodmann area prime relay center for limbic projections origi-
40, for Wernicke aphasia) and the posteroinfe- nating from the amygdala and the basal fore-
rior frontal lobe (Brodmann area 44 for Broca brain. The reciprocal inputs are the most promi-
aphasia). However, transcortical aphasia does nent cortical connections, originating from
not directly involve these areas. Most investiga- second-order sensory association and paralim-
tors agree that receptive transcortical aphasia in- bic association areas, including the cingulate
volves the posterior-inferior temporal gyrus, ie, cortex, temporal pole, and parahippocampal
Brodmann area 37. Transcortical motor aphasia area. The frontal lobe is an integrator and ana-
occurs with left frontal lobe lesions. Echolalia is lyzer of highly complex multimodal cortical ar-
common in these latter aphasias, whereas right eas, including limbically processed information.
visual deficits are common in the sensory Experimental animals demonstrated unusual
transcortical aphasias. behavior after ablation of both frontal lobes.
Some patients with Wernicke aphasia are able Some of the most dramatic symptoms, including
to mimic certain movements, especially midline automatic nonpurposeful behaviors with a ten-
commands such as opening the mouth, closing dency to chew randomly on objects, led to the
the eyes, and standing up. Another common conclusion that the frontal lobe was important
phenomenon, best described as a crowding ef- for the integration of goal-directed movement.
fect, occurs when patients seem to be able to fol- Investigations in the 1950s began to define the
low the first command and perhaps the second, importance of the frontal lobe in analyzing stim-
but their performance deteriorates with subse- uli. Lesions in the frontal lobe resulted in loss of
quent testing. The cause may be partially intact normal social interchange, personal internal re-
language function becoming overwhelmed by inforcement, and judgment. Therefore, patients
persistent use. The aphasia also seems worse are unable to modify behavior despite poten-
when patients are anxious or upset. tially harming or embarrassing effects of their ac-
tions. Furthermore, patients tend to repeat auto-
FRONTAL LOBE DYSFUNCTION matic behaviors that do not result in conclusive
The frontal lobe comprises approximately actions, as seen in perseveration testing.
30% of brain mass occupying the motor area In humans, one of the earliest descriptions of
(Brodmann area 4), the premotor cortex (Brod- frontal lobe damage included significant person-
mann areas 6 and 8), and significant prefrontal ality changes and “release of animal instincts.”
areas. Significant prefrontal areas are distinct Subsequent reports described patients with apa-

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Figure 2-5 Dominant Hemisphere Language Dysfunction


Clinical syndromes
related to site of region

Broca aphasia Global aphasia

Wernicke aphasia Occipital region

Inferior
Conduction aphasia Angular gyrus temporal lobe

Wernicke Conduction Inferior


Broca aphasia aphasia aphasia Angular gyrus temporal lobe Occipital region Global aphasia

Dysarthria Normal, Occasional


Pronunciation, stuttering, fluent, Normal Normal pause in Normal
Very
speech rhythm effortful loquacious word finding
abnormal

Missed syllables, Use of wrong


Speech agrammatical, or nonexistent
Some wrong
Often normal
Occasional
Normal
Very
content telegraphic words
words circumlocution abnormal

Abnormal but Very


Repetition better than Abnormal Abnormal Normal Normal Normal
of speech abnormal
spontaneous
Comprehension
of spoken Very Slightly Very
Normal abnormal abnormal Normal Normal Normal abnormal
language
Comprehension Not as good Abnormal but Often Very Very Very
of written as for spoken better than
normal abnormal Normal abnormal abnormal
language language for spoken
Clumsy, Penmanship OK Occasional Very abnormal, Very
Writing agrammatical, but misspelling spelling and Normal Normal
misspelling and inaccuracies language errors spelling errors abnormal

Better than Occasional Often Very Occasionally Very


Naming spontaneous Wrong names abnormal abnormal abnormal
wrong names abnormal
speech
Slight hemipar-
Sometimes Slight hemi- esis, trouble
Hemiplegia, paresis, neglect Hemianopsia,
Other hemianopsia calculating, — color anomia Hemiplegia
apraxia of right-sided
and apraxia finger agnosia,
stimuli hemianopsia

Central sulcus Supplemental


Lateral view of the motor cortex Superior
Primary parietal lobule
forebrain: functional regions motor
cortex
Frontal
eye fields Premotor Primary
cortex
somatosensory
cortex Wernicke
Primary area
trigeminal Secondary
Broca region of somatosensory Visual
area cortex association
motor somatosensory
cortex cortex areas of cortex
Primary Multisensory
association
Auditory areas of cortex
cortex Primary
visual
cortex

Lateral fissure

NEUROLOGIC EXAMINATION
46
COGNITIVE AND LANGUAGE EVALUATION

thy and disturbed emotions. Elucidation of the larly, when asked to draw a circle, they may draw
frontal lobe connections, particularly the medial- many superimposed circles. The classic grasp re-
basal portion, shows that the limbic system pro- sponse is also a form of perseveration. The exact
vides significant input to that area. Autonomic pathophysiologic mechanisms underlying these
centers originating in the brainstem and hypo- dysfunctions are not well defined. However, it is
thalamus also have significant connections with thought that perseveration inertia is a form of dis-
the basal frontal lobe. When these connections inhibition; the patient is unable to differentiate
are disrupted, aggressive, impulsive, and uncon- between activities because of an inability to ap-
trolled behavior results. propriately process many sensory and motor in-
Frontal lobe syndromes are classified clini- puts normally handled in the frontal lobes.
cally, anatomically, and neuropsychologically As the vignette in this chapter illustrates, pa-
into lateral and medial-basal groups. Patients tients with frontal lobe dysfunction typically
with lateral frontal lesions experience significant have preserved memory and no obvious pri-
difficulties in executing a purely motor function, mary motor or sensory deficits. They may have
with general slowing of speech, gait, or other visual fixation problems from frontal visual field
previously learned motor action, and are some- involvement but do not experience severe visual
times described as behaving “like a bump on a disturbance. Sometimes, patients may lose the
log.” Individuals with medial-basal lesions dis- details of moving objects or misinterpret a com-
play prominent behavioral and emotional diffi- plicated drawing; an individual shown a picture
culties and disturbances of consciousness. of an airplane may misinterpret it as a bird. Their
Broca aphasia is the classic form of frontal limited visual scanning and fixation and their im-
lobe language dysfunction, exhibited as a non- pulsive nature may produce erroneous interpre-
fluent stuttering, very effortful, agrammatic tation of stimuli. This disturbance in frontal lobe
speech in association with poor naming but with function extends to a variety of tasks, including
normal comprehension. Often, these individuals memory. A patient with severe frontal lobe dys-
have an associated apraxia and weakness of the function may recognize that his sister is a family
right side of the face and right hand. member but then refer to her as his mother, be-
cause of resemblance in hair or height or other
Testing isolated features. When given a set of 3 words to
One of the simplest frontal lobe tests is se- memorize and then given another set of 3
quencing movements, ie, rapid alternating words, these patients often repeat the first set.
movements. Although this test may serve several Even when told of the mistake, the patient is un-
purposes during the neurologic examination, it is able to correct it. These patients also have diffi-
most useful in frontal lobe syndrome diagnosis. culties associating words with certain pictures
One set of 3 motor movements includes tapping and recalling them in a specific order. Patients
with the hand on the thigh, forming the letter O with frontal lobe dysfunction become severely
with the index finger and the thumb, and forming impaired as a result of disconnection of an im-
the letter T by placing an open hand on top of portant analyzer that processes sensory and mo-
the other. With the rapid alternating movement tor inputs. Frontal lobe syndromes at the earliest
sequences, the patient is asked to place the hand stages may be difficult to diagnose. Sometimes,
in 3 different positions: flat, then sideways, and fi- they are misinterpreted, and occasionally, a psy-
nally with the fist clenched. Many variations of chiatric illness is diagnosed. Traditional imaging
these tests exist. Patients with frontal lobe dys- studies often help to demonstrate a focal patho-
function fail them, either by performing haphaz- logic process, although even sophisticated
ard movements or by perseverating on the same modalities do not always demonstrate a specific
movement. Other tests for eliciting frontal lobe lesion.
dysfunction include copying certain randomly
repeating shapes. Such patients suddenly start TEMPORAL LOBE DYSFUNCTION
drawing the previous shape repeatedly even Wernicke aphasia is the best-known neuro-
when instructed to draw a different shape. Simi- logic presentation of a dominant posterior tem-

NEUROLOGIC EXAMINATION
47
COGNITIVE AND LANGUAGE EVALUATION

poral lobe lesion. It is characterized by fluent, of- to formulate memory. Memory seems to involve
ten nonsensical jargon, many neologisms, poor both hemispheres. Right temporal lesions result
naming ability, and difficulty comprehending in disturbance of imprinting and storage of mem-
spoken language. More anterior portions of the ory traces, whereas left temporal lesions pro-
superior temporal gyrus, the Heschl transverse duce disturbance of delayed recall. These types
gyrus, receive auditory inputs. Therefore, the of memory mechanisms have also been called
most important connections of the superior tem- involuntary and voluntary memory, referring to
poral gyrus are those involving complex analy- right and left hemispheric functions, respec-
ses of sound. Lesions in the dominant superior tively. Separating testing for involuntary and vol-
temporal gyrus can lead to word deafness. Un- untary memory is beyond the scope of this text.
like patients with typical Wernicke aphasia in
many aspects, these patients are able to recog- Testing
nize written words. Linguistically, word deafness Documenting long-term and short-term recall
deficit is an inability to differentiate phonemes, and new learning are the basic elements of mem-
sounds that allow differentiation of closely re- ory testing. Remote memory testing can involve
lated words. In English, phonemic differentiation asking the patient about relevant past events
occurs between fricatives such as F and V or Z such as birthdays or anniversaries. Short-term
and SH and nonfricatives such as zipper and tip- memory can be tested with a pure measure of at-
per. In other languages (eg, French), phonemes tention and immediate memory, the Digit Span
are vowels. Some patients with word deafness Forward test. It is administered by asking the pa-
may also be unable to recognize music. Amusia tient to repeat a series of numbers with increas-
can occur with unilateral lesions of the temporal ing length, from 3 digits up to 8 digits. The Digit
lobe. Span Backward test, giving the patient a series of
The inferior and medial temporal gyri are func- numbers to repeat in reverse order, tests the abil-
tionally less well understood. Connections of the ity to manipulate information in memory. Nam-
inferior temporal gyrus include input from visual ing 3 objects and then asking the patient to re-
processing areas and connections with the pari- peat the list 3 to 5 minutes later can easily test
etal cortex. The meaning of these connections learning. These memory problems may be dra-
and their contributions to certain cognitive func- matic and accompanied by confabulations, sim-
tions are not well understood. Prominent ilar to Korsakoff syndrome.
changes in monkey behavior follow the removal Word list testing is a nonspecific test of recall,
of both temporal lobes. More focal lesions in- learning, and executive function. The patient is
volving the medial parts of the temporal lobes asked to produce, over 1 minute, a list of words
produce sudden outbursts of rage, fluctuating beginning with a specific letter or in a specific
levels of consciousness, inability to acquire new category. This task requires input from multiple
skills, and disappearance of previously learned cortical areas and tests several cognitive deficits.
skills. Similar deficits occur in patients with tem-
poral lobe tumors or temporal lobe epilepsy. PARIETAL LOBE DYSFUNCTION
Prominent deficits include a heightened anxiety Sensory alterations are typically characterized
level and short-term memory difficulties. Mem- by a loss of cortical sensory modalities (chapter
ory and learning are complex processes and re- 1). One of the most dramatic changes with non-
quire integration of information at multiple levels dominant parietal lobe pathology is neglect syn-
from multiple sources. The ability to retain infor- drome, ie, asomatognosia. This mind-body dis-
mation starts with registering, integrating, retain- sociation manifests with curious behavioral
ing, recalling, and reproducing the information. changes, sometimes including a form of confab-
Memory may not become encoded if one of ulation. For example, when an affected patient is
these steps fails. asked about his paretic arm, he may state that it
Often, a neurodegenerative process presents is not paretic and proceed to demonstrate the
with confusion and disturbed thinking and per- motor function of the normal arm. When his ne-
ception, compromising the processes that help glected and paretic arm is shown to him, he does

NEUROLOGIC EXAMINATION
48
COGNITIVE AND LANGUAGE EVALUATION

not recognize it as his arm. In addition, many pa- “boy and the cookie jar” drawing and asking
tients perceive distortion of the size and shape of them to describe what they see. Gerstmann syn-
their limb. The neglect, often associated with a drome, found in dominant posteroinferior pari-
relatively blunted affect, may be difficult to dif- etal lobe dysfunction, results in right and left con-
ferentiate from visual field deficits, especially in fusion, finger agnosia, and acalculia. These
the early stages of the disease. However, neglect problems are easily tested by asking the patient
seems to be more prominent with exposure to to raise his or her right or left hand or identify the
multiple stimuli, ie, double simultaneous stimula- examiner’s crossed right or left hand. Similarly,
tion. Smaller parietal lobe lesions may manifest asking the patient to show his thumb or index fin-
as geographic disorientation. For example, pa- ger and perform several levels of calculation
tients may not be able to find their way around help to diagnose left parietal lesions.
their home. Some patients with dominant hemispheric le-
Apraxia, a form of movement construction fail- sions may have alexia or other language difficul-
ure, is defined as an inability to perform pur- ties. Although it is uncommon and difficult to
poseful movement in the absence of elementary recognize as a language problem, alexia is con-
sensory or motor disturbance. Ideational apraxia firmed when patients are able to write but can-
is characterized by patients who cannot create not read their writing (alexia without agraphia).
the image of the required movement. The re- Reading numbers is usually more preserved than
sponsible lesion is located near the parieto-oc- reading letters or words. Some patients also
cipital junction, although some frontal lobe le- have difficulties identifying colors. Occasionally,
sions may mimic it. Motor apraxia causes the adjacent occipital lobe is involved; most pa-
disturbances of coordination of previously tients have a contralateral visual field deficit.
learned motor movements and results from le- Alexia is related to inability to relay visual infor-
sions just anterior to the parieto-occipital sulcus. mation to the angular gyrus. Therefore, a lesion
The evaluation of patients with possible apraxia in the splenium of the corpus callosum or under-
involves asking them to perform certain tasks: lying white matter may interrupt this topo-
pretending to hold a toothbrush and brushing graphic information to the language areas in the
the teeth or pretending to hold a comb and dominant hemisphere.
combing the hair. In patients, oral apraxia devel-
ops as a manifestation of inferior post–central OCCIPITAL LOBE DYSFUNCTION
gyrus lesion, testable by asking the patient to At the most elementary level, occipital lobe le-
perform or mimic certain movements, such as sions cause visual field defects (Figure 2-7). Rudi-
chewing, smiling, or kissing. Dominant hemi- mentary visual information is processed in the
spheric lesions account for most of these aprax- striate cortex, or Brodmann visual area 17. Com-
ias. Dressing apraxia, another common sympto- plex visual information is processed more anteri-
matic apraxia, is a combination of apraxia and a orly, relying on inputs from multiple cortical ar-
topographical disorientation usually seen in non- eas. The complex nature of visual processing
dominant parietal lesions. An extension of spa- and integration of visual information in memory
tial dysfunctions is the inability to copy three-di- and orientation suggest that a vision disturbance
mensional figures, such as a cube. may lead to several neuropsychological abnor-
malities. The primary visual areas project to
Testing midtemporal areas, temporal polar regions, and
There are multiple tests for nondominant the parahippocampal gyrus. These regions re-
hemispheric dysfunction (Figure 2-6). These in- ceive extensive input from other sensory
clude asking the patient to mark the numbers of sources and limbic structures that influence tag-
a clock on a large circle covering an entire sheet ging emotional and motivational significance to
of paper, followed by drawing the hands of a the stimulus. Aside from the visual field defects
clock to indicate 10:10, or asking the patient to discussed in chapter 1, patients may experience
draw transecting lines that fill an entire sheet. Al- image distortion or shifting, or persistence of an
ternatives include showing patients the classic image when the eyes have already moved from

NEUROLOGIC EXAMINATION
49
COGNITIVE AND LANGUAGE EVALUATION

Figure 2-6 Nondominant Hemisphere Higher Cortical Dysfunction


A. Constructional dyspraxia and spatial disorientation

Clock face drawn by patient Patient asked to copy Draws this House drawn by patient

B. Neglect of left-sided stimuli

Patient shown picture Sees this Patient shown printed page Sees this
C. Anosognosia Not recognizing
(unawareness deficit, patient
of deficit) insists on trying
to walk and falls,
Patient with obvious but still fails
left hemiplegia. to recognize
Asked, “What is wrong deficit.
with you?”
Answers, “Nothing
is wrong, I am
perfectly all right.”

D. Motor impersistence

Patient asked to
raise arms over head
and to keep them up
Raises arms but
then drops them
quickly

E. Abnormal recognition of nonlanguage cues


(facial expression, voice tone, mood)
Patient shown Patient answers,
picture. Asked, “I don’t know,
“Which is the they are all
happy face?” the same.”

NEUROLOGIC EXAMINATION
50
COGNITIVE AND LANGUAGE EVALUATION

Figure 2-7 Occipital Lobe Functional Anatomy


Visual Pathways in the Parietal
and Temporal Lobes

Parietal lobe Spatial visual pathway: positional


relationship between objects in
visual scene, analysis of motion

Frontal lobe
Middle temporal area:
direction selective and
motion responsive

MT Occipital
V3 lobe
V2

V4
V3 V1
V2

V4: shape and color


Temporal lobe perception
Object recognition pathway:
high resolution and form

Partial Sensory and


Autonomic Seizures

Formed visual hallucinations


(posterior, temporal, parietal, occipital,
visual association cortex, temporal limbic cortex)

Unformed visual hallucination


(occipital lobe)

the object. Usually, these phenomena are ob- ter group, usually elderly patients who often
served in occipital and temporal lesions. More have severely compromised vision.
dramatic “realistic” images are described in pa- Visual agnosias are not related to visual per-
tients with temporal lobe lesions and in some in- ception but to the incorporation of visual data
dividuals without a specific cerebral structural with other relevant information. Patients with
abnormality. Bonnet syndrome applies to the lat- the most basic form of visual agnosia cannot rec-

NEUROLOGIC EXAMINATION
51
COGNITIVE AND LANGUAGE EVALUATION

ognize objects shown to them but can easily rec- REFERENCES


ognize them by touch. Other rare agnosias in- Damasio AR, Damasio H, Van Hoesen GW. Prosopagnosia:
clude prosopagnosia, in which patients cannot anatomic basis and behavioral mechanisms. Neurology.
1982;32:331-341.
recognize faces or understand the meaning of fa- Damasio AR, Geschwind N. Anatomical localization in clini-
cial expression. In Balint syndrome, there is a cal neuropsychology. In: Vinken PJ, Bruyn GW, Klawans
profound inability to scan visual space or grasp HL, eds. Handbook of Clinical Neurology. Vol 45. Amster-
an object in space. In these visual agnosia syn- dam, The Netherlands: Elsevier; 1985:7-22.
Denny-Brown D. The frontal lobes and their functions. In:
dromes, visual information seems to be discon-
Feiling A, ed. Modern Trends in Neurology. New York, NY:
nected from brain areas that associate signifi- Hoeberharper; 1951:13-89.
cance with that information. It is unclear Landis T, Cummings JL, Benson F, Palmer EPL. Loss of topo-
whether this occurs in the somatosensory, lan- graphic familiarity. An environmental agnosia. Arch Neu-
guage, or other higher-order visual areas. Most rol. 1986;43:132-136.
Mesulam MM. Principles of Behavioral Neurology. Philadel-
of the lesions that cause visual agnosias, such as phia, Pa: FA Davis; 1985.
diffuse anoxia after cardiac arrest or occipital Papez JW. A proposed mechanism of emotion. Arch Neurol
lobe infarcts, are bilateral. Psychiatry. 1937;38:725-743.

NEUROLOGIC EXAMINATION
52
Chapter 3

Retina and Optic Nerve


Ippolit C. A. Matjucha and Robert J. Updaw II

RETINA
Anatomy and Physiology
The retina, the neural tunic of the eye, contains 9 types of cells arranged in 10 distinct layers (Figure
3-1). Its function is 3-fold: to detect visible light, transduce the photic energy of light into neural signals,
and perform an initial analysis of visual information before sending it to the brain for further process-
ing. Many types of cells participate in accomplishing these tasks.

Vascular Supply photoreceptor outer segments. Retinitis pigmen-


The outer retina receives blood via the chorio- tosa is characterized by progressive night blind-
capillaris. The remaining retina, however has an ness (nyctalopia). Pigmentosa is named for the
additional vascular source; the central retinal characteristic black clumps of pigment found
artery sends branches into the eye through the within the peripheral retina known as bone
optic disk (Figure 3-2). Although significant varia- spicules. As retinitis pigmentosa advances, only
tions are encountered, in general, 4 branches of a central island of vision may remain, which may
the central retinal artery emanate from the disk: su- eventually be extinguished.
perior nasal, superior temporal, inferior nasal, and The pathognomonic retinal pattern of visual
inferior temporal. field loss in retinitis pigmentosa is characterized
by the classic ring scotoma, with a midperipheral
Retinal Disease loss sparing the center and far periphery. Ring sco-
The topographic layering of the retina allows a tomata are encountered in other retinal diseases,
disease process (eg, inflammation) to destroy one particularly the toxic retinopathies of chloro-
group of photoreceptors and leave an immedi- quine, hydroxychloroquine, or phenothiazines.
ately adjacent group unaffected. Visual field de-
fects based on retinal disorders have sharp, steep Macular Degeneration
borders that may be irregular or ragged edged. Few retinal diseases have more epidemiologic
The shape of visual field deficits due to vascu-
impact than age-related macular degeneration, an
lar compromise is largely predictable, being rela-
ischemic disorder. Macular degeneration pro-
tively consistent with the location of the retinal
duces painless loss of central vision in the elderly.
occlusion. Because branch retinal arteriolar oc-
Two types exist: a nonexudative or “dry” type, and
clusions affect the nerve fiber layer, defects ex-
tend beyond the local occlusion in an outwardly an exudative or “wet” variety when choroidal neo-
expanding arcuate or sectoral pattern because all vascularization intrudes into the macular retina.
nerve fibers arcing through the ischemic area are Characteristic of the nonexudative variant are
affected. For example, with branch occlusions of retinal drusen (from the German term for geode
the retinal artery supplying the superior retina, crystals). Ophthalmoscopically, drusen are
the inferior field of vision within that eye is lost. small, discrete, extracellular deposits recognized
as yellow lesions clustered around the macula.
Retinitis Pigmentosa These lesions gradually become larger, more nu-
Frequently, a genetic disorder, retinitis pig- merous, and confluent, with adjacent retinal at-
mentosa, is characterized by rod and cone dys- rophy. Visual acuity and disturbed color vision
trophies and is often from a gene mutation for loss ensues from the inability to support the
rhodopsin or peripherin, a glycoprotein on the metabolic activity of the photoreceptors.

NEURO-OPHTHALMOLOGY
54
RETINA AND OPTIC NERVE

Figure 3-1 The Retina and the Photoreceptors

Retinal Layers Cells


Visual Receptors Inner limiting
Nerve fiber layer
A. Eyeball Lens Cornea membrane
Iris Axons at surface of
Suspensory Ciliary body Ganglion cell retina passing via
ligament layer optic nerve, chiasm,
Inner plexiform and tract to lateral
layer geniculate body
Anterior Posterior Ganglion cell
chamber chamber Inner nuclear Müller cell
containing layer (supporting glial
Ora aqueous humor cell)
serrata Bipolar cell
Outer plexiform
Vitreous humor Amarcine cell
layer
Horizontal cell
Optic Outer nuclear Rod
nerve layer
Retina Cone
Choroid Photoreceptor Pigment cells
Sclera layer of choroid
Fovea Pigment B. Section
epithelium through retina
©
Rod Photoreceptor Cone Photoreceptor
Synaptic Horizontal Outer
ending cell plexiform
fully layer
Synaptic Bipolar
polarized
ending C. Rod in dark D. Rod in light cell
depolarized Photons of light

Rhodopsin all-cis retinal Nucleus

Inner Inner
Nucleus
segment segment
all-trans retinal
Intracellular
transduction via
phosphodiesterase Mitochondria
Current Ca2+ ion flow
flow + Opsin
hydrolysis of modulates light Cilium
adaptation Ca2+
Na+ all-cis intracellular Photopigments
permeability retinal cGMP Outer
Outer cone opsins
increased segment
through Vitamin A segment (blue, green,
decreased red plus
cGMP-gated Na+ permeability II-cis retinal)
Na+ channels Plasma Pigment
membrane epithelium

NEURO-OPHTHALMOLOGY
55
RETINA AND OPTIC NERVE

Figure 3-2 Retinal Architecture and Perimetry


Minor arterial circle of iris
Cornea Major arterial circle of iris
Anterior chamber
Blood vessels of ciliary body
Scleral venous sinus
(Schlemm canal) Bulbar conjunctiva and
conjunctival vessels
Iridocorneal angle
Anterior ciliary artery
Posterior chamber and vein
Zonular fibers Ciliary Muscular artery
Lens body and vein
Iris
Ora serrata
Superior macular Extrinsic eye
arteriole and venule Retina muscle
Superior temporal Vitreous
Long posterior
retinal arteriole Superior nasal chamber
Choroid ciliary artery
and venule retinal arteriole
Vorticose vein
and venule Sclera
Episcleral artery and vein
Macula Optic disc
Retinal artery and vein
and fovea
centralis Long posterior ciliary artery
Inferior nasal Short posterior ciliary arteries
retinal arteriole
Central retinal artery and vein
and venule
Inferior temporal Optic nerve (II)
retinal arteriole Inferior macular
and venule arteriole and venule

Right retinal vessels: ophthalmoscopic view

Topography of retinal nerve fibers


Macular nerve fibers course
Arcuate nerve fibers from directly to optic disc.
temporal periphery of retina
must arc around macular Optic disc (blind spot)
bundle.

Nasal retina
Temporal retina
Median horizontal raphe.
Inferior and superior Nerve fibers of nasal retina
arcuate fibers meet at but course directly to optic disc.
do not cross.
Macula (fixation point)

Schema of retinal neuroarchitecture


Nerve fiber layer
Ganglion cell layer
Internal plexiform layer
Internal nuclear layer
External plexiform layer
External nuclear layer
Photoreceptor layer
Pigmented epithelium
Optic nerve

NEURO-OPHTHALMOLOGY
56
RETINA AND OPTIC NERVE

OPTIC NERVE fore meeting at the optic chiasm, just above the
Anatomy of the Optic Disk and Nerve pituitary gland.
The optic nerve nominally begins when the ax-
ons of the ganglion cells turn 90°, changing ori- Disorders
entation from horizontal along the inner retinal The many diseases affecting the optic nerve
surface to vertical through the retina and out of produce characteristic patterns of visual field
the eye via the scleral canal (Figure 3-3). The gan- loss, depending on where the optic nerve is af-
glion cells receive input from bipolar and fected. Generally, defects occur with disease of
amacrine cells. The gathering of axons at the the disk or the anterior optic nerve, the orbital
canal forms the optic disk of the fundus, also optic nerve, and the prechiasmal or “junctional”
called the optic nerve head. optic nerve. Glaucoma, optic neuritis, anterior is-
Numerous axons carrying information from chemic optic neuropathy, optic nerve drusen,
the foveal retina form a thick area in the nerve papilledema, and congenital dysplasia occur
fiber layer as they gather at the temporal quad- within the anterior optic nerve. Diseases of the
rant of the disk. This area, the papillomacular orbital optic nerve include posterior ischemic
bundle, is located between the disk and the optic neuropathy, optic neuritis, compressive
fovea. The papillomacular fibers enter the tem- optic neuropathy, toxic optic neuropathy, and
poral optic disk; diseases that produce temporal genetic or metabolic optic neuropathies. Junc-
pallor of the disk, particularly multiple sclerosis tional optic nerve disease is usually due to com-
(MS), therefore produce a central scotoma on vi- pression.
sual field testing (chapter 1).
The inferior and superior arcuate bundles, 2 Anterior Optic Nerve Pathoanatomical
additional groupings of retinal nerve fibers, relay Correlations
information from the non–foveal macula and en- Visual field defects from anterior optic nerve
ter the disk at its inferior and superior poles. The disease (eg, glaucoma) typically consist of para-
remaining nerve fibers constitute the nasal nerve central, Bjerrum, Seidel, arcuate, nasal step, and
fiber bundle, entering the disk in a radial fashion. altitudinal scotomata. The scotomata represent
The more superior and inferior fibers of the nasal levels of depletion of the inferior or superior ar-
bundle contain axons from the temporal retinal cuate bundles. If the damage is minimal or small,
periphery; the more central fibers contain axons an elongated blind spot (Seidel scotoma) or par-
from the nasal periphery. tial arcuate loss (Bjerrum scotoma) may result.
The optic nerve begins at the optic disk and With more significant disease, a complete arcu-
courses to the optic chiasm. It has several por- ate scotoma or more extensive altitudinal loss in-
tions: intraocular, intraorbital, and intracranial. volving the entire superior or inferior field nasal
The first part of the intracranial segment runs to the blind spot can be seen. Because of the hor-
through the bony optic canal. When the optic izontal raphe, such cases often show a sudden
nerve leaves the interior of the eye, the axons “step” change, called a nasal step, in the visual
pass through a reticulated opening in the sclera field on crossing the nasal horizontal meridian; it
called the lamina cribrosa and then undergo a is a dependable sign of anterior optic nerve dis-
major anatomical change. ease.
Its previously nonmyelinated fibers now be- The papillomacular bundle consists of macu-
come myelinated with CNS myelin, explaining lar fibers entering the temporal aspect of the op-
why the optic nerve is affected by MS. The optic tic disk. As these neurons travel into the orbit,
nerve is therefore misnamed; it is not a periph- they comprise a central position in the orbital op-
eral nerve but a white matter tract of the brain. It tic nerve. Because no central optic nerve artery
is also bathed in CSF and invested with arach- exists, blood reaches these axons from vessels
noid tissue, pia mater, and dura mater that fuses on the exterior of the nerve. A partial watershed
with orbital connective tissue, forming the optic of blood flow is produced, in which the most
nerve sheath. After leaving the optic canal, the metabolically active axons on the optic nerve
optic nerves course below the frontal lobes be- (the papillomacular bundle) are the farthest from

NEURO-OPHTHALMOLOGY
57
RETINA AND OPTIC NERVE

Figure 3-3 Anatomy of Optic Nerve


Retinal
nerve fibers
Optic nerve layers
Retina
Nerve fiber
Choroid layer

Sclera
Prelaminar
Central retinal layer
vessels

Vascular circle of Laminar


Zinn-Haller layer
Short posterior
ciliary a.
Lamina cribrosa
Retrolaminar
Nerve fiber bundles layer

Pial layer

Arachnoid
layer
Dura
mater

Disc Disc

Cup Cup
Clinical appearance
Thin, atrophic
Healthy, thick neural rim
neural rim
Small optic cup Large, deep cup
with visible lamina
cribrosa

Normal
lamina
cribrosa Distorted
lamina
cribrosa

In glaucoma, normal
architecture of lamina
cribrosa disrupted,
neural rim eroded,
cup-to-disc ratio
increased
Normal Glaucoma

NEURO-OPHTHALMOLOGY
58
RETINA AND OPTIC NERVE

the blood supply. These neurons are therefore syndrome of monocular visual loss with pain
regarded as particularly fragile and prone to in- usually means the lesion is within the orbital por-
jury. tion of the optic nerve. Therefore, central sco-
Hence, central and centrocecal (extending to tomata are the classic finding, although periph-
the blind spot) scotomata are seen in metabolic, eral loss and scotomata more typical of anterior
toxic, and compressive optic nerve diseases. Al- or junctional optic nerve lesions are routinely
though the entire optic nerve is exposed to the seen. Similarly, ensuing pallor of the optic disk is
effects of compression, toxic exposure, or meta- typically temporal pallor, involving fibers of the
bolic disease, often only these core fibers are in- papillomacular bundle that occupies the central
jured, producing the characteristic central sco- orbital nerve.
toma. Intravenous methylprednisolone (1 g/d for 3
days, followed by oral prednisone) is recom-
Glaucoma mended for accelerating visual recovery and
Glaucoma is a family of slowly progressive op- providing some protective effect against new
tic neuropathies usually related to high intraocu- MS events, perhaps up to 2 years. However, it
lar pressures and progressive cavitating (“cup- does not improve the eventual degree of recov-
ping”) atrophy of the optic disk. Visual loss starts ery of the optic neuritis; possible inherent risks
in the midperiphery as circular or arcuate sco- with this medication must be considered. Alter-
tomata, which, over years in severe cases, first native treatment with oral prednisone alone is
abolish most of the peripheral vision, and then contraindicated because it may predispose to
abolish the central vision. No restorative therapy occurrence of MS relapse earlier than if the con-
exists. dition is left untreated.
Treatment concentrates on slowing or stop-
ping progression by decreasing intraocular pres- Anterior Ischemic Optic Neuropathy
sure through medications, laser surgery, or ocu- Anterior ischemic optic neuropathy is a stroke
lar surgery. Most cases are asymptomatic until affecting the optic nerve at the disk. Patients usu-
visual loss is severe; therefore, detection of pa- ally experience sudden, severe, painless, monoc-
tients at risk by routine screening of intraocular ular visual loss, often on awakening (Figure 3-5).
pressure and optic nerve contour is vital, espe- Examination classically reveals an altitudinal (su-
cially for patients with a family history of glau- perior or inferior) visual field loss, with a unilater-
coma or another significant risk factor (eg, his- ally swollen, hemorrhagic disk.
tory of severe ocular trauma) (Figure 3-4). Usually, the combination of constricted optic
nerve anatomy (a small crowded disk) and noc-
Optic Neuritis turnal systemic hypotension are the responsible
Often the presenting sign and one of the most mechanisms. There is no recognized therapy for
common MS features, optic neuritis typically the involved eye. To prevent contralateral in-
produces a brief prodrome of periocular pain, volvement, daily antiplatelet therapy (eg, 81 mg
mounting over days and worsened by eye move- aspirin) is recommended.
ments. Ensuing visual loss is often sudden and se-
vere, with perceived worsening over days; how- Giant Cell Arteritis
ever, the degree of visual loss varies. One of the major neurologic emergencies, gi-
Examination demonstrates central vision, visual ant cell arteritis, places patients at risk for rapid—
field, and color vision loss in the affected eye and sometimes within a few days or hours—total vi-
a relative afferent pupillary defect; however, ini- sual loss in one or both eyes. Few cases of
tially the optic nerve usually looks normal, until anterior ischemic optic neuropathy result from
partial pallor begins some weeks later. Recovery this serious disorder, which primarily affects indi-
of vision, typically to near normal, occurs mainly viduals older than 65 years and rarely occurs in
over a 2- or 3-month period. younger persons (Figure 3-5B). Some individuals
Although MS produces visual loss by affecting have a premonitory warning of incipient visual
the white matter within the visual pathways, the loss with transient blurred vision that leads them

NEURO-OPHTHALMOLOGY
59
RETINA AND OPTIC NERVE

Figure 3-4 Optic Disc and Visual Field Changes in Glaucoma


Early
Right eye
nasal side

Funduscopy: notching of contour of physiologic Perimetry: slight enlargment of physiologic


cup in optic disc with slight focal pallor in blind spot (1); development of a secondary,
area of notching; occurs almost invariably in superonasal field defect (2) which corresponds
superotemporal or inferotemporal (as shown) to nerve fiber damage in area of
quadrants inferotemporal notching

Minimally advanced
Right eye
nasal side

3 4

Funduscopy: increased notching of rim of cup; Perimetry: localized constriction of superonasal


thinning of rim of cup (enlargement of cup); visual field (3) because of progessive damage to
deepening of cup; lamina cribrosa visible in inferotemporal fibers; superior arc-shaped scotoma
deepest areas (Bjerrum scotoma) develops (4)

NEURO-OPHTHALMOLOGY
60
RETINA AND OPTIC NERVE

Figure 3-5A Multiple Sclerosis: Ocular Manifestations

Sudden unilateral
blindness, self-limited
(usually 2 to 3
weeks). Patient
covering one eye,
suddenly realizes
other eye is partially
or totally blind.

Visual fields reveal central scotoma due


to acute retrobulbar neuritis.

Figure 3-5B Giant Cell Arteritis: Ocular Manifestations

Senior citizen with sudden monocular


visual blurring or blindness, associated
with malaise, scalp tenderness, and
myalgia. The erythrocyte sedimentation
is very elevated, usually 60 to 120 min/h.

Anterior ischemic optic neuropathy

NEURO-OPHTHALMOLOGY
61
RETINA AND OPTIC NERVE

to seek care. Typically, patients may have noted When visual loss occurs, it starts with blind
general malaise, weight loss, and headaches spot enlargement (Figure 1-5B), a nonspecific
with scalp tenderness, and they often have a pre- and often reversible change. In chronic or severe
ceding diagnosis of polymyalgia rheumatica. cases, visual field loss resembling that of glau-
Sometimes, patients also report jaw claudication coma can ensue, sometimes rapidly. Treatment
characterized by masticatory muscle pain with consists of acetazolamide (typically 1 g/d in di-
chewing. Rarely, these patients present with or vided doses) to reduce production of CSF; occa-
later have a stroke, usually within the extracere- sionally, other diuretics are used. When medical
bral portions of the vertebral artery. The primary treatment fails, two surgical options exist: optic
examination finding is a prominent, thickened, nerve sheath fenestration or lumboperitoneal
and tender superficial temporal artery. CSF shunt.
Every such patient requires immediate evalua-
tion. ESR is an excellent screening test because TREATMENT
most patients have significant increases, 60 to New classes of medication have been devel-
110 mm/h, normal less than 20 mm/h. Ideally, an oped for the treatment of glaucoma, and new
immediate temporal artery biopsy is indicated. treatments attempting to slow macular degener-
However, because it is not often possible on an ation range from novel lasers to simple dietary
emergency basis, high-dose (80 mg/d) pred- supplements. However, the goals of restorative
nisone must be initiated pending the results of or regenerative treatment for extensive retinal or
optic nerve disease remain elusive.
the biopsy. If the pathologic findings demon-
strate giant cell granulomas and interruption of
the elastic membrane, treatment must be main-
FUTURE DIRECTIONS
Diagnostic advances in retinal and optic nerve
tained for at least 3 to 6 months. Usually, gradual
disease have focused on new imaging and elec-
dose reduction on an alternate-day basis lessens
trophysiologic modalities for these structures and
the well-known adverse effects of cortico-
on identification of genetic markers and other spe-
steroids; disease activity is monitored by the ESR.
cific risk factors. Computer-driven laser scanning
devices permit the determination of nerve-fiber
Papilledema
layer thickness in the retina, allow tomographic
Papilledema (Figure 1-5A), optic nerve eleva- optical “sectioning” of the retina, and may even an-
tion and swelling, is usually due to high intracra- alyze the neural content of the retina using the op-
nial pressure. Often, patients have no symptoms tical birefringence of the arrayed nerve fibers.
of visual compromise, although with chronic in- These advanced techniques allow better detec-
tracranial pressure increases, some report visual tion and localization of structural abnormalities.
obscurations with transient blurring, particularly Gross whole-field (Ganzfeld) electroretinogra-
with postural changes. Many primary patho- phy is complemented by focal and multifocal
physiologic mechanisms are associated with pa- techniques that test smaller areas of retina, so
pilledema, including CNS tumor (with edema or that focal but critical losses (ie, in the fovea) are
obstructive hydrocephalus), meningitis, certain not “masked out” by predominantly normal sig-
medications (eg, tetracycline or vitamin A), or in- nals from most of the retina. Earlier and more
tracranial venous obstruction. certain diagnosis of more limited forms of retinal
Papilledema is occasionally seen, without ex- disease is therefore possible.
planation, in obese women of childbearing age The slow task of finding dependable genetic
and is called idiopathic intracranial hyperten- markers for optic nerve and retinal diseases has
sion or pseudotumor cerebri (chapter 71). experienced some small, remarkable successes
Treatment is directed against known causes. In (eg, Leber hereditary optic atrophy), suggesting
the idiopathic group, treatment is initially de- hope in more common diseases. The study of pa-
ferred if the condition is mild with no progressive tients with common eye diseases to seek out
visual loss or debilitating headache. new risk factors (eg, thin corneas predisposing to

NEURO-OPHTHALMOLOGY
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RETINA AND OPTIC NERVE

glaucoma) continues to help determine which Hedges TR, Friedman D, Horton J, Newman SA, Striph G,
patients need the most aggressive treatment. Kay MC. Neuro-ophthalmology. In: Weingeist TA,
Liesegang TJ, Slamovits TL, eds. Basic and Clinical Science
Course, 1997-1998. San Francisco, Calif: American Acad-
REFERENCES emy of Ophthalmology; 1997:51-55.
Bron AJ, Tripathi RC, Tripathi BJ. Wolff‘s Anatomy of the Eye Tripathi RC, Chalam KV, Chew EY, et al. Fundamentals and
and Orbit. 8th ed. Boston, Mass: Chapman & Hall (Kluwer principles of ophthalmology. In: Weingeist TA, Liesegang
Academic); 1997. TJ, Slamovits TL, eds. Basic and Clinical Science Course,
Harrington DO, Drake MV. The Visual Fields: Text and Atlas of 1997-1998. San Francisco, Calif: American Academy of
Clinical Perimetry. 6th ed. St. Louis, Mo: CV Mosby; 1991. Ophthalmology; 1997:76-92.

NEURO-OPHTHALMOLOGY
63
Chapter 4

Optic Chiasm, Optic Tract,


Lateral Geniculate Nucleus,
and Optic Radiations
Ippolit C. A. Matjucha and Robert J. Updaw II

OPTIC CHIASM

Clinical Vignette
A 51-year-old woman presented with worsening vision over many months. She reported no other significant
medical history.
On checking visual acuity, the examiner discovered that the patient could see only the left half of the eye chart
with her right eye and only the right half with her left eye. A gross confrontation visual field check confirmed a dense
bitemporal hemianopia. The examiner also noted that the woman had facial hypertrichosis and enlargement of her
brow, nose, lips, and jaw and that the patient’s rings and shoes no longer fit properly. Acromegaly, as occurs from
abnormally high circulating levels of human growth hormone produced by a pituitary tumor, was diagnosed. MRI
confirmed the lesion compressing the optic chiasm.

Bitemporal hemianopsia is characteristic of an rior to the chiasm is the third ventricle. The inter-
optic chiasm lesion, usually caused by pituitary nal carotid arteries flank the optic chiasm laterally
tumors, although occasionally a craniopharyn- and then bifurcate into the anterior and middle
gioma is the primary lesion. The optic chiasm cerebral arteries. The anterior cerebral arteries
(from the Greek letter x) represents the “Great and the anterior communicating artery are ante-
Divide” of the afferent visual system. Visual field rior to the optic chiasm. Within the chiasm, axons
defects are typically categorized into 3 anatomi- from the temporal retina (nasal field) comprise its
cal areas. Prechiasmatic defects affect the visual lateral aspect, remaining ipsilateral, whereas the
field of the ipsilateral eye only and typically result nasal retinal fibers decussate, carrying the tem-
from retinal or optic nerve pathology. Chias- poral field information to the contralateral side.
matic disorders classically lead to bitemporal Inferior nasal fibers decussate within the chiasm
hemianopia (also, hemianopsia), with loss of the more anteriorly than superior ones. As the infe-
right lateral field in the right eye and left lateral rior nasal retinal fibers approach the posterior as-
field in the left eye. Postchiasmatic defects pro- pect o the chiasm, the fibers shift to occupy the
duce homonymous hemianopias, with defects lateral aspect of the contralateral optic tract.
appearing more congruous (equal for both eyes)
the farther posteriorly the lesion is located. Vascular Supply
The arterial blood supply of the optic chiasm is
Anatomy derived from the circle of Willis, particularly the
The optic chiasm is an X-shaped intersection of carotid artery trunks and its branches, the supe-
the optic nerve axons located above the pituitary rior hypophyseal arteries, derived from the supr-
body within the sella turcica of the sphenoid aclinoid trunk of the carotids. A “prechiasmal
bone, which is covered by the diaphragm sellae plexus,” the hypophyseal portal system, and
(Figure 4-1). The chiasmatic cistern is located be- branches of the anterior cerebral arteries also
tween the chiasm and the diaphragm sella. Supe- contribute to the chiasmal blood supply.

NEURO-OPHTHALMOLOGY
64
OPTIC PATHWAYS

Figure 4-1

NEURO-OPHTHALMOLOGY
65
OPTIC PATHWAYS

Figure 4-2 Disorders Affecting Optic Chiasm


A. Lesions of anterior chiasm

LE RE
Optic nerve Lesion
Anterior
Chiasm

Optic tract
Posterior

Anterior chiasm tumor compressing optic nerve at its entrance to chiasm results in junctional scotoma consisting of a
central visual field loss in eye ipsilateral to lesion and a superior temporal defect in the opposite eye.
B. Lesions of central chiasm

Pituitary
adenomas

LE RE
Anterior
Lesion Optic nerve

Chiasm

Posterior Optic tract

Pituitary adenomas are most common lesion to affect chiasm. Central lesions which compress mid chiasm affect crossing
nasal retinal fibers, resulting in the typical bitemporal hemianopsia on visual field examination.
C. Lesions of posterior chiasm

Aneurysm

LE RE
Optic nerve Anterior

Chiasm
Lesion
Optic tract

© Posterior

Posterior communicating artery aneurysm affecting posterior chiasm combines features of both a chiasmatic and an optic
tract lesion, resulting in a posterior junctional scotoma—an incongruous (less dense in ipislaterial eye) hemianopic field loss
contralateral to lesion.

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OPTIC PATHWAYS

Venous drainage comprises 2 primary areas: Posterior optic chiasm lesions lead to a poste-
the superior aspect into the anterior cerebral rior junctional scotoma, which displays the fea-
veins and the inferior aspect draining into the in- tures of chiasmal and optic tract lesions. The clas-
fundibular plexus and thus to the paired basal sic finding is incongruous (less dense in the
veins of Rosenthal. ipsilateral eye) hemianopic visual field loss con-
tralateral to the lesion—from involvement of the
Disorders nascent optic tract and an inferotemporal visual
Pituitary adenomas are generally benign neo- field loss in the ipsilateral eye—from pressure on
plasms and the most common of the numerous the posterior chiasm affecting the late-crossing
diseases affecting the optic chiasm (Figure 4-2). superotemporal retinal fibers. Such defects oc-
Aneurysm, often of the anterior or posterior cur in disorders located near the anterior aspect
communicating artery, and trauma, especially of the third ventricle that approach the chiasm
from severe deceleration injury to the frontal from its posteriomedial aspect. The incongruous
skull, are other important etiologies. nature of the hemianopic defect is caused by the
Lesions of the anterior chiasm compress the incomplete intermixing of the decussating fibers
optic nerve at its entrance to the chiasm, result- on entering the optic tract with their corre-
ing in a junctional scotoma, a central visual field sponding uncrossed fibers from the contralateral
loss in the eye ipsilateral to the lesion and a su- eye.
perotemporal defect in the other, a combination
of the visual field characteristics of an optic OPTIC TRACT AND LATERAL
nerve lesion with chiasmal disease. The field loss GENICULATE NUCLEUS
in the contralateral eye is from involvement of Optic Tract
the anterior chiasm; early crossing fibers from Axons contained within the optic tract are
the inferonasal retina of the contralateral eye are those emanating from the retinal ganglion cells,
affected. Because the inferior nasal fibers decus- which have yet to synapse. Nevertheless, after
sate in the anterior aspect of the chiasm, the sco- they leave the chiasm for the optic tract, they
toma of the contralateral eye is typically super- nominally become part of the “posterior visual
otemporal. Central chiasmal lesions most pathway” (Figure 4-3). Axons that leave the optic
commonly produce a bitemporal hemianopsia chiasm and go to the lateral geniculate nucleus
that ensues when the optic chiasm is com- (LGN) within the thalamus form the optic tract,
pressed or damaged midsaggitally at its decussa- which resembles a round band, approaching the
tion. Such lesions preferentially affect the cross- LGN via the anterior limb of the internal capsule.
ing nasal retinal fibers responsible for temporal It courses between the tuber cinerum and the
vision, as in the vignette in this chapter. anterior perforated substance, continuing poste-
Progressive visual field loss from an expanding riorly as a band of flattened fibers around the
sellar tumor characteristically begins in the up- cerebral peduncles to synapse within the LGN.
per temporal field, likely from preferential com- A relatively small number of nonvisual retinal
pression of the inferior chiasm by the underlying origin fibers within the optic tract accompany
pituitary tumor exerting pressure on the chiasm the optic nerve and chiasm and supply the affer-
through the diaphragm sellae. Typically, this ent stimulus to the pupillomotor center within
deficit begins with visual acuity depression the pretectal nucleus. These axons for the pupil-
within the superotemporal quadrant, particularly lary fibers bypass the LGN laterally en route to
affecting perception of colors and small objects; the midbrain.
the remaining field remains normal. As the tumor
enlarges, the upper temporal quadrant becomes Lateral Geniculate Nucleus
an absolute peripheral depression, but central The LGN is a thalamic relay nucleus that
fixation is still spared. With further progression, serves as the synapse point of the retinal gan-
the lower nasal field is affected. Finally, the su- glion cells. It comprises 6 gray matter layers sep-
perotemporal field is affected, and, if progres- arated by 5 white matter layers. The layers are
sion continues, the whole field disappears. folded over, forming a bend or small knee. Each

NEURO-OPHTHALMOLOGY
67
OPTIC PATHWAYS

Figure 4-3

To visual cortex (area 17)


From visual cortex
To visual cortex (areas 18, 19)

Suprachiasmatic Pulvinar
nucleus
Pretectum

Superior colliculus

Nucleus of accessory
optic tract

Lateral geniculate
body

Pontine
tegmental reticular
nucleus

Inferior olive

To preganglionic
sympathetic neurons
(T1-2) that project to
the superior cervical ganglion
and regulate melatonin secretion
from the pineal gland

layer has a retinotopic organization, creating a terior parietal cortex, occipital cortex, and other
map of the contralateral hemifield. The ratio of thalamic nuclei. The LGN may use these nonreti-
geniculate cells to retinal axons is approximately nal elements to “screen” the visual input, gating
1:1. Retinal input to the LGN comprises only one certain inputs to the visual cortex while blocking
fifth of its afferent fibers. The remainder comes other signals, depending on the state of these
from the mesencephalic reticular formation, pos- other inputs.

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OPTIC PATHWAYS

The same vessels that supply the posterior chi- the primary visual cortex. After they leave the lat-
asm nourish the anterior one third of the optic eral geniculate body, they continue through the
tract: the internal carotid, middle cerebral, and posterior limb of the internal capsule. Most
posterior communicating arteries. The blood fibers take a fairly direct path to the calcarine cor-
supply of the posterior two thirds of the optic tex, following the curve of the internal capsule
tract is derived from the anterior choroidal through the parietal lobe to the occipital lobe.
artery, a branch of the internal carotid that runs However, the most inferior axons (Meyer loop)
posteriorly near the optic tract. The lateral genic- that carry visual information from the opposite
ulate body receives blood from the posterior superior field detour laterally around the lateral
cerebral artery and the posterior communicating ventricles and through the posterior temporal
arteries. lobe (Figure 4-4). Therefore, stroke or injury con-
fined to this portion of the temporal lobe affects
Disorders only this portion of the optic radiations. Meyer
Like all components of the posterior visual loop fibers rejoin the rest of the optic radiations
pathways, optic tract lesions are characterized by after their detour.
homonymous hemianopia. However, these le-
sions have a unique propensity to lead to com- Vascular Supply
bined homonymous hemianopia with pupillary Five primary arteries supply blood to the optic
changes and optic disk pallor. Total lesions of the radiation: the anterior and posterior choroidal ar-
optic tract, by affecting the pupillary afferents teries, the middle and posterior cerebral arteries,
within the tract, produce a mild relative afferent and the calcarine artery (Figure 4-5). The anterior
papillary defect in the contralateral eye because choroidal artery supplies the anterior portion of
slightly more crossed fibers exist within the tract the optic radiations, the optic tract, and the lateral
from that eye than uncrossed fibers from the ipsi- geniculate body. The anterior optic radiations are
lateral eye. When wallerian degeneration ensues, also fed by a meshwork of branches from the pos-
pallor characteristic of optic tract lesions develops terior choroidal arteries. The middle portion of the
in the optic disks. The ipsilateral eye, losing axons optic radiations, however, is fed via the deep optic
from the retina temporal to the fovea, has chiefly branch of the middle cerebral artery, which lies lat-
superior and inferior polar atrophy, whereas the eral to the ventricle. The posterior portion of the
contralateral eye, losing the interior of the papillo- optic radiation is fed by the posterior cerebral
macular bundle and the axons from the retinal artery and one of its branches, the calcarine artery.
nasal to the optic nerve, has pallor in the temporal
and nasal poles (“bow tie” atrophy). Disorders
As with the chiasm, neoplasms, aneurysms, Tumors, stroke, and demyelination are chief
and trauma are the typical lesion mechanisms. In injury mechanisms to the optic radiations. Le-
the optic tract and LGN, strokes are relatively un- sions confined to the temporal lobe can only af-
common. Depending on the severity of the le- fect the Meyer loop of the optic radiations. The
sion, the field loss is a complete homonymous resulting visual field defect is typically a superior
hemianopia or an incomplete incongruous one. wedge, with one side located at the vertical
In either instance, the visual field defect affects meridian, and the second edge being less sharp.
both eyes in the field of vision contralateral to This defect, resembling a “slice” removed from
the affected site. Incomplete lesions often pro- the superior visual field, has been termed a “pie
duce wedge-shaped defects, with the point of in the sky” defect. When encountered, it pro-
the wedge encroaching on the center, a “dagger vides strong evidence of a temporal lobe origin.
into fixation.” Often, other neurologic deficits compatible with
temporal lobe dysfunction confirm the visual
OPTIC RADIATIONS field findings. Generally, these lesions are mildly
Anatomy incongruous.
The optic radiations are myelinated axons em- Conversely, if a parietal lesion affects the optic
anating from cell bodies in the LGN, coursing to radiations anteriorly, an inverse lesion sparing

NEURO-OPHTHALMOLOGY
69
OPTIC PATHWAYS

Figure 4-4
Central darker circle
represents macular zone.
Overlapping Lightest shades represent
visual fields monocular fields.
Each quadrant is
a different color.

Projection on Projection on
left retina right retina

Optic (II)
nerves
Optic
chiasm
Projection on left Projection on right
dorsal lateral dorsal lateral
geniculate nucleus Meyer Optic Meyer geniculate nucleus
Ipsilateral Ipsilateral
loop tracts loop
6 6
5 5
4 Lateral 4
3 geniculate 3
2 bodies 2
1 1

Contralateral Contralateral

Projection on left
occipital Projection on right
lobe occipital
lobe

Calcarine
fissure
with

the temporal lobe “wedge” occurs; however, body, parietal lobe, or occipital lobe. However,
such lesions are rarely encountered. Occasion- the rare optic tract lesions also produce a mild
ally, a larger or more posterior parietal lesion can contralateral relative afferent pupil defect and
affect all of the optic radiations when the Meyer contralateral bow tie atrophy of the optic disk.
loop has rejoined the other fibers, producing a Thus, when present, these additional findings
complete homonymous hemianopia. help to localize the responsible lesion. Similarly,
Complete hemianopias are “non-localizing”; because areas within the parietal lobe contribute
by examining the visual field, there is no evi- to pursuit eye movements, patients with a com-
dence whether they are secondary to a lesion at plete homonymous hemianopia from a parietal
the level of the optic tract, lateral geniculate lesion may show altered or absent pursuit move-

NEURO-OPHTHALMOLOGY
70
OPTIC PATHWAYS

Figure 4-5 Arteries of Brain: Inferior Views

Distal medial striate artery


(recurrent artery of Heubner)
Anterior communicating artery
Anterior cerebral artery
Middle cerebral artery
Posterior communicating artery
Anterior choroidal artery
Optic tract
Posterior cerebral artery
Cerebral crus
Lateral geniculate body
Posterior medial choroidal artery
Posterior lateral choroidal artery
Choroid plexus of lateral ventricle
Medial geniculate body
Pulvinar of thalamus
Lateral ventricle

ments in the direction toward the lesion. Such become a practical addition to current diagnos-
defects are best demonstrated clinically with a tic tools.
standard optokinetic nystagmus drum.
REFERENCES
FUTURE DIRECTIONS Hedges TR, Friedman D, Horton J, Newman SA, Striph G,
Advances in diagnosis have focused on im- Kay MC. Neuro-ophthalmology. In: Weingeist TA,
proved imaging techniques. For example, diffu- Liesegang TJ, Slamovits TL, eds. Basic and Clinical Science
Course, 1997-1998, San Francisco, Calif: American Acad-
sion MRI scanning allows for better detection of
emy of Ophthalmology; 1997:55-70.
early, focal brain edema after stroke. Functional Miller NR, Newman NJ, eds. Walsh and Hoyt’s Clinical Neuro-
MRI scanning, allowing the MRI scan to detect ophthalmology. 5th ed. Baltimore, Md: William & Wilkins;
pH or neurochemical abnormalities focally, may 1998.

NEURO-OPHTHALMOLOGY
71
Chapter 5

Primary Visual Cortex


Ippolit C. A. Matjucha and Robert J. Updaw II

Clinical Vignette
A 64-year-old gynecologist, while operating, suddenly had difficulty seeing to the right. He had to turn his head
to see the full operative field. The next day, he saw his ophthalmologist, who found evidence of a dense right
homonymous hemianopsia.
A subsequent neurologic consultation was otherwise unremarkable. MRI demonstrated a hypodensity in the left
occipital lobe. ECG and transesophageal echocardiography results were normal. A 48-hour Holter monitor docu-
mented 7 periods of intermittent atrial fibrillation. Anticoagulation was initiated. The patient was advised to have
his wife do all the driving.

This vignette typifies an embolus to the left terneurons. The thickest stria is layer IV, compris-
posterior cerebral artery causing a left occipital ing almost half the depth of the visual cortex.
lobe infarct. Although occasionally such patients Highly branched stellate cells exist superficially
improve, often individuals have no substantial within layer IVa. The Gennari stripe comprises
resolution of function. Prevention of further layer IVb, containing myelinated axons from af-
strokes is needed, by treating the atrial fibrilla- ferent visual (geniculate) cells and cortical asso-
tion or, if unsuccessful, using anticoagulants to ciation fibers. Pyramidal and granule cells and gi-
prevent further atrial thrombi from forming. ant pyramidal (Meynert) cells occur more deeply
Complete driving restriction is essential because at IVc. Layer V is a densely cellular region with
of the total inability to perceive objects in the variously sized pyramidal cells. Layer VIa is a less
densely lost field. cellular superficial portion, and layer VIb con-
tains a varied neuronal population.
ANATOMY
Axons of the optic radiations synapse with the Vascular Supply
primary visual cortex. A unique white stripe or The blood supply of the striate cortex primar-
stria (stripe of Gennari for the discovering ily derives from the calcarine artery, a branch of
anatomist), representing a myelin-rich cortical the posterior cerebral artery, and sometimes
layer, is easily seen in gross sections through the the middle cerebral artery, or anastamoses
cortex with its layered, highly structured organi- from it (Figure 5-2). Usually, the calcarine artery
zation of V1, also known as the primary visual supplies most of the visual area. However, in
cortex, the striate cortex, or Brodmann area 17. 75% of cases, other arteries contribute: the pos-
Primarily located on the mesial surface of the oc- terior temporal or parietooccipital arteries, occa-
cipital lobe within and surrounding the calcarine sionally aided by the middle cerebral artery.
fissure, the most posterior aspect of V1 typically
wraps around the posterior (occipital) pole for a Topographic Organization
short distance (Figure 5-1). Specific anatomical correlations are the pri-
Microscopically, the visual cortex is arranged mary clinical features pertinent to the striate cor-
in 6 laminae, running from the surface to a depth tex: visual information from the left visual field in
of slightly greater than 2 mm. The most superfi- each eye is projected to the right visual cortex
cial, layer I, primarily contains glial cells. Layers II (and conversely); the superior visual field is pro-
and III contain pyramidal cells and small in- jected into the inferior half of V1 (and con-

NEURO-OPHTHALMOLOGY
72
PRIMARY VISUAL CORTEX

Figure 5-1A Functional Organization of the Cerebral Cortex


Central sulcus Supplemental
motor cortex Superior
parietal lobule
Primary motor
cortex
Frontal
eye fields Premotor Primary
cortex somatosensory
cortex
Primary Wernicke
trigeminal Secondary area
Broca region of somatosensory Visual association
area cortex
motor somatosensory areas of cortex
cortex cortex Primary Multisensory
Auditory association
Lateral fissure cortex areas of cortex Primary
visual
cortex

Parietal lobe

Spatial visual pathway: positional


Figure 5-1B Visual Cortex
Parietal lobe

Frontal lobe Spatial visual pathway: positional


relationship between objects in
visual scene, analysis of motion

Middle temporal area:


direction selective and
motion responsive
MT
V3
V2 Occipital
lobe

V4 V1
V3
V2

Temporal lobe
V4: shape and color perception

Object recognition pathway: high resolution and form

Figure 5-1C Medial Aspect of the Cerebral Cortex


Primary motor cortex Precentral sulcus

Paracentral lobule
Supplemental Parietal
motor cortex Frontal
Limbic Somatosensory association cortex
Limbic cingulate
cortex Corpus callosum
Occipital

Visual association cortex


Thalamus
Primary visual cortex

Pituitary gland

Calcarine
fissure

Cerebellum
Pons
Medulla oblongata
73
PRIMARY VISUAL CORTEX

Figure 5-2 Arterial Supply of Cerebral Cortex

Anterior cerebral artery

Middle cerebral artery

Posterior cerebral artery

versely); and the most central visual field is pro- to the periphery. Up to 50% of the cortex may
jected most posteriorly, whereas the peripheral correspond to the central 10° of vision; in fact,
field is located anteriorly within V1. the most central 1° of vision uses as much cortex
“Cortical magnification” in V1 results in much posteriorly as the most peripheral 50°. Cortical
more cortex dedicated to the central area than magnification is considered a reflection of the

NEURO-OPHTHALMOLOGY
74
PRIMARY VISUAL CORTEX

evolutionary importance of precise central vi- tics of visual field defects from V1 lesions pro-
sion to survival. vide a diagnostic set leading to an anatomical lo-
Ocular dominance columns run at right angles calization even before imaging procedures. The
to the cortical surface. Within a column, visual in- hemianopic visual field loss from V1 lesions, if in-
put is derived from one eye only; in the immedi- complete, shows close congruence of deficit be-
ate neighboring cortical surface, perhaps only tween the eyes. The small size and close proxim-
0.5 mm from that column, another column de- ity of the left and right ocular dominance
riving input from the other eye is encountered. columns make it impossible to selectively dam-
Monocular occlusion during the early postna- age the visual field of only one eye.
tal period demonstrates that the columns of the The extreme temporal visual field of each eye
occluded eye grow smaller, whereas the represents an exception to this principle. Be-
columns of the open eye enlarge. Subsequent cause the nasal visual field extends only approx-
uncovering of the occluded eye does not restore imately 65°, the remaining lateral field on each
the equality of the columns, which is considered side is supplied solely by the ipsilateral eye. This
central to understanding critical periods in visual “temporal crescent” of the visual field corre-
development. The failure of that development is sponds to the most anterior aspect of V1, abut-
designated amblyopia. ting the occipitoparietal fissure, where ocular
A hierarchy exists to the processing of visual dominance columns are absent, because all in-
information at a cellular level. The striate cortex put comes from the contralateral eye’s nasal
has different cell types that respond to increas- retina. Therefore, in a lesion of the anterior stri-
ingly specific stimuli, including simple cells, ate cortex, if only the most temporal 25° is af-
which have the same light/dark, center/sur- fected, a “monocular homonymous defect” may
round response profile as retina and lateral result.
geniculate nucleus cells. Additionally, complex Because of the specialized nature of V1, le-
and hypercomplex cells respond best to a light sions there affect vision and no other neurologic
stimulus that is not a spot but a line at a particu- function. In addition to the extreme congruity of
lar angle or a specific length to achieve an opti- the visual field defects of the 2 eyes, striate cor-
mal cell response. tex lesions have no signs of anterior visual path-
This hierarchical structure suggests that addi- way involvement, ie, no optic pallor and no rela-
tional cell types, probably located in extrastriate tive afferent papillary defect. Typically, central
association cortices, respond to more specific acuity is not affected. This deficit isolation differs
and complex stimuli until, eventually, the puta- from parietal lesions that typically affect both vi-
tive “grandmother cell”—the unique cell that sual field (homonymous contralateral loss) and
fires when a picture of one’s grandmother is eye movements (loss of contralateral lateral sac-
seen—is encountered. Although instructive and cade, manifesting as uncorrected ipsilateral drift
fanciful, this concept is incorrect; single-neuron on testing with an opticokinetic nystagmus drum
deaths in the brain occur daily, but the inability to turning toward the affected side).
recognize a single person when other individu- Rarely, bilateral occipital cortical lesions occur
als are recognized normally is not a described simultaneously. Generalized systemic hypoten-
disease state. There may be “higher” association sion, such as from a cardiac arrest or basilar or bi-
cortices, with groups of cells producing specific lateral posterior cerebral artery occlusion,
patterns of neuronal activation that represent causes bilateral ischemic damage. Similarly, di-
the anatomical correlate for perceptual recogni- rect or contrecoup traumatic damage occurs
tion. when both occipital poles are injured. Initially, bi-
lateral occipital pole lesions may be confused
DISEASES OF THE STRIATE CORTEX with bilateral optic nerve lesions because an ap-
Striate cortex lesions, like other neurologic le- parent “central scotoma” is found in each eye.
sions, can be classified into ischemic, neoplastic, However, careful visual field mapping along the
and demyelinating disease; infection, especially vertical axis demonstrates a discontinuity, a ver-
from septic emboli, is rare. Clinical characteris- tical step. The vertical step is expected because

NEURO-OPHTHALMOLOGY
75
PRIMARY VISUAL CORTEX

these cortical injuries should not be absolutely driven by orientation of stimulus, whereas V3
symmetric, so the extent of visual field loss contains cells that favor specific direction of
should vary in size between the left and right stimulus motion. V2, V4, and VP (ventral parietal
hemifields. The size difference is seen most eas- area) are influenced by stimulus color and thus
ily at the vertical meridian, resulting in a keyhole may influence color vision. V5 or the middle
defect. Like temporal crescent defects, keyhole temporal area seems to select for certain stimu-
defects are characteristic of occipital lobe le- lus speeds. V6, perhaps correlated with the hu-
sions. man parietooccipital visual cortex, varies from
Macular sparing in visual field defects is ex- V1 in not having retinotopic organization or cen-
pected with incomplete striate cortex destruc- tral magnification and may be a visual/so-
tion and preservation of the posterolateral ex- matosensory interface. The macaque medial su-
tension around the occipital pole, where the perior temporal area seems to help decipher
total loss of the cortex is certain (eg, surgical re- complex visual motion.
moval), and as a testing artifact from inconstant
fixation of the tested eye on the target center. FUTURE DIRECTIONS
The anatomy of V1, with the most central visual Research continues to describe new areas of
field represented on the posterior pole rather visual association cortex that subserve specific
than on the mesial occipital surface and often aspects of vision, increasing the possibility of rec-
supplied by the middle rather than the posterior ognizing deficits experienced by patients with le-
cerebral artery, may make it likely that even le- sions in these areas and guiding investigations of
sions affecting most of V1 may miss the most an- poorly understood visual problems, such as vi-
terior, central vision area. Such sparing is due to sual hallucinations and palinopsia (persistence
relative anatomical and vascular isolation of that of visual sensation after the stimulus has ended
cortical area from areas representing more pe- or moved).
ripheral vision. Advancing imaging techniques may help to
improve sensitivity and precision in diagnosis.
EXTRASTRIATE ASSOCIATION Specific protection of neurons after ischemic or
VISUAL AREAS traumatic injury remains at the far horizon of re-
Brodmann areas 18 and 19, immediately adja- search, as do regenerative therapies for neural
cent to area 17, in the area surrounding the cal- tissue.
carine fissure, were termed the parastriate or as-
sociation visual cortex on the assumption that REFERENCES
they functioned to “associate” the visual data Horton JC, Hoyt WF. The representation of the visual field in
from V1 with brain areas regarding spatial orien- human striate cortex: a revision of the classic Holmes
tation, recognition, and language. Human corre- map. Arch Ophthalmol. 1991;109:816-824.
lates of the described macaque areas remain un- Hubel DH. Eye, Brain and Vision. New York, NY: WH Free-
man; 1995. Scientific American Library; No. 22.
certain. Nicholls JG, Martin AR, Wallace BG, Fuchs PA. From Neuron
In brief, areas V2 and V3 are adjacent to V1, to Brain. 4th ed. Sunderland, Mass: Sinauer Associates;
sharing its retinotopic organization. V2 cells are 2001.

NEURO-OPHTHALMOLOGY
76
Chapter 6

Cranial Nerve III: Oculomotor


Ippolit C. A. Matjucha

Clinical Vignette
A 37-year-old woman presented with a 2-day history of disturbed vision on upward gaze, vertical binocular
diplopia, and headache. One month previously, she had experienced the same symptoms for 5 days. Sinusitis was
diagnosed, and an antibiotic was prescribed.
Examination demonstrated impaired upward gaze in the right eye and poor downward gaze and adduction. She
had ptosis on the right, and the pupil was slightly larger and reacted poorly compared with the left.
MRI yielded normal results, but catheter angiography demonstrated a posterior communicating artery (P-com)
aneurysm. At craniotomy that same night, the neurosurgeon clipping the 5 ⫻ 10-mm aneurysm reported fresh and
old clot around the aneurysm that was compressing the right oculomotor nerve. The patient had an uneventful re-
covery, with gradual resolution of the neuro-ophthalmologic findings.

The oculomotor nerves course from the ventral The pupil is dilated and immobile (paralysis of the
midbrain to the orbits. CN-III provides the general sphincter pupillae), and the eye is focused for the
far point and cannot accommodate for near by
somatic motor efferent innervation controlling up-
(paralysis of the ciliary muscle).*
per lid elevation and most of the extraocular
movements upward, medially, and downward. In
addition, CN-III carries the general visceral motor ANATOMICAL CORRELATIONS
(parasympathetic) efferent innervation responsi- Oculomotor Nucleus
ble for pupillary constriction and accommodation The CN-III nucleus is a lepidopteroid collec-
(near focus) of the crystalline lens. tion of 9 subnuclei located in the center of the
Any individual who has an oculomotor rostral midbrain at the level of the superior colli-
(CN-III) paresis must be evaluated for a P-com culi (Figure 6-2). The most ventral of these sub-
aneurysm (Figure 6-1A). Often, such patients nuclei is the central caudate nucleus, a midline
present with a concomitant severe headache, structure that innervates both levator palpebrae
described as the worst one they have ever had. muscles. Because of the bilateral innervation,
When patients have diabetes and the pupil is un- when CN-III is injured at the nucleus, the pa-
affected, the lesion may be related to a mi- tient’s eyelids exhibit bilateral blepharoptosis or
crovasculopathy of CN-III (Figure 6-1B). How- are normal, depending on whether the injury ex-
ever, this diagnosis of exclusion cannot be made tends to the central caudate nucleus.
until imaging studies rule out an aneurysm. Similarly, axons from the right and left medial
columns, the subnuclei serving, respectively, the
CLINICAL PRESENTATION left and right superior rectus muscles, immedi-
The following description from the early 20th ately decussate across the midline so each
century captures the classic presentation of a innervates the contralateral superior rectus—
CN-III palsy: hence the clinical presentation of a nuclear CN-
III palsy wherein the contralateral eye displays
Oculo-motor paralysis ... presents a characteristic defective upward gaze, with the remaining ocu-
picture. The upper lid hangs loosely down (pto-
lar motor deficits seen ipsilaterally.
sis) and has to be drawn up with the finger to give
a view of the eyeball, which is deflected strongly The other 6 subnuclei likewise form 3 left-and-
outward and somewhat down, because the two right pairs but innervate ipsilateral extraocular
muscles not paralyzed—the [lateral] rectus and
the superior oblique—draw it in this direction. *Duane, page 310.

NEURO-OPHTHALMOLOGY
77
CRANIAL NERVE III: OCULOMOTOR

Figure 6-1A Ophthalmologic Manifestations of Cerebral Anerurysms

Retinal changes

Oculomotor palsy: Ptosis, eye turns laterally and inferiorly, pupil dilated; common finding
with cerebral aneurysms, especially carotid-posterior communicating aneurysms

Neuromuscular disorders
Abducens palsy: Affected eye
turns medially. May be first
manifestation of intracavernous Optic atrophy may develop
carotid aneurysm. Pain above as a result of pressure
eye or on side of face may be on the optic (II) nerve from
secondary to trigeminal (V) a supraclinoid carotid,
nerve involvement. ophthalmic, or anterior
cerebral aneurysm.

Basilar a.
Tentorium (divided)
R. trochlear (IV) n.
R. oculomotor (III) n.
R. trigeminal (V) n.
Posterior clinoid process
Middle fossa

R. internal carotid a. Pons

Papilledema may be caused


R. ophthalmic a. by increased intracranial
pressure secondary to
rupture of a cerebral
R. optic (II) n. aneurysm.

R. anterior Cerebellum
cerebral a.

R. middle R. superior
cerebral a. cerebellar a.

Aneurysm
R. posterior
communicating a.
Hemorrhage into optic (II)
R. posterior nerve sheath after rupture
cerebral a. of aneurysm may result
Temporal lobe
(elevated) in subhyaloid hemorrhage
with blood around disc.

NEURO-OPHTHALMOLOGY
78
CRANIAL NERVE III: OCULOMOTOR

Figure 6-1B Ocular Complications of Diabetes

VI

III

IV

Attempted left gaze in left


sixth cranial nerve palsy

NEURO-OPHTHALMOLOGY
79
CRANIAL NERVE III: OCULOMOTOR

Figure 6-2
Oculomotor (III), Trochlear (IV), and Abducent (VI) Nerves: Schema

Long ciliary nerve Ciliary ganglion


Short ciliary nerves Posterior ethmoidal nerve Abducent nucleus
Anterior ethmoidal nerve Sensory root of ciliary ganglion
Superior oblique muscle Trochlear nucleus
Sympathetic root of ciliary ganglion
Levator palpebrae Oculomotor nucleus
superioris muscle Superior division of
Superior oculomotor nerve
Accessory oculomotor
rectus muscle Frontal nerve (cut) (Edinger-Westphal)
nucleus (parasympathetic)
Lacrimal nerve (cut)

Nasociliary nerve Trochlear nerve (IV)

Oculomotor nerve (III)

Ophthalmic nerve (V1)

Infraorbital nerve Abducent


nerve (VI)
Zygomatic nerve (cut) Pterygopalatine
Mandibular nerve (V3)
Inferior oblique muscle ganglion
Inferior division of Internal carotid artery
Ciliary muscle and nerve plexus
oculomotor nerve
Dilator muscle of pupil Maxillary nerve (V2)
Medial rectus muscle
Sphincter muscle of pupil
Lateral rectus muscle and
Inferior rectus muscle abducent nerve (turned back)
Efferent fibers
Parasympathetic root Cavernous plexus
Afferent fibers
of ciliary ganglion
Sympathetic fibers Common tendinous ring
Parasympathetic fibers

NEURO-OPHTHALMOLOGY
80
CRANIAL NERVE III: OCULOMOTOR

muscles. The ventral nucleus, intermediate col- caudal extension also affect the brachium con-
umn, and dorsal nuclei, respectively, control the junctivum, producing ipsilateral cerebellar
medial rectus (eye adduction), inferior oblique ataxia, called Claude syndrome. When damage
(intortion and some elevation), and inferior rec- extends ventrally into the basis pedunculi, corti-
tus (depression). cospinal tract dysfunction causes Weber syn-
The CN-III nucleus receives numerous affer- drome: hemiplegia contralateral to the CN-III
ents, including inputs from the paramedian pon- palsy.
tine reticular formation for horizontal eye move-
ment, the rostral interstitial nucleus of the medial Subarachnoid Oculomotor Nerve
longitudinal fasciculus for vertical and torsional The nascent oculomotor nerve emerges from
movements, and the vestibular nuclei. Other af- the medial surface of the cerebral peduncle to
ferents come from the superior colliculi, the oc- enter the interpeduncular cistern. It crosses the
cipital cortex, and the cerebellum. cistern for approximately 5 mm, passing under
Sometimes considered a subnucleus of CN-III, the posterior cerebral artery. It follows beneath
the Edinger-Westphal nucleus abuts the others the P-com for 10 mm, and then pierces the dura
rostrodorsally, residing at the ventral edge of pe- underneath the P-com en route to the cavernous
riaqueductal gray matter. The Edinger-Westphal sinus. This short segment of CN-III forms the
nucleus supplies the cholinergic efferents pro- pathophysiologic territory for an important
ducing pupillary constriction and ciliary muscle pupillary finding in clinical neurology. Typically,
contraction (lens accommodation). Afferents in obtunded or comatose patients, when cere-
from the pretectal nuclei mediate the pupillary bral edema leads to uncal herniation, the ipsilat-
light reflex, whereas inputs influencing pupil con- eral anterior temporal lobe is pushed medially
striction and lens accommodation in response to and downward through the tentorial window.
near visual stimulus originate from striate and The uncus of the temporal lobe compresses the
prestriate cortex and the superior colliculus. ipsilateral posterior cerebral artery downward
When the pupillary fibers join the oculomotor onto CN-III, with a resultant ipsilateral sluggish
nerve, they move exteriorly and dorsally within pupillary light reaction. With external compres-
the nerve, a clinical continuation of the spatial re- sion of CN-III, pupillary motor fibers typically are
lation of the Edinger-Westphal nucleus to CN-III. affected earlier than extraocular motor fibers, an
observation usually attributed to the exterior po-
Oculomotor Fasciculus sition of the pupil fibers on the surface of CN-III.
Axons from the CN-III nucleus gather into a As compression worsens, a fully dilated pupil de-
fascicle and sweep ventrally in an arc curving to- velops. Shortly thereafter, other signs of oculo-
ward the medial surface of the cerebral pedun- motor nerve dysfunction usually occur. There-
cle, passing through the red nucleus. The cere- fore, periodic checking of pupil function in
bral peduncle at this level carries the comatose patients remains an indispensable
corticospinal tract axons that innervate the con- clinical tool.
tralateral somatic musculature; the red nucleus
provides secondary motor inputs contralaterally. Cavernous Sinus and Superior Orbital Fissure
Bordering the red nucleus is the superior cere- The cavernous sinus is part of the intracranial
bellar peduncle (brachium conjunctivum), cau- venous system. It receives blood from the oph-
dally comprising cerebellocortical axons aiding thalmic vein and sphenoparietal sinus, transmit-
motor control ipsilaterally, and the medial lem- ting this flow to the superior and inferior petrosal
niscus laterally that relays contralateral somatic sinuses. The left and right cavernous sinuses are
afferents. connected via the intracavernous plexus; they
CN-III fasciculus lesions at the red nucleus pre- also communicate with the basilar sinus and the
sent as oculomotor palsy with crossed pterygoid and foramen ovale plexuses. The cav-
hemitremor, Benedikt syndrome. If the lesion ernous sinus resides lateral to the pituitary gland,
extends to the medial lemniscus, there is also resting atop the roof and lateral wall of the sphe-
contralateral hypesthesia. Similar lesions with noid sinus. Besides venous blood, the space con-

NEURO-OPHTHALMOLOGY
81
CRANIAL NERVE III: OCULOMOTOR

tains the intracavernous portions of CN-III, -IV, crovascular” vasculopathies—not an aneurysm.


and -VI; the ophthalmic branch of CN-V and its Typically, these palsies have a favorable progno-
maxillary nerve posteriorly; the internal carotid sis and uncomplicated recovery within 2 to 4
artery; and the sympathetic nerve fibers invest- months.
ing the adventitia of the internal carotid artery. Whether a spared pupil reliably excludes an
CN-III, -IV, -VI and the ophthalmic nerve all leave aneurysm deserves discussion. Certainly, with in-
the cavernous sinus to enter the orbit via the su- stances of an incomplete extraocular CN-III
perior orbital fissure. palsy, the absence of pupil involvement must not
Given the confluence of these structures into be considered evidence of a benign etiology.
this relatively small sinus, neurologic disorders in This is particularly true in nonelderly patients
this area are prone to produce multiple CN with few microvascular risk factors. The relative
palsies, often with pain in the ophthalmic distrib- ease of access to MRI and MRA has removed
ution. If the pathologic process is extensive, this concern. Still, two caveats exist about nonin-
signs of venous obstruction in the orbit also de- vasive neuroimaging: an aneurysmal complete
velop. extraocular CN-III palsy without pupillary in-
volvement is exceedingly rare, but a negative
Orbit noninvasive study does not guarantee absence
CN-III typically divides into superior and infe- of aneurysm.
rior branches within the anterior cavernous si- Open or closed head injuries also may lead
nus, thus entering the orbit as two distinct struc- to oculomotor nerve palsies. The suspected lo-
tures. The superior branch supplies the superior cus of injury is the dural entrance, wherein the
rectus and levator palpebrae muscles. The infe- nerve is fixed in relation to the petrous bone, al-
rior branch provides somatic innervation to the lowing a violent shift of the intracranial contents
medial and inferior recti and the inferior oblique to produce traction injury to CN-III. Typically,
and supplies the parasympathetic pupil inputs to these are associated with severe frontal deceler-
the ciliary ganglion, located superiolaterally to ation impact (eg, unrestrained occupant in a mo-
the midorbital optic nerve. The parasympathetic tor vehicle accident) with loss of consciousness.
axons from the Edinger-Westphal nucleus In cases of CN-III palsies with pupillary involve-
synapse here, with the postsynaptic neurons ment, concomitant with trivial injuries, an under-
providing visceral motor control to the iris lying pathology must be sought with appropriate
sphincter and the ciliary muscles via the short cil- imaging.
iary nerves. The prognosis for recovery in traumatic CN-III
At the orbital apex, the optic nerve and oph- palsy is guarded; if recovery occurs, it is usually
thalmic artery enter the orbit via the optic fora- marked by aberrant regeneration. In such im-
men just nasally to the superior orbital fissure. perfect healing, axonal regrowth is misdirected
such that nerve branches no longer innervate
DIFFERENTIAL DIAGNOSIS their expected muscle groups. The best-known
Posterior communicating artery aneurysms example is the pseudo-Graefe sign, in which the
are the most common cause of acute headaches branch of CN-III that normally innervates the in-
with signs of CN-III palsy with pupillary involve- ferior rectus now synkinetically innervates the le-
ment. The typical location of these aneurysms is vator palpebrae, causing the upper lid to lift on
the origin of P-com at the internal carotid artery downward gaze (clinically simulating the lid lag,
(Figure 6-3). Up to 30% of acquired CN-III or Graefe sign, of Graves orbitopathy). Internal
palsies develop from such aneurysms, and 90% motor efferents can likewise be involved, result-
of aneurysms present with CN-III palsy. ing in a change of pupil size as gaze is shifted.
However, not all patients with acquired CN-III Duane syndrome is an example of a congeni-
palsies have aneurysms. Pupillary findings pro- tal aberrant innervation. In affected individuals,
vide a paramount distinguishing feature. In ap- attempted lateral eye movement results in simul-
proximately 80% of CN-III palsy cases, the pupil taneous stimulation of the medial and lateral
is spared, and the cause is diabetes or other “mi- recti, causing variable eye movement, measur-

NEURO-OPHTHALMOLOGY
82
CRANIAL NERVE III: OCULOMOTOR

Figure 6-3 Posterior Communicating Artery Aneurysm


Compressing Cranial Nerve III

Internal carotid a.

Cavernous sinus
Oculomotor (III) n. (divided)

Trochlear (IV) n.

Trigeminal (V) n.

Abducens (VI) n.
Oculomotor (III) n. (divided)

Posterior communicating a.

Posterior cerebral a.
Basilar a.

able globe retraction into the orbit, and conse- pupil dysfunction is often encountered when the
quent pseudoptosis. A prenatal abducens nerve carotid artery wall is involved and is noticeable
dysgenesis or injury may result in subsequent because the associated CN-III palsy often spares
misdirection in these cases. The congenital na- the pupil. The usual etiology is an idiopathic cav-
ture of this condition is most easily deduced by ernous sinus inflammation, although infection
the absence of symptomatic diplopia in lateral and tumor must be considered. Most instances
gaze despite the presence of noncomitant stra- relate to the spectrum of inflammatory pseudo-
bismus. tumor. Neuroimaging confirms the diagnosis.
Cavernous sinus thrombosis, often as a septic Treatment with high-dose corticosteroids is indi-
complication of central facial cellulitis, is a dreaded cated.
clinical entity. A septic phlebitis of the facial vein or Cavernous sinus syndrome is usually a
pterygoid plexus is the usual intermediary be- chronic noninflammatory process characterized
tween cellulitis and infectious thrombosis. by a cranial polyneuropathy. Typically, it affects
Acute pain occasionally develops associated CN-III, -IV, and -VI and the ophthalmic branch of
with an aneurysm, especially at the origin CN-V. Slowly growing tumors within the cav-
of the meningohypophyseal trunk. Such ernous sinus are in the differential. The clinical
aneurysms classically present with primary CN- history often includes chronically increasing
III synkinesis, ie, aberrant regeneration without diplopia, sometimes with pain or numbness in
antecedent palsy. Because this type of aneur- the CN-V ophthalmic distribution. Therefore, any
ysm exists within a venous space, a ruptured patient presenting with diplopia, initially thought
aneurysm may produce a high-flow carotid cav- to be related to a cranial mononeuropathy, must
ernous fistula, a lesion having less devastating have careful examination of the adjacent CN to
sequellae than ruptured subarachnoid exclude involvement of other CNs. Similarly, pa-
aneurysms. However, one case of a subarach- tients presenting with new upper facial pain
noid aneurysm presenting with primary CN-III must always be checked for impaired eye move-
synkinesis has been reported. ments and corneal hypesthesia to exclude early
Tolosa-Hunt syndrome is a painful ophthal- cavernous sinus syndrome. Superior orbital fis-
moplegia without venous obstruction that typi- sure syndrome is indistinguishable from cav-
cally varies in degree over days. Sympathetic ernous sinus syndrome.

NEURO-OPHTHALMOLOGY
83
CRANIAL NERVE III: OCULOMOTOR

Lesions producing diminished vision with in- accommodation but without ptosis or extraocu-
ternal (ie, pupillary involvement) ophthalmople- lar immobility. Patients report of photophobia in
gia, external ophthalmoplegia, and pain with bright light and blurred near vision.
corneal hypesthesia characterize orbital apex The postsynaptic axons reinnervate the iris
syndrome. In simplified terms, this syndrome sphincter and ciliary muscles. Their regenera-
clinically is characterized by findings of Tolosa- tion is apparently directed at random, such that
Hunt with a concomitant compressive optic previous ciliary fibers find their way to the iris or
neuropathy. Inflammation and tumor are the ciliary body. When regrowth is complete, over a
usual etiologies. few months, the resulting pupil is typically mid-
Because CN-III divides into superior and infe- dilated, constricts poorly to light, reacts slowly
rior rami just before its entrance into the orbit, ip- but more fully to accommodative stimulus, and
silateral dysfunction of the superior rectus and le- redilates slowly, ie, tonically. Signs of incom-
vator palpebrae muscles seems to indicate an plete iris reinnervation also occur, including ir-
orbital or anterior cavernous sinus pathologic regular pupillary shape from persisting segmen-
site. However, more proximal intracranial dis- tal denervation and a hypersensitivity to
ease is often responsible. acetylcholine analogs from muscarinic receptor
Inferior division palsies sometimes occur from up-regulation.
a presumed orbital lesion. However, these
palsies usually spare the pupil, the results of REFERENCES
imaging studies are normal, and spontaneous re- Arle JE, Abrahams JM, Zager EL, Taylor C, Galetta SL. Pupil-
covery is observed—in all ways mimicking the sparing third nerve palsy with preoperative improvement
from a posterior communicating artery aneurysm. Surg
microvascular CN-III palsies discussed in this Neurol. 2002;57:423-426.
chapter. Duane A. Motor anomalies of the eye. In: Fuchs HF. Text-
Adie tonic pupil is another example of aber- book of Ophthalmology. 8th ed. Duane A, trans. Philadel-
rant regeneration affecting a facet of CN-III func- phia, Pa: JB Lippincott; 1924:267-364.
tion with a probable intraorbital location within McFadzean RM, Teasdale EM. Computerized tomography
angiography in isolated third nerve palsies. J Neurosurg.
the ciliary ganglion. Typically, no specific etiol- 1998;88:679-684.
ogy exists. In the acute setting, Adie pupil pre- Miller NR, Newman NJ, eds. Walsh and Hoyt’s Clinical Neuro-
sents as unilateral parasympathetic ocular den- ophthalmology. 5th ed. Baltimore, Md: William & Wilkins;
ervation, with consequent mydriasis and loss of 1998.

NEURO-OPHTHALMOLOGY
84
Chapter 7

Cranial Nerve IV: Trochlear


Ippolit C. A. Matjucha

Clinical Vignette
A workman, bent over head-down, sustained a scalp laceration over the left occiput when a coworker dropped
a tool from above. Diplopia and headache subsequently developed.
Examination revealed poor depression of the right eye in leftward gaze. Prismatic spectacle lenses were pre-
scribed to alleviate the diplopia. After a few months, the patient reported that his vision had returned to normal.

This vignette describes a seemingly mild CLINICAL PRESENTATION


closed head injury with isolated injury to the Patients with trochlear palsy have impaired
trochlear nerve (CN-IV), often the most benign of ability to depress the eye on the involved side, a
the cranial neuropathies, particularly those re- hypertropia. Worse when the eye is in downward
lated to extraocular muscle function. The CN-IV or medial gaze or when the head is inclined to-
nuclei are located in the lower midbrain, off mid- ward the side of palsy, it emphasizes the weak-
line at the ventral edge of the periaqueductal ness of the depressor function of the superior
gray, at the level of the inferior colliculi (Figure 7- oblique.
1). The nuclei are crossed; the left trochlear nu- With a superior oblique palsy, head tilt toward
cleus innervates the right superior oblique and the palsied side emphasizes the rotary compo-
vice versa (Figure 1-2). The nucleus is ventrally nent of the diplopia, magnifying the hypertropia
bordered by the medial longitudinal fasciculus. because superior rectus action is unopposed.
The proximity of these structures predisposes to This pattern of incomitant strabismus is summa-
a syndrome of trochlear palsy with an internu- rized as “hypertropia worse with gaze away and
clear ophthalmoplegia from lesions in the lower with tilt toward the affected side.”
midbrain tegmentum. Patients with CN-IV palsy often adopt a sec-
Axons emanating from the trochlear nucleus ondary torticollis, offering a diagnostic clue.
arc laterally and dorsally into the tectum of the “The tilting of the head lowers the image on the
midbrain, where they cross the midline. These side toward which the head is tilted ... so that the
decussated fibers emerge beneath the inferior twin images are brought to the same level, and
colliculus at the medial border of the brachium moreover it corrects the obliquity of the false im-
conjuctivum as CN-IV. The primary (central) neu- age by inclining with it.”* Because this posture
rons of the pupillary sympathetic chain pass minimizes the visual consequences of a CN-IV
through the midbrain in the central tegmental palsy, congenital CN-IV palsies are often undiag-
tract en route to the ciliospinal center of Budge- nosed for decades. Patients may have a diagno-
Waller. sis in adulthood after asthenopia or intermittent
CN-IV completely decussates and exits the diplopia develops or when they seek treatment
brainstem from its dorsal aspect, a unique site for torticollis. The characteristic head tilt in child-
among CNs. It enters the orbit via the superior hood photographs often confirms the congenital
orbital fissure and innervates the superior nature of the palsy.
oblique muscle, which depresses the eye in ad-
duction and intorts the eye. *Duke-Elder, pages 4047-4048.

NEURO-OPHTHALMOLOGY
85
CRANIAL NERVE IV: TROCHLEAR

Figure 7-1A
Oculomotor (III), Trochlear (IV), and Abducent (VI) Nerves: Schema
Long ciliary nerve Ciliary ganglion
Short ciliary nerves Abducent nucleus
Posterior ethmoidal nerve
Anterior ethmoidal nerve
Superior oblique muscle Sensory root of ciliary ganglion Trochlear nucleus
Levator palpebrae Sympathetic root of ciliary ganglion
superioris muscle Oculomotor nucleus
Superior division of
Superior oculomotor nerve Accessory oculomotor
rectus muscle (Edinger-Westphal)
Frontal nerve (cut) nucleus (parasympathetic)
Lacrimal nerve (cut)
Trochlear nerve (IV)
Nasociliary nerve
Oculomotor nerve (III)

Ophthalmic nerve (V1)

Infraorbital nerve Abducent


nerve (VI)
Zygomatic nerve (cut) Pterygopalatine
ganglion Mandibular nerve (V3)
Inferior oblique muscle
Internal carotid artery
Ciliary muscle Inferior division of
and nerve plexus
oculomotor nerve
Dilator muscle of pupil Maxillary nerve (V2)
Medial rectus muscle
Sphincter muscle of pupil
Lateral rectus muscle and
Inferior rectus muscle abducent nerve (turned back)
Efferent fibers
Afferent fibers Parasympathetic root Cavernous plexus
of ciliary ganglion
Sympathetic fibers Common tendinous ring
Parasympathetic fibers

Levator palpebrae Trochlear


Superior oblique m.
superioris m. (IV) nerve

Superior rectus m.
Oculomotor
(III) nerve Medial rectus m. Abducens
Lateral rectus m.
(VI) nerve
Inferior rectus m.
Inferior oblique m.

NEURO-OPHTHALMOLOGY
86
CRANIAL NERVE IV: TROCHLEAR

Figure 7-1B
Nerves of Orbit

Superior view Medial branch Supraorbital nerve


Lateral branch

Supratrochlear nerve Levator palpebrae superioris muscle

Medial rectus muscle Superior rectus muscle


Lacrimal gland
Superior oblique muscle
Lacrimal nerve
Infratrochlear nerve
Lateral rectus muscle
Nasociliary nerve
Frontal nerve
Trochlear nerve (IV)
Maxillary nerve (V2)
Common tendinous ring
Meningeal branch
of maxillary nerve
Ophthalmic nerve (V1)
Mandibular nerve (V3)
Optic nerve (II)
Lesser petrosal nerve
Internal carotid artery
and nerve plexus Meningeal branch
of mandibular nerve
Oculomotor nerve (III)
Greater petrosal nerve
Trochlear nerve (IV)
Trigeminal (semilunar)
Abducent nerve (VI) ganglion
Tentorial nerve (meningeal)
Tentorium cerebelli branch of ophthalmic nerve

Long ciliary nerves


Superior view:
levator palpebrae superioris, Short ciliary nerves
superior rectus, and superior
oblique muscles partially Lacrimal nerve
cut away Ciliary ganglion

Supratrochlear nerve (cut) Parasympathetic root of ciliary


ganglion (from inferior branch
Supraorbital nerve of oculomotor nerve)
branches (cut)
Sympathetic root of ciliary
Infratrochlear nerve ganglion (from internal
carotid plexus)
Anterior ethmoidal nerve
Sensory root of ciliary ganglion
Optic nerve (II) (from nasociliary nerve)
Posterior ethmoidal nerve Branches to inferior
and medial rectus muscles
Superior branch of
oculomotor nerve (III) (cut) Abducent nerve (VI)

Nasociliary nerve Inferior branch of


oculomotor nerve (III)
Internal carotid plexus
Lacrimal nerve
Trochlear nerve (IV) (cut) Frontal nerve (cut)
Oculomotor nerve (III)
Ophthalmic nerve (V1)
Abducent nerve (VI)

NEURO-OPHTHALMOLOGY
87
CRANIAL NERVE IV: TROCHLEAR

DIFFERENTIAL DIAGNOSIS the dura below the CN-III entrance, coming to


Midbrain Lesions the cavernous sinus via its lateral wall. At the su-
Traumatic unilateral and bilateral CN-IV perior orbital fissure, CN-IV courses superolater-
palsies are frequently encountered. A high ally to the oculomotor nerve, entering the orbit.
frontal impact creates contrecoup forces caus- Perhaps because of their relatively fixed loca-
ing a traction injury at the origin of the nerves or tion within the lateral wall of the cavernous sinus,
a contusion injury of the tectum against the cere- the trochlear and trigeminal nerves can be in-
bellar tentorium. MRI demonstration of tectal jured concomitantly. Patients with a posteriorly
subarachnoid hematoma in a traumatic draining carotid-cavernous fistula sometimes pre-
trochlear palsy supports this theory. Similar force sent with painful superior oblique dysfunction,
directions can be generated by deceleration in presumably due to local cavernous distention.
falls when the victim incurs occipital impact, or
with coccygeal impact transmitted up a straight DIAGNOSTIC APPROACH AND
spinal column. The amount of force needed to TREATMENT
produce traumatic CN-IV palsy seems variable. A diagnosis is made when the characteristic
Although an acquired trochlear palsy after mi- pattern of worsening hypertropia in downward
nor head trauma prompts suspicion of an undi- gaze, contralateral gaze, and ipsilateral head tilt
agnosed mass lesion, producing a “pathologic” is discovered. In patients with other CN coin-
palsy in an already damaged nerve, clinical ex- volvement, neuroimaging is required.
perience includes patients with acquired CN-IV Patients with isolated CN-IV palsies may be
palsies who had relatively minor trauma but no observed for spontaneous improvement over 3
preexisting pathology. CN-IV palsy has occurred to 4 months, especially if the history indicates a
in benign intracranial hypertension and after likely etiology (trauma or known diabetes). Non-
lumbar puncture—presumably due to tractional resolution over time usually prompts imaging un-
mechanisms—both with CN-VI coinvolvement, less congenital CN-IV palsy is strongly suggested
and in spontaneous intracranial hypotension by history and examination findings (eg, vertical
with CN-III coinvolvement. fusional amplitude greater than 4 diopters).
A lesion interrupting both the predecussation Therapy may include the use of prismatic
trochlear fasciculus and the ipsilateral sympa- glasses to shift “second images” to line up with
thetics within the tectum produces an ipsilateral primary images, eliminating diplopia. However,
Horner syndrome with crossed CN-IV palsy. The the utility of prismatic glasses in CN-IV palsy is
confluence of the 2 trochlear fasciculi at their de- limited because only 1 prismatic strength can be
cussation provides a locus wherein a single le- ground into the spectacles, but different
sion may produce bilateral trochlear palsies with strengths are needed for different gazes. Many
the seeming paradox of no head tilt. These pa- patients prefer strabismic surgical treatment of
tients demonstrate a left hypertropia on right nonresolving palsies; it offers greater range of
gaze and left head tilt, and right hypertropia on gaze without diplopia.
left gaze and right head tilt. They may adopt a
chin-down head tilt. Various pathologies at the REFERENCES
inferior dorsal midbrain cause bilateral CN-IV Duke-Elder WS. Neurology of Vision: Motor and Optical
palsies. Anomalies. St Louis, Mo: CV Mosby; 1949. Duke-Elder
WS. Text-book of Ophthalmology; vol 4.
Trochlear Nerve Lesions Jacobson DM, Warner JJ, Choucair AK, Ptacek LJ. Trochlear
nerve palsy following minor head trauma: a sign of struc-
After emerging below the corpora quadrigem-
tural disorder. J Clin Neuroophthalmol. 1988;8:263-268.
ina, CN-IV sweeps around the brainstem toward Miller NR, Newman NJ, eds. Walsh and Hoyt’s Clinical Neuro-
its ventral aspect, situated lateral and inferior to ophthalmology. 5th ed. Baltimore, Md: Williams & Wilkins;
CN-III. After a distance of 75 mm, CN-IV enters 1998.

NEURO-OPHTHALMOLOGY
88
Chapter 8

Cranial Nerve VI: Abducens


Ippolit C. A. Matjucha

Clinical Vignette
A 78-year-old Asian-born woman was admitted to the hospital for worsening mentation and gait problems. Neuro-
imaging and initial eye examination results were negative. The patient became only partially responsive to verbal
commands. A treating physician then noted that one eye no longer abducted normally; hours later, the patient
could not abduct either eye, and fundus examination showed bilateral papilledema.
Lumbar puncture revealed markedly high intracranial pressure and chemical and cellular abnormalities of the
CSF consistent with tubercular meningitis.

The sixth cranial nerve (CN-VI), similar to CN- eral CN-III nuclear complex. These internuclear
IV, innervates a single extraocular muscle, the lat- neurons control the near-simultaneous stimula-
eral rectus, which is the primary abductor for the tion of the contralateral medial rectus during ip-
eyes. Patients with CN-VI paresis have an inward silateral abducens nerve stimulation to produce
deviation of the affected eye, a noncomitant es- lateral horizontal gaze.
otropia. Temporal eye movement beyond mid-
line is lost or reduced. ABDUCENS FASCICULUS
Patients with abducens palsies, especially par- From its position laterally abutting the para-
tial and mild palsies, sometimes adopt a head median pontine reticular formation, the CN-VI
turn toward the affected side to minimize nucleus first sends its motor afferents medially
diplopia by keeping the eye adducted. In more toward the MLF and then ventrally, passing
severe palsies, this strategy often fails or is un- through the paramedian pontine reticular forma-
comfortable given the large turn required, so pa- tion and the undecussated corticospinal tract to
tients present with one eye shut, patched, or reach the ventral surface of the brainstem at the
covered. inferior lip of the pons.
The vignette at the beginning of this chapter of On exiting the ventral pons, the abducens
bilateral abducens nerve palsies is typical of lep- nerve ascends between the pons and the clivus
tomeningeal pathology, likely due to increased within the subarachanoid pontine cistern. After it
intracranial pressure. However, treatable enters the dura, CN-VI continues up the clivus to
chronic granulomatous inflammatory processes the posterior clinoid. It travels over the petrous
sometimes deserve consideration also. ridge to lie beneath the inferior petrosal sinus
and then enters the cavernous sinus via the
ABDUCENS NUCLEUS Dorello canal, just medial to the Meckel cave,
The CN-VI nucleus, located just beneath the which houses the gasserian ganglion.
facial colliculi in the inferior pons is enveloped After CN-VI is within the cavernous sinus, it
by the turning CN-VII fascicular fibers of the fa- passes forward, adjacent to the internal lateral
cial genu and contains two physiologically—but aspect of the carotid artery. Here, it likely, albeit
not topographically—distinct groups of neurons briefly, carries the majority of the sympathetic ax-
(Figure 8-1). One group innervates the ipsilateral ons to innervate the pupil. More anteriorly, the
lateral rectus; the other sends axons across the abducens passes these neurons onto the oph-
midline to the contralateral medial longitudinal thalmic nerve to follow its nasociliary branch to
fasciculus (MLF). These latter axons ascend in the ciliary ganglion; the sympathetic fibers pass
the MLF to the ventral nucleus of the contralat- through the ganglion without synapse, entering

NEURO-OPHTHALMOLOGY
89
CRANIAL NERVE VI: ABDUCENS

Figure 8-1A
Nerves of Orbit
Superior view Medial branch Supraorbital nerve
Lateral branch
Supratrochlear nerve Levator palpebrae superioris muscle

Medial rectus muscle Superior rectus muscle

Superior oblique muscle Lacrimal gland


Lacrimal nerve
Infratrochlear nerve
Lateral rectus muscle
Nasociliary nerve
Frontal nerve
Trochlear nerve (IV)
Maxillary nerve (V2)
Common tendinous ring Meningeal branch
Ophthalmic nerve (V1) of maxillary nerve
Mandibular nerve (V3)
Optic nerve (II)
Lesser petrosal nerve
Internal carotid artery
and nerve plexus Meningeal branch
of mandibular nerve
Oculomotor nerve (III)
Greater petrosal nerve
Trochlear nerve (IV) Trigeminal (semilunar)
Abducent nerve (VI) ganglion
Tentorial nerve (meningeal)
Tentorium cerebelli
branch of ophthalmic nerve
Long ciliary nerves
Superior view: Short ciliary nerves
levator palpebrae superioris, superior
rectus, and superior oblique muscles Lacrimal nerve
partially cut away Ciliary ganglion
Supratrochlear nerve (cut) Parasympathetic root of ciliary
ganglion (from inferior branch
Supraorbital nerve of oculomotor nerve)
branches(cut)
Sympathetic root of ciliary ganglion
Infratrochlear nerve
(from internal carotid plexus)
Anterior ethmoidal nerve
Sensory root of ciliary ganglion
Optic nerve (II) (from nasociliary nerve)
Posterior ethmoidal nerve Branches to inferior
Superior branch of and medial rectus muscles
oculomotor nerve (III) (cut) Abducent nerve (VI)
Nasociliary nerve Inferior branch of
Internal carotid plexus oculomotor nerve (III)
Lacrimal nerve
Trochlear nerve (IV) (cut)
Frontal nerve (cut)
Oculomotor nerve (III)
Ophthalmic nerve (V1)
Abducent nerve (VI)

Coronal section
through cavernous sinus

Cavernous sinus Optic chiasm

Oculomotor nerve (III) Posterior


communicating artery
Trochlear nerve (IV)
Internal carotid
Abducent nerve (VI) artery

Ophthalmic nerve (V1) Hypophysis


(pituitary gland)
Maxillary nerve (V2)
Sphenoidal sinus

Nasopharynx

90
CRANIAL NERVE VI: ABDUCENS

Figure 8-1B Central Control of Eye Movements


Occipital
eye fields
(Brodmann
areas 17, 18, 19)
Excitatory endings Frontal eye fields
Inhibitory endings (Brodmann area 8)

Interstitial
nucleus of
Cajal

Superior Superior
colliculus oblique m.
Superior
Oculomotor rectus m.
nucleus
Medial longitudinal
fasciculus

Abducens
Oculomotor (III) n.
internuclear
neuron Trochlear (IV) n.
Medial rectus m. Lateral
rectus m.
Trochlear Corticoreticular fibers
nucleus

Medial longitudinal
fasciculi

Medial
longitudinal Ascending tract
fasciculi of Deiters
Abducens
nucleus Superior

Medial Vestibular Inferior Inferior


Lateral nuclei oblique m. rectus m.
Inferior

Vestibular n.

Abducens (VI) n.
Parapontine reticular formation (lateral gaze center)

NEURO-OPHTHALMOLOGY
91
CRANIAL NERVE VI: ABDUCENS

the eye via the short ciliary nerves. Additional the facial fasciculus, adding ipsilateral facial palsy
sympathetic fibers bypass the ciliary ganglion, to the presentation (Millard-Gubler syndrome).
entering the eye as the long ciliary nerves. Anterior inferior cerebellar artery occlusion
typically produces more lateral damage charac-
CLINICAL PRESENTATION teristically to the vestibular nuclei, the auditory
Primary nuclear CN-VI lesions typically have nerve, CN-VII, the paramedian pontine reticular
concomitant ipsilateral facial nerve involvement. formation, the spinothalamic tract, and the mid-
Interestingly, clinical lesions of the CN-VI nu- dle cerebellar peduncle and possibly extending
cleus do not result in isolated ipsilateral lateral dorsally to the cerebellar hemisphere and ros-
rectus dysfunction. Instead, because there are 2 trally to the CN-V nucleus. The combined deficits
functionally separate types of neurons within the produce a lateral inferior pontine syndrome of
abducens nucleus, an ipsilateral gaze palsy de- nystagmus (with beats or fast phase directed ip-
velops, with inability to move both eyes to the af- silaterally), vertigo, gaze palsy, facial paralysis
fected side. This gaze palsy occurs because le- and hypesthesia, deafness, and ataxia, all with
sions involving the CN-VI nucleus affect neurons crossed analgesia.
headed for the lateral rectus and the contralat- Foville syndrome develops within this same
eral third nerve nucleus via the MLF. territory. It is characterized by gaze or abducens
Larger lesions affecting the CN-VI nucleus and palsy (CN-VI nucleus or fasciculus) with ipsilat-
the ipsilateral MLF interrupt the crossed internu- eral dysgeusia (nucleus of the tractus solitarius),
clear neurons from the opposite CN-VI nucleus, facial analgesia (spinal tract of CN-V), Horner
with consequent inability to adduct the ipsilat- syndrome (central tegmental tract), and deaf-
eral eye in horizontal gaze. This combined lesion ness (auditory nerve).
produces the Fisher “one-and-a-half” syndrome: CN-VI, the carotid artery, and sympathetic
ipsilateral gaze palsy and internuclear ophthal- pupil fibers are situated closely within the cav-
moplegia. As with other internuclear ophthalmo- ernous sinus, accounting for another syndrome.
plegia variants, it can be distinguished from syn- The expanding artery of intracavernous carotid
dromes produced by true adduction palsy dissection or aneurysm compresses the ab-
because patients maintain the ability to adduct ducens nerve and the sympathetic fibers within
both eyes for near stimulus (convergence). Con- the cavernous sinus, producing painful ab-
vergence is spared in internuclear ophthalmo- ducens palsy with ipsilateral Horner syndrome.
plegia because neither the upper midbrain path- Other pathologic processes within this region
ways producing convergence nor CN-III is sometimes produce a similar clinical picture.
affected.
Other CN-VI Lesions
DIFFERENTIAL DIAGNOSIS Processes that affect the anterior midline brain
Arterial Vascular Syndromes stem also deserve consideration in the differen-
Paramedian basilar artery branch occlusion tial diagnosis, including various posterior fossa
causes infarction of the medial and ventral struc- tumors or inflammatory processes that affect the
tures of the inferior pons, producing ipsilateral abducens nerve during its ascent of the clivus.
gaze palsy (paramedian pontine reticular forma- Tumors such as chordoma that favor the midline
tion involvement), hemifacial paralysis (CN-VII), skull base and grow slowly occasionally present
limb ataxia and nystagmus (involvement of mid- as isolated or bilateral CN-VI palsy. Other tumors
dle cerebellar peduncle and possibly vestibular requiring consideration include meningiomas,
nuclei efferents), crossed paralysis (corticospinal chondrosarcomas, eosinophilic granulomas, na-
tract), and crossed tactile hypesthesia (medial sopharyngeal carcinoma, and metastases. Al-
lemniscus). More focal lesions may produce though all these tumors may produce isolated
Raymond syndrome (abduction palsy and CN-VI palsies, most often, they produce addi-
crossed hemiplegia) from abducens fascicular in- tional neurologic deficits.
jury at the corticospinal tract, whereas similar le- Gradenigo syndrome is characterized by a
sions with some lateral extension also involve painful abducens palsy resulting from mastoiditis

NEURO-OPHTHALMOLOGY
92
CRANIAL NERVE VI: ABDUCENS

and petrositis complicating chronic otitis media. brain begins to cause downward brainstem her-
The infectious process erodes the bone, affect- niation, CN-VI is stretched and loses function,
ing the abducens nerve and the gasserian gan- perhaps from compressive ischemia. Similarly,
glion; CN-VII is sometimes also involved as it downward shift of the pons in relation to the
passes through the mastoid bone en route to the petrous ridge is thought to account for CN-VI
stylomastoid foramen. A combined trigeminal- palsies sometimes seen in intracranial hypoten-
abducens-facial nerve syndrome can be pro- sion, whether of spontaneous or post–lumbar
duced by other entities, particularly tumors, that puncture origin.
affect this region.
Unilateral or bilateral CN-VI palsy is also REFERENCES
commonly recognized as a false localizing sign Lyle DJ. Neuro-ophthalmology. Springfield, Ill.: Charles C
in patients with increased intracranial pres- Thomas; 1945.
sure, whether idiopathic or from tumor. A tumor Rizzo JF, Lessell S, eds. Neuro-ophthalmology. In: Albert DM,
distant from CN-VI can produce abducens Jakobiec FA. Principles and Practice of Ophthalmology.
Vol 4. Section XI. Philadelphia, Pa: WB Saunders;
palsies if intracranial pressure is increased suffi- 1994:2387-2712.
ciently. The sharp turn of CN-VI across the Victor M, Ropper AH. Adam‘s and Victor‘s Principles of Neu-
petrous ridge may make it vulnerable. As the rology. 7th ed. New York, NY: McGraw-Hill; 2001.

NEURO-OPHTHALMOLOGY
93
Chapter 9

Cranial Nerve I: Olfactory


Michal Vytopil and H. Royden Jones, Jr

Clinical Vignette
A 68-year-old widower reported to his girlfriend that he had been having difficulty smelling food for a few
months. He was uncertain about the nature of the onset of this difficulty. His family had noted personality changes,
especially disinhibition, although he denied it. He had no headaches, changes in vision, or seizures. He was no
longer speaking with 2 of his 3 children.
Neurologic examination results demonstrated anosmia. His behavior was inappropriate, with almost complete
fixation on sexual desires, stating that he wanted someone to help him so he could participate in such during most
of his waking hours. However, his Mini-Mental Status Examination results were normal, he had no papilledema or
optic atrophy, and his gait was normal, as was the remainder of the results of a complete neurologic examination.
Brain MRI demonstrated a large olfactory groove meningioma (Figure 9-1). The patient went to a neurosurgeon,
who elected to observe the tumor; it did not change in size over a 2-year period.
The patient never regained his sense of smell, his behavior became increasingly inappropriate, and he declined
neuropsychologic evaluation to determine whether he had cognitive impairment. His family began calling into
question seemingly inappropriate financial decisions.

Although the benign neoplasm in this vignette When identifying odors, humans rely on
had likely been present for years, it was not discov- volatile substances entering their nasal cavity to
ered until the patient became aware of his inability excite receptors. Olfactory receptor cells are
to smell. Family strife resulted from behavioral bipolar sensory neurons whose dendrites form
changes that were possibly related to the sub- a delicate sensory carpet on the superior
frontal tumor. Nevertheless, this previously astute aspect of the nasal cavity (Figure 9-2). The thin,
individual functioned independently and ada- unmyelinated axons of the bipolar sensory cells
mantly declined further investigation except semi- collectively form CN-I and travel through the
annual neurologic visits to monitor tumor size. cribriform plate into the olfactory bulb at the
The olfactory nerve (CN-I) provides the sense base of the fronto-orbital lobe. Within the bulb,
of smell. It is an important warning system be- CN-I fibers synapse with the dendrites of the
cause it enables identification of spoiled foods or large mitral cells, whose axons constitute the
noxious chemicals. Moreover, it significantly olfactory tract passing along the base of the
contributes to quality of life because it is largely frontal lobe and projecting directly into the pri-
responsible for many desirable sensations, such mary olfactory cortex in the temporal lobe with-
as food and beverage flavors. Unfortunately, out connecting to a thalamic nucleus (Figure
many neurologists think of olfactory function 9-3). Although this may seem like an aberration
testing (chapter 1) as somewhat redundant, and to sensory connections, the direct relay to the
consequently, it is seldom performed ade- limbic structures of the brain may have served
quately. Neglecting to perform smell evaluation an evolutionary function in lower animals and,
may compromise rare patients. Occasional seri- later, primates. In humans, the primary olfac-
ous but treatable conditions, most notably olfac- tory cortex includes the uncus, hippocampal
tory groove meningiomas, may present with CN- gyrus, amygdaloid complex, and entorhinal cor-
I compromise as their first symptom. tex.

CRANIAL NERVES
96
CRANIAL NERVE I: OLFACTORY

Figure 9-1 Subfrontal Meningioma

T1-weighted, gadolinium-enhanced sagittal and coronal MR images show a large enhancing mass on the skull
base displacing and compressing the olfactory apparatus.

DIFFERENTIAL DIAGNOSIS predilection for males, even in sporadic and au-


The sense of smell can be disrupted at any site tosomal forms. In addition to the isolated pri-
along the olfactory pathway. Therefore, an olfac- mary olfactory form, Kallmann syndrome may
tory impairment is not necessarily equivalent to be associated with other congenital deficits,
a CN-I lesion. Several relatively common condi- such as cleft palate, lip/dental agenesis, and
tions that spare CN-I create various olfactory dis- neural hearing loss.
turbances, including upper respiratory infec-
tions, nasal sinus disease, and, less frequently, Acquired Disorders
damage to olfactory bulb, tract, or cortex. Together, upper respiratory infection and
Disturbances of smell are congenital or ac- nasal and paranasal sinus disease account for
quired, with the latter being more common. Al- more than 40% of olfactory disturbances and
though most olfactory dysfunctions occur bilat- are the most frequent causes. These intranasal
erally, unilateral anosmia is an important sign processes mechanically prevent volatile chemi-
that should prompt MRI to exclude an olfactory cal stimuli from reaching the olfactory sensory
groove tumor. epithelium and activating the receptors. Be-
cause they do not lead to direct damage to the
Congenital Disorder CN-I pathways, they are called transport or con-
In Kallmann syndrome, anosmia results from ductive olfactory disorders. The high frequency
congenital hypoplasia of olfactory lobes and oc- of conductive disorders underscores the neces-
curs in conjunction with hypogonadotropic hy- sity for thorough otorhinolaryngologic evalua-
pogonadism. Most cases are sporadic, but famil- tions in patients with an olfactory dysfunction.
ial cases have been reported suggesting various Head trauma is responsible for approximately
inheritances: X linked, autosomal dominant, or 20% of all cases of smell disturbances via direct
autosomal recessive. Some of the responsible damage to CN-I or the frontobasal cerebral cor-
genes have been identified. There is a strong tex associated with olfaction. The incidence of

CRANIAL NERVES
97
CRANIAL NERVE I: OLFACTORY

Figure 9-2 Olfactory Receptors


Cribriform plate of
Olfactory bulb ethmoid bone

A. Distribution of
olfactory epithelium
(blue area)

Lateral nasal wall Septum


B. Schema of
section through
olfactory mucosa

Cribriform plate

Schwann cell

Olfactory gland

Unmyelinated
olfactory axons

Basement
membrane

Sustentacular cells

Endoplasmic
reticulum

Nucleus

Olfactory cells

Dendrites

Terminal bars
(desmosomes)
Olfactory rod
(vesicle)
Villi

Cilia

Mucus

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98
CRANIAL NERVE I: OLFACTORY

Figure 9-3 Olfactory Pathways


Subcallosal (parolfactory) area
Efferent fibers Septal area and nuclei
Afferent fibers Fibers from contralateral olfactory bulb
Granule cell (excited by Fibers to
and inhibiting to mitral contralateral Anterior
and tufted cells) olfactory bulb commissure
Mitral cell Medial olfactory
stria
Recurrent process
Olfactory trigone
Tufted cell and olfactory
Periglom- tubercle
erular cell Anterior
Glomerulus perforated
substance
Olfactory
nerve fibers Habenula
Lateral olfactory
stria
Lateral olfactory
tract nucleus
Piriform lobe
Hippocampal
fimbria
Dentate gyrus
Uncus
Parahippocampal
Olfactory epithelium Olfactory tract gyrus
Olfactory nerves Anterior olfactory nucleus Amygdala
(in phantom)
Olfactory bulb Cribriform plate of ethmoid bone
Entorhinal area

anosmia after head trauma is approximately 7% sensory nerve. Recovery eventually occurs in
to 30%, depending on injury severity. Olfactory some but not all individuals.
dysfunction after head trauma can present as Olfactory groove meningiomas are rare but
unilateral or bilateral anosmia or hyposmia. It is may result in significant morbidity unless diag-
hypothesized that most olfactory dysfunctions in nosed and treated early. Usually slow growing,
blunt head trauma result from shearing of CN-I they represent 8% to 18% of all intracranial
as it passes through the cribriform plate. Hence, meningiomas. Although unilateral or bilateral ol-
occipital and side blows are more dangerous to factory dysfunction is thought to be their first
olfaction than are frontal ones. More substantial symptom, few patients present with disturbances
damage, such as contusion of the olfactory bulb in their sense of smell, probably because of a
or cortical and subcortical olfactory areas, may slow and orderly growth with resultant gradual
occur in severe head traumas with anterior fossa decline in olfactory function. Moreover, because
fracture. most meningiomas are unilateral and cause ol-
The average life span of olfactory receptor factory loss confined to the ipsilateral side, pa-
neurons is 30 days; they are continuously re- tients retain olfactory function on the contralat-
placed by fresh cells growing into the olfactory eral side and are usually unaware of any loss.

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99
CRANIAL NERVE I: OLFACTORY

Consequently, most cases are not diagnosed un- cluding opiates (codeine, morphine), antiepilep-
til the tumor is large enough to cause other symp- tic drugs (carbamazepine, phenytoin), and im-
toms resulting from pressure on the frontal lobes munosuppressive agents, that, similar to radia-
and optic tracts, such as headache, visual distur- tion, disrupt the physiologic turnover of receptor
bances, personality changes, and memory im- cells.
pairment. Therefore, early diagnosis of olfactory Abuse of cocaine via intranasal snorting may
meningiomas remains challenging, and most tu- lead to septal perforation and direct trauma to
mors, as in the vignette, become relatively large CN-I.
(eg, ⬎4 cm in diameter) at diagnosis.
An olfactory groove tumor, most often a menin- REFERENCES
gioma, may rarely result in the Foster-Kennedy De Kruijk JR, Leffers P, Menheere PP, et al. Olfactory function
syndrome, characterized by unilateral optic atro- after mild traumatic brain injury. Brain Inj. 2003;17:73-78.
phy and contralateral papilledema. Optic atrophy Dode C, Levilliers J, Dupont JM, et al. Loss-of-function muta-
tions in FGFR1 cause autosomal dominant Kallmann syn-
results from direct pressure of the neoplasm on drome. Nat Genet. 2003;33:463-465.
the optic nerve, whereas increased intracranial Doty R. Olfaction. Annu Rev Psychol. 2001;52:423-452.
pressure produces contralateral papilledema. Doty RL, Yousem DM, Pham LT, et al. Olfactory dysfunction
in patients with head trauma. Arch Neurol. 1997;54:1131-
Other Entities 1140.
Finelli PF, Mair R. Disturbances of taste and smell. In: Bradley
Normal aging is associated with a progressive WG, Daroff RB, Fenichel GM, Marsden CD, eds. Neurol-
decline in the ability to appreciate and discrimi- ogy in Clinical Practice: Principles of Diagnosis and Man-
nate odors. agement. 2nd ed. Boston, Mass: Butterworth-Heinemann;
Hallucinations of smell, although not disor- 1996:243-250.
ders of CN-I, are important in differential diagno- Green P, Rohling ML, Iverson GL, et al. Relationships be-
tween olfactory discrimination and head injury severity.
sis of an olfactory dysfunction. They can present Brain Inj. 2003;17:479-496.
in psychiatric conditions (depression, psy- Rubin G, Ben David U, Gornish M, et al. Meningiomas of the
chosis), as part of alcohol withdrawal syndrome, anterior cranial fossa floor: review of 67 cases. Acta Neu-
or in complex partial epilepsy (chapter 25). In rochir (Wien). 1994;129:26-30.
the latter, hallucinations are called uncinate Sweeney PJ, Hanson MR. The cranial neuropathies. In:
Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds.
crises, are almost invariably of foul character, Neurology in Clinical Practice: Principles of Diagnosis and
and often accompany symptoms typical for focal Management. 2nd ed. Boston, Mass: Butterworth-Heine-
temporal lobe epilepsy, including staring and mann; 1996:1721-1732.
various automatisms. Turetsky BI, Moberg PJ, Yousem DM, et al. Reduced olfac-
Some neurodegenerative disorders are asso- tory bulb volume in patients with schizophrenia. Am J Psy-
chiatry. 2000;157:828-830.
ciated with an impaired sense of smell: Parkin- Welge-Luessen A, Temmel A, Quint C, et al. Olfactory func-
son disease, Alzheimer disease, Huntington dis- tion in patients with olfactory groove meningioma. J Neu-
ease, and, inexplicably, motor neuron disease. rol Neurosurg Psychiatry. 2001;70:218-221.
Olfactory discrimination can also be adversely
affected by many medications and drugs, in-

CRANIAL NERVES
100
Chapter 10

Cranial Nerve V: Trigeminal


Michal Vytopil and Edward Tarlov

ANATOMY
The trigeminal cranial nerve (CN-V) has 3 major divisions: ophthalmic, maxillary, and mandibular (Fig-
ures 10-1 and 10-2). CN-V is the major sensory nerve of the face, mouth, and nasal cavity. It also sup-
plies motor and proprioceptive fibers to the muscles involved in chewing. It has a complex course orig-
inating within the pons from 2 nuclei, a large sensory and smaller motor give rise to sensory and motor
roots that emerge from the midlateral pons. Both roots continue toward the trigeminal ganglion on the
bottom of the middle cranial fossa, where the general sensory fibers have their cell bodies. Distal to the
ganglion, the 3 sensory divisions exit the cranium through the superior orbital fissure, foramen rotun-
dum, and foramen ovale. The motor component passes through the trigeminal ganglion and accom-
panies the mandibular division.

CN-V Nuclei on the floor of the middle cranial fossa. Central


The motor nucleus is located in the mid pons, processes of neuronal cell bodies constitute the
medial to the large sensory nucleus. The sensory large sensory root that enters the pons and pro-
nucleus is the largest, begins rostrally within the jects into the pontine trigeminal and spinal tract
midbrain, and extends caudally through the nuclei. Peripheral processes divide into the 3
pons and medulla into the second segment of sensory divisions that exit the skull.
the cervical spinal cord (Figure 10-3). It is subdi-
vided into 3 portions. The mesencephalic por- Sensory Divisions
tion contains cell bodies of sensory fibers carry-
The ophthalmic division collects touch, pain,
ing proprioceptive information from the
temperature, and proprioceptive information
masticatory muscles. The pontine trigeminal por-
tion, also called the principal sensory nucleus, is from the upper third of the face, adjacent si-
thought to receive only tactile stimuli and, there- nuses, and scalp regions. These nerve branches
fore, to subserve exclusively light touch. The course posteriorly in the orbit toward the supe-
spinal tract nucleus is considered primarily to re- rior orbital fissure, where they enter the skull.
lay pain and temperature. The maxillary division carries sensory infor-
mation from the skin overlying the maxilla, side
Principal Sensory Component of the forehead, medial cheek, and side of the
The sensory component of CN-V conveys nose, upper lip, palate, upper teeth, nasophar-
general sensation from the facial skin and scalp ynx, and meninges of the anterior and middle
to the top of the head, tragus of the ear, and an- cranial fossae.
terior wall of the external auditory meatus. Also, The mandibular division primarily provides
it provides general sensation from the mouth, in-
sensory innervation for the skin overlying the
cluding the tongue and teeth, nasal and
lower jaw (with the exception of the angle of the
paranasal sinuses, and meninges of the anterior
jaw innervated by the second and third cervical
and middle cranial fossae.
nerves), cheeks, chin and lower lip, mucous
Trigeminal Ganglion membrane of the mouth, gums, inferior teeth,
The cell bodies of almost all sensory CN-V anterior two thirds of the tongue, side of the
fibers, with the exception of proprioceptive head, anterior wall of the external auditory mea-
fibers, are located within the trigeminal ganglion. tus, external wall of the tympanic membrane,
The ganglion sits in a depression, a Meckel cave, and temporomandibular joint.

CRANIAL NERVES
101
CRANIAL NERVE V: TRIGEMINAL

Figure 10-1 Trigeminal Nerve (V): Schema


Trigeminal nerve (V) ganglion and nuclei
Efferent fibers Ophthalmic nerve (V1)
Afferent fibers Motor nucleus
Tentorial (meningeal) branch Mesencephalic nucleus
Proprioceptive fibers
Parasympathetic fibers Nasociliary nerve
Principal sensory nucleus
Sympathetic fibers Lacrimal nerve
Sensory root of Spinal tract and nucleus
ciliary ganglion
Frontal nerve
Ciliary ganglion
Posterior ethmoidal nerve
Long ciliary nerve
Short ciliary nerves
Anterior ethmoidal nerve
Supraorbital nerve
Supratrochlear nerve
Infratrochlear nerve
Internal nasal branches
and
External nasal branches
of anterior ethmoidal nerve
Maxillary nerve (V2)
Meningeal branch
Zygomaticotemporal
nerve
Zygomaticofacial nerve
Zygomatic nerve
Infraorbital nerve
Pterygopalatine
ganglion
Superior
alveolar
branches of
infraorbital nerve Facial nerve (VII)
Nasal branches
Chorda
(posterior superior
lateral, nasopalatine tympani nerve
and posterior
superior medial)
Nerve (Vidian) of
pterygoid canal
(from facial nerve [VII]
and carotid plexus)
Pharyngeal branch Superficial
Greater and lesser temporal branches
palatine nerves Articular branch
Deep temporal nerves and anterior
(to temporalis muscle) auricular nerves
Lateral pterygoid Auriculotemporal nerve
and masseteric nerves Submandibular Inferior
ganglion Parotid branches
Tensor veli palatini and alveolar nerve
medial pterygoid nerves Mylohyoid nerve Otic ganglion Meningeal branch
Buccal nerve Mandibular nerve (V3) Tensor tympani Lesser petrosal nerve (from
Mental nerve nerve glossopharyngeal nerve [IX])
Inferior dental plexus
Lingual nerve

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102
CRANIAL NERVE V: TRIGEMINAL

Figure 10-2A Ophthalmic (V1) and Maxillary (V2) Nerves Sensory

Communicating branch Anterior ethmoidal nerve

Posterior ethmoidal nerve Supraorbital nerve


Supratrochlear nerve
Long and short ciliary nerves
Lacrimal gland
Ciliary ganglion
Infratrochlear nerve
Lacrimal nerve (from nasociliary nerve)
Cutaneous branch
Nasociliary nerve of lacrimal nerve

Frontal nerve Zygomaticotemporal


nerve
Tentorial nerve Zygomaticofacial
nerve
Ophthalmic nerve (V1)
External
nasal
Trigeminal
branch of
(semilunar) ganglion
anterior
ethmoidal
Trigeminal nerve
nerve (V)

Meningeal
branch
Foramen
rotundum Infraorbital
nerve
Foramen
ovale Anterior superior
alveolar nerve
Mandibular nerve (V3)
Nasal
branch
Maxillary nerve (V2)
Mucous membrane
Zygomatic nerve of maxillary sinus

Nerve (vidian) of pterygoid canal Dental and gingival

Pterygopalatine ganglion Superior dental plexus

Greater and lesser palatine nerves Infraorbital nerve


entering infraorbital canal
Ganglionic branches to pterygopalatine ganglion Middle superior alveolar nerve

Posterior superior alveolar nerve

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103
CRANIAL NERVE V: TRIGEMINAL

Figure 10-2B Mandibular Nerve (V3) Sensory and Motor


Anterior division Temporal fascia
and temporalis muscle Posterior
Lateral view Posterior division Deep temporal nerves
Anterior
Foramen ovale
Masseteric nerve
Meningeal branch
Lateral pterygoid
Foramen spinosum nerve and muscle
Middle
meningeal artery
Auriculotemporal
nerve
Posterior
auricular nerve
Facial nerve (VII)
Chorda tympani Buccal nerve
nerve and buccinator
Lingual nerve muscle (cut)

Inferior alveolar nerve (cut) Submandibular


ganglion
Nerve to mylohyoid
Sublingual
Medial pterygoid muscle (cut) gland
Digastric muscle (posterior belly) Mylohyoid
muscle (cut)
Stylohyoid muscle
Hypoglossal nerve Mental nerve

Submandibular gland
Inferior alveolar nerve (cut)
Sublingual nerve Digastric muscle (anterior belly)
Medial view
Motor root
Trigeminal (semilunar) Sensory root
ganglion Geniculum
Ophthalmic nerve (V1) Tympanic cavity
Maxillary nerve (V2)
Chorda tympani nerve
Mandibular nerve (V3)
Facial nerve (VII)
Anterior division
Tensor tympani
Tensor veli palatini muscle and nerve
nerve and muscle
Lesser petrosal nerve
Otic ganglion
Chorda tympani nerve Auriculotemporal nerve

Medial pterygoid Maxillary artery


nerve and muscle (cut)
Mylohyoid nerve
Pterygoid hamulus
Inferior alveolar nerve entering
Lingual nerve mandibular foramen

CRANIAL NERVES
104
CRANIAL NERVE V: TRIGEMINAL

Figure 10-3 Cranial Nerve Nuclei in Brainstem: Schema


Oculomotor nerve (III)
Posterior phantom view
Red nucleus
Superior colliculus
Oculomotor nucleus
Accessory oculomotor
(Edinger-Westphal) nucleus
Lateral geniculate body

Mesencephalic nucleus Trochlear nucleus


of trigeminal nerve
Trochlear nerve (IV)
Principal
sensory nucleus of
trigeminal nerve Motor nucleus of
trigeminal nerve

Trigeminal nerve Trigeminal


(V) and ganglion nerve (V)
and ganglion
Facial nerve (VII)
and geniculate
ganglion Abducent nucleus

Vestibulocochlear Facial nucleus


nerve (VIII)
(Geniculum)
of facial
nerve

Superior
Anterior and inferior
Cochlear salivatory
nuclei Posterior nuclei

Vestibular
nuclei Glosso-
pharyngeal
Glossopharyngeal nerve (IX)
nerve (IX)
Vagus nerve (X)
Vagus nerve (X) Accessory nerve (XI)*

Spinal tract and spinal Nucleus ambiguus


nucleus of trigeminal nerve
Posterior (dorsal) nucleus
Solitary tract nucleus of vagus nerve (X)

Dorsal vagal nucleus Hypoglossal nucleus

Spinal nucleus
Efferent fibers of accessory nerve
Afferent fibers
Mixed fibers

*Recent evidence suggests that the accessory nerve lacks a cranial root and has no connection to the vagus nerve.
Verification of this finding awaits further investigation.

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CRANIAL NERVE V: TRIGEMINAL

Motor Component Typically, the pain is intermittent and rarely oc-


The motor nucleus, originating within the mid curs at night. It may be provoked by simple daily
pons, receives its major input from primary pro- activities, including talking, chewing, shaving, or
prioceptive neurons in the mesencephalic sub- drinking hot or cold liquids, or any facial touch or
nucleus, creating a monosynaptic reflex arch sensory stimulation.
similar to spinal reflexes, assessable by eliciting In many patients, perhaps the majority, myelin
the jaw jerk. Motor nucleus axons exit the pons loss within the posterior root of the CN-V leads
as the motor root medial to the sensory root, to this pain. In younger individuals, a primary
passing through the trigeminal ganglion and ex- CNS demyelinating disorder, particularly multi-
iting the skull via the foramen ovale. Extracra- ple sclerosis, is occasionally operative. Another
nially, this root combines with the sensory common cause is an artery that has lengthened
mandibular division forming the mandibular during adult life and become tortuous. An
nerve, which provides branches innervating the ectatic loop of such an artery, usually a branch of
muscles of mastication: the masseter, tempo- the superior cerebellar artery, compresses the
ralis, medial and lateral pterygoid, mylohyoid, trigeminal posterior root, causing the pain.
and anterior digastric muscles. Trigeminal neuralgia can result from other condi-
tions causing pressure on CN-V, including
TRIGEMINAL NEURALGIA acoustic neuromas, meningiomas, AVM, or
aneurysms compressing the posterior root of the
Clinical Vignette CN-V.
MRI is generally appropriate, particularly in
A 48-year-old woman experienced lightninglike at-
tacks of electrical shock–like, intense pain in the left younger persons who may have unexpected
cheek. They usually occurred without warning but multiple sclerosis. It also helps exclude the rare
were often precipitated by talking, chewing, washing mass lesions previously mentioned.
her face, or applying lipstick to the left upper lip. She
was free of pain between attacks. Neurologic examina-
tion results were within normal limits.
Treatment
Treatment with carbamazepine (200 mg 3 times
Several treatments are available for trigeminal
daily) helped initially, but relapse necessitated dosage
increases to 800 mg and 1000 mg/d. Although the pa- neuralgia, regardless of the cause. Most patients
tient’s pain was controlled at the higher dosage, she respond initially to carbamazepine (600 mg
became lethargic and forgetful and could not function. daily), which stabilizes cell membranes and in-
The neurosurgical consultant advised microvascular creases the threshold of neural stimulation.
decompression. Teflon was placed between the artery Phenytoin, gabapentin, and lioresal are also
and the nerve to maintain decompression. Postopera- used. When these fail, surgery is considered.
tively, the patient experienced significant relief.
If the diagnosis is accurate, surgical decom-
Disabling, brief paroxysms of pain within the pression is often successful. Effective surgical
CN-V distribution are some of the worst pains an procedures include microvascular decompres-
individual may experience. Also called tic sion of the trigeminal sensory root, percuta-
doulereux, trigeminal neuralgia is not diagnos- neous radiofrequency rhizotomy, and percuta-
able by any test, so diagnosis must come from its neous trigeminal ganglion balloon compression.
characteristic history. Patients are almost always The latter two imply partial and irreversible de-
adults and often senior citizens. Typically, they struction of the trigeminal ganglion.
experience spontaneous, paroxysmal, unilateral, Nonparoxysmal steady pain, post-traumatic
often provocable, disabling pain, usually in the pain, pain after dental procedures, and pain
lower face and primarily within the second or that is not strictly within the trigeminal zone,
third trigeminal divisions, rarely involving the first including pain at the vertex, side of the neck,
division. Knowledge of the anatomy and facial or retroauricular sources, are not trigeminal
distribution of CN-V and an awareness of the neuralgia. These other types of pain do not re-
character of trigeminal neuralgia are essential for spond effectively to trigeminal neuralgia treat-
accurate diagnosis (Figure 10-4). ments.

CRANIAL NERVES
106
CRANIAL NERVE V: TRIGEMINAL

Figure 10-4 Trigeminal Neuralgia


Sensory distribution of trigeminal (V) nerve
Trigeminal (semilunar) ganglion

Ophthalmic n.
Frontal n.

Nasociliary n.
Lacrimal n.
Supraorbital nn.
Ant. and post.
ethmoidal nn.
Int. nasal nn.
Ext. nasal n.

Maxillary n.
Zygomatico-
temporal n.
Zygomaticofacial n.
Infraorbital n.
Mandibular n.
Sup. alveolar nn.
Auriculotemporal n.
Sup. dental and Buccal n.
gingival branches
Lingual n.
Post. nasal nn.
Inf. alveolar n.
Palatine nn.
Inf. dental and gingival branches
Pharyngeal branch Mental n.

Ophthalmic n. zone

Zones of skin
innervation of Maxillary n. zone
trigeminal nerve
divisions, where
pain may occur
in trigeminal Common
neuralgia trigger points

Mandibular n. zone

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107
CRANIAL NERVE V: TRIGEMINAL

OTHER CN-V LESIONS Table 10-1


Clinical Presentation and Diagnostic Differential Diagnosis of CN-V Lesion
Approach
Origin
Facial numbness is the most common symp-
tom of CN-V neuropathy and may be produced Brainstem: stroke, brain stem gliomas, multiple
by a lesion at any site along the sensory path- sclerosis, or syringobulbia
way: the nuclei within the brainstem, sensory Intracranial: trigeminal neurinoma, acoustic
root, trigeminal ganglion, or primary divisions of neurinoma, meningioma, granuloma, metastasis,
CN-V. The division or nerve in question is identi- herpes zoster, carotid or basilar aneurysms,
fied by testing sensation in the distribution of in- trigeminal sensory neuropathy
dividual branches. Skull base lesions: metastasis, nasopharyngeal
Unilateral loss of the corneal reflex is a clini- carcinoma, lymphoma, basilar meningitis
cally useful sign of CN-V neuropathy, although its Trigeminal divisions and nerve branches: trauma,
localizing value is low. Masticatory weakness, metastasis, spreading skin tumor, salivary tumor,
difficult to test when the change is subtle, occurs vasculitis, leprosy
when the motor nucleus, root, or mandibular di- Drug intoxication: trichloroethylene,
vision is damaged. hydroxystilbamidine
Facial pain, numbness, or both are the hall-
marks of most CN-V lesions, including herpes
zoster. The possibility of a neoplasm infiltrating trigeminal ganglion becomes reactivated, affect-
the nerve must be considered with severe and ing the ophthalmic division. Most patients pre-
persistent facial pain. Impairment of general sen- sent with a characteristic periorbital vesicular
sation from the tongue and palate carried by CN- rash and severe neuralgic pain within the oph-
V may also cause taste disturbances, even thalmic division (Figure 10-5). Similar to other
though the special sensory fibers providing taste herpes zoster syndromes, the pain may precede
sensation are supplied by the facial and glos- eruption of cutaneous lesions. Permanent visual
sopharyngeal nerves. Both general and special impairment is the most serious outcome of oph-
sensation to the tongue and palate are necessary thalmic zoster infection. Antiviral agents such as
for fully functional taste. acyclovir are the main therapy. Rarely, an ipsilat-
eral middle cerebral artery infarction may subse-
Differential Diagnosis quently develop in these patients.
The primary differential diagnosis includes In trigeminal sensory neuropathy, the trigem-
herpes zoster, dental trauma, and other cranial inal ganglion cell bodies are the primary patho-
injuries, the most frequent etiologies in devel- logic target. Although the pathogenesis of this
oped countries, followed by neuropathies due ganglionopathy is not understood, an associa-
to neoplasms and idiopathic causes. Worldwide, tion between trigeminal sensory neuropathy
Hansen disease is a common cause of CN-V neu- and connective tissue disorders is recognized.
ropathy (Table 10-1). Presumably, circulating autoantibodies attack
the ganglion cells whose blood-brain barrier is
Clinical Entities more permeable to large molecules than is the
CN-V divisions and branches are exposed to blood-brain barrier elsewhere in the peripheral
injuries especially from invasive dental treat- nervous system. Systemic sclerosis and mixed
ments, fractures of facial bones or the cranium, connective tissue disorder typically have numb-
and other invasive procedures in the face and ness that begins around the mouth and spreads
neck. Trauma is among the most common eti- slowly over months, involving all CN-V divisions,
ologies of CN-V lesions and typically results in unilaterally or bilaterally. Sometimes the oph-
anesthesia in the area supplied by the affected thalmic division is spared. In Sjögren syndrome,
branch. trigeminal involvement is common and usually
Herpes zoster ophthalmicus occurs when a seen as part of a more widespread sensory gan-
latent varicella-zoster virus infection in the glionopathy.

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CRANIAL NERVE V: TRIGEMINAL

Figure 10-5 Varicella Zoster with Probable Keratitis

Herpes zoster

Trigeminal neurinomas are rare (0.2% of in- age to adjacent structures. Thus, tumors growing
tracranial tumors and 2% to 3% of intracranial downward into the posterior fossa may lead to
neurinomas), usually benign, well-demarcated, cerebellar ataxia and lesions of CN-VII and CN-
and slowly growing neoplasms. Most frequently, VIII, manifesting with facial palsy, tinnitus, or hear-
they arise near the trigeminal ganglion, usually ex- ing loss. In contrast, neurinomas exerting pres-
tending into the middle and posterior cranial fos- sure upward on the cavernous sinus lateral wall
sae. Rarely, they arise exclusively from one of the produce CN-II, -III, -IV, and -VI lesions.
sensory divisions and spread extracranially. Rare Cerebellopontine angle tumors, typically
instances of malignant schwannomas originating acoustic neurinomas, initially involve CN-VIII
within the trigeminal ganglion are described. (Figure 10-6). Enlarging tumors may compress
Most neurinomas reach a diameter of up to 2.5 the trigeminal sensory root and lead to facial
cm before diagnosis because of their slow and numbness or pain with subsequent ipsilateral
asymptomatic growth. At presentation, patients loss of corneal reflex.
typically report numbness and paresthesias Other neoplasms include meningiomas, epi-
within the CN-V distribution. Trigeminal neuralgia dermoids, lymphomas, hemangioblastomas,
or burning intermittent pain is less common. gangliocytomas, chondromas, and sarcomas.
Rarely, sensory problems are accompanied by Tumors involving the face, such as squamous
other neurologic symptoms resulting from dam- cell carcinoma, microcystic adenexal carci-

CRANIAL NERVES
109
CRANIAL NERVE V: TRIGEMINAL

Figure 10-6 Acoustic Neurinoma Compressing Trigeminal Nerve*

*
V

VII

VIII

IX

Large acoustic neurinoma filling cerebellopontine angle, distorting brainstem and cranial nerves V, VII, VIII, IX, X

noma, and keratoacanthoma, have a proclivity tomeninges. Lymphoproliferative malignancies


for invading cutaneous nerves because of their and metastatic breast cancer are the most com-
innate neurotropism. Similarly, certain skull base mon etiologies.
tumors, such as nasopharyngeal carcinoma, sali-
vary gland adenocarcinoma, and metastatic dis- REFERENCES
ease, sometimes also invade various trigeminal Elias WJ, Burchiel KJ. Trigeminal neuralgia and other neuro-
divisions. The numb chin syndrome consists of pathic pain syndromes of the head and face. Curr Pain
unilateral numbness of the chin and adjacent Headache Rep. 2002;6:115-124.
Hughes RAC. Diseases of the fifth cranial nerve. In: Dyck PJ,
lower lip. Although seemingly harmless, it is usu- Thomas PK, Griffin JW, Low PA, Poduslo JF, eds. Peripheral
ally an ominous sign of primary or metastatic Neuropathy. 3rd ed. Philadelphia, Pa: WB Saunders Co;
cancer involving the mandible, skull base, or lep- 1993;801-817.

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CRANIAL NERVE V: TRIGEMINAL

Loeser JD. Tic douloureux. Pain Res Manag. 2001;6:156-165. ophthalmicus in Olmsted County, Minnesota: have sys-
Maillefert JF, Gazet-Maillefert MP, Tavernier C, et al. Numb temic antivirals made a difference? Arch Ophthalmol.
chin syndrome. Joint Bone Spine. 2000;67:86-93. 2003;121:386-390.
Nager GT. Neurinomas of the trigeminal nerve. Am J Oto- Starr CE, Pavan-Langston D. Varicella-zoster virus: mecha-
laryngol. 1984;5:301-333. nisms of pathogenicity and corneal disease. Ophthalmol
Rosenbaum R. Neuromuscular complications of connective Clin North Am. 2002;15:7-15.
tissue diseases. Muscle Nerve. 2001;24:154-169. Sweeney PJ, Hanson MR. The cranial neuropathies. In:
Rowe JG, Radatz MW, Walton L, et al. Gamma knife stereo- Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds.
tactic radiosurgery for unilateral acoustic neuromas. J Neurology in Clinical Practice: Principles of Diagnosis and
Neurol Neurosurg Psychiatry. 2003;74:1536-1542. Management. 2nd ed. Boston, Mass: Butterworth-Heine-
Severson EA, Baratz KH, Hodge DO, et al. Herpes zoster mann; 1996;1721-1732.

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111
Chapter 11

Cranial Nerve VII: Facial


Michal Vytopil, Peter J. Catalano, and H. Royden Jones, Jr

Lesions of the facial nerve (CN-VII) are one of the most frequent cranial mononeuropathies. As one
of the most complex cranial nerves, CN-VII has a long course, multiple functions, and 4 primary com-
ponents. Motor fibers constitute the largest component and serve the primary function of CN-VII: in-
nervating the muscles of facial expression. Unilateral, complete facial weakness is the hallmark of al-
most all facial neuropathies. CN-VII also contains autonomic fibers that initiate lacrimal, salivary, and
mucous secretions; special sensory fibers that collect taste sensations from the anterior two thirds of
the tongue; and general sensory fibers that innervate the external auditory canal and a small skin area
behind the ear.

When a patient presents with facial weakness, glionic parasympathetic fibers arise from the su-
differentiation should be made between periph- perior salivatory nucleus, relay through the
eral facial nerve lesions and CNS facial pterygopalatine and submandibular ganglions,
processes. With the latter, when the patient is re- and eventually provide the efferent function for
laxed, subtle suggestions of a facial nerve lesion lacrimation and salivation. The other intermedi-
may be appreciated by noting the affected na- ate nerve fibers carrying taste and general so-
solabial fold flattening. Brain lesions are often as- matic sensation have their primary cell bodies in
sociated with other findings that can help with lo- the geniculate ganglion and terminate within the
calization. For example, a small lesion near the nucleus solitarius and the spinal tract of CN-V, re-
Broca area may result in motor aphasia and fa- spectively.
cial weakness. Larger lesions affecting a signifi-
cant portion of a hemisphere, as with large hemi- Peripheral CN-VII
spheric strokes, cause a constellation of Both roots of CN-VII leave the brainstem to
symptoms, including face, arm, and leg weak- enter the temporal bone via the internal auditory
ness; gaze deviation; and neglect or aphasia. meatus, where they accompany the auditory
Posterior limb lesions of the internal capsule re- nerve (CN-VIII) passing through the internal au-
sult in face, arm, and leg weakness. ditory canal (Figure 11-1). CN-VII continues to
the periphery through the facial canal; this seg-
ANATOMY ment has 5 parts, based on their relation to
Intrapontine Portion surrounding anatomical structures. The labyrin-
CN-VII consists of 2 primary roots (Figure 11- thine segment passes above the labyrinth and
1). The larger division carries somatic motor leads anterolaterally to the geniculate ganglion,
fibers and has its origin within the facial nucleus containing the cell bodies of CN-VII afferents. At
in the caudal pons, where it lies adjacent to the this site, the canal abruptly turns posteriorly and
spinal tract of the trigeminal nerve (CN-V). It then forms the external genu of CN-VII. The greater
passes dorsally and rostrally, curves around the petrosal nerve originates here; it carries pregan-
abducens nerve (CN-VI) nucleus (internal genu), glionic parasympathetic fibers to the ptery-
and exits the brainstem at the bulbopontine an- gopalatine ganglion, where they synapse and
gle between CN-VI and CN-VIII. subsequently direct postganglionic fibers to the
Its smaller component, the intermediate nerve lacrimal gland.
of Wrisberg (the nervus intermedius), contains a The tympanic segment of CN-VII travels pos-
combination of autonomic, special sensory teriorly and laterally along the medial wall of the
(taste), and general sensory fibers. Its pregan- middle ear. At the posterior wall of the middle

CRANIAL NERVES
112
CRANIAL NERVE VII: FACIAL

ear, the facial canal changes its course and trav- presumably, bilateral central innervation of the
els inferiorly toward the exit at the stylomastoid motor neurons for the upper face, in contrast to
foramen. This vertical portion is named the mas- the classic contralateral input to the lower facial
toid segment; it has 2 important branches: prox- muscles, is responsible for this valuable clinical
imally, the stapedius nerve arises to innervate tool. In the uncommon circumstance where
the stapedius muscle; more distally, the chorda there is bilateral corticobulbar tract involvement,
tympani branches and exits the facial canal and, as in various suprabulbar palsies, facial move-
after traversing the middle ear, joins the lingual ment is retained in response to emotional stimuli
nerve belonging to the third division of CN-V. but absent as a voluntary effort.
The chorda tympani contains preganglionic Intramedullary pontine lesions affect the fa-
parasympathetic fibers that synapse within the cial motor nucleus and often the contiguous
submandibular ganglion to innervate the sub- fibers of CN-VI that traverse over the facial nu-
mandibular and sublingual glands. The chorda cleus. These often lead to concomitant ipsilateral
tympani also carries taste fibers. Their cell bod- paralysis of facial expression with diminished lat-
ies originate within the geniculate ganglion, me- eral gaze as in a brainstem glioma.
diating taste sensation from the anterior two Cerebellopontine (CP) angle tumors, if they
thirds of the tongue. are large acoustic neuromas, have conjoint CN-
Soon after leaving the skull at the stylomas- VIII and CN-VII fiber involvement manifested by
toid foramen, the distal CN-VII gives rise to sev- diminished hearing, sometimes initially present-
eral small motor branches innervating the poste- ing with tinnitus and later presenting with pe-
rior auricular, occipital, digastric, and stylohyoid ripheral facial paresis (Figure 11-3). Occasionally,
muscles (Figure 11-2). The main motor trunk of when these lesions are large, concomitant in-
CN-VII then passes through the parotid gland to volvement of the ipsilateral CN-V fibers causes
terminate as the temporal, zygomatic, buccal, unilateral facial numbness, sometimes only ini-
mandibular, and cervical branches. The first tially signaled by the loss of the corneal reflex.
two innervate the muscles involved in moving In addition to an ipsilateral facial palsy, rela-
the forehead, closing the eyes, and wrinkling the tively proximal intracanicular CN-VII palsies
nose. Muscles of the lower face and neck are pri- have an associated motor fiber dysfunction of
marily innervated by the latter 2 branches. CN- the stapedius muscles characteristically associ-
VII subserves all muscles of facial expression ex- ated with hyperacusis, an increased sensitivity to
cept the levator palpebrae superioris, and sound that is particularly noticeable while using
therefore, CN-VII impairment, with a resultant a telephone. When CN-VII is injured along its
asymmetric facies, is a major social and cosmetic course from the facial nucleus to the geniculate
impediment. ganglion, lacrimation, salivation, taste sensation
for the anterior two thirds of the tongue, and so-
CLINICAL CORRELATIONS AND matic sensation for the external auditory canal
ENTITIES are also impaired.
CN-VII can be damaged at any level along its When the lesion is more distally situated, be-
complex course. Almost all CN-VII pathologies, tween the geniculate ganglion and the stapedius
regardless of the lesion site, produce a facial nerve, all of the above findings occur, but
musculature paralysis. The degree of facial paral- lacrimation is spared. If damage occurs between
ysis with various dysfunctions of other CN-VII the stapedius nerve and the chorda tympani, sali-
components causes symptoms that help to iden- vation and taste are impaired, but changes in
tify the lesion site. lacrimation and hyperacusis are not present. CN-
An upper motor neuron dysfunction causes VII lesions distal to the chorda tympani only pro-
facial palsy by depriving the facial nucleus of its duce ipsilateral facial weakness.
cortical input. Because there is bilateral innerva-
tion of the frontalis and orbicularis oculi, these Clinical Vignette
muscles are spared with an upper motor neuron
An 18-year-old, previously healthy woman experi-
lesion such as a cerebral infarct. The mechanism enced modest pain in her right ear; 1 day later, voices
of this phenomenon is not fully appreciated, but

CRANIAL NERVES
113
CRANIAL NERVE VII: FACIAL

Figure 11-1 Facial Nerve: Intracranial Course


Facial Nerve: VII
Afferent fibers Geniculum of facial nerve Facial canal
(site of geniculate ganglion) Tympanic cavity
Greater petrosal nerve
Chorda tympani nerve
Cochlear (spiral) ganglion
Head of malleus
Vestibular nerve

Cochlear nerve Incus

Motor root of facial nerve


and intermediate nerve

Vestibulocochlear
nerve (VIII)

Medulla oblongata
(cross section)

Ampulla of lateral
semicircular duct

Ampulla of superior
semicircular duct
Internal
Medial auditory Utricle
meatus
Vestibular Superior Ampulla of posterior
nuclei Anterior semicircular duct
Cochlear
(diagrammatic) Inferior Posterior nuclei Saccule
Lateral Inferior cerebellar
peduncle (to cerebellum) Superior division
of vestibular nerve
Inferior division
Vestibular ganglion

Tympanic segment of facial nerve


Head of malleus Superior ligament of malleus

Epitympanic recess Superior ligament of incus

Anterior process of malleus Short limb of incus

Chorda tympani nerve Posterior mallear fold


Anterior mallear fold
Posterior ligament of
Tensor tympani muscle incus

Handle of malleus Long limb of incus

Tympanic Chorda tympani nerve


membrane
(pars tensa) Lenticular process of
incus
Pharyngotympanic
(auditory) tube Facial nerve (VII)

Internal carotid artery

CRANIAL NERVES
114
CRANIAL NERVE VII: FACIAL

Figure 11-2 Facial Nerve (VII): Schema

Greater petrosal nerve Facial nerve (VII)


Deep petrosal nerve Internal acoustic meatus
Geniculate ganglion
(from internal carotid plexus) Intermediate nerve of Whisberg
Internal carotid plexus Motor nucleus of facial nerve
Lesser petrosal nerve (on internal carotid artery)
Nerve (Vidian) of pterygoid canal Superior salivatory nucleus
Otic ganglion Solitary tract nucleus
Pterygopalatine ganglion

Facial muscles
Frontal belly (frontalis)
of occipitofrontalis
Orbicularis oculi
Corrugator supercilii
Zygomaticus major
Zygomaticus minor
Temporal branc

Procerus
Levator labii
superioris
Levator labii
superioris
hes

alaeque nasi
Levator
anguli oris
Nasalis

Zygomati
Depressor c
septi nasi Occipital
Tas tongue belly
Orbicularis of Buccal
oris branches (occipitalis) of
Occipital occipitofrontalis
Depressor muscle
branch of
ch

anguli oris
bran

posterior
Depressor labii auricular
l
Cervica

inferioris nerve
Mentalis Branches to auricular muscles
(Risorius) Posterior auricular nerve
al
(not shown) Marginibular Nerve to stapedius muscle
mand h
Buccinator branc Stylomastoid foramen
Platysma Tympanic plexus
Tympanic nerve (Jacobson)
Sublingual gland (from glossopharyngeal nerve)
Submandibular gland Glossopharyngeal nerve (IX)
Submandibular ganglion Digastric muscle (posterior belly)
Lingual nerve (from trigeminal nerve) Stylohyoid muscle
Efferent fibers Chorda tympani nerve Caroticotympanic nerve (from internal carotid plexus)
Afferent fibers
Parasympathetic fibers
Sympathetic fibers

CRANIAL NERVES
115
CRANIAL NERVE VII: FACIAL

Figure 11-3 Cerebellopontine Angle Tumor


Trigeminal
(V) n. Tentorium
Petrous ridge

Transverse
Porus acusticus sinus
(opening of internal
auditory meatus)

Cut bone

Dura
mater

A. Pre-operative compressing
CN-VII, -VIII (not visualized)

Tumor covered by arachnoid

Cranial nn. IX, X, XI

Cerebellum retracted

B. Postoperative tumor bed revealing VI, VIII

Cut end of superior vestibular n.

Cut end of inferior vestibular n.

Flattened and widened facial (VII) n.

Flattened and widened cochlear n.

Loop of anterior inferior cerebellar a.

Depressed tumor bed in brainstem

Cut end of vestibular n.

CRANIAL NERVES
116
CRANIAL NERVE VII: FACIAL

seemed to be louder than normal while she was talking mouth. Less commonly, decreased taste, or hy-
on the phone. The next day, she noted that her right peracusis, is the first symptom. A preceding dull
eye did not close, and she could not smile on that side.
aching pain behind the ear is a common initial
Tearing was diminished in her right eye, and she could
not taste things on the right side of her tongue. problem.
On examination, the patient was unable to wrinkle her Facial asymmetry is unequivocally present;
forehead, smile on the right, or close her right eyelids, al- the affected frontalis is smooth and cannot be
though the eye rolled upward when she attempted to do normally corrugated, whereas the angle of the
so. She had diminished tearing and loss of taste sensation mouth appears depressed even in repose. Ever-
on the anterior two thirds of the right side of her tongue. sion of the lower lid, responsible for excessive
All other examination results were normal.
tearing (epiphora) in some patients, and an in-
A steroid dose pack was given at the initial evalua-
ability to completely close the eyelids (lagoph-
tion. The patient experienced gradual return of total
CN-VII function over the next 2 months. thalmos) result from orbicularis oculi weakness.
The Bell phenomenon refers to the eyeball turn-
Idiopathic Facial Palsy ing up without eyelid closure despite attempted
This vignette describes a benign, idiopathic fa- eye closure (Figure 11-4). Whether the palsy is
cial palsy (Bell palsy) (Figure 11-4). The lesion accompanied by taste disturbances may help to
had a proximal location, denoted by the loss of distinguish lesions proximal and distal to the
total motor function on one side of the patient’s chorda tympani branch. For example, a pure mo-
face involving the frontalis and orbicularis oculi tor lesion may suggest a lesion at the distal part
muscles, the lower facial muscles, and the loss of of the facial canal or within the parotid gland,
stapedius muscle and lacrimal gland function. whereas when all 4 primary functions are af-
Her benign outcome is typical of 85% to 90% of fected, an unusual proximal lesion, or even a
individuals with acute and relatively total Bell pontine lesion when concomitant ocular abduc-
palsy within a few days after symptom onset. tion is weak, is a possibility.
Bell palsy is one of the most common and dis-
tinctive entities in clinical neurology. Typically, Differential Diagnosis
patients present with acute unilateral weakness It is important to initially exclude other causes
of all mimetic muscles, and the lesions evolve, of- of unilateral facial paralysis and determine
ten over several hours to just a few days. Al- whether the lesion has an upper or a lower mo-
though Bell palsy is usually benign, its dramatic tor neuron source. Patients with upper motor
appearance creates significant distress in many neuron paralysis primarily present with an asym-
patients who are concerned that it may repre- metric smile, unilateral drooling, or both,
sent a stroke or that permanent facial disfigure- whereas in peripheral facial palsy, all muscula-
ment will result. ture innervated by CN-VII is affected.
The pathogenesis of Bell palsy is generally Inflammatory conditions present with acute
thought to be associated with edema, entrap- facial palsy. Facial palsy in Lyme disease, sar-
ment, and resulting ischemia of CN-VII as it coidosis, and Guillain-Barré syndrome is often
passes through its bony canal. The specific but not always bilateral. Other features distin-
pathophysiology is unknown; a reactivation of guishing these conditions are usually present. In
herpes simplex and varicella-zoster virus latent some instances of Ramsay-Hunt syndrome, uni-
infection within the geniculate ganglion is hy- lateral facial paralysis, secondary to herpes
pothesized. The combination of possible edema zoster, may precede the typical herpetic vesicles
and infection provide the basis for treatment. in the external auditory canal and is clinically in-
distinguishable from classic idiopathic Bell palsy.
Clinical Presentation The diagnosis requires detection of a 4-fold in-
Patients become aware of the weakness when crease in the antibody titer against the virus. The
a family member or friend notes the facial asym- typically acute onset of Bell palsy is another criti-
metry, when they notice an inability to close an cal differential feature in favor of an idiopathic or
eye, or when they experience difficulty holding infectious mechanism versus the evolutionary
saliva, food, and fluids in the affected side of the nature of neoplastic processes.

CRANIAL NERVES
117
CRANIAL NERVE VII: FACIAL

Figure 11-4 Bell Palsy


Course and distribution of facial (VII) nerve
Facial Sites of leisons and
Occipito- Orbicularis oculi m. (VII) n. their manifestations
frontal m. Lacrimal gland
Acoustic
1. Intracranial and/or
Pterygopalatine Pons (VIII) n.
Corrugator internal auditory meatus.
ganglion
super- All symptoms of 2, 3,
cilii m. Geniculate and 4, plus deafness
ganglion due to involvement of
Greater eighth cranial nerve.
petrosal n.
Temporal 2. Geniculate ganglion.
branch 2
1 All symptoms of 3 and
4, plus pain behind ear.
Herpes of tympanum
Stapedius m. and of external auditory
Tympanum 3 meatus may occur.
Stylo-
Lingual n. mastoid
3. Facial canal.
formen
All symptoms of 4, plus
loss of taste in anterior
tongue and decreased
Tongue Posterior
salivation on affected
auricu-
side due to chorda
lar n.
tympani involvement.
4 Chorda Hyperacusia due to
tympani effect on nerve branch
Parotid gland to stapedius muscle.

Buccal branch 4. Below stylomastoid


Marginal foramen (parotid gland
mandibular branch tumor, trauma).
Facial paralysis (mouth
Cervical branch draws to opposite side;
Orbicularis on affected side,
oris m. Zygomatic branch patient unable to close
Levator Sublingual eye or wrinkle forehead;
gland Platysma m. food collects between
anguli oris m.
teeth and cheek due
Submandibular ganglion
Depressor Risorius m. to paralysis of
anguli oris m. Submandibular gland buccinator muscle).

Buccinator m.

In patient’s attempts
to smile or bare
teeth, mouth draws
to unaffected side.
Patient cannot wink,
close eye, or wrinkle
forehead on
affected side.

Hyperacusia: patient holds phone away from


ear because of painful sensitivity to sound

CRANIAL NERVES
118
CRANIAL NERVE VII: FACIAL

Slowly progressive evolution of a unilateral ity of the underlying CN-VII injury. The degree
CN-VII weakness suggests a neoplasm. Neo- of injury ranges from mild (pure demyelinating
plasms may be located proximally and focally, conduction block) to severe (axon loss and re-
near the brainstem, and later may be associated sulting wallerian degeneration). With a de-
with other cranial neuropathies, as with focal CP myelinating conduction block, the most com-
angle tumors. Or these may occur diffusely in the mon type (up to 90% of Bell palsy cases), there
leptomeninges, such as with metastatic carci- is no associated axon loss, and therefore, re-
noma or lymphoma and affect multiple cranial covery is prompt, complete, and without synki-
nerves, including the facial. An evolving, progres- nesis. The remaining patients have axonal dam-
sive distal CN-VII lesion suggests a parotid tumor. age with wallerian degeneration, resulting in
slow and incomplete recovery that requires re-
Treatment and Prognosis generating axons to reinnervate paralyzed mus-
Treatment is controversial because of the cles.
good prognosis in most Bell palsy cases. Corti- EMG provides valuable prognostic informa-
costeroid use seems to reduce the duration of tion, especially if it is not performed until ap-
paralysis and risk of permanent impairment. The proximately 3 weeks after onset. By then, it is
typical regimen, if started within 7 days after the possible to distinguish between nerve fibers that
onset of palsy, is 1 mg/kg oral prednisone, up to have undergone wallerian degeneration and
60 mg/d for 6 days and then tapering over the those that are only temporarily blocked. A signif-
next 4 days. Limited evidence suggests that the icantly reduced amplitude of CN-VII compound
combination of acyclovir and prednisone has muscle action potential and abundant fibrilla-
greater complete recovery rates than pred- tion potentials in facial muscles indicate the for-
nisone alone. The common dosage of acyclovir mer, whereas a demyelinating conduction block
is 2000 mg/d for 10 days. There is insufficient ev- is typically partially resolved by that time, evi-
idence to support primary surgical CN-VII de- denced by absent or scarce fibrillation poten-
compression in Bell palsy. tials.
Great care is required to protect the exposed
cornea that is subject to trauma from incomplete Infectious Facial Palsies
eye closure. Additionally, a dry and unprotected Varicella-Zoster Virus
cornea is susceptible to development of trophic Ramsay-Hunt syndrome, from reactivation of
defects. Therefore, eye patching, particularly at the varicella-zoster virus within the geniculate
night, and artificial tears are warranted. ganglion, is the second most common cause of
The recovery rate from Bell palsy follows 2 atraumatic facial palsy. Clinically, it is character-
patterns: most patients begin to regain facial ized by the triad of acute facial palsy, neuralgic
strength within 3 weeks after onset, but in some, pain, and eruption of herpetic vesicles within the
the recovery is delayed until 3 to 6 months after external auditory canal, ipsilateral palate, and an-
onset. The overall prognosis is good; most pa- terior two thirds of the tongue. The areas of pain
tients (80-85%) recover completely, but the rest and rash are appropriate to the general sensory
may have synkinesis, residual weakness, tearing, innervation of the afferent CN-VII branches. The
or contracture. Synkinesis is the most frequent geniculate ganglion cell bodies host the latent
permanent sequela, resulting from misdirection varicella-zoster virus infection.
of regenerating axons that grow into muscles The prognosis for Ramsay-Hunt syndrome is
that they initially did not innervate. It clinically worse than that of idiopathic Bell palsy, with fre-
manifests as synchronized movement of differ- quent complete paralysis, incomplete recovery,
ent muscles that normally do not contract to- and residual synkinesis. Therefore, aggressive
gether. Typically, there is subtle eye closure with treatment with acyclovir (750 mg/d IV or 4000
smiling, or a lip or chin twitch with blinking. This mg/d oral), in combination with prednisone, is
is rarely disabling. indicated. The best long-term results are ob-
The rate, completeness, and quality of recov- tained when treatment is started within 3 days af-
ery from Bell palsy are determined by the sever- ter onset.

CRANIAL NERVES
119
CRANIAL NERVE VII: FACIAL

Lyme Disease mended. If meningitis is clearly present, treat-


ment should be more aggressive. Facial palsy
Clinical Vignette prognosis in Lyme disease is excellent; most pa-
tients recover completely.
A 32-year-old woman presented with recent left facial
drooping and an associated 1-week history of
headaches and photosensitivity. Other Infections
Her temperature was 38°C (100.6°F). There was a 10- Peripheral facial paralysis may occur with in-
cm circular rash on the medial aspect of her right thigh. fectious mononucleosis, caused by Epstein-Barr
Her neck was slightly rigid; no intraauricular vesicles virus, and poliomyelitis, caused by an en-
were noted. Neurologic examination results demon- terovirus.
strated an isolated left peripheral CN-VII weakness.
Several infectious conditions involving the
Brain CT results were unremarkable. Lumbar punc-
ture revealed a WBC count of 23/mm3, primarily lym-
temporal bone can also cause peripheral facial
phocytes, with a normal protein level and a slightly de- paralysis, such as acute and chronic otitis media
creased glucose level. CSF and serum Lyme antibody and osteomyelitis of diverse etiologies, includ-
test results were positive. ing tuberculosis and syphilis. Similarly, acute
This vignette is typical of Bell palsy secondary bacterial or tuberculous meningitis may affect
to Lyme disease (neuroborreliosis). Although rel- multiple cranial nerves including CN-VII. Lep-
atively uncommon, it should always be consid- rosy is a common cause of facial palsy in en-
ered, particularly in endemic areas. demic areas.
Facial paralysis is the most common focal
manifestation of neuroborreliosis; 40% of these Granulomatous Disorders
patients have cranial neuropathies, and approxi- Sarcoidosis is a disease of unknown etiology
mately 80% have CN-VII involvement. Multiple characterized by histopathologic findings of
cranial nerves are affected in one fifth of those nonnecrotizing granulomas within multiple or-
with a cranial neuropathy; two thirds of this 20% gans. Unilateral or bilateral CN-VII palsy with hy-
with multiple cranial neuropathies primarily peracusis and dysgeusia, thought to result from
have bilateral facial palsy. Additional clues (ie, granulomatous meningitis, is the most frequent
erythema migrans, possible exposure to disease neurologic manifestation. The prognosis is favor-
transmitting ticks) suggesting neuroborreliosis in able; most patients recover completely after
patients with acute facial palsy warrant further steroid treatment.
studies. Wegener granulomatosis is a systemic dis-
Essential studies include titers of anti–Borrelia ease characterized by necrotizing, granulo-
burgdorferi antibodies in blood and CSF and matous lesions of the upper and lower respira-
standard CSF analyses. Although the latter may tory tract, glomerulonephritis, and systemic
demonstrate a pleocytosis with lymphocytic pre- necrotizing vasculitis. Of the primary systemic
dominance, the results of this analysis are some- vasculitides, only Wegener granulomatosis is as-
times normal. Facial paralysis may also occur be- sociated with a significant frequency of cranial
fore seroconversion, eg, early in the disease neuropathies. CN-VII involvement, usually oc-
before antibody testing results become positive.
curring in conjunction with other cranial neu-
When clinical suspicion of Lyme disease is high,
ropathies, may reflect direct granulomatous in-
follow-up serologic tests are indicated.
vasion of the temporal bone or granulomatous
Although it is generally presumed that these
basilar meningitis. Because the 2-year fatality
CN-VII lesions are from basilar meningitis, some
rate of untreated Wegener granulomatosis is
patients with Lyme disease have a pure motor fa-
greater than 90%, aggressive immunotherapy is
cial paresis without dysgeusia, hyperacusis, or
warranted on diagnosis.
associated CSF pleocytosis. This suggests that
the facial neuropathy in some patients results
from a more distal CN-VII lesion. Traumatic Facial Palsy
Optimal treatment remains controversial; a Facial paralysis may occur in traumatic frac-
regimen of oral or IV antibiotics is recom- tures of petrous temporal bone. Also, facial palsy

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may result from surgery of the middle ear, Biopsy results demonstrated a malignant parotid
surgery of the parotid gland, or mastoidectomy. gland adenocarcinoma with extension beyond the
capsule at surgery.
Neoplasms The absence of clinical hyperacusis and
Several primary and metastatic malignancies preservation of taste function in the patient in
may cause a facial palsy. Carcinomatous this vignette pointed to a pure motor lesion dis-
meningitis usually affects multiple cranial tal to the chorda tympani branch. Unfortunately,
nerves; the most common sources are the lung, the progressive nature of the temporal profile of
the breast, and gastrointestinal cancers and lym- the illness was not appreciated at the first pre-
phomas. Typically, these tumors have an aggres- sentation. Whether the parotid tumor would
sive clinical course; those that present with an have been identified before extending beyond
isolated CN-VII lesion soon demonstrate signs its capsule is unknown but emphasizes the im-
of multiple cranial or spinal nerve root involve- portance of determining the temporal profile of
ment or both. each patient’s presentation, because early diag-
Certain benign tumors may exert chronic ex- nosis can be lifesaving. This man died within a
trinsic pressure on CN-VII. Schwannomas from few years.
the vestibular portion of CN-VIII, typically occur-
ring within the acoustic meatus at the CP angle, Uncommon Mass Lesions
or less commonly meningiomas at similar sites, Cholesteatomas are another rare mass lesion
do not affect CN-VII initially. When they eventu- at the CP angle that deserve consideration in pa-
ally do so, they tend to predominantly and subtly tients with slowly evolving facial paralysis. Other
affect sensory fibers; motor fibers are more re- uncommon entities include pontine gliomas,
silient to chronic deformation. Therefore, the arachnoid cysts, lipomas, and hemangiomas.
only sign of CN-VII involvement may be rela-
tively minor numbness behind the ear, on the
floor of the ear canal, in the posterior inferior Neuromuscular Disorders With Facial Palsy
quadrant of the eardrum (Hitselberger sign), or a The motor portion of the CN-VII nucleus with
combination of these sites. The change in hear- nuclei of CN-V, -IX, -X, -XI, and -XII may be in-
ing leads to the diagnosis. Signs of a motor CN- volved in various motor neuron diseases, such as
amyotrophic lateral sclerosis and bulbospinal
VII lesion do not occur until these lesions be-
muscular atrophy (Kennedy disease).
come large.
CN-VII is affected in 33% to 50% of Guillain-
Malignant distal infiltration of CN-VII is seen
Barré syndrome cases, often bilaterally. Al-
with parotid tumors (Figure 11-5).
though usually first evident when limb weakness
is severe, CN-VII lesions may develop at any dis-
Clinical Vignette ease stage, including as the presenting sign.
A 62-year-old man initially experienced subtle weak- Miller-Fisher syndrome, a variant of Guillain-
ness of his left lower face while shaving. Within 2 Barré syndrome, is characterized by ophthal-
months, he became aware of a more profound weak- moplegia, ataxia, and areflexia. However, in-
ness of his entire left face, particularly his ability to volvement of cranial nerves other than CN-III, IV,
close the left eye. He did not report any other symp-
toms. A neurologist reassured him that he had “be-
and VI occurs in more than 50% of cases. Facial
nign” Bell palsy. When his symptoms seemed to weakness has been reported in approximately
worsen, he sought a second opinion. 25% of published Miller-Fisher syndrome cases,
Examination results demonstrated weakness in all underscoring the important clinical overlap be-
left CN-VII divisions without synkinesis. The remainder tween classic ascending Guillain-Barré syn-
of the head, neck, and otoscopic examination results drome and Miller-Fisher syndrome.
were unremarkable. Audiologic test results were nor- Some primarily muscular diseases may cause
mal, including the left acoustic/stapedius reflex. A
corneal reflex was sluggish on the left but present bilat- bilateral facial weakness, typically accompa-
erally. Palpation of the cheek demonstrated some full- nied by wasting. In adult-onset myotonic dys-
ness in the left parotid gland. trophy, muscles innervated by CN-III and CN-V,

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CRANIAL NERVE VII: FACIAL

Figure 11-5 Facial Nerve Branches and Parotid Gland

Temporal
branches
Parotid
gland
Zygomatic
branches
Posterior
auricular
nerve
Parotid duct

Main trunk of
facial nerve Buccal branches
emerging from
stylomastoid foramen

Marginal mandibular
Nerve to posterior branch
belly of digastric
muscle and to
stylohyoid muscle
Cervical branch

Medial pterygoid muscle


Horizontal section
Ramus of mandible
Main trunk of facial nerve
Masseter muscle

Mastoid process
Parotid gland

Temporofacial division Temporal branch

Posterior auricular nerve


Zygomatic branches
Main trunk of facial nerve

Nerve to posterior belly


of digastric muscle and Buccal branches
to stylohyoid muscle

Cervicofacial division
Marginal mandibular branch

Cervical branch

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CRANIAL NERVE VII: FACIAL

Figure 11-6 Seventh Nerve Hemangioma

A B

(A and B) Axial and coronal post—gadolinium-


enhanced, T1-weighted, fat-saturated images
demonstrate enlargement and enhancement of the
geniculate ganglion (thin arrows). (C) Coronal thin
section CT of petrous bone shows smooth enlarge-
C ment of the geniculate region (arrowheads).

such as the levator palpebrae superioris and the ment is likely to be an early, slowly progressing
temporalis, are also involved. Therefore, ptosis sign.
and jaw weakness often also occur. Congenital
myotonic dystrophy may present with bilateral Recurrent CN-VII Palsy
facial diplegia and is sometimes associated with Recurrence occurs in approximately 10% of
severe neonatal hypotonia. Facial weakness oc- Bell palsy cases, a circumstance necessitating ex-
curs in 95% of patients with facioscapulo- tensive diagnostic evaluation to exclude under-
humeral dystrophy who are younger than 30 lying causes, especially neoplasms and basilar
years. It affects predominantly the orbicularis meningeal involvement.
oris and is often asymmetric. Although facial Melkersson-Rosenthal syndrome, an autoso-
weakness is rarely the presenting problem, mal dominant hereditary disorder, is character-
most patients with facioscapulohumeral dystro- ized by a triad of facial palsy, facial edema, and a
phy reveal long histories of difficulties whistling furrowed tongue (lingua plicata) but often
or blowing balloons. Therefore, facial involve- exhibits an incomplete penetrance. Each com-

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CRANIAL NERVE VII: FACIAL

ponent may occur independently or in combina- around the eyes and spreads to other ipsilateral
tion. Patient history is characterized by recurrent facial muscles, especially in the perioral region. It
attacks of facial paralysis, often beginning during is strictly confined to muscles innervated by CN-
childhood. Attacks can also include facial VII; preceding CN-VII lesions are rare. Paroxysms
swelling, particularly affecting the upper lip. The are often induced by voluntary or reflex facial
tendency to recur is the only feature of facial movements, stress, and fatigue and may persist
paralysis that distinguishes it from most Bell palsy during sleep. The most common pathogenetic
cases. mechanism of hemifacial spasm seems to be vas-
Hereditary liability to pressure palsies is an cular compression of CN-VII by an aberrant arte-
allelic disorder with the Charcot-Marie-Tooth IA rial loop near the brainstem. Therefore, detailed
neuropathy, caused by deletion of the region imaging studies including MRA are essential for
containing the peripheral-myelin-protein 22 diagnosis of hemifacial spasm. Less frequent
gene. It manifests with recurrent acute painless pathophysiologic mechanisms include tumors
palsies from nerve lesions at sites of compres- and localized infectious processes. Botox injec-
sion or increased exposure. Although the typical tions are an effective symptomatic treatment.
presentation is of peroneal or ulnar neuropathy, Surgical decompression is an alternative some-
recurrent facial paralysis occasionally occurs. times leading to remission.

CN-VII Hyperactivity DIAGNOSTIC MODALITIES


Several positive symptoms occur from exces- Diagnostic modalities include imaging studies
sive reactivity of CN-VII; synkinesis, facial myo- that may define the presence of contiguous or
kymia, and hemifacial spasm are the most fre- rarely intrinsic CN-VII lesions and testing modali-
quent. ties used to study the various functions of CN-
Synkinesis is frequently observed subsequent VII.
to aberrant reinnervation in patients with an-
tecedent severe Bell palsy; an inappropriate fa- Imaging Studies
cial movement results, ie, concomitant blinking The 2 primary imaging options are MRI and
while smiling. Ephaptic transmission, or “artifi- CT. MRI is best at imaging the CP angle and the
cial synapse”, may arise at a lesion site where de- parotid gland. CT is the choice to image the tem-
polarization of the injured fibers acts as a stimu- poral bone and its facial (fallopian) canal. MRI
lus to the intact portion of the nerve. must include primary and gadolinium enhance-
Facial myokymia is characterized by subtle, ment images. The concentration of contrast
continuous, undulating movement of facial mus- agent within lesions is of diagnostic importance.
cles. The movements are usually unilateral, sub- A proximal lesion at the CP angle is easily iden-
tle, often confined to 1 to 2 facial muscles, and tified with brain MRI. There may be unexpected
sometimes accompanied by facial contracture relatively diffuse leptomeningeal enhancement
or weakness. Observed mainly in multiple scle- when a facial neuropathy is the inciting lesion
rosis, it much less commonly reflects an intrinsic leading to a diagnosis of metastatic carcinoma or
brainstem tumor, particularly pontine gliomas. lymphoma.
In the former, it is usually self-limited and abates Primary facial neuromas also strongly en-
after several weeks. Some cases of facial hance with contrast. It is crucial that the ordering
myokymia are thought to be from an antibody- physician indicate a diagnosis of facial palsy, re-
mediated specific subtype of voltage-gated questing an evaluation of CN-VII along its entire
potassium channels. The specific antibody iden- course, not just the intracranial portion.
tified in some patients with facial myokymia also Extracranial lesions must also be considered
occurs with Isaac syndrome. in the imaging evaluation of facial
Hemifacial spasm consists of intermittent weakness/palsy. If the neoplasm appears to be
paroxysms of rapid, irregular, clonic twitching fa- distal to the stylomastoid foramen, as with a
cial movements. The attack typically starts highly malignant adenocarcinoma of parotid

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CRANIAL NERVE VII: FACIAL

gland, MRI of the face may identify this tumor. REFERENCES


Bone erosion or destruction versus remodeling Ang KL, Jones NS. Melkersson-Rosenthal syndrome. J Laryn-
is another important distinction that can be eval- gol Otol. 2002;116:386-388.
Grogan PM, Gronseth GS. Practice parameter: steroids, acy-
uated only on a bone window CT. Slow-growing clovir, and surgery for Bell’s palsy (an evidence-based re-
benign lesions remodel bone, whereas bone view): report of the Quality Standards Subcommittee of
erosion is more indicative of an aggressive or the American Academy of Neurology. Neurology.
malignant process. 2001;56:830-836.
Grose C, Bonthius D, Afifi AK. Chickenpox and the genicu-
late ganglion: facial nerve palsy, Ramsay Hunt syndrome
Intrinsic CN-VII Topognostic Testing Studies and acyclovir treatment. Pediatr Infect Dis J. 2002;21:615-
Intrinsic CN-VII topognostic testing studies are 617.
based on the presence or absence of specific Hanson MR, Sweeney PJ. Disturbances of lower cranial
anatomical branch-point functions. With mod- nerves. In: Bradley WG, Daroff RB, Fenichel GM, Marsden
CD, eds. Neurology in Clinical Practice: Principles of Diag-
ern imaging studies, these are used less often but
nosis and Management. 2nd ed. Boston, Mass: Butter-
are occasionally valuable. worth-Heinemann; 1996;251-263.
Motor fibers of CN-VII innervate the many stri- Keane JR. Bilateral seventh nerve palsy: analysis of 43 cases
ated facial muscles, including the stapedius. Its and review of the literature. Neurology. 1994;44:1198-
motor innervation is the most proximal branch 1202.
Knox GW. Treatment controversies in Bell palsy. Arch Oto-
of this group. An abnormality in the efferent limb laryngol Head Neck Surg. 1998;124:821-823.
of the acoustic reflex arc localizes the lesion Renault F, Quijano-Roy S. Congenital and acquired facial
proximal to this branch. The absence of a unilat- palsies. In: Jones HR, De Vivo DC, Darras BT, eds. Neuro-
eral acoustic reflex suggests an afferent or effer- muscular Disorders of Infancy, Childhood, and Adoles-
cence: A Clinician’s Approach. Philadelphia, Pa: Butter-
ent problem. If auditory function is normal on au-
worth-Heinemann; 2003;277-300.
diometric testing, the afferent arc is normal. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol
The Schirmer test of lacrimal flow depends Neurosurg Psychiatry. 2001;71:149–154.
on an intact geniculate ganglion, the site of the Sweeney PJ, Hanson MR. The cranial neuropathies. In:
most proximal anatomical branch point along Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds.
Neurology in Clinical Practice: Principles of Diagnosis and
the course of CN-VII, giving rise to the greater su- Management. 2nd ed. Boston, Mass: Butterworth-Heine-
perficial petrosal nerve. The greater superficial mann; 1996;1721-1732.
petrosal nerve carries autonomic fibers to the Ter Bruggen JP, van der Meche FG, de Jager AE, et al. Oph-
lacrimal gland. Decreased lacrimation based on thalmoplegic and lower cranial nerve variants merge into
each other and into classical Guillain-Barre syndrome.
Schirmer testing suggests a problem with the
Muscle Nerve. 1998;21:239-242.
lacrimal gland, the greater superficial petrosal Verzijl HT, van der Zwaag B, Cruysberg JR, et al. Mobius syn-
nerve, or CN-VII proximal to the ganglion. An as- drome redefined: a syndrome of rhombencephalic malde-
sociated facial palsy eliminates the former 2 pos- velopment. Neurology. 2003;61:327-333.
sibilities.

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Chapter 12

Cranial Nerve VIII: Vestibular


Kinan K. Hreib, Judith White, and Marie C. Lucey

Clinical Vignette
A 65-year-old woman came to the emergency department with a chief complaint of “dizziness.” At 3:00 AM, she
had awoken to an odd feeling in her head, which was accompanied by nausea. As she turned to her right to ask
her husband for help, she experienced a severe spinning sensation with increased nausea followed by vomiting.
The symptoms lasted for a few minutes. However, in the car and subsequently in the emergency department, any
head and neck movement precipitated recurrent symptoms. Her medical history included diabetes mellitus, hy-
pertension, and a remote TIA manifested by right-sided weakness.
Her blood pressure was 180/90 mm Hg. She appeared pale and uncomfortable and refused to open her eyes or
move her head during the examination. The findings of her neurologic examination were normal, with the excep-
tion that she was hesitant to get off the examining cart to allow gait testing. Brain MRI results were normal, exclud-
ing an acute cerebellar infarction. A subsequent otolith particle-repositioning (Epley) maneuver successfully allevi-
ated her symptoms.

This vignette describes a classic case of an in- to whether things actually move in front of the
dividual with acute benign paroxysmal posi- patient’s eyes helps the patient define this symp-
tional vertigo (BPPV). In most patients, this an- tom. Determining whether vertigo is precisely
noying disorder can be successfully treated by a present is helpful because any acute unilateral
simple maneuver. However, the possibility of a peripheral or central vestibular pathologic fea-
stroke or other cerebellar lesion must be consid- ture causes vertigo. Typical associated clinical
ered before making this diagnosis. findings include sudden precipitous onset, nau-
Dizziness is a common nonspecific symptom. sea and vomiting, nystagmus, and postural dyse-
In patients older than 75 years, it is the most quilibrium.
common medical complaint that brings individu- Gaze-dependent nystagmus occurs in pro-
als to a physician; dizziness is the third most cesses that affect the ipsilateral cerebellum. Verti-
common symptom among all age groups. In the cal nystagmus seen with upward gaze is often the
United States, there are 8 million visits annually result of disease in the cerebellum or tegmentum.
for dizziness; chronic dizziness affects 16% of Downward gaze vertical nystagmus is most often
the self-reported population. found in processes at the foramen magnum level,
When patients report dizziness, one of the pri- especially Chiari malformations. Optokinetic nys-
mary challenges is to define its precise character. tagmus refers to a reflexive slow movement of the
Feeling faint or lightheaded and experiencing loss eye (pursuit) and a cortically driven corrective fast
of equilibrium, vertigo, unsteady gait, and fainting movement (saccade). Patients with parietal lobe
can all be grouped under a patient’s ill-defined de- lesions lose the fast, saccadic elements of the op-
scription of “dizziness,” although these symp- tokinetic response when the strip is moved in the
toms often suggest different etiologies. Clarifica- direction of the abnormal hemisphere.
tion of the precise historical details—onset,
duration, positional and other exacerbating fac- ANATOMY
tors, and associated symptomatology—is essen- The vestibulocochlear nerve, CN-VIII, is actu-
tial to determine the likely cause. ally composed of 2 nerves: the vestibular and
Vertigo is the illusory perception of motion. cochlear nerves. The vestibular nerve is respon-
Patients describe it as a sensation similar to that sible for efferent and afferent fibers that control
experienced on a merry-go-round. An inquiry as balance and equilibrium. The cochlear nerve,

CRANIAL NERVES
126
CRANIAL NERVE VIII: VESTIBULAR

Figure 12-1 Vestibular Receptors


A. Membranous labyrinth B. Position within base of skull
Canals
Superior superior Posterior
Vestibular Plane of
semicircular horizontal
ganglion Utricle canal canal and
Vestibular and utricle
cochlear divisions Saccule
of vestibulo-
30˚
cochlear n. Horizontal
semicircular 60˚
canal
Cochlear duct Plane
(scala media) of saccule
Plane of Superior
superior canal
Horizontal
Maculae
Cristae within Posterior
ampullae semicircular 90˚
canal
Posterior
Plane of
C. Section of crista posterior canal
Opposite wall
of ampulla E. Structure and innervation
of hair cells Excitation
Gelatinous cupula Kinocilium
Kinocilium Inhibition
Hair tufts
Hair cells Stereocilia Basal body Stereocilia
Nerve fibers Cuticle Cuticle Basal body
Basement
membrane Hair cell
Hair cell (type I) (type II)
Supporting cells Supporting
D. Section of macula cells
Afferent nerve
Otoconia calyx Efferent nerve
Gelatinous otolithic ending
Efferent nerve
membrane
ending Afferent nerve
Hair tuft
Hair cells calyx
Basement
Supporting cells membrane Myelin sheath
Basement membrane
Nerve fibers Myelin sheath

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CRANIAL NERVE VIII: VESTIBULAR

also called the auditory nerve, carries the effer- artery. The acute postural instability with a pos-
ent and afferent fibers for hearing. The vestibular terior inferior cerebellar artery infarction is usu-
system provides specific sensory input and influ- ally so severe that independent ambulation is
ences motor function in reference to postural not possible. Similarly, patients with multiple
control (Figures 11-1 and 12-1); the latter depends sclerosis with lesions in the brainstem may pre-
on interrelated mechanisms, including percep- sent with acute vertigo and gait dysfunction.
tion of position and motion in relation to gravity In contrast, patients with peripheral vestibular
and orientation of the head and body in relation disorders have preserved ambulation, although
to vertical during quiet stance. Other vestibular they may have feelings of dysequilibrium and be
functions include the selection of appropriate frightened to move as noted in the vignette. If
sensory cues for postural orientation in various
carefully brought into the upright posture, most
sensory environments; this aids in controlling the
of these individuals can ambulate well.
center of gravity when the body is static or mov-
Therefore, for patients presenting with vertigo
ing and stabilizes the head during bodily move-
who concomitantly cannot ambulate indepen-
ments. Because the vestibular system primarily
provides sensory information about the head on dently, and particularly those with vascular risk
the body, the CNS must rely on other sensory factors, brain imaging is mandated to rule out
modalities to determine overall body position cerebellar infarction or multiple sclerosis. Other
and movement. than difficulty walking, there may be no cerebel-
The visual system provides multiple informa- lar or central findings with a posterior inferior
tion modes about head position and movement cerebellar artery infarction. This diagnosis is par-
with respect to the environment, the direction of ticularly important because acute postinfarction
vertical, and low frequency information regard- swelling or hemorrhage within the cerebellar
ing slow or static tilts. Joint position and muscle hemisphere can cause brainstem compression
stretch also contribute to the somatosensory sys- and death (chapters 20 and 62).
tem, which provides information about the rela-
tive alignment of body segments with each other PERIPHERAL NERVOUS SYSTEM
and the support surface. Postural control in- DISORDERS
volves the combination of the complex organi- Acute peripheral vestibular dysfunction
zation of this sensory information, a “central set” causes vertigo by interrupting the normal tonic
based on previous experience and biomechani- discharge of 1 labyrinth. The matched tonic in-
cal constraints. Normally, to maintain proper put of both vestibular end organs is processed
body alignment over the support base, the indi- centrally to mediate head stability. Unilateral re-
vidual generates a motor output via the vestibu- duction in vestibular input is interpreted as turn-
lospinal and corticospinal systems.
ing. In the intact vestibular system, upright head
During the patient’s initial evaluation, it is im-
rotation causes a reduction in horizontal semi-
portant to differentiate a CNS lesion from a pe-
circular canal firing rate on one side, paired with
ripheral localization by determining whether any
an increased firing rate on the other side. With
associated neurologic deficits exist (Figure 12-3).
acute unilateral vestibular loss, the reduced firing
CNS DISORDERS rate simulates the normal response to turning,
Brainstem dysfunction typically includes generating fast phase nystagmus away from the
prominent dysmetria, diplopia, dysphagia, affected ear. The nystagmus is usually more pro-
dysarthria, perioral numbness, or weakness. Of nounced in gaze toward the affected side and re-
patients with risk factors for stroke who present duced in gaze away from the affected side (law
to emergency medical settings with isolated ver- of Alexander). Veering or tilting toward the side of
tigo, nystagmus, and postural instability, 25% lesion may be present, from effects on the
have an inferior cerebellum infarction within the vestibular-spinal, vestibular-ocular, and vestibular-
territory of the posterior inferior cerebellar cerebellar pathways.

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CRANIAL NERVE VIII: VESTIBULAR

Figure 12-2
Causes of Vertigo
(classified by region)

Temporal lobe
Tumor or other
lesion causing
seizures

Medulla oblongata
Infarction caused
by vertebral
artery disease
Multiple sclerosis

Cerebellum
Trauma
Infarction
Hemorrhage

Vestibulocochlear
(VIII) nerve
Neuritis
Acoustic neurinoma

ETIOLOGIC CLASSIFICATION OF fistula, and central pathophysiologic mecha-


PERIPHERAL VESTIBULOPATHIES nisms need to be excluded.
Etiologic classification of peripheral vestibu- Ménière disease, secondary to an imbalance
lopathies is initially based on symptoms and the of the inner ear’s endolymph, is characterized by
presence of hearing loss. When symptoms per- recurrent episodes of vertigo lasting for hours,
sist for days to weeks, and concomitant cochlear associated with hearing loss and tinnitus. Rarely,
symptoms such as hearing loss and tinnitus are infectious processes, including syphilis, Lyme
present, a diagnosis of labyrinthitis is made disease, and HIV, mimic Ménière disease. Simi-
once other causes are excluded. Although larly, certain central processes such as acoustic
labyrinthitis is presumably of viral origin, certain neuroma must be excluded. Studies suggest that
structural pathologic conditions including ero- bilateral Ménière disease may have an autoim-
sive cholesteatoma, temporal bone trauma or mune basis.

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CRANIAL NERVE VIII: VESTIBULAR

Vestibular neuritis is characterized by pro- Chronic vestibulopathies are less likely to


longed vertigo without hearing loss. This is a cra- cause vertigo, because their duration allows for
nial mononeuropathy, limited to the vestibular CNS compensation. Acoustic neuromas and
division of CN-VIII. Diagnosis is often difficult in other slow-growing neoplasms affecting CN-VIII
patients with recurrent true vertiginous episodes may cause unilateral tinnitus, hearing loss, and
lasting hours, without associated cochlear symp- abnormal hypoactive caloric responses on
tomatology. electronystagmogram. However, these tumors
Initially, it is important to exclude verte- rarely present with vertigo.
brobasilar TIAs, particularly in those with vascu- Bilateral vestibular problems do not typically
lar risk factors, including young persons with re- cause vertigo. Because vestibular input is bilater-
cent neck injury predisposing the individual to a ally reduced, there is no sensation of turning.
vertebral artery dissection. However, it is rare for However, disruption of bilateral vestibular input
vertigo to be the sole manifestation of a TIA, em- affects the vestibular-ocular reflex, which stabi-
phasizing the importance of a careful history. lizes visual perception during head motion.
The patient may overlook seemingly less impor- Vestibulotoxic agents, such as aminoglycosides,
tant symptoms that could lead to a diagnosis and alcohol, and heavy metals, can also lead to tran-
may focus on the vertigo. Occasionally, early sient or permanent vestibular damage. Bilateral
Ménière disease is diagnosed in some patients vestibular hypo-function can occur in otherwise
when hearing loss eventually develops. healthy adults (idiopathic) or can result from a
genetic predisposition.
Oscillopsia, or failure to stabilize vision during
TYPES OF VERTIGO head movement, can cause bobbing visual per-
Benign paroxysmal positional vertigo is usu-
ception while walking, with decline in dynamic
ally intense, short-lived, and reliably reproduced visual acuity. Because some patients call this
by positional maneuvers. Typically, patients with “dizziness,” the history differentiates such from
BPPV report rotational vertigo lasting for seconds true vertigo. Oscillopsia is typically seen in pa-
when lying down, arising quickly, bending, look- tients with the Arnold-Chiari syndrome, a devel-
ing up, or reaching or when extending the neck or opmental condition often associated with sy-
lying back at the hairdresser or dentist. BPPV re- ringomyelia and syringobulbia. This symptom
sults from otoconial/statoconium (otolith) debris complex may also occur in patients with other le-
entering the semicircular canals and rendering sions involving the brainstem, particularly at the
them gravitationally sensitive. Normally, the foramen magnum. It may be rarely observed in
canals respond to angular acceleration (turning) patients with multiple sclerosis or those receiv-
by the fluid in the canal transducing inertial mo- ing ototoxic drugs. If it involves only 1 eye, the
mentum by pressing against the crista and stimu- possibility of ocular muscle myokymia is a be-
lating the vestibular neurons. Solid debris acts as a nign consideration.
plunger or weight, disrupting the normal function.
Immediate treatment with particle reposition- DIAGNOSTIC APPROACH
ing maneuvers is effective in 80% to 90% of When one sensory vestibular mechanism is
cases (Figure 12-3). Although most cases of absent, the remaining sensory inputs are used to
BPPV involve the posterior canal, rarely horizon- elicit postural reactions. Other neurologic disor-
tal or anterior canal involvement will cause vari- ders including stroke, Parkinson disease, cere-
ation in the observed nystagmus pattern. Risk bellar pathologic conditions, or peripheral neu-
factors for BPPV include recent head trauma ropathy may affect the potential of the CNS for
(which can be relatively minor), otologic surgery compensation. Careful musculoskeletal evalua-
or disease, habitual unusual positioning such as tion can define the presence of impairments in
is a daily occurrence for plumbers, mechanics, muscle strength, particularly the large truncal
and yoga enthusiasts, or advanced age. Preva- and proximal lower extremity musculature, and
lence studies suggest that 10% of community- in postural alignment, such as scoliosis, kyphosis,
dwelling elderly may have BPPV. or lower extremity contractures.

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Figure 12-3 Canalith Repositioning


(Epley Maneuver)
Right ear
Superior
Head rotated 450 toward
right ear, patient moves
from seated to supine position.

Posterior

Lateral Utricle

Particles

Utricle Posterior
Utricle
Lateral Particles

Superior
Particles
Posterior Superior Lateral

Vertigo is provoked. Head is rotated rostral-


Dix-Hallpike test ceases. caudal. Left ear is down.

Posterior Particles Particles


Superior
Lateral
Lateral
Utricle

Utricle
Superior Posterior

Head and body are rotated


further so head is down.

With left shoulder down,


patient is brought to a
seated position.

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Dynamic posturography is a complex testing The Berg Balance Scale, another useful evalu-
modality providing useful information to define ation modality, uses 14 test-specific items rated 0
the extent to which a patient is able to use visual, to 4 that measure postural control during func-
somatosensory, and vestibular input for postural tionally related tasks. These require anticipatory
control. However, to date, the value of this test is abilities and are performed only while sitting and
still open to question, although it is sometimes a standing. Test score is a good predictor of elderly
useful tool for designing rehabilitation strategies. fall status. Scores less than 45 were associated
The clinical test of sensory interaction and with an increase risk for falls; scores less than 36
balance uses a combination of 3 visual and 2 were associated with a 100% risk of falls.
support surface conditions to clinically measure
a patient’s sensory interaction for postural stabil- TREATMENT
ity. The Romberg test, sharpened Romberg with Rehabilitation
eyes open and eyes closed, and unilateral stand- Many vestibular rehabilitation programs pro-
ing with eyes open and eyes closed are not spe- vide a range of treatment modalities aimed to fa-
cific for postural deficits secondary to vestibular cilitate acute recovery and ongoing compensa-
pathologic conditions. However, patients with tion programs for patients with degrees of
vestibular damage may demonstrate increased residual vestibular deficit. Some are useful for
sway or falling during these tests. acute or chronic vestibular lesions and are
Dynamic tests, such as floor walking with the equally applicable to vertigo, dizziness, and
eyes closed, measure tandem walking for up to dysequilibrium.
10 steps. Persons with acute or chronic vestibu-
lar disorders may fail this test based on estab-
Pharmacologic Therapy
lished age-related norms.
Recent studies suggest that methylpred-
Several performance tests are used to estab-
nisolone significantly improves the recovery of pe-
lish a baseline function analysis and measure
ripheral vestibular neuritis, whereas valacyclovir
outcome in individuals with impairments of sta-
does not. Treatment was begun with 100 mg daily
tic and dynamic postural control. These include
with reduction by 20 mg every third day, eventu-
the Timed Up and Go Test, the Dynamic Gait In-
ally down to 10 mg daily. Vestibular suppressant
dex, and the Berg Balance Scale. medications such as meclizine, scopolamine, and
The Timed Up and Go test measures the time benzodiazepines are useful for relief of acute
required to rise from a standard chair, walk 10 symptoms of vertigo, dizziness, and dysequilib-
feet, return to that chair, and sit. The norm for rium. However, long-term use interferes with cen-
neurologically intact, older adults is 10 to 12 sec- tral vestibular compensation mechanisms.
onds. The results may be a predictor for falls in
community-dwelling elders. There is a maximum Nonpharmacologic Therapy
of 14 seconds for elders at minimal risk for falls Vestibular compensation results from active
and less than 30 seconds for elders who are de- neuronal changes in the cerebellum and the
pendent on assistance for ambulation in the brainstem in response to sensory conflicts cre-
community. There is no threshold established for ated by vestibular pathology. Despite sponta-
patients with vestibular disorders. neous “recovery,” patients still experience dise-
The Dynamic Gait Index measures a patient’s quilibrium, motion-provoked vertigo, or both
ability to modify gait in response to 8 different because the system, inhibited by the cerebellum,
task demands during ambulation. Each task is is unable to respond appropriately to
given a score of 0 to –3, with a maximum of 24 labyrinthine input produced by normal head
points. A mean score of 21 ⫾ 3 is defined for movement.
older adults with no neurologic impairments or Because movement provokes symptoms of
history of imbalance. In contrast, a score of 11 ⫾ dysequilibrium and vertigo, patients with vestibu-
4 is found in older adults with a history of falls but lar disorders may restrict their activity level and
no neurologic disorders. trunk and head movements to avoid these symp-

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CRANIAL NERVE VIII: VESTIBULAR

toms. This provides for greater short-term com- also affect outcomes. Elderly patients often require
pensatory stability but interferes with long-term longer treatment times to reach maximum benefit.
recovery if patients are not challenged to in- Similarly, they generally require more therapist
crease movement to facilitate vestibular com- contact in addition to a home exercise program.
pensation. Educating patients about vestibular Patients with chronic vestibular disorders may also
function encourages and reassures them to find help through the Vestibular Disorders Associ-
safely increase their activity level even though ation, http://www.vestibular.org.
early recovery movement provokes symptoms.
Initially, an assistive device such as a cane or REFERENCES
walker may be recommended. Sensory input Fregley AR, Graybiel A, Smith MJ. Walk on floor eyes closed
through the upper extremity from a cane or light (WOFEC): a new addition to an ataxia test battery. Aero-
space Med. 72;43:395-399.
touch through fingertips can reduce postural
Furman J, Cass S. Benign paroxysmal positional vertigo. N
sway in patients without vestibular function. Engl J Med. 1999;341:1590-1596.
Motor organization exercises also improve Furman J, Whitney S. Central causes of dizziness. Phys Ther.
standing, ambulation, and functional activities 2000;80:179-187.
such as moving at various speeds, changing di- Herdman S. Advances in the treatment of vestibular disor-
ders. Phys Ther. 1997;77:602-618.
rections, and maneuvering around obstacles.
Horak F. Clinical measures of postural control in adults. Phys
These exercises also help to promote normal Ther. 1987;67:1881-1885.
timing and coordination to maintain stability. Horak FB, Jones-Rycewiccz C, Black FO, Shumway-Cook A.
Weekly therapy visits for 4 to 12 weeks help to Effects of vestibular rehabilitation on dizziness and imbal-
monitor the effectiveness of assigned home ex- ance. Otolaryngol Head Neck Surg. 1992;106:175-180.
Hotson J, Baloh R. Acute vestibular syndrome. N Engl J Med.
ercise programs. Treatment success also de-
1998;339:680-685.
pends on the nature of the primary underlying Norrving B, Magnusson M, Holtas S. Isolated acute vertigo in
neurologic dysfunction. Peripheral vestibular dis- the elderly: vestibular or vascular disease? Acta Neurol
orders such as BPPV and stable vestibular hypo- Scand. 1995;91:43-48.
function are most amenable to treatment. In Mathias S, Nayak U, Issacs B. Balance in elderly patients: the
“Get Up & Go” test. Arch Phys Med Rehabil. 1986;67:387-
contrast, individuals with primary CNS disorders
389.
have poorer outcomes. However, many of these Podsialdo D, Richardson S. The timed “Up & Go”: a test of ba-
patients still demonstrate reduced symptomatol- sic functional mobility for frail elderly persons. J Am Geri-
ogy with treatment. atr Soc. 1991;39:142-148.
Other factors influencing treatment effective- Shumway-Cook A, Brauer S, Woollacott M. Predicting the
probability for falls in community-dwelling older adults us-
ness include the degree of initial disability and a
ing the timed up and go test. Phys Ther. 2000;80:896-903.
more recent time of onset. Comorbidities, such as Strupp M, Zingler VC, Arbusow V, et al. Methylprednisolone,
underlying musculoskeletal dysfunction and other valacyclovir, or the combination for vestibular neuritis.
neurologic impairments, and patient compliance N Engl J Med. 2004;351:354-361.

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133
Chapter 13

Cranial Nerve VIII: Auditory


Ellen Choi and Peter J. Catalano

Clinical Vignette
A 68-year-old man presented with sudden onset of unilateral right-sided hearing loss. He stated that this was pre-
ceded by several months of constant ringing in his right ear. He had a history of hypertension and type II diabetes
mellitus that was well controlled by oral hypoglycemic agents. He had no recent head trauma or previous surgeries.
His only medications included atenolol and glyburide. There was no family history of hearing loss. He did not have
a history of excess noise exposure or recent travel. No other otolaryngologic symptoms were reported.
On examination, his tympanic membranes (TMs) were bilaterally normal. The Weber test lateralized the tuning
fork to the side opposite his hearing loss. Rinne test results were normal. The rest of the head and neck examina-
tion findings were unremarkable.
CBC results were normal, and results of a fluorescent treponemal antibody absorption blood test were negative.
Autoimmune screening tests were not ordered.
The patient received a baseline audiogram demonstrating a right ear sensorineural hearing deficit predominantly
in the higher frequencies. Brain MRI failed to demonstrate any mass lesion. Because of its asymmetric distribution,
a brainstem auditory evoked response (BAER) study was performed and revealed a prolonged interaural wave I-III
latency, compatible with a retrocochlear process.

In this vignette, because of the patient’s his- transmitted through the oval window at the foot-
tory of diabetes and the absence of a tumor or plate of the stapes, causing a fluid wave to travel
other more specific lesion, his sudden hearing through the endolymphatic fluid of the cochlea
loss may be attributed to a microvascular infarct of the inner ear. The fluid waves vibrate the or-
of the auditory nerve. However, similar to other gan of Corti’s basilar membrane, thus stimulating
cardiovascular processes, there is no difference inner and outer hair cells. Hair cells, receptors of
in the incidence of hearing loss between patients the sensorineural system, transmit action poten-
with and without diabetes; therefore, the etiol- tials to the bipolar neurons, the bodies of which
ogy in this case is unknown. are in the spiral ganglion.
Afferent fibers projecting toward the CNS
ANATOMY comprise the auditory nerve (Figure 13-2). They
The vestibulocochlear nerve, CN-VIII, is actu- travel to the dorsal and ventral cochlear nuclei
ally composed of 2 nerves: the vestibular and located in the caudolateral part of the pons.
cochlear nerves. The vestibular nerve is respon- Most of the secondary neurons project con-
sible for efferent and afferent fibers that control tralaterally over the midline to the superior oli-
balance and equilibrium (chapter 12). The vary nucleus and then travel up the lateral lem-
cochlear nerve, also called the auditory nerve, niscus into the inferior colliculus of the midbrain.
carries the efferent and afferent fibers for hear- Decussating fibers from the cochlear nucleus to
ing. To understand dysfunction of the auditory the superior olivary nucleus are located in the
nerve, a brief description of the human hearing trapezoid bodies and also in the base of the
mechanism is required. pons. Fibers from the inferior colliculus continue
Sound waves travel through the external audi- to travel rostrally to the medial geniculate body
tory canal and vibrate the TM, which produces of the thalamus and then terminate at the audi-
motion of the middle ear ossicles (incus, malleus, tory cortex, located in the transverse temporal
and stapes) (Figure 13-1). The vibrations are gyri of Heschl.

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CRANIAL NERVE VIII: AUDITORY

Figure 13-1 Pathway of Sound Reception


Frontal section
Facial nerve (VII) (cut)

Limbs of stapes Base of stapes in oval (vestibular) window


Prominence of lateral semicircular canal Vestibule
Incus Semicircular ducts, ampullae, utricle,
and saccule
Tegmen tympani
Facial nerve (VII) (cut)
Malleus (head)
Vestibular nerve
Epitympanic recess
Cochlear nerve
Auricle
Internal acoustic
meatus

Vestibulocochlear
nerve (VIII)

External
acoustic meatus Helicotrema Nasopharynx
Tympanic membrane Scala vestibuli
Tympanic cavity Cochlear duct
Cochlea
containing
Promontory spiral organ
(Corti)
Round (cochlear) window
Scala tympani
Pharyngotympanic (auditory) tube

Note: Arrows indicate course of sound waves.

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CRANIAL NERVE VIII: AUDITORY

Figure 13-2 Afferent Auditory Pathways

Acoustic area of
temporal lobe cortex

Medial geniculate body

Brachium of
inferior colliculus

Inferior colliculus

Midbrain

Correspondence
between cochlea
and acoustic area
of cortex:
Low tones
Middle tones
Lateral lemnisci
High tones
Nuclei of
lateral
Medulla oblongata
lemnisci

Superior olivary
complex
Dorsal cochlear nucleus
Inferior cerebellar peduncle
Ventral cochlear nucleus
Cochlear division of
vestibulocochlear nerve

Dorsal
acoustic
Intermediate stria
acoustic stria Reticular
formation Inner Outer

Trapezoid body (ventral acoustic stria) Spiral ganglion Hair cells

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CRANIAL NERVE VIII: AUDITORY

CLINICAL PRESENTATION must be determined. Only a few processes, such


History as otosclerosis and otitic meningitis, involve
Auditory nerve dysfunction usually results in both areas. Typically, tinnitus and vertigo are in-
tinnitus, sensorineural hearing loss (SNHL), or ner ear symptoms and indicate involvement of
both. SNHL is hearing deficit from dysfunction of the cochlea, vestibular labyrinth, or auditory
the cochlea (sensory), the auditory nerve nerve or a combination of these structures.
(neural), or any part of the central auditory path- Hearing loss associated with otalgia, otorrhea,
way. Hearing loss can result from pathologic con- headache, and aural fullness is most likely in-
ditions anywhere along the anatomic pathway flammatory and can be confirmed on physical
for hearing. It may involve the auditory nerve (eg, examination. Concomitant tinnitus, vertigo, or
acoustic tumors), or the auditory nerve may be both suggest the ominous extension of the in-
uninvolved (serous otitis media). A targeted his- flammatory process to the inner ear or beyond.
tory and physical examination narrow the diag- In this setting, a formal audiogram is indicated to
nosis. The temporal profile of symptom onset (ie, determine whether the perceived hearing loss is
sudden, progressive, fluctuating, or stable) is crit- secondary to a middle ear effusion or an addi-
ical. tional sensorineural component. The latter is an
Tinnitus often presents concomitant with otolaryngologic emergency.
SNHL but can be the only presenting symptom. With both ototoxicity and Ménière disease,
Tinnitus is classified into 2 subgroups. Subjective concomitant vestibular symptoms, tinnitus, aural
tinnitus, the most common, is heard solely by the fullness, or a combination of these symptoms
patient. It can range from soft fluctuating ringing may accompany hearing loss. In conditions such
noise to loud constant roaring that can be debili- as presbycusis and noise-induced hearing loss,
tating. The cause is usually unknown; however, vestibular symptoms are less likely to be part of
subjective tinnitus is associated with exposure to the presentation.
loud noise, ototoxic drugs (such as aspirin, cis- Neurologic or ophthalmologic complaints,
platinum, and aminoglycosides), acoustic tu- or both, sometimes accompany primary oto-
mors, Ménière disease, and cochlear otosclero- logic symptoms. These occur with diseases
sis. Objective tinnitus is heard by the patient and such as multiple sclerosis (MS) or expanding
the examiner and is usually not a sign of auditory neoplastic lesions that may lead to combined
nerve dysfunction. Middle ear effusion, as in facial nerve, trigeminal nerve, or ophthalmo-
serous otitis media, can magnify vascular pulsa- logic symptoms.
tions from the nearby internal carotid artery and Systemic diseases can contribute to hearing
produce vascular tinnitus. Pulsatile tinnitus is usu- loss and tinnitus. Ototoxic drugs (aminoglyco-
ally secondary to vascular causes, such as arteri- sides, salicylates, or loop diuretics) are important
ovenous malformations or glomus tumors. Click- causes of SNHL, tinnitus, and vestibular symp-
ing tinnitus is secondary to temporomandibular toms. Trauma to the temporal bone area, result-
joint disease, palatal myoclonus, or spontaneous ing in labyrinth or auditory nerve injury, can cre-
contraction of the middle ear muscles. ate auditory nerve dysfunction. Diving and flying
Laterality of hearing loss is essential in the as- may cause barotrauma, leading to rupture of the
sessment. Bilateral hearing loss occurs in cochlear membranes with subsequent hearing
processes such as ototoxicity, noise exposure, loss. Occupational and recreational noise expo-
and presbycusis (hearing loss related to aging). sure damages the cochlea’s outer hair cells, cre-
Unilateral hearing loss raises the concern of neo- ating high-frequency hearing loss. It is also im-
plastic, vascular, neurologic, or infectious etiolo- portant to establish whether a family history of
gies. Fluctuation of hearing is seen in Ménière hearing loss exists because this can be an impor-
disease, whereas progressive or sudden hearing tant mechanism.
loss occurs with acoustic neuromas and viral
neuritis, respectively. Physical Examination
Whether the hearing loss involves a process in Cerumen impaction or foreign bodies are eas-
the external or middle ear versus the inner ear ily identified on inspection of the external audi-

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137
CRANIAL NERVE VIII: AUDITORY

tory canal. Pneumatic otoscopy is used to assess A basic audiogram with pure tones and
quality (color, lucency, and mobility) of the TM speech discrimination evaluation determines the
and defines conductive hearing loss as a reason type and amount of hearing loss. (Serous effu-
for hearing deficit. Decreased TM mobility can sion does not exclude a sensorineural compo-
be attributed to ossicular fixation, such as oto- nent to the hearing loss.) Unilateral decrease or
sclerosis, or middle ear effusion, as in otitis me- asymmetry in speech discrimination, high-
dia. Middle ear or expanding jugular foramen tu- frequency hearing loss, or acoustic reflex abnor-
mors can present as a mass behind the TM and malities suggest a retrocochlear lesion, warrant-
can cause conductive hearing loss. ing further testing.
Tuning fork tests assess whether the hearing Gadolinium-enhanced MRI is specifically indi-
loss is conductive or sensorineural (Figure 13-3) cated when history, symptoms, and audiometric
(chapter 1). During the head and neck examina- tests strongly suggest retrocochlear disease. MRI
tion, a complete cranial nerve examination must is the diagnostic “gold standard” for tumors
also be performed to assess other potential cra- causing hearing loss. For patients presenting
nial nerve abnormalities. Facial nerve weakness with asymmetric hearing loss, especially if sud-
may be attributed to viral infections, such as her- den, MRI is warranted to exclude acoustic neu-
pes zoster oticus, or expanding neoplasms in the romas or other cerebellopontine tumors. MRI
internal auditory canal or cerebello-pontine an- with gadolinium can detect 2- to 3-mm tumors
gle, such as meningiomas or facial neuromas. within the temporal bone. It can also detect vas-
Auscultation of the areas around the orbit and cular disease and the lesions of MS.
ear may detect pulsatile tinnitus. Brainstem auditory evoked response is a useful
objective and quantitative test when a retro-
cochlear deficit is suspected. It can suggest the
Etiologies of SNHL
site-of-lesion from the cochlea to the inferior col-
Ototoxic drugs, excess noise exposure, and
liculus in the pons. BAER studies were initially
autoimmune diseases affect the cochlea, the pri-
considered highly sensitive for retrocochlear
mary sensory organ of hearing, by damaging the
causes; however, as with most tests, false-nega-
hair cells within it. The quality of the hearing loss
tive and false-positive results are possible. The
is usually described as decreased sensitivity to
BAER uses electrodes attached to the patient’s
pure tones with preserved speech discrimina-
head and clicking sounds emitted through ear-
tion.
phones. The sounds elicit action potentials
Hearing loss caused by retrocochlear lesions
through the peripheral and central auditory path-
of the nerve fibers of CN-VIII or its central audi-
ways, and the EEG activity is measured and aver-
tory projections begins as decreased speech dis-
aged by a computer. Right and left ear waveform
crimination with relatively normal pure tone sen-
morphologic appearance and latencies are com-
sitivity. Decreased speech discrimination is not
pared. Interaural differences suggest pathologic
exclusive to retrocochlear lesions; it is also ob-
conditions. Five wave peaks characterize the
served with extensive hair cell damage.
BAER, corresponding to specific anatomic points
within the auditory pathway: I, CN-VIII action po-
DIAGNOSTIC APPROACH tential; II, cochlear nucleus; III, olivary complex;
Standard laboratory blood tests are not rou- IV, lateral lemniscus; and V, inferior colliculus. A
tinely obtained for hearing loss, unless a particu- change in peak morphologic appearance and la-
lar cause is suspected. Usually, the fluorescent tency helps localize the pathologic condition.
treponemal antibody absorption blood test or
the microhemagglutination test for Treponema DIFFERENTIAL DIAGNOSIS
pallidum or both should be ordered, because Usually idiopathic, sudden SNHL is generally
syphilis is often asymptomatic and is a treatable defined as loss that develops in 12 hours or less.
cause of SNHL. Routine tests for other systemic However, a broad differential includes Ménière
diseases are not useful unless prompted by the disease, neoplasms, vascular disorders, viral in-
history and physical examination. fections, MS, and rarely, hematologic disorders.

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CRANIAL NERVE VIII: AUDITORY

Figure 13-3 Hearing Test: Weber and Rinne

Weber Test

Tone referred Tone referred


to poorer ear Poorer Better
ear to better ear
indicates ear indicated
conductive perceptive
impairment. impairment.

Rinne Test
Stage 1
Stage 2

Tone heard longer by air conduction = Rinne positive: indicates perceptive loss
Tone heard longer by bone conduction = Rinne negative: indicates conductive loss

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139
CRANIAL NERVE VIII: AUDITORY

Figure 13-4 patients during the disease. Progressive hearing


Vestibular Schwannoma loss generally results from stretching or com-
pression of the cochlear nerve as the tumor
grows. In contrast, when the hearing loss is pre-
cipitous, it is thought to be secondary to occlu-
sion of the internal auditory artery supplying the
cochlea. Tinnitus with acoustic neuromas is typi-
cally high pitched, continuous, and unilateral.
Paradoxically, vestibular symptoms are not seen
frequently with vestibular nerve Schwannomas
because as these lesions grow, the contralateral
vestibular system adjusts to the imbalance, pre-
venting any prolonged vestibular symptoms.
Larger tumors occasionally lead to facial or
trigeminal nerve symptoms, such as facial paral-
ysis or paresthesias, respectively.
Before MRI, BAERs were the diagnostic test of
choice for acoustic neuromas, with a sensitivity
Axial T1-weighted, post-gadolinium-enhanced fat-satu-
rated image shows an enhancing mass widening the me- of 93% to 98%; but, the sensitivity is significantly
dial left internal auditory canal and extending into the CP lower with tumors less than 1 cm (58%). With
angle with distortion of the pons. MRI, smaller tumors are detected in patients
The right side is normal (arrowhead).
who have had normal BAERs.

Ménière Disease Vascular Etiologies


Ménière disease is an idiopathic process char- Vertebrobasilar stroke is another cause of sud-
acterized by episodic vertigo, fluctuating SNHL, den, unilateral SNHL. This mechanism has po-
tinnitus, aural fullness, or a combination of these tential devastating effects. Distinguishing
symptoms. Vestibular symptoms are usually the whether hearing loss results from microvascular
main complaint. Patients with Ménière disease disease or a brainstem infarct is vital. The ante-
likely constitute less than 5% of all patients with rior inferior cerebellar artery supplies blood to
SNHL. However, a condition called cochlear the inferolateral portion of the pons, CN-VII, the
Ménière produces only SNHL and is considered spinal trigeminal tract, and the inferior cerebel-
a “diagnosis of exclusion” because there are no lum. A stroke from occlusion of this artery causes
specific tests for it. an infarct of the ipsilateral pons, creating a myr-
iad of symptoms: ipsilateral hearing loss, vestibu-
lar symptoms, gait ataxia, facial paralysis, and
Neoplasms contralateral loss of pain and temperature sensa-
In any case of sudden, unilateral hearing loss, tion in the extremities and the ipsilateral face.
neoplastic lesions, although rare, belong in the CT is the initial imaging study of choice to ex-
differential until excluded by diagnostic and ra- clude a hemorrhagic infarct, particularly within
diologic testing. Acoustic neuromas are benign the cerebellum and brainstem. MRI provides
tumors arising from the Schwann cells of CN-VIII better definition when available.
and account for 6% of all intracranial tumors. Unilateral hearing loss also occurs secondary
These occur on the vestibular portion of CN-VIII, to occlusion of the cochlear blood supply, includ-
involving its adjacent cochlear division by com- ing the internal auditory artery, a terminal branch
pression against the bony walls of the internal au- of the anterior inferior cerebellar artery, or the
ditory canal. Less commonly, neuromas can also basilar artery. Occlusion usually occurs sec-
arise directly from the cochlear nerve. ondary to compression by an acoustic neuroma
Hearing loss is the most commonly reported in the internal auditory canal, but a thrombotic or
symptom, occurring in approximately 95% of embolic event can also occlude the artery.

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CRANIAL NERVE VIII: AUDITORY

Microvascular disease may also cause sud- tion of the disease, begins at higher frequencies
den, unilateral hearing loss and, depending on and can progress to bilateral cochlear and
the patient’s history, can be attributed to differ- vestibular dysfunction. The exact causal mecha-
ent systemic diseases. Atherosclerosis and hy- nism is unknown; however, proposed theories
pertension may cause microvascular disease, include microvascular disease, direct spirochetal
causing hearing loss. Patients with diabetes have infiltration of the perilymph, and temporal bone
an increased incidence of SNHL. The link be- osteitis. Diagnostic tests for syphilis, a treatable
tween diabetes and hearing loss makes intuitive cause of SNHL, include the fluorescent trepone-
sense, because neuropathic and microvascular mal antibody absorption blood test.
disease processes exist in persons with diabetes.
Hematologic Disorders
Multiple Sclerosis
Leukemia, sickle cell anemia, polycythemia,
Patients with MS present with a variety of neu-
and macroglobulinemia can also cause sudden
rologic symptoms, depending on the location of
SNHL, usually from sludging, hemorrhage into
the lesions. SNHL appears as a retrocochlear
the inner ear, or microthrombi. CBC can exclude
manifestation in approximately 4% to 10% of pa-
hematologic etiology.
tients with MS. However, it is rare for SNHL to be
the initial presentation. Usually, the hearing loss
Presbycusis
is sudden and resolves in weeks with treatment.
The most common cause of slowly progres-
If MS is suspected, CSF evaluation may be help-
sive, bilateral, symmetric, high-frequency hear-
ful; increased IgG index and oligoclonal bands in
ing loss that develops with increasing age, pres-
gel electrophoresis suggest MS. Audiometric
bycusis originates from a pathologic condition
testing can show normal or decreased speech
that decreases the number of hair cells within the
discrimination in proportion with pure tone
organ of Corti. It has an almost universal inci-
threshold. MRI is the radiologic study of choice
dence in the elderly. Multiple factors determine
because periventricular white matter lesions on
its progression rate. Three of the most common
the inferior colliculus or cochlear nucleus are vis-
are genetic predisposition, nerve toxins, particu-
ible on T2-weighted images.
larly medications, and history of long exposure
to loud noises.
Infections
Various viral and bacterial infections can TREATMENT
cause sudden hearing loss. Herpes zoster oticus When the primary causal mechanism for hear-
usually affects the facial nerve, creating herpetic ing loss is identified, such as an acoustic neu-
skin eruption around the auricle and in the ex- roma or syphilis, therapy is straightforward and
ternal auditory canal, and a facial palsy. Measles potentially remediable depending on when the
and mumps previously led to hearing loss, al- lesion is diagnosed in the illness course. In vas-
though vaccination for these childhood viruses cular lesions with infarction of the auditory
has eliminated them in economically privileged nerve, there is no treatment. However, for com-
countries. mon disorders such as presbycusis, a variety of
Nonspecific viral processes, often following a high-technology, hearing-enhancing modalities
flulike illness, are considered a common cause of can be designed to meet the patient’s needs,
sudden deafness, especially when no vestibular with the aid of an otologist.
symptoms coexist. The mechanism of action of
the presumed viral illness is unknown; therefore, REFERENCES
this etiology is considered a “diagnosis of exclu- Cummings CW, Haughey B. Otolaryngology: Head and Neck
sion.” Surgery. 3rd ed. St Louis, Mo: Mosby-Year Book Inc; 1999.
Duck SW, Prazma J, Bennett PS, Pillsbury HC. Interaction be-
Otosyphilis is defined as a positive syphilis tween hypertension and diabetes mellitus in the patho-
serologic result in the setting of unexplained genesis of sensorineural hearing loss. Laryngoscope.
SNHL. The hearing loss, usually a late manifesta- 1997;107:1596-1605.

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CRANIAL NERVE VIII: AUDITORY

Fletcher SD, Cheung SW. Syphilis and otolaryngology. Oto- Schmidt RJ, Sataloff RT, Newman J, Spiegel JR, Myers DL. The
laryngol Clin N Am. 2003;36:595-605. sensitivity of auditory brainstem response testing for the
Ho SY, Kveton JF. Acoustic neuroma: assessment and man- diagnosis of acoustic neuromas. Arch Otolaryngol Head
agement. Otolaryngol Clin N Am. 2002;35:393-404. Neck Surg. 2001;127:19-22.
Rudick RA. Multiple sclerosis and demyelinating conditions Sismanis A. Pulsatile tinnitus. Otolaryngol Clin N Am.
of the central nervous system. In: Goldman L, Ausiello D, 2003;36:389-402.
eds. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Swartz MH. Textbook of Physical Diagnosis: History and Exam-
Pa: WB Saunders Co; 2004:2320-2327. ination. 4th ed. Philadelphia, Pa: WB Saunders Co; 2002.

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Chapter 14

Cranial Nerve IX,


Glossopharyngeal, and Cranial
Nerve X, Vagus: Swallowing
Eva M. Michalakis, Allison Gudis Jackson, and Peter J. Catalano

Clinical Vignette
A 70-year-old man with a history of hypertension and paroxysmal atrial fibrillation presented to the emergency
department with acute onset of slurred speech, left-sided paresis, left neglect, and a left inferior quadrant anopsia.
MRI revealed a right middle cerebral artery stroke. An initial oral peripheral and cranial nerve examination revealed
bilateral depressed gag reflex and diminished soft palate elevation, left central facial weakness, reduced labial re-
traction, and tongue deviation to the right side with protrusion, ie, weakness of the left tongue movement . The pa-
tient was to receive nothing per mouth (NPO) and was referred to a speech pathologist.
Clinical swallowing evaluation demonstrated hoarse and moderately dysarthric speech that retained fair intelli-
gibility. Graduated sized boluses of thin liquids, nectar thick liquids, and purees were administered. The patient had
significant difficulty with oral containment, bolus formation, and posterior transport though the oral cavity, with left
side greater than right. Posterior placement on the right side facilitated oral swallowing. He also had a reduced oro-
labial seal that benefited from a straw. Attempts to administer a soft solid bolus were unsuccessful. Because of the
severity of oral deficits, he was not given solids; he had delayed triggering of oropharyngeal swallow.
There were mild vocal changes after liquids, characterized by a wet vocal quality, indicative of laryngeal pene-
tration and possible silent aspiration. The patient was able to clear with cues to use a throat clear/re-swallow strat-
egy. Use of a chin tuck swallowing posture with nectar thick liquids eliminated clinical signs of aspiration.
He was placed on a modified pureed diet with nectar thick liquids, and medications were crushed in applesauce.
Aspiration precautions included remaining upright 90° during and 45 minutes after all oral intake, with single, small
boluses. Swallowing strategies included a chin tuck swallowing posture and right posterior placement of food in
the oral cavity to decrease anterior leakage and assist in oral transport. The patient was referred for a flexible en-
doscopic evaluation of swallowing with sensory testing, an objective assessment of swallowing function, to further
assess dysphagia, exclude silent aspiration, determine appropriate diet modifications, and identify additional inter-
vention strategies. The testing demonstrated bilateral vocal cord movement, right ⬎ left. Sensory testing revealed
severe left laryngopharyngeal sensory deficit. Accordingly, and because of the silent aspiration, the patient re-
mained at risk for aspiration pneumonia.

Swallowing functions are important to sur- larly Guillain-Barré syndrome), neuromuscular


vival; malfunctioning prevents adequate nutri- junction dysfunction (especially myasthenia
tion intake and may predispose to significant as- gravis), and myopathies such as oculopharyn-
piration with a risk for potentially fatal geal or myotonic dystrophy and dermatomyosi-
pneumonia. Dysphagia can be the presenting tis can also compromise swallowing function.
sign of several nervous system disorders that
negatively affect swallowing. PHYSIOLOGY
Dysphagia may occur with CNS disorders in- Swallowing is a complex process involving
cluding acute cerebral infarction, brainstem motor control with sensory feedback from many
strokes, Parkinson disease, and bulbar disorders anatomic structures within the oral cavity, phar-
such as multiple sclerosis, motor neuron disease, ynx, larynx, and esophagus (Figure 14-1). The
syringobulbia, or primary pontomedullary or trigeminal (CN-V), facial (CN-VII), glossopharyn-
meningeal tumors. Similarly, peripheral disor- geal (CN-IX), vagus (CN-X), and hypoglossal
ders including peripheral nerve lesions (particu- (CN-XII) cranial nerves are involved. “Normal

CRANIAL NERVES
143
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

Figure 14-1 Neuroregulation of Deglutition

V to tensor veli palatini muscle

X (XI) to levator veli palatini muscle

Pharyngeal plexus
V from soft palate

V from tongue (lingual nerve)


V to mylohyoid and ant. belly of digastric
IX from soft palate, fauces, pharynx

(Mylohyoid IX to stylopharyngeus
nerve) X From pharynx, larynx, upper esophagus
From lower esophagus and GI tract

X To muscles of pharynx, larynx, upper esophagus


To muscle of lower esophagus and GI tract

XII to muscles of tongue and geniohyoid


Ansa hypoglossi to infrahyoid muscles
Sympathetic efferents
Recurrent Afferents
laryngeal Sympathetic efferents
nerve
Afferents

Soft palate (slight)

Pharyngeal wall

Anterior pillar
Tonsil
Posterior pillar

Posterior part
of tongue
Sympathetic efferents
thoracic greater splanchnic nerve

Afferents
Areas from which deglutition
reflex may be excited (stippled)

Celiac ganglion

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144
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

Figure 14-1 Neuroregulation of Deglutition (continued)

Thalamus

Hypothalamus

V
Principal sensory nucleus of V
Motor nucleus of V

VII
IX Deglutition center

Nucleus of XII
X
XI
Dorsal nucleus of X
(motor and sensory)
XII
Nucleus of solitary tract
Stellate C1
ganglion and Nucleus ambiguus
2

T4
Thoracic
sympathetic
ganglionic Dorsal root
chain ganglion
T5

Key
T6
Sympathetic efferents

Parasympathetic efferents
T7 Somatic efferents

Afferents (and CNS connections)

Indefinite paths
T8

T9

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CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

swallow” comprises 2 major components, bolus provide critical sensory feedback from the larynx
transport and airway protection. The swallowing and esophageal inlet.
process is typically classifed into 4 phases. The pharyngeal phase begins with the bolus
The oral preparatory phase is voluntary motor passing into the throat, triggering the swallowing
function during which food or liquid is taken into reflex and causing several pharyngeal physio-
the mouth, masticated (CN-V), and mixed with logic actions to occur simultaneously, allowing
saliva to form a cohesive bolus (Figure 14-2). This food to pass into the esophagus. Once pharyn-
phase requires coordination of lip closure and geal swallowing is elicited, essential functions of
labial seal (CN-VII), tension in the labial and buc- airway protection occur. Intrinsic laryngeal mus-
cal musculature closing the anterior and lateral cles innervated by CN-X close the larynx at the
sulci (CN-VII), rotary mandible motion for chew- aryepiglottic, false vocal, and true vocal folds,
ing (CN-V3), lateral rolling tongue motion (CN- creating a seal to separate the airway from the di-
XII) to position food, and bulging of the soft gestive tract. The laryngeal vestibule is then pro-
palate forward to seal the oral cavity posteriorly tected from foreign material aspiration. The
while widening the nasal airway to prepare food tongue (CN XII) is the major force pushing the
for the swallow (CN-IX). Tongue mobility is the bolus through the pharynx. Synergistic actions
most important neuromuscular function in- with CN-X produce pharyngeal peristalsis as it in-
volved in this first phase. The mid and lower divi- nervates the pharyngeal constrictors and carries
sions of CN-V provide sensory feedback for po- afferents from the lower pharynx.
sitioning the bolus. Saliva derived from the CN-IX mediates the sensory portion of the pha-
parotid, sublingual, and submandibular glands, ryngeal gag but innervates just 1 muscle, the sty-
innervated by secretomotor fibers of CN-IX and lopharyngeus. The absence of the gag reflex is not
-VII, contain digestive enzymes that act as an the sole indicator of a patient’s swallowing abili-
emollient to soften the bolus. ties. A study of the risk of aspiration in 14 patients
The oral phase of swallowing is initiated when with dysphagia with absent gag reflex demon-
the tongue (CN-XII) sequentially squeezes the strated that 86% tolerated a modified diet. Addi-
bolus posteriorly against the hard palate, begin- tionally, the gag reflex was absent in 13% of 69
ning propulsion into the oropharynx. Lips and nondysphagic control group individuals.
buccal muscles contract (CN-V and CN-VII) with Poor airway protection and delayed triggering
elevation of the velum (CN-V and CN-X) provid- of pharyngeal swallow may cause aspiration.
ing the valving process that generates pressure When swallowing is inefficient and aspiration oc-
to seal the nasopharynx, preventing reflux and curs, a reflexive cough needs to occur as respira-
nasal regurgitation. CN-V is responsible for the tory defense against foreign matter. The cough
afferent (sensory) feedback for the entire oral reflex is induced by irritation of afferent CN-IX
cavity and tongue. The soft palate (CN-IX) critical and CN-X sensory fibers in the larynx, trachea,
to containing the bolus within the oral cavity dur- and larger bronchi (Figure 14-3). If a reflexive
ing the oral preparatory phase, moves posteri- cough is not elicited in response to foreign ma-
orly, allowing the bolus to pass through the fau- terial within the airway, silent aspiration results;
cial arches and simultaneously preventing the it is radiographically documented in 50% of as-
bolus from entering the nasopharynx. The swal- piration cases.
lowing reflex is triggered as the bolus passes the CN-IX is the primary afferent of the swallowing
anterior tonsillar pillars, which initiates the pha- response, whereas CN-X is the secondary affer-
ryngeal phase. ent; both nerves terminate in the swallowing
Taste for the anterior two thirds of the tongue center located in the medulla within the nucleus
is carried by CN-VII, whereas the afferent CN-IX solitarius. Sensory events initiating swallowing
controls taste for the posterior one third of the occur with stimulation to jaw, posterior tongue,
tongue and the posterior pharyngeal wall. CN-X faucial pillars, and upper pharynx and are medi-
supplies primary innervation to the palatal mus- ated through CN-V, CN-IX, and CN-X. These af-
cles, pharyngeal constrictors, laryngeal muscula- ferent fibers converge on the nucleus solitarius
ture, and cricopharyngeus. Afferent fibers also in the medulla and communicate with the nu-

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146
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

cleus ambiguous via interneurons stimulating pneumonia. Concomitant risk factors include
the motor response. chronic obstructive pulmonary disorders, con-
The esophageal phase occurs with the pas- gestive heart failure, feeding tubes, dependence
sage of the bolus through the cricopharyngeal for oral care and feeding, medications, de-
sphincter, moving over the closed airway and creased laryngopharyngeal sensation, and a re-
passing the pharyngoesophageal segment into duced level of alertness. Any patient with aspira-
the esophagus via the cricopharyngeal sphincter tion risk needs to be placed on aspiration
at the proximal esophagus (Figure 14-2). This precautions and referred for swallowing evalua-
area contains the cricopharyngeus muscle that tion before initiation of oral intake.
normally keeps the esophagus closed. CN-X me-
diates the action of the cricopharyngeus, which DIAGNOSTIC APPROACH
relaxes to allow food to pass from the hypophar- Formal assessment of swallowing function
ynx into the esophagus. through various examinations evaluates the
Elevation and anterior movement of the larynx severity of dysfunction and identifies therapeutic
is the significant mechanical force contributing strategies and intervention. Clinical swallowing
to the opening of the cricopharyngeal sphincter, evaluation includes the assessment of patient
which in conjunction with the relaxation of the history combined with observations regarding
cricopharyngeus muscle opens the pharyngoe- mental and respiratory status. Oral, peripheral,
sophageal segment, permitting the passage of and cranial nerve examinations are conducted.
food into the esophagus. The sphincter must oth- Various food consistencies are administered
erwise remain closed to prevent the entrance of with close evaluation of the oral preparatory,
air into the stomach and reflux from the esopha- oral, and pharyngeal phases of swallowing.
gus into the hypopharynx. CN-X, specifically the Based on this evaluation, swallowing strategies
efferent fibers from the dorsal nucleus, innervate may be implemented or the need for objective
the involuntary muscles of the esophagus, stom- studies may be identified.
ach, small intestine, and portions of the large in- Flexible endoscopic evaluation of swallowing
testine. with sensory testing allows direct evaluation of
motor and sensory aspects of the pharyngeal
CLINICAL PRESENTATION swallow. It requires transnasal passage of a
Dysphagia, or difficulty swallowing, can result fiberoptic laryngoscope into the hypopharynx to
from many causes, including neurologic disor- view the larynx and surrounding structures. La-
ders; viral, bacterial, or fungal infections of the ryngeal airway protection and the integrity of
upper airway; surgeries or disease processes the oropharyngeal swallow are assessed.
that do not directly involve the oral, pharyngeal, A comprehensive swallowing evaluation is
or laryngeal structures; and psychogenic mecha- then performed. The patient is given various food
nisms. Aging and medications may exacerbate consistencies tinted with food coloring to en-
dysphagia. Some antidepressant medications hance visualization. Similar to the modified bar-
cause xerostomia (reduced salivary flow), affect- ium swallow (MBS), compensatory strategies and
ing bolus formation. Medications causing postures are attempted when indicated to facili-
drowsiness or lethargy may significantly affect tate improved swallowing function and decrease
swallowing. Common signs of dysphagia include the risk of aspiration pneumonia. Velopharyngeal
pocketing of food in the oral cavity, drooling, closure, anatomy of the base of the tongue and
wet vocal quality during meals, episodes of hypopharynx, abduction and adduction of the vo-
coughing with throat clearing, and shortness of cal folds, pharyngeal musculature, and the pa-
breath during meals. tient’s ability to manage secretions are assessed.
Aspiration, the primary concern when dealing Laryngopharyngeal reflux can also be visualized.
with patients with dysphagia, is technically de- Modified barium swallow, also called videoflu-
fined as entrance of a foreign substance below oroscopy or videopharyngogram, is a functional
the level of the vocal cords, into the trachea. As- evaluation requiring active patient participation.
piration is a dangerous precursor of aspiration Before scheduling MBS, laryngopharyngeal sen-

CRANIAL NERVES
147
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

Figure 14-2 Deglutition

Bolus Bolus

1. Tip of tongue in contact with


anterior part of palate. Bolus
is pushed backward in groove Transverse Genioglossus
between tongue and palate. intrinsic musculature muscles
Soft palate is being drawn of tongue
upward. Bulge has begun to 2. Bolus lying in groove on lingual
form in upper part of posterior dorsum formed by contraction of
pharyngeal wall (Passavant ridge) genioglossus and transverse
and approaches rising soft palate. intrinsic musculature of tongue.

Soft palate

Root of
tongue
Vallecula
Epiglottis
turned down
(sectioned)
Thyroid cartilage uum of bo
Resid lus
Aryepiglottic fold
Ventricular fold
Bolus Ventricle of larynx
Vocal fold
Cricoid cartilage

6. Soft palate has been pulled


down and approximated to root
of tongue by contraction of
pharyngopalatine muscles
(posterior pillars), and by pressure
of descending “stripping wave.” 7. Laryngeal vestibule
Oropharyngeal cavity closed by is closed by
contraction of upper pharyngeal approximation of
constrictors. Cricopharyngeus aryepiglottic and
muscle is relaxing to permit entry ventricular folds,
of bolus into esophagus. Trickle of preventing entry of
food enters also laryngeal aditus food into larynx
but is prevented from going farther (coronal section:
by closure of ventricular folds. AP view).

CRANIAL NERVES
148
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

Figure 14-2 Deglutition (continued)


Soft
palate
Root of
tongue
Bolus

Bolus Epiglottis
turned
down
Bolus Laryngeal
aditus

3. Gradually pressing 4. Bolus has


more of its dorsal reached
surface against hard vallecula. 5. Epiglottis is tipped down
palate, tongue pushes Hyoid bone over laryngeal aditus
bolus backward into oral and larynx move but not completely closing
pharynx. Soft palate is upward and it. Bolus flows in two
drawn upward to make forward. Epiglottis is streams around each side of
contact with Passavant ridge, tipped downward. ”Stripping wave” epiglottis to piriform fossae.
closing off nasopharynx. Receptive space in on posterior pharyngeal wall Streams will then unite to
oral pharynx forms by slight forward moves downward. enter esophagus. Trickle of
movement of root of tongue. Contraction of food may enter laryngeal
stylopharyngeus and upper pharyngeal aditus (viewed from behind).
constriction muscles draws pharyngeal wall
upward over bolus.

8. “Stripping wave”
has reached vallecula
and is pressing out 9. ”Stripping 10. All
last of bolus therefrom. wave” has structures
Cricopharyngeus passed pharynx. of pharynx
muscle has relaxed and Epiglottis is have
bolus has largely beginning to turn returned to
passed into esophagus. up again as hyoid resting
bone and larynx descend. position
Communication with nasopharynx as “stripping wave” passes on
has been reestablished. down into esophagus, pushing
bolus before it.

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149
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

Figure 14-3A Glossopharyngeal Nerve (IX): Schema


Efferent fibers Spinal tract and spinal nucleus of trigeminal nerve
Afferent fibers Solitary tract nucleus
Parasympathetic fibers Tympanic nerve (Jacobson) Nucleus ambiguus
Tympanic cavity and plexus Inferior salivatory nucleus
Stylomastoid foramen Geniculate ganglion
of facial nerve
Caroticotympanic nerve (from internal carotid plexus)
Greater petrosal nerve
Deep petrosal nerve
Nerve (vidian) of pterygoid canal
Lesser petrosal nerve
Pterygopalatine ganglion
Mandibular nerve (V3)
Otic ganglion
Auriculotemporal nerve
Parotid gland
Tubal branch of tympanic plexus
Pharyngotympanic (auditory)
tube and pharyngeal opening

Stylopharyngeus muscle (and branch


from glossopharyngeal nerve) Glossopharyngeal
nerve (IX)

Jugular foramen
Communication to
auricular branch of
vagus nerve
Superior and
Inferior ganglia of
Glossopharyngeal nerve
Taste and
somatic Communication to facial
sensation: nerve (VII)
posterior
1
⁄3 of tongue Vagus nerve (X)

Superior cervical
sympathetic ganglion
Sympathetic trunk

Carotid branch of
glossopharyngeal nerve
Pharyngeal plexus
Internal carotid artery
Pharyngeal, tonsillar, and lingual Carotid sinus
branches of glossopharyngeal nerve
Carotid body
Pharyngeal branch of vagus nerve Common carotid artery
External carotid artery

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150
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

Figure 14-3B Vagus Nerve (X): Schema

Posterior nucleus of vagus


nerve (parasympathetic
and visceral afferent)
Glossopharyngeal nerve (IX)

Meningeal branch of vagus nerve


Solitary tract
Auricular branch of vagus nerve nucleus (visceral
afferents
Pharyngotympanic (auditory) tube including taste)

Levator veli palatini muscle Spinal tract and


spinal nucleus of
Salpingopharyngeus trigeminal nerve
muscle (somatic afferent)
Palatoglossus
muscle
Palatopharyngeus
muscle Nucleus ambiguus
(motor to pharyngeal
Superior and laryngeal muscles)
pharyngeal
constrictor Cranial root of accessory
muscle nerve
Vagus nerve (X)
Stylopharyngeus muscle Jugular foramen
Superior ganglion of vagus nerve
Middle pharyngeal constrictor muscle
Inferior ganglion of vagus nerve
Inferior pharyngeal constrictor muscle Pharyngeal branch of vagus nerve (motor
to muscles of palate and lower pharynx;
Cricothyroid muscle sensory to lower pharynx)
Trachea Communicating branch of vagus nerve to
carotid branch of glossopharyngeal nerve
Esophagus Pharyngeal plexus
Right subclavian artery Superior laryngeal nerve:
Internal branch (sensory and parasympathetic)
Right recurrent laryngeal nerve External branch (motor to cricothyroid muscle)

Left recurrent laryngeal nerve (motor to Superior cervical cardiac branch of vagus nerve
muscles of larynx except cricothyroid;
sensory and parasympathetic to larynx Inferior cervical cardiac branch of vagus nerve
below vocal folds; parasympathetic,
efferent and afferent to upper esophagus Thoracic cardiac branch of vagus nerve
and trachea)
Efferent fibers
Afferent fibers
Parasympathetic fibers

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151
CRANIAL NERVES IX, X: GLOSSOPHARYNGEAL, VAGUS

sation should be evaluated via flexible endo- mon sequelae of stroke-induced dysphagia. They
scopic evaluation of swallowing with sensory also increase the risk of aspiration.
testing to assess the risk of barium aspiration. Apraxia of the swallow mechanism with unco-
MBS, performed in conjunction with radiologic ordinated muscle movements, characterized by
examination, differs from the standard barium reduced bolus formation and inability to manip-
swallow. Patients ingest graduated sized boluses ulate the bolus and trigger timely sequenced
of various consistencies mixed with barium in the swallow, may also occur. If the nucleus am-
upright position. The primary purpose of MBS is biguus is damaged in a brainstem stroke, ipsilat-
to determine appropriate therapeutic interven- eral paralysis of the larynx, pharynx, and palate
tion strategies to facilitate safe and efficient swal- may lead to a severe pharyngeal phase dyspha-
lowing function. Aspiration and silent aspiration gia. If the nucleus ambiguus is spared, but there
can also be detected. Images are taken in lateral is unilateral tongue, face, or jaw weakness with
and anterior-posterior projections to focus on concomitant loss of sensation in the affected
oropharyngeal swallowing anatomy and physiol- side of the oral cavity, a more severe dysphagia
ogy, with screening for esophageal motility and may result.
esophageal or pharyngeal reflux. The patient Optimal management of neurogenic dyspha-
need not be NPO for MBS. gia requires a multidisciplinary approach. Clini-
cians must be aware of the natural history of the
TREATMENT underlying disorder, with consideration for even-
Patient candidacy for oral intake requires con- tual improvement (ie, cerebrovascular accident
sideration of 3 major components: safety of versus further degeneration of the swallowing
swallow, efficiency and ability to maintain nutri- process as is typical for amyotrophic lateral scle-
tional support through swallowing, and quality rosis). Concomitant respiratory disorders and
of life. For some patients, particularly those who psychosocial aspects of eating must also be con-
are neurologically compromised, safety of swal- sidered.
low is grossly impaired, heightening the risk of Placement of a percutaneous esophagogas-
aspiration pneumonia. Additionally, many of trostomy can be lifesaving in neurologic disor-
these patients are bedridden and have cognitive ders associated with severe dysphagia that have
impairment or decreased levels of alertness. In significant potential for recovery, such as brain-
this setting, even small amounts of aspiration stem strokes. Even in patients with fatal illness
cannot be tolerated. Other patients may present such as amyotrophic lateral sclerosis, a percuta-
with a good swallow, but may fatigue with chew- neous esophagogastrostomy can provide sus-
ing and consecutive swallowing (as is seen with tained comfort and maintain nutrition while sig-
myasthenia gravis), which impedes swallowing nificantly lessening the risk of fatal aspiration
safety and the potential to tolerate a full oral diet. pneumonia.
Eating becomes hard work, making consump-
tion of enough calories difficult.
REFERENCES
Efficiency and ability to maintain nutritional Hughes TAT, Wiles CM. Clinical measurement of swallowing
support through swallowing is the second treat- in health and in neurogenic dysphagia. Q J Med.
ment consideration. Damage to descending cor- 1996;89:109-116.
ticobulbar fibers can occur from stroke, head in- Langmore SE, Skarupski KA, Park PS, Fries BE. Predictors of
aspiration pneumonia in nursing home residents. Dyspha-
jury, or multiple sclerosis. Stroke can result in
gia. 2002;17:298-307.
mild-severe dysphagia depending on the site and Larson C. Neurophysiology of speech and swallowing.
size of the lesion and the accompanying deficits. Semin Speech Lang. 1985;6:275-289.
Unilateral hemispheric stroke is a common, tem- Logemann JA. Evaluation and Treatment of Swallowing Disor-
porary cause of dysphagia. However, delayed ders. 2nd ed. Austin, Tex: Pro-Ed; 1998.
McConnell FMS, Cerenko D, Mendelson MS. Manofluor-
oral transit times, delayed or absent triggering of
graphic analysis of swallowing. Otolaryngol Clin N Am.
the pharyngeal swallow, and poor pharyngeal 1988;21:625-635.
bolus propulsion with decreased sensory aware- Zemlin WR. Speech and Hearing Science: Anatomy and Phys-
ness in the oral and pharyngeal cavities are com- iology. 4th ed. Boston, Mass: Allyn and Bacon; 1998.

CRANIAL NERVES
152
Chapter 15

Cranial Nerve X, Vagus: Voice


Disorders
Timothy D. Anderson

Clinical Vignette
A 62-year-old man with an unremarkable history except for 50 pack-years of cigarette smoking presented with a
hoarse, breathy voice. He had lost his voice suddenly 3 months earlier but had gradually regained some vocal func-
tion. He noted that speaking tired him quickly and that he frequently ran out of breath in mid-sentence.
Physical examination revealed a healthy male in no apparent distress with a breathy, hoarse voice. The remain-
der of the examination showed normal findings. Otolaryngology consultation revealed left vocal fold paralysis with
partial compensation. CT with contrast of the brain, neck, and upper chest showed a large mass in the left upper
lung. A biopsy specimen revealed a primary lung adenocarcinoma.

Although the larynx is usually considered the cricoid. Vocal ligaments stretch from the ary-
source of speech, speech production requires tenoids to the thyroid cartilage. Muscles insert-
precise coordination of multiple organ systems. ing on the arytenoids move the vocal folds and
Contraction of the abdominal musculature, di- arytenoids together for speech and swallowing,
aphragm, and chest wall provides a power and apart for respiration. Although the ary-
source for the voice. The larynx acts as a pres- tenoids’ motion is multidimensional, knowledge
sure regulator and vibratory source. The phar- of the intrinsic laryngeal muscles and their func-
ynx, tongue, nose, and mouth shape these vibra- tions is important for diagnosis (Table 15-1).
tions into recognizable speech and singing. Note that the cricothyroid muscle is the only in-
However, the larynx is the most easily injured of trinsic laryngeal muscle innervated by the supe-
these systems, and most vocal problems origi- rior laryngeal nerve (SLN), and the posterior
nate within it. cricoarytenoid is the only vocal fold abductor.
The motor supply of the laryngeal muscles be-
gins in the nucleus ambiguous (Figure 15-1).
ANATOMY OF THE LARYNX These fibers travel within the vagus nerve (CN-X)
The skeleton of the larynx consists of thyroid as it exits the cranium via the jugular foramen,
and cricoid cartilages. The arytenoid cartilages traveling through the neck within the carotid
articulate with the posterior portion of the sheath (Figure 15-2). High in the neck, the SLN

Table 15-1
Laryngeal Muscle Innervation, Action, and Vocal Function*
Muscle Innervation Action Vocal Function

Lateral cricoarytenoid RLN Adduction Speech


Posterior cricoarytenoid RLN Abduction Respiration
Thyroarytenoid RLN Adduction and shortening Fine voice control
Cricothyroid SLN Lengthening Increase pitch

*RLN indicates recurrent laryngeal nerve; SLN, superior laryngeal nerve.

CRANIAL NERVES
153
CRANIAL NERVE X, VAGUS: VOICE DISORDERS

Figure 15-1 Vagus Nerve (X): Schema


Posterior nucleus of vagus
nerve (parasympathetic
and visceral afferent)
Glossopharyngeal nerve (IX)

Meningeal branch of vagus nerve Solitary tract


nucleus (visceral
Auricular branch of vagus nerve afferents
including taste)
Pharyngotympanic (auditory) tube
Spinal tract and
Levator veli palatini muscle spinal nucleus of
Salpingopharyngeus trigeminal nerve
muscle (somatic afferent)

Palatoglossus
muscle

Palatopharyngeus
muscle Nucleus ambiguus
(motor to pharyngeal
Superior and laryngeal muscles)
pharyngeal
constrictor Cranial root of accessory
muscle nerve
Vagus nerve (X)
Stylopharyngeus muscle
Jugular foramen
Middle pharyngeal constrictor muscle Superior ganglion of vagus nerve
Inferior ganglion of vagus nerve
Inferior pharyngeal constrictor muscle
Pharyngeal branch of vagus nerve (motor
Cricothyroid muscle to muscles of palate and lower pharynx;
sensory to lower pharynx)
Trachea
Communicating branch of vagus nerve to
Pharyngeal plexus carotid branch of glossopharyngeal nerve

Efferent fibers
Afferent fibers
Parasympathetic fibers

splits from CN-X and travels medially. It splits DISORDERS OF VOICE


again into internal and external branches. The in- Recurrent Laryngeal Nerve
ternal branch pierces the thyrohyoid membrane Recurrent laryngeal nerve damage usually
and provides sensory innervation to the pharynx causes vocal fold immobility on the side of in-
and larynx. The external branch travels lower in jury. Depending on the position of the vocal
the neck past the superior pole of the thyroid fold, symptom severity varies greatly. The most
gland to innervate the cricothyroid muscle. common symptoms are a breathy, hoarse
The recurrent laryngeal nerve (RLN) takes a voice and ineffective cough. If the paralyzed
more tortuous path. It separates from CN-X, vocal fold is in the midline, the only symptom
loops around the aortic arch on the left and the may be vocal fatigue and slight breathiness.
brachiocephalic artery on the right, and travels Most patients eventually compensate some-
back toward the larynx in the tracheoesophageal what. The normal vocal fold may cross the mid-
groove bilaterally. It passes under the thyroid line slightly, or the patient may use muscles
gland and inserts into the larynx under the thy- around the larynx to squeeze the vocal folds
roid cartilage, innervating all other intrinsic laryn- shut. If accessory muscles are used to speak,
geal muscles. Both these nerves are vulnerable to muscle fatigue and neck pain may develop af-
injury and have distinct symptoms when injured. ter prolonged talking.

CRANIAL NERVES
154
CRANIAL NERVE X, VAGUS: VOICE DISORDERS

Figure 15-2 Innervation of Larynx


Vagus nerve Hyoid bone
Nucleus Thyrohyoid
Superior laryngeal membrane
nerve
Internal branch
External branch
Superior
laryngeal
nerve (SLN)

Thyroid cartilage

Vagus nerve

Left recurrent
laryngeal nerve
(RLN)
Right recurrent
laryngeal nerve
(RLN)

Posterior view
Paralysis of Normal right
left vocal fold vocal fold

Aortic
arch Lesions disrupting
recurrent laryngeal
nerve result in
paralysis of ipsilateral
vocal fold.

Anterior view
Causes of Neurologic Disorders of Voice
CNS vascular CN-XII
occlusion involving
vagal nuclei Neck tumor
(thyroid
parathyroid)
Lung Neck
tumor surgery Amyotrophic
CNS tumors
involving vagus n. lateral sclerosis
involving nucleus
of CN-XII
N-M
junction

Dopamine Neck lesions involving


Parkinson disease
vagus, RLN, or SLN © Myasthenia gravis

CRANIAL NERVES
155
CRANIAL NERVE X, VAGUS: VOICE DISORDERS

Common causes of vocal fold paralysis in- Although most common in Parkinson disease,
clude thyroid, lung, or neck tumors; cerebrovas- vocal fold bowing can occur in several other neu-
cular accidents; CN-X tumors; and surgery near rologic disorders. The voice is weak and breathy,
CN-X or the RLN. Less common causes include and accessory muscle compensation is common,
thyroiditis (causing inflammation of the RLN), in- causing neck pain and vocal fatigue. Medical
fectious diseases such as Lyme and syphilis, or di- treatment of the underlying disease is the first
abetic or other neuropathies. therapy. Speech therapy is also frequently suc-
Diagnostic evaluation of vocal fold paralysis cessful. Surgery is sometimes necessary to
includes imaging of the brain, neck, and upper straighten and strengthen the bowed vocal folds.
chest, serologic testing, and thyroid function Neuromuscular disorders, most commonly
tests; electromyography of the laryngeal mus- myasthenia gravis, can affect the laryngeal mus-
cles may confirm the diagnosis. Even with exten- cles. A rare form of myasthenia gravis isolated to
sive investigation, the cause of vocal fold paraly- the laryngeal muscles has been described. The
sis sometimes cannot be determined. Treatment most common symptom is vocal fatigue. Patients
is usually directed at moving the paralyzed vocal generally respond to medical therapy.
fold to the midline. Reinnervation procedures
have been described but are not widely used be-
Other Neurologic Voice Disorders
cause of inconsistent results.
Neurologic damage to other portions of the vo-
cal production system can create voice com-
Superior Laryngeal Nerve plaints. Tongue dysarthria, from cerebrovascular
The classic symptom of SLN dysfunction is the accident, amyotrophic lateral sclerosis, or hy-
inability to raise the vocal pitch. Patients also fre- poglossal nerve damage, is one of the most com-
quently have weak voices that tire easily. If the in- mon. Paralysis of the palatal muscles can give a
jury includes the external SLN branch, patients hypernasal voice. Although tongue and pharyn-
may also have poor laryngeal sensation leading geal neurologic disorders can cause voice prob-
to aspiration. High CN-X lesions and cerebrovas- lems, swallowing problems associated with these
cular accidents cause combined SLN and RLN in- disorders are often more problematic and require
juries. These patients are at high risk for aspiration urgent diagnosis and management (chapter 14).
because they can neither close the larynx nor
sense when they are about to aspirate. Common REFERENCES
causes of SLN dysfunction include thyroiditis and Anderson TD, Mirza N. Immediate percutaneous medializa-
thyroid surgery. Infections such as Lyme and tion for acute vocal fold immobility. Laryngoscope.
2001;111:1318-1321.
syphilis can also cause SLN dysfunction. How-
Berke GS, Gerratt B, Kreiman J, Jackson K. Treatment of
ever, most cases of SLN paresis are idiopathic. Parkinson hypophonia with percutaneous collagen aug-
mentation. Laryngoscope. 1999;109:1295-1299.
Boutsen F, Cannito MP, Taylor M, Bender B. Botox treatment
Other Neurologic Disorders of the Larynx in adductor spasmodic dysphonia: a meta-analysis. J
Spasmodic dysphonia is a task-specific dysto- Speech Lang Hear Res. 2002;45:469-481.
nia affecting the larynx. Patients have irregular Chhetri DK, Gerratt BR, Kreiman J, Berke GS. Combined ary-
voice breaks when trying to speak, but many can tenoid adduction and laryngeal reinnervation in the treat-
ment of vocal fold paralysis. Laryngoscope. 1999;109:
sing normally. Its cause is unknown. Treatment in-
1928-1936.
volves identifying the involved muscles and para- Dursun G, Sataloff RT, Spiegel JR, Mandel S, Heuer RJ, Rosen
lyzing them with injections of botulinum toxin. DC. Superior laryngeal nerve paresis and paralysis. J
Patients with laryngeal tremor have regular Voice. 1996;10:206-211.
voice breaks and a tremulous voice. Laryngeal Mao VH, Abaza M, Spiegel JR, Mandel S, Hawkshaw M,
Heuer RJ, Sataloff RT. Laryngeal myasthenia gravis: report
examination reveals regular contractions of the
of 40 cases. J Voice. 2001;15:122-130.
laryngeal muscles at rest and during phonation. Ramadan HH, Wax MK, Avery S. Outcome and changing
Singing is not spared. It can be difficult to distin- cause of unilateral vocal cord paralysis. Otolaryngol Head
guish spasmodic dysphonia from tremor. Neck Surg. 1998;118:199-202.

CRANIAL NERVES
156
Chapter 16

Cranial Nerve XI: Spinal Accessory


Michal Vytopil and H. Royden Jones, Jr

Clinical Vignette
A 23-year-old medical student noted swollen lymph nodes in the left posterior triangle of his neck. He was oth-
erwise asymptomatic. The student health service told him there was nothing wrong except that he was overly con-
cerned. Within a short time, he became quite fatigued. He sought an opinion from a respected internist. The pres-
ence of abnormal left posterior cervical and axillary nodes was confirmed; the remaining findings of his clinical
examination were normal. Results of a mononucleosis spot test were normal and results of liver function studies
were abnormal; therefore, excision of 1 cervical node was performed.
During the procedure, the surgeon queried the student about the risks of this minor operation. The student cor-
rectly replied that it is important to exercise caution so as not to cut the spinal accessory nerve (CN-XI) because
paralysis of the trapezius muscle would result. When he returned to his rotation on the chief of surgery’s service,
the student was informed by the intern that the chief, on hearing about his missing student, commented that there
was a 50% 5-year mortality rate among people undergoing this procedure.
The professor of pathology could not arrive at a diagnosis and sent the node to the Armed Forces Institute of
Pathology and to the Mayo Clinic for more definitive opinions. They did not believe it represented a lymphoma; 6
weeks later, the test for infectious mononucleosis was positive. The editor of this text notes that it is now 44 years
since he had this biopsy.

This vignette exemplifies that careful consider- hemiparesis will not “be able” to turn the head to
ation of every patient complaint is warranted, the “affected” side, even though each SCM is re-
even of those in the medical profession. The pa- sponsible for the contralateral function (the right
tient was labeled with “medical studentitis,” and SCM for a left hemiparesis, and vice versa). This
a peer created emotional turmoil for him until a anatomic paradox becomes a useful evaluative
benign mechanism was established. Occasion- tool, especially when considering patients
ally, patients undergoing similar procedures ex- whose weakness is doubted. However, patients
perience iatrogenic laceration of CN-XI, some- whose nonanatomical weakness is corroborated
times leading to significant shoulder pain and should not be abandoned. They may be calling
atrophy of the unilateral trapezius muscle with for support or emotional help, as was one pa-
some scapular winging. The more proximally in- tient who later proved to be a victim of incest.
nervated sternocleidomastoid muscle (SCM) is
spared. Cervical lymph node biopsies may be ANATOMY
the most common cause of CN-XI palsy. CN-XI is primarily a motor nerve innervating
Of the CNs, CN-XI is probably the least crucial the SCM and trapezius muscles in the neck and
despite its interesting, primarily motor, function back (Figure 16-1). In contrast to other CNs, its
for the neck and shoulder. It also has an intrigu- lower motor neuron cell bodies are located pri-
ing functional array because one of the 2 major marily within the spinal cord. The accessory nu-
muscles it innervates, the SCM, originates on the cleus is a cell column within the lateral anterior
occiput; when one side contracts it turns the gray column of the upper 5 or 6 cervical spinal
head, seemingly paradoxically, in the opposite cord segments. Proximally it lies nearly in line
direction, ie, turning to the left requires a right with the nucleus ambiguus and caudally within
SCM contraction and vice versa. the dorsolateral ventral horn. Originating from
The uncommon patients who have conver- the accessory nucleus, the rootlets emerge from
sion hysteria or malingering with a purported the cord and unite to form the trunk of CN-XI. It

CRANIAL NERVES
157
CRANIAL NERVE XI: SPINAL ACCESSORY

Figure 16-1 Accessory Nerve (XI): Schema


Nucleus ambiguus
Cranial root of accessory nerve (joins vagus nerve
and via recurrent laryngeal nerve supplies muscles of
Vagus nerve (X) larynx, except cricothyroid)*
Spinal root of
accessory nerve Jugular foramen
Foramen
magnum Superior ganglion
of vagus nerve

Accessory nerve (XI)*

Inferior ganglion
of vagus nerve

C1 spinal nerve

C2 spinal nerve

Accessory nerve
(to sternocleidomastoid
and trapezius muscles)

Sternocleidomastoid muscle (cut)

C3 spinal nerve

C4 spinal nerve

Trapezius muscle

Efferent fibers
Proprioceptive fibers

*Recent evidence suggests that the accessory nerve lacks a cranial root and has no connection to the vagus nerve.
Verification of this finding awaits further investigation.

CRANIAL NERVES
158
CRANIAL NERVE XI: SPINAL ACCESSORY

extends rostrally through the foramen magnum when patients abduct the arms. In contrast,
into the posterior cranial fossa. Intracranially, it scapular winging from long thoracic nerve palsy
accompanies caudal fibers of the vagus nerve with weakness of serratus anterior is most promi-
(CN-X) and leaves the skull through the jugular nent on forward elevation of the arms (Figure
foramen. 16-3).
CN-XI then descends in close proximity to the Typically, the patient often also experiences a
internal carotid artery and internal jugular vein painful paresis secondary to direct nerve injury
(Figure 16-2). During its extracranial course, CN- during various procedures or disorders or, if de-
XI is joined by some fibers derived from the third layed, by entrapment of the nerve within scar tis-
and fourth upper cervical ventral rami. Some of sue or structural lesions such as tumors.
these cervical fibers may innervate the caudal EMG is important for confirming that the ob-
trapezius, whereas the proximal trapezius and served lesion is confined to the distribution of
the entire SCM muscle may be primarily inner- CN-XI. Additionally, a gadolinium-enhanced MRI
vated by CN-XI. Having emerged from the mid- is appropriate if any question exists of a lesion
point of the posterior border of the SCM, CN-XI more widespread than a simple CN-XI neuropa-
crosses the posterior triangle of the neck superfi- thy.
cial to the levator scapulae. Importantly, this is
where CN-XI is closely related to the superficial
cervical lymph nodes. Further caudally, approxi- DIFFERENTIAL DIAGNOSIS
mately 5 cm above the clavicle, it passes into the The close association of CN-XI with superficial
anterior border of the trapezius muscle, which it cervical lymph nodes renders it vulnerable to ia-
also innervates. trogenic damage during various surgical proce-
There is a minor afferent component to CN-XI. dures within the posterior triangle, including
These fibers seem to have a primary propriocep- lymph node biopsy and radical neck surgical dis-
tive function for the 2 muscles that CN-XI inner- section. Damage also rarely occurs after carotid
vates. endarterectomy or jugular vein cannulation be-
CN-XI is described as having a cranial root in cause of the nerve’s proximity to large neck ves-
addition to the spinal roots described above. The sels. Various disorders at the anatomical level of
cranial root consists of a few fibers originating in the anterior horn cell are within the differential,
the caudal portion of the nucleus ambiguus, including motor neuron disease, syringomyelia,
which run with the 11th CN intracranially, then and poliomyelitis.
through the jugular foramen. Intraspinal and intracranial portions of CN-XI
may be affected by intrinsic spinal cord lesions,
CLINICAL PRESENTATION AND posterior fossa meningiomas, or metastases. Be-
DIAGNOSTIC APPROACH nign tumors such as an enplaque meningioma at
Lesions located intracranially or proximally to the base of the brain or metastatic tumors at the
the innervation of the SCM cause weakness of jugular foramen or foramen magnum may im-
the SCM and the trapezius. Damage to the nerve pinge on CN-XI; however, they usually concomi-
in the posterior triangle of the neck spares the tantly affect CN-X and sometimes even the hy-
SCM and results in weakness of the trapezius poglossal nerve that exits through the adjacent
only. If the SCM is weak, the patient experiences hypoglossal foramen. Rarely, radiation injury af-
weakness when turning the head to the opposite fects CN-XI with treatment of adjacent tumors.
side. Because most individuals with CN-XI palsies
Involvement of the trapezius manifests as present with shoulder or neck pain or both, le-
drooping of the shoulder and mild scapular sions at the level of the cervical nerve roots or
winging away from the chest wall with slight lat- brachial plexus require consideration. Patients
eral displacement. Weakness in shoulder eleva- with neuralgic amyotrophy may have concomi-
tion and arm abduction above horizontal is typi- tant involvement of CN-XI and the phrenic
cal. Winging is apparent, with arms hanging nerve. These plexopathies and radiculopathies
along the trunk, and becomes accentuated have an acute onset.

CRANIAL NERVES
159
CRANIAL NERVE XI: SPINAL ACCESSORY

Figure 16-2 Cervical Plexus In Situ


Facial artery Parotid gland
and vein
Submandibular Great auricular
gland nerve
Mylohyoid Lesser occipital
muscle nerve
Hypoglossal Sternocleidomastoid
nerve (XII) muscle (cut, turned up)
Digastric muscle Stylohyoid muscle
(anterior belly) Digastric muscle
Lingual artery (posterior belly)

External carotid artery C2 spinal nerve (ventral ramus)

Internal carotid artery Accessory nerve (XI)

Thyrohyoid muscle C3 spinal nerve (ventral ramus)

Superior thyroid artery Levator scapulae muscle


Omohyoid muscle (superior Middle scalene muscle
belly) (cut) Anterior scalene muscle
Ansa cervicalis Superior root C5 spinal nerve
Inferior root
(ventral ramus)
Sternohyoid muscle Transverse cervical
artery
Sternothyroid muscle Phrenic nerve
Internal jugular vein
Common carotid artery
Inferior thyroid artery
Vagus nerve (X) Omohyoid muscle (inferior
belly) (cut)
Vertebral artery
Brachial plexus
Thyrocervical trunk Dorsal scapular artery
Subclavian artery and vein Suprascapular artery

PROGNOSIS times surgical exploration is helpful. The time


For patients with benign traumatic lesions, the frame for reinnervation is similar to that for any
likelihood of reinnervation is good unless the 2 peripheral nerve: 1 mm per day or 3 cm per
CN-XI segments are widely separated. Some- month.

CRANIAL NERVES
160
CRANIAL NERVE XI: SPINAL ACCESSORY

Figure 16-3 Clinical Findings in Cranial Nerve XI Damage


Lesion proximal Weakness Weakness
to sternocleido- of SCM turning head to
C1 mastoid (SCM) opposite side
innervation
C2
Drooping of
Spinal C3 Lesion in posterior shoulder and
accessory Weakness mid scapular
triangle of
nerve of trapezius winging;
C4 neck
(distal to SCM weakness in
Sternocleido-
C5 innervation) shoulder
mastoid
elevation
muscle (SCM) Clinical presentation and arm
Trapezius varies with abduction above
muscle location of horizontal
damage.

Comparison of clinical findings in CN-XI and long thoracic nerve damage


CN-XI damage Spinal
accessory
Mild nerve (CN-XI)
Mild scapular
shoulder winging Trapezius
droop muscle
Normal Normal Scapula

Marked
scapular
winging

Arms at side Arms in abduction


Spinal accessory (CN-XI) nerve lesions cause weakness
of trapezius muscle on involved side and present with
mild shoulder droop. Weakness of shoulder elevation
and scapular winging most pronounced on arm abduction
Long thoracic nerve damage
Winging Normal
of scapula
Scapula

Serratus
anterior m.
(helps
stabilize
scapula)

Long thoracic n. Forward elevation of arms with


push against stationary object

Lesion of long thoracic nerve causes weakness


of serratus anterior muscles with winging of scapula
most pronounced when pushing against stationary
object with arms in forward elevation.

CRANIAL NERVES
161
CRANIAL NERVE XI: SPINAL ACCESSORY

REFERENCES Sarma S, Sekhar LN, Schessel DA. Nonvestibular schwanno-


Berger PS, Bataini JP. Radiation-induced cranial nerve palsy. mas of the brain: a 7-year experience. Neurosurgery.
Cancer. 1977;40:152-155. 2002;50:437-448.
Brown H. Anatomy of the spinal accessory nerve plexus: rel- Sweeney PJ, Hanson MR. The cranial neuropathies. In:
evance to head and neck cancer and atherosclerosis. Exp Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds.
Biol Med (Maywood) 2002;227:570-578. Neurology in Clinical Practice: Principles of Diagnosis and
Finsterer J, Erdorf M, Mamoli B, et al. Needle electromyogra- Management. 2nd ed. Boston, Mass: Butterworth-Heine-
phy of bulbar muscles in patients with amyotrophic lateral mann; 1996:1721-1732.
sclerosis: evidence of subclinical involvement. Neurology. Thomas PK, Mathias CJ. Diseases of the ninth, tenth,
1998;51:1417-1422. eleventh, and twelfth cranial nerves (1993). In: Dyck PJ,
Friedenberg SM, Zimprich T, Harper CM. The natural history Thomas PK, Griffin JW, Low PA, Poduslo JF, eds. Peripheral
of long thoracic and spinal accessory neuropathies. Mus- Neuropathy. 3rd ed. Philadelphia, Pa: WB Saunders Co;
cle Nerve. 2002;25:535-539. 1993:869-885.
Hanson MR, Sweeney PJ. Disturbances of lower cranial Wilson-Pauwels L, Akesson EJ, Stewart PA. Accessory nerve.
nerves. In: Bradley WG, Daroff RB, Fenichel GM, Marsden In: Wilson-Pauwels L, Akesson EJ, Stewart PA, eds. Cranial
CD, eds. Neurology in Clinical Practice: Principles of Diag- Nerves: Anatomy and Clinical Comments. Philadelphia, Pa:
nosis and Management. 2nd ed. Boston, Mass: Butter- BC Decker; 1988:139-145.
worth-Heinemann; 1996:251-263. Woodward G, Venkatesh R. Spinal accessory neuropathy
Kim DH, Cho YJ, Tiel RL, et al. Surgical outcomes of 111 spinal and internal jugular thrombosis after carotid endarterec-
accessory nerve injuries. Neurosurgery. 2003;53:1106-1112. tomy. J Neurol Neurosurg Psychiatry. 2000;68:111-112.

CRANIAL NERVES
162
Chapter 17

Cranial Nerve XII: Hypoglossal


Michal Vytopil and H. Royden Jones, Jr

Despite being the most distal of the 12 paired CNs, the hypoglossal nerve (CN-XII) controls what is
teleologically an important human function: the final common pathway for language implementation.
Phylogenetically, CN-XII has major significance because of its role in food intake. As with any CN, CN-
XII is susceptible to numerous pathologic processes.

ANATOMY carotid system. Severe bilateral carotid stenosis was di-


CN-XII carries motor fibers that supply all in- agnosed. Sequential carotid endarterectomies were
performed 6 days apart at another hospital. Immedi-
trinsic and most extrinsic tongue muscles, ie, the ately after the second surgery, the patient noted
hyoglossus, styloglossus, genioglossus, and ge- speech and swallowing difficulties.
niohyoid (Figure 17-1). Its fibers originate from Neurologic examination demonstrated dysarthric
the hypoglossal nucleus beneath the floor of the speech, particularly for lingual sounds. The patient had
fourth ventricle. In its intramedullary course, CN- bilateral tongue weakness with inability to protrude or
XII axons pass ventrally to the lateral side of the elevate the tongue but no atrophy or fasciculation.
medial lemniscus emerging from the medulla in EMG 5 weeks after surgery demonstrated considerable
diminution in the number of motor units with fibrilla-
the ventrolateral sulcus between the olive and
tion potentials confined to the tongue and infrahyoid
the pyramid. The rootlets unite to form CN-XII, muscles. The patient’s speech and swallowing gradu-
which exits the skull through the hypoglossal ally improved over the subsequent 6 months.
foramen within the posterior cranial fossa.
After exiting the skull, CN-XII runs medial to The close proximity of CN-XII to the carotid
CN-IX, -X, and -XI. It continues between the in- artery makes it vulnerable to concomitant le-
ternal carotid artery and internal jugular vein, sions. Typically, acute carotid artery dissection is
and deep into the posterior belly of the digastric accompanied by signs of Horner syndrome, an
muscle. It then loops anteriorly, coursing on the associated cranial XII neuropathy, or both, and
lateral surface of the hyoglossus muscle, and sometimes by dysgeusia. However, swallowing
later, it divides to supply the intrinsic and extrin- and speech functions are not greatly affected un-
sic muscles of the ipsilateral tongue. til bilateral lesions develop, as in the vignette in
The anterior primary ramus of the spinal nerve this chapter. Both CN-XII nerves were damaged
C1 sends fibers to accompany CN-XII for a short sequentially by the carotid endarterectomies.
distance; these fibers later connect with the
fibers of C2 and C3 anterior primary rami, form- CLINICAL PRESENTATION
ing a loop called ansa cervicalis. This innervates Straight protrusion of the tongue is accom-
the scalenus group, rectus capitis, longus capitis, plished by balanced action of both genioglossus
longus colli, and the levator scapulae muscles of muscles. Therefore, bilateral CN-XII lesions im-
the neck. pair tongue protrusion causing dysarthria and
swallowing difficulties. Unilateral CN-XII lesions
Clinical Vignette do not interfere with swallowing or speech but
cause the tongue to deviate toward the side of
A 67-year-old man presented with a 2-week history the lesion when the patient attempts to protrude
of slurring of speech and difficulty swallowing liquids.
Two months earlier, he had experienced a few transient
the tongue (Figure 17-2). A lower motor neuron
episodes of aphasia lasting 15 to 20 minutes consistent lesion of CN-XII typically results in atrophy, fasci-
with a diagnosis of transient ischemic attacks of the left culation, and increased furrowing of the ipsilat-

CRANIAL NERVES
163
CRANIAL NERVE XII: HYPOGLOSSAL

Figure 17-1A Hypoglossal Nerve (XII): Schema

Hypoglossal nerve (XII) Meningeal branch


(in hypoglossal canal)

Superior Hypoglossal nucleus


Intrinsic longitudinal
muscles Transverse
of tongue and vertical Styloglossus
Inferior muscle
longitudinal

Occipital condyle

Inferior ganglion of
vagus nerve

Ventral rami of
C1, 2, 3 form
ansa cervicalis
of cervical plexus

Superior cervical
sympathetic
ganglion

Superior root of
ansa cervicalis
Genioglossus
muscle
Internal carotid artery
Geniohyoid muscle

Hyoglossus muscle Inferior root of ansa cervicalis

Thyrohyoid muscle Ansa cervicalis

Omohyoid muscle Internal jugular vein


(superior belly)
Sternohyoid muscle Common carotid artery

Sternothyroid muscle

Omohyoid muscle (inferior belly) Efferent fibers


Afferent fibers

CRANIAL NERVES
164
CRANIAL NERVE XII: HYPOGLOSSAL

Figure 17-1B Hypoglossal Nerve Intermedullary Course


Medulla—Level of the Obex Nucleus cuneatus

Nucleus gracilis
External (lateral)
Obex
cuneate nucleus Nucleus
solitarius Central canal
Inferior cerebellar
peduncle with Dorsal motor
dorsal nucleus of X
Level of spinocerebellar
section tract Nucleus
CN-XII
Tractus
solitarius
Spinal tract Internal
CN-V arcuate
fibers
Spinal nucleus
CN-V
Medial
Spinothalamic/ longitudinal
spinoreticular fasciculus
tract
Tectospinal
tract
Nucleus
ambiguus Medial lemniscus
Inferior olivary nucleus
CN-XII Pyramid Medial accessory olivary nucleus

Medulla—Level of the Inferior Olive


Nucleus cuneatus
External cuneate
nucleus Nucleus solitarius

Tractus solitarius

Inferior Dorsal motor nucleus of X


cerebellar Choroid
peduncle plexus

Level of Fourth
section Spinal tract ventricle
CN-V
Nucleus
Spinal nucleus CN-XII
CN-V

CN-X Medial
Spinothalamic/ longitudinal
spinoreticular tract fasciculus

Nucleus ambiguus Tectospinal


tract
Inferior olivary nucleus Medial
Dorsal accessory olivary nucleus lemniscus
CN-XII Medial accessory olivary nucleus
Pyramid

CRANIAL NERVES
165
CRANIAL NERVE XII: HYPOGLOSSAL

Figure 17-2 Hypoglossal Nerve (CN-XII)


Sites of lesions affecting
hypoglossal nerve (CN-XII)
Motor
cortex

Lesion

Subtle weakness of tongue may be tested by asking


patient to press tongue against cheek (shown) or
against a tongue depressor.

CN-XII

Lesion Nucleus
CN-XII Lesions of
nucleus of
CN-XII or nerve
proper result
in ipsilateral
CN-XII deficit.

Patient with
Atrophy right sided
CN-XII lesion

Fasciculation with
E. Hatton
©
When hypoglossal nerve or its nucleus If hypoglossal nerve is affected on one side,
is damaged, atrophy and fasciculation the tongue often deviates toward the side
of the tongue are noted on evaluation. of the lesion on protrusion (due to imbalance
of genioglossus contraction).

CRANIAL NERVES
166
CRANIAL NERVE XII: HYPOGLOSSAL

eral side of the tongue. In contrast, a unilateral meningeal enhancement should be checked.
upper motor neuron lesion may result in devia- The latter is typically seen with metastatic tu-
tion of the tongue contralateral to the lesion mors, sarcoidosis, or other rare leptomeningeal
without evidence of atrophy or fasciculation. infiltrating lesions such as tuberculosis.
CSF analysis is indicated if an infiltrative
DIFFERENTIAL DIAGNOSIS process is clinically suspected or suggested from
Anterior horn cell disorders may affect the hy- MRI. CSF analysis must include routine studies
poglossal nucleus, as in motor neuron disease or and cytologic analysis for malignant cells.
poliomyelitis. Other intramedullary processes EMG is indicated when the above studies are
such as syringobulbia or intramedullary tumors unremarkable. Unfortunately, an asymmetrically
may also lead to a tongue paresis. Syringobulbia atrophied tongue is most commonly the pre-
is frequently associated with the Arnold-Chiari senting sign of motor neuron disease.
malformation. Lower motor neuron tongue
weakness of sudden onset may be a manifesta- REFERENCES
tion of a rare form of brainstem infarction but is Berger PS, Bataini JP. Radiation-induced cranial nerve palsy.
usually accompanied by other features, most no- Cancer. 1977;40:152-155.
tably contralateral hemiparesis and involvement Combarros O, Alvarez de Arcaya A, Berciano J. Isolated uni-
lateral hypoglossal nerve palsy: nine cases. J Neurol.
of other lower CNs. 1998;245:98-100.
Although tongue weakness secondary to in- DePaul R, Abbs JH, Caligiuri M, et al. Hypoglossal, trigemi-
trinsic medullary lesions is usually bilateral and is nal, and facial motoneuron involvement in amyotrophic
accompanied by other features, isolated unilat- lateral sclerosis. Neurology. 1988;38:281-283.
eral hypoglossal paralysis should always suggest Gutrecht JA, Jones HR. Bilateral hypoglossal nerve injury af-
ter bilateral carotid endarterectomy. Stroke. 1988;19:261-
a neoplasm. A primary tumor of CN-XII, such as
262.
neurinoma, may be the cause. Intracranial Hanson MR, Sweeney PJ. Disturbances of lower cranial
metastases, particularly bronchial or breast car- nerves. In: Bradley WG, Daroff RB, Fenichel GM, Marsden
cinomas, lymphomas, or benign lesions such as CD, eds. Neurology in Clinical Practice: Principles of Diag-
meningiomas, chordomas, or cholesteatomas nosis and Management. 2nd ed. Boston, Mass: Butter-
occasionally affect CN-XII. Glomus jugulare tu- worth-Heinemann; 1996:251-263.
King AD, Leung SF, Teo P, et al. Hypoglossal nerve palsy in na-
mor, a relatively rare lesion, occasionally pre-
sopharyngeal carcinoma. Head Neck. 1999;21:614-619.
sents in this clinical context. Sometimes, no Sarma S, Sekhar LN, Schessel DA. Nonvestibular schwanno-
mechanism is identified. mas of the brain: a 7-year experience. Neurosurgery.
Neck trauma with or without spontaneous 2002;50:437-448.
dissection of the internal carotid is a rare cause Spector GJ, Druck NS, Gado M. Neurologic manifestations
of glomus tumors in the head and neck. Arch Neurol.
of CN-XII neuropathy. Similar to some other
1976;33:270-274.
CNs, CN-XII may be affected by radiation ther- Sweeney PJ, Hanson MR. The cranial neuropathies. In:
apy. Bradley WG, Daroff RB, Fenichel GM, Marsden CD, eds.
Glossodynia is a controversial syndrome char- Neurology in Clinical Practice: Principles of Diagnosis and
acterized by burning pain in the tongue. The Management. 2nd ed. Boston, Mass: Butterworth-Heine-
condition occurs in middle-aged patients and oc- mann; 1996:1721-1732.
Thomas PK, Mathias CJ. Diseases of the ninth, tenth,
curs more frequently in women. Although vita-
eleventh, and twelfth cranial nerves (1993). In: Dyck PJ,
min B deficiency, including B12, has been sug- Thomas PK, Griffin JW, Low PA, Poduslo JF, eds. Peripheral
gested as a cause, most cases are thought to be Neuropathy. 3rd ed. Philadelphia, Pa: WB Saunders Co;
psychogenic. 1993:869-885.
Wesselmann U, Reich SG. The dynias. Semin Neurol.
1996;16:63-74.
DIAGNOSTIC APPROACH
Wilson-Pauwels L, Akesson EJ, Stewart PA. Accessory nerve.
MRI of the brain base and neck are the diag- In: Wilson-Pauwels L, Akesson EJ, Stewart PA, eds. Cranial
nostic tests of choice. If these tests do not define Nerves: Anatomy and Clinical Comments. Philadelphia, Pa:
evidence of a specific mass lesion, lepto- BC Decker; 1988:147-151.

CRANIAL NERVES
167
Chapter 18

Primary and Secondary Headache


Jose A. Gutrecht and Edward Tarlov

Clinical Vignette
A 40-year-old woman presented to her internist because of increasing severity of long-standing headaches. These
headaches occurred without warning and were characterized by unilateral, throbbing pain aggravated by minor
physical activities such as bending over or climbing stairs. She had nausea, photophobia, and was unable to work
for 1 to 2 days until the headache subsided. Chocolate often precipitated a headache, and the patient’s family his-
tory was positive for migraines. Her neurologic and general medical examination was normal. CT scan of the brain
was normal.

Headache is one of the most common symp- logic pathology, or secondary to some cerebral
toms in medicine, often a primary presenting or extracerebral pathology. The differentiation
complaint. It can be the presentation in many pri- between primary and secondary is critical; it dic-
mary neurologic illnesses and in some serious tates the diagnostic approach and guides treat-
systemic disorders. The preceding vignette is ment and prognosis.
typical of one of the most common headache
syndromes (migraine) that bring patients to med- PRIMARY HEADACHE DISORDERS
ical attention. More serious causes requiring spe- Migraine
cific treatment must not be overlooked, such as Migraine is the most common type of
brain tumors, ruptured aneurysms, low cere- headache that leads patients to seek medical
brospinal pressure syndromes, subdural hema- care. Its prevalence is almost 10% (30 million
toma, meningitis, and temporal arteritis. The pri- persons) in the US population, with a female to
mary pain-sensitive structures in the head male ratio of 3:1. First-degree relatives of patients
include the leptomeninges, arteries, and nasal si- with migraine seem to be at higher risk. Fre-
nuses (Figure 18-1). quently, migraine begins during puberty. It is
Assessment of a patient presenting with a sig- more prevalent in individuals aged 25 to 55
nificant headache starts with the detailed history. years and is one of the leading causes of chronic
Essential characteristics should be defined: any suffering and disability in this population.
premonitory symptoms, manner of onset (eg, Patients with migraine often have prodromal
precipitous or gradual), provoking factors, pain symptoms of emotional or mood disturbances
characteristics, the associated clinical circum- such as irritability, elation or depression, general
stances and symptoms, duration, and degree of symptoms of fatigue, thirst, anorexia, fluid reten-
disability. Family and social history, current med- tion, food cravings, or other neurologic phenom-
ications, drug allergies, and review of systems ena, including drowsiness or even enhanced
are also paramount. A detailed neurologic and alertness (Figure 18-2). Many individuals with mi-
general medical examination is essential to the graine have premonitory focal disturbances that
evaluation, particularly with individuals having a mimic a TIA or stroke. Of migraine patients, ap-
recent or precipitous onset or experiencing proximately 15% have an aura preceding the
changes in headache characteristics. Ancillary pain phase. This presentation was formerly re-
laboratory and neuroradiologic testing are often ferred to as classic migraine; however, the new
indicated. classification system identifies it as migraine with
Headache syndromes must first be classified aura. The aura comprises focal neurologic symp-
as primary, without significant underlying neuro- toms, most commonly visual (⬎95% of all auras).

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170
PRIMARY AND SECONDARY HEADACHE

Figure 18-1 Pain—Sensitive Structures and Pain Referral


Pain
sensation Dural sinus

Middle
meningeal a.

Temporal a.

Proximal
cerebral
aa.

Tentorium Anterior head


cerebelli Afferent nerves from
Internal intracranial and extracranial
and structures of anterior 2/3 of
external head and somatic pain
carotid aa. afferent nerves from
forehead and scalp are
carried by ophthalmic nerve.
These neurons refer pain
from intracranial structures
to forehead, scalp, or
retrobulbar sites.

Ophthalmic
(V1) n.
Central pain
pathway Spinal nucleus
of trigeminal (V) n.

Spinal ganglia C1-3

Dura of posterior
fossa Posterior head
Afferent nerves from
occipital region, ear,
and neck and from dura
Vertebro- of posterior fossa and
basilar aa. vertebrobasilar arteries
are carried by dorsal roots
of C1-3 spinal ganglia,
accounting for pain
referral to these sites.

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PRIMARY AND SECONDARY HEADACHE

Figure 18-2 Mechanisms in Migraine


Central mechanisms Peripheral mechanisms
Pain perception
Migraine may be initiated by
afferent stimulation from central
centers in cortex, thalamus, and
hypothalamus or by peripheral
afferent stimulation via trigeminal
nerve or cervical roots C1-3

Periaqueductal
gray matter
Nucleus
raphe
Local defect in endogenous pain control
dorsalis
system prevents inhibition of pain
Trigeminal
stimulation (disinhibition) in spinal
Locus nerve pain
nucleus of trigeminal nerve.
ceruleus pathway

Unopposed pain stimulation in


spinal nucleus of trigeminal
nerve

V1
Peripheral
V2 inflow

Central Trigeminal V3
pain (V) n.
Trigeminal vascular reflex
pathway Afferent stimulation of pain centers in
spinal nucleus of trigeminal nerve
Facial increased and perpetuated by cycle of
(VII) parasympathetic dilation of internal
Nucleus and external carotid arteries mediated via
raphe n.
facial nerve, resulting in stimulation of
magnus pain centers by trigeminal nerve afferents.

Parasympathetic (vasodilation) outflow


Impaired inhibition in endogenous
pain control system
Second-
order Pain stimulation in spinal nucleus of trigeminal
neuron nerve via afferent input from higher sources
and via cervical roots C1-3

C1-3 pain pathway

Adapted from Lance

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PRIMARY AND SECONDARY HEADACHE

Figure 18-3 Migraine


Attack
Severe, throbbing
headache; unilateral
Sonophobia Transient
at first but may Vertigo
aphasia
spread to opposite
side Photophobia Photophobia
Pallor
Local erythema
may be present Speaks in low Thick
voice to avoid speech
aggravating
Pallor, perspiration pain

Chills

Tremor

Unilateral
numbness
or weakness

Some other manifestations


of aura, which may occur
individually or in combination

Vomiting may occur.


Scintillating Scotoma and Fortification Phenomena
Scintillating
edge

Fortification
Early phase: isolated paracentral pattern Spread of scotoma to involve entire
scintillating scotoma unilateral visual field

Wavy lines (heat shimmers) Metamorphopsia

Wavy line distortions in part of visual field Distortions of form, size, or position of objects
similar to shimmers above hot pavement or environment in part of visual field

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PRIMARY AND SECONDARY HEADACHE

These visual phenomena occur in a homony- ity. These criteria must be met for at least 5 sepa-
mous or hemifield distribution. Typically, sco- rate episodes. Concomitantly, it is crucial for the
tomata, either scintillating simple flashes often physician to ensure that specific organic patho-
called stars (phosphenes) or geometric patterns physiologic mechanisms are excluded before
known as a fortification spectra characterize confirming a diagnosis of migraine. Therefore,
these symptoms. careful evaluation of the patient presenting with
Other types of migrainous auras include sen- the first few headaches is essential. Similarly, any
sory, motor, or rarely, involvement of higher cor- change in the character of the individual head-
tical functions, including language. Most auras aches warrants a fresh investigation of the un-
develop slowly over several to 20 minutes, in- derlying mechanism.
volve anatomically different areas, and last less Migraine pathophysiology is related to a com-
than 1 hour. For example, the patient may ini- bination of meningeal blood vessel dilatation,
tially experience numbness in the fingers that secondary activation of trigeminal pathways,
gradually spreads up the arm to the face and and subsequent release of vasoactive neuropep-
sometimes even down the leg. Occasionally, in tides (Figure 18-3). These promote neurogenic
some individuals, the auras may not be followed inflammation and pain perception at thalamic
by the headache phase. Less commonly, the and cortical brain levels.
aura phase consists of a more complex sympto-
matology. For example, in basilar artery mi- Special Considerations
graine, symptoms may include dysarthria, ver- Two thirds of women with migraines primarily
tigo, ataxia, diplopia, and hearing deficits. In experience them just before or during menses.
confusional migraine, cognitive deficits are These headaches are often associated with other
more prominent. Ocular nerve palsies are the premenstrual dysphoric symptoms. Estrogen
hallmark of ophthalmoplegic migraine. Hemi- withdrawal most likely influences this major mi-
plegic migraine has a variably prominent unilat- graine subgroup.
eral weakness. Migraine may worsen early in pregnancy but
Careful neurologic evaluation is mandatory tends to improve during the later trimesters, par-
before migraine can be safely diagnosed in indi- ticularly in women with migraine primarily related
viduals with a focal aura; this diagnosis, in these to their menstrual cycle. Migraine prevalence de-
circumstances, is one of exclusion. The patho- creases with age. Women with migraines that oc-
physiology of the aura may be related to neu- cur earlier in life generally experience a significant
ronal events of “spreading depression” and con- decrease in episode frequency after menopause.
comitant decrease in cerebral blood flood. Great care must be used in the evaluation and
Although auras are thought to typify migraine treatment of “migraine“ headache in the elderly.
and to help differentiate migraines from other The incidence of headaches as a manifestation
headaches, most migraine patients never experi- of other illnesses significantly increases with age.
ence an aura. Headaches may occur as an adverse effect of
The pain phase and its associated symptoms medications, and the use of antimigraine med-
support the clinical classification of headaches ication may be more hazardous.
as migraine. According to the 1988 guidelines of
the Headache Classification Committee of the Treatment
International Headache Society, the headache There are 2 primary steps in the care of mi-
must last 4 to 72 hours (either untreated or un- graine patients: treatment of an acute headache
successfully treated) and must be associated and prevention of subsequent events. The initial
with nausea and/or vomiting, or photophobia, goal is prompt pain relief without recurrence,
and/or phonophobia. The headache must be with minimal, if any, untoward adverse effects,
characterized by 2 of the following 4 symptom while restoring baseline function. For mild to
characteristics: unilateral location, throbbing moderately severe nondisabling pain, oral
pulsatile quality, moderate or worse degree of NSAIDs, acetylsalicylic acid (aspirin), or aceta-
severity, or intensified by routine physical activ- minophen is recommended for short-term treat-

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PRIMARY AND SECONDARY HEADACHE

ment. Caffeine may enhance the effect of these the pain occurred in the daytime. “I become very rest-
various medications. Antiemetics are often use- less during the episode, which generally lasts no more
than 1 hour. A few years ago, I had similar headaches
ful in conjunction with analgesics.
for about 4 or 5 weeks.”
However, in patients with more severe dis-
abling migraines, oral, injectable, intranasal, or As evident from the initial interview, a cluster
quick-dissolve oral serotonin 1B/1D receptor ag- headache is one of the most severe headaches a
onist (“triptan”) preparations are the medications patient can experience, with the exception of an
of choice. Standard doses are 6.25 to 12.5 mg al- acute ruptured berry aneurysm. Fortunately,
motriptan, 25 to 100 mg sumatriptan, 10 mg riza- cluster headaches occur much less commonly
triptan (5 mg if the patient is taking a ␤ blocker), than migraine, to which they are unrelated.
and 2.5 to 5 mg zolmitriptan. These doses may be These recurrent headaches usually first occur in
repeated after 2 hours if necessary. These prepa- the third decade, affecting men more often than
rations are also recommended for patients with women (male to female ratio is 2-7:1). Unlike in
milder headaches whose migraines are disabling migraine, a family history of cluster headache is
because they are refractory to the simpler anal- uncommon. Distinctive clinical features include
gesics. When the above-mentioned therapeutic abrupt recurrence once or twice daily, often
options are ineffective in patients with the most awakening the patient from sleep. These
severe migraines, opiate category medications headaches are always severe with a unilateral
such as intranasal butorphanol or IM meperidine distribution around one eye and orbit, lasting up
are often used in EDs. However, the possibility of to 1 hour. Most patients with cluster headaches
untoward sedation and more importantly, subse- experience concomitant autonomic dysfunc-
quent overuse and dependence, must be consid- tion, including partial Horner syndrome, lacrima-
ered. Nonnarcotic treatments, such as ketorolac tion, and nasal congestion ipsilateral to the
and antiemetics, are still preferred. headache (Figure 18-4). Patients are often rest-
Prophylactic treatment, the other aspect of less during the episode. Alcohol ingestion is a
the migraine therapeutic algorithm, is indicated frequent precipitant.
for patients with frequent headache (⬎3 dis- The periodicity of cluster headaches, likely
abling headaches/month lasting more than 1-3 from dysfunction at the medial hypothalamic ar-
days). Other indications for preventive therapies eas of the brain, is one of the most important di-
include poor response or adverse effects from agnostic characteristics. The patient reports a clin-
immediate treatment medications, need for ical course of daily headache recurrence for 6 to
medications with potential for abuse and depen- 12 weeks, with periods of cluster recurring every
dency, or patient preference when one migraine several months to few years. The underlying
can significantly interfere with daily activities or pathophysiology seems to be activation of the
responsibilities. Various antidepressants, ␤ trigeminal vascular and parasympathetic systems.
blockers, calcium channel blockers, sodium val- Like migraine, the 2 parts to the treatment al-
proate, and methysergide are the most effective gorithm are short-term and preventive therapy.
medications. Experimentation often dictates the Effective short-term headache therapies include
most effective medication for the individual pa- sublingual triptan, 25 to 50 mg oral in-
tient. domethacin 3 times daily, or oxygen inhalation
at 4 to 6 L/min for 15 minutes. Preventive ther-
Cluster Headache apy must also be used. Verapamil is the drug of
choice for prophylaxis of cluster headaches.
Other beneficial drugs include sodium val-
Clinical Vignette proate, lithium, short-term corticosteroids, and
A 36-year-old man reported, “For the last 10 days, I methysergide, although now in short supply. In
have had an absolutely terrible pain behind my left eye 10% of the patients with cluster headaches, a
that wakes me up every night in the early morning chronic form develops; combined-drug pro-
hours, almost like clockwork.” He stated, “The pain is
intense, I have tearing from the eye, and my nose feels
grams may be advised for these patients. Very
congested. I am told my eye looks red.” Occasionally, rarely, in well-selected patients, surgical ablation

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PRIMARY AND SECONDARY HEADACHE

Figure 18-4 Cluster Headache and Chronic Paroxysmal Hernicrania


Cluster headache

Temporal artery
bulging and pulsating
Severe headache,
pain behind eye
Unilateral ptosis,
swelling, and redness
of eyelid
Miosis, conjunctival
injection
Tearing

Nasal congestion,
rhinorrhea
Flushing of side
of face, sweating

Large, strong, muscular man typical


patient. Face may have peau d’orange
skin, telangiectasis.

Attacks typically nocturnal; average


frequency 1-3 in 24 hours, lasting
15 minutes-3 hours Chronic paroxysmal hemicrania (CHP)

Attacks typically nocturnal;


average frequency 10-30 in
24 hours, lasting 5-20 minutes

CO C1

Flexion and rotation N CH3


of neck may
precipitate attack.

CH3O CH2COOH

Typical patient is woman (30-40 y). Symptoms of Diagnostic for CHP, prompt
unilateral headache (lacrimation, rhinorrhea, and miosis), and absolute response to
which are common in cluster, are absent in CHP. indomethacin

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PRIMARY AND SECONDARY HEADACHE

or radiofrequency rhizotomy of the trigeminal for abuse are analgesics, ergotamine, barbitu-
nerve offer other options. rates, and opiates.
Patient education is of utmost importance. The
Tension Headache patient must understand the pathophysiologic
Tension headache is the most common mechanisms and agree to cooperate if treatment
headache type. In 1988, the Headache Classifica- is to succeed. Step 1 is withdrawal of the offend-
tion Committee of the International Headache So- ing medication. Patient support mechanisms to
ciety determined that a diagnosis of tension control anxiety and a management plan for
headache requires the presence of at least 2 of the headache recurrence are needed. Hospitaliza-
following pain characteristics: a nonpulsating tion with a series of IV dihydroergotamine treat-
quality, mild or moderate nondisabling intensity, ments may be necessary for short-term pain con-
bilateral location, and no aggravation by routine trol. Comorbid etiologic factors—depression,
physical activity. In addition, these patients do not psychosocial stresses, and poor sleep—require at-
experience nausea or vomiting and have no pho- tention. Follow-up prophylactic treatments for
tophobia or phonophobia. Tension headaches both groups may be nonpharmacologic, includ-
last fewer than 7 days. ing emotional support, counseling, physical
Careful evaluation is indicated in every patient therapy, relaxation exercises, heat, or massage.
suspected of having tension headache. Exclusion Antidepressants often provide the key pharma-
of structural, infectious, or metabolic disorders is cologic treatment.
essential. Although sometimes features of mi-
graine are present, they are a minor part of the Paroxysmal Hemicranias
clinical picture. Specific triggering factors are also These are unusual primary headaches, unilat-
less common than with migraine. Chronic tension eral and short lived (⬍1 hour), which occur in a
headaches are diagnosed when they recur more chronic or episodic manner. Typically, the pain
than 15 times per month. The precise pathophys- has a severe throbbing or boring quality and often
iology is unknown. It is probably a heterogeneous recurs during the same day. These headaches are
disorder with various etiologic factors, including associated with ipsilateral cranial autonomic dys-
pericranial muscular tension (Figure 18-5), oro- function but occur more often in women than
mandibular dysfunction, psychosocial stress, anx- men. Furthermore, these headaches have a
iety, depression, and analgesic drug overuse. Ten- greater daily recurrence and less of a cluster pat-
sion headache treatment usually requires only tern than cluster headaches. They usually re-
over-the-counter analgesics, including NSAIDs, spond well to 25 to 50 mg indomethacin 3 times
and nonpharmacologic intervention, including daily.
relaxation techniques, massage, or heat applica-
tion. Prophylactic medication is indicated for fre- Hypnic Headaches
quent recurrence. Headaches of this type tend to occur in the el-
derly population and affect women more than
Chronic Daily Headaches men, particularly at night, waking patients during
Some individuals experience frequent tension REM stages of sleep. Such headaches are gener-
(“rebound”) headaches. If they recur more than alized, throbbing, and without autonomic fea-
15 days per month, the diagnosis of chronic ten- tures. They may last up to 1 hour and may recur
sion-type headache or chronic daily headaches through the night.
applies. Patients with migraines recurring more
than 15 days per month are said to have either Cold Stimulus Headaches
chronic migraine or transformed migraine. Many These are the classic “ice cream headaches.”
patients in these 2 clinical groups repeatedly and Typically, these benign headaches occur during
inappropriately use acute headache medica- the rapid ingestion of cold food or drink, are lo-
tions for longer than 3 months, complicating cated in the forehead, and subside in a few min-
treatment. Medications known to have potential utes without medication.

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PRIMARY AND SECONDARY HEADACHE

Figure 18-5 Muscle Contraction Headache


Soreness of scalp; pain
on combing hair
Intermittent, recurrent, or constant head Occipital
pain, often in forehead, temples, or back tension
of head and neck; commonly described
as “bandlike,” “tightness,” or “viselike”

Bandlike
constriction
Temporal tightness
or pressure

Pressure on
contracted
muscle may Rigidity
augment pain. of neck

Psychogenic factors:
emotional conflict
and depression
often seen
Sleep disturbances common; in chronic
diurnal incidence: headache headache
occurs most often between
4 and 8 AM and 4 and 8 PM

Local trigger factors of muscle


contraction headache

Muscle spasm

Muscle spasm

Temporomandibular
joint dysfunction
Cervical spine
osteoarthritis

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PRIMARY AND SECONDARY HEADACHE

Benign Exertional Headaches sential to patient management. Often, immedi-


Physical exercise is always the precipitating ate laboratory, neuroradiologic testing, or both
factor in benign exertional headaches. They may are crucial.
develop during acute straining (eg, weight lift- Whether this approach applies to every pa-
ing) or after sustained exercise such as running. tient with headaches is debatable. However,
These headaches are usually generalized and with the widespread availability and relatively in-
throbbing and often respond to 25 to 50 mg in- expensive nature of brain CT, it is judicious to im-
domethacin 3 times daily taken either after the age patients who have experienced recent onset
exercise or prophylactically once a typical pat- of a significant headache, including those with
tern is established. ␤ Blockers may also be used. normal clinical examinations. Significant neuro-
However, the possibility of serious disorders logic disorders, such as subarachnoid hemor-
such as arterial dissection, aneurysmal rupture, rhage in all age groups, a third ventricular cyst in
and even an intracranial mass must be excluded. younger individuals, or subdural hematomas in
CT, MRI, and MRA may be appropriate. the elderly, provide examples wherein brain CT
may diagnose serious neurologic conditions
Benign Cough Headaches when the most careful neurologic examinations
These brief, generalized headaches typically are normal.
occur in an older population and may represent
another variety of exertional headaches. They re- Giant Cell (Temporal) Arteritis
spond to 25 to 50 mg indomethacin 3 times
daily. Clinical Vignette
A 70-year-old man reported a recent onset of mild,
Coital Headaches generalized headaches. The patient’s history further re-
Typically, these headaches develop as dull, gen- vealed some malaise, slight fever, and weight loss. He
eralized pain during sexual activity or as an acute had significant blurred vision, affecting his ability to read
severe pain during orgasm. As with other parox- with his left eye. Fortunately, his eyesight returned fully
ysmal headaches, such as cough or exertional within 20 minutes. This led him to seek medical atten-
tion. Questionable tenderness over the temporal arter-
types, a search for underlying systemic or in- ies was noted on neurologic examination, the results of
tracranial pathology, particularly a ruptured which were otherwise normal. A temporal artery biopsy
aneurysm, is needed before diagnosing a benign demonstrated the classic findings of giant cell arteritis.
process. Administering 25 to 50 mg in- The symptoms resolved with treatment with corticos-
domethacin 3 times daily is useful for many indi- teroids in gradually diminishing doses during the next 8
months.
viduals if ingested before the activity.
Headache is the most common and promi-
SECONDARY HEADACHE nent presenting symptom of giant cell arteritis,
DISORDERS also called temporal arteritis, a serious disorder
Although most headaches occur in the ab- in the elderly with potentially major complica-
sence of underlying intracranial or systemic tions, particularly permanent blindness. The pa-
pathology, some result from more serious illness. tient in the above vignette is a classic example
The neurologist is often the initial physician con- whose early identification and treatment pre-
tacted by such patients. Because of the painful vent blindness from developing. The pain is usu-
nature of the headaches, the clinical presenta- ally nonspecific, throbbing or continuous, and
tion may be the patient’s only expressed con- sometimes so mild that its potential significance
cern. A careful history is mandatory to achieve is easily overlooked. These patients may have
an accurate diagnosis and eventual patient man- subtle and intermittent visual blurring early in the
agement. The temporal profile and age at illness (Figure 18-6). Systemic complaints includ-
headache onset, sex, family history, precipitating ing general malaise, myalgias, and arthralgias are
factors (triggers), pain character, and associated common, frequently providing important diag-
symptoms require analysis. The history is fol- nostic clues. Although often present, temporal
lowed by neurologic examination and testing es- artery tenderness may be relatively minor.

HEADACHE
179
PRIMARY AND SECONDARY HEADACHE

Figure 18-6 Giant-Cell (Temporal) Arteritis,


Polymyalgia Rheumatica

Temporal cephalalgia,
Pain on chewing scalp tenderness

Loss of weight, Visual disturbances


weakness Blindness may
develop rapidly.

Low-grade fever,
malaise

Symmetric pain
and stiffness of
shoulder and hip
girdle muscles
Anterior ischemic optic neuropathy

Elevated
sedimentation
rate

Hypochromic
anemia

Biopsy specimen of superficial temporal artery: almost total


obliteration of lumen with some recanalization. High-power
Rigid, tender, nonpulsating temporal insert shows infiltration with lymphocytes, plasma cells, and giant
arteries may be visible or palpable. cells; fragmentation of elastica.

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PRIMARY AND SECONDARY HEADACHE

Early diagnosis is paramount because the ar- gency imaging is also needed: CT, possibly with
teritis may precipitously cause unilateral or bilat- MRI or MRA if there is concern about hemor-
eral permanent visual loss. Furthermore, the ar- rhage. If these are normal and no evidence of a
teritis may be widespread with involvement mass lesion exists, a CSF analysis is indicated to
beyond the temporal arteries to the extracere- check for a subarachnoid hemorrhage or signs of
bral vertebral artery with an uncommon poten- an infectious process (chapters 24, 63, and 64).
tial for posterior circulation stroke.
ESR and C-reactive protein evaluation pro- Idiopathic Intracranial Hypertension
vides the laboratory means supporting this po- (Pseudotumor Cerebri)
tential diagnosis. Typically, the ESR is significantly Considered a nonspecific headache syn-
increased to 60 to 110 mm/h, although there are drome, pseudotumor cerebri is usually associ-
rare exceptions. Biopsy of a long temporal artery ated with diplopia, papilledema, and an associ-
segment is indicated in every patient suspected ated otherwise benign increased intracranial
of having temporal arteritis. Because temporal CSF pressure. Typical patients are young, mor-
arteritis is a chronic disorder, it requires relatively bidly obese, otherwise healthy women. Neu-
long-term corticosteroid treatment. Prompt diag- roimaging studies are mandatory to exclude the
nosis and treatment initiation are required to other possible causes of increased intracranial
prevent serious complications. Treatment must pressure (chapter 71).
not be delayed by the biopsy. Corticosteroid
treatment does not alter the pathologic findings Low CSF Pressure Headache
if the biopsy is performed within a few days of
therapy initiation. The usual treatment of giant Clinical Vignette
cell arteritis is oral corticosteroids. The initial A vigorous, healthy, 61-year-old physician noted the
dose is 40 to 60 mg/d prednisone, continued for onset of progressively severe headaches over a 3-week
1 to 2 years at smaller doses. period. These were precipitated by going down stairs,
straining, and sneezing and were particularly excruciat-
ing while landing on a lake in a float plane. This jarring
Brain Hemorrhage, Infections, and Tumors
produced such severe pressurelike headaches each
Subdural, intracerebral hematoma; subarach- time the plane bounced on the water surface that this
noid hemorrhage; meningitis; and brain tumors gentleman chose to seek further diagnostic evaluation.
are important causes of secondary headaches. Until then, he had ignored the initial headaches be-
Each of these entities needs consideration in pa- cause he was in otherwise excellent health. His neuro-
logic examination was normal despite his severe pain
tients who experience the recent onset of a new,
each time he bent forward.
different type, or changing pattern of headaches.
The results of initial MRI studies were unremarkable;
Each of these important disorders is discussed however, there was marked leptomeningeal enhance-
elsewhere in this text; however, a few comments ment after the administration of gadolinium. CSF
are warranted here. demonstrated RBCs and WBCs with a slightly in-
Every individual who has a precipitous onset creased protein level. Cytology results were normal.
of “the worst headache of my life” warrants a The results of intracerebral and cervical MRA studies
were normal.
careful evaluation in the ED, or the neurologist’s
or primary care physician’s office. A careful gen- The headache that occurs subsequent to a
eral evaluation is essential, including whether lumbar puncture for CSF analysis or spinal anes-
the patient is febrile. A neurologic examination thesia is the classic example of a low CSF pres-
should evaluate mental status, language func- sure headache. Occasionally, a similar headache
tion, muscle stretch reflexes, and plantar stimula- occurs subsequent to a closed head injury such
tion for Babinski signs and should search for as walking into a low doorway or from a contact-
signs of meningismus, pupillary changes, or ex- sport injury. Typically, the patient does not im-
traocular muscle palsies. A careful funduscopic mediately associate the onset of the pain with
examination should evaluate for increased in- the previous trauma. Therefore, this history is not
tracranial pressure, papilledema, or subhyaloid recalled until detailed inquiry by the suspicious
hemorrhages. Regardless of the findings, emer- physician based on headache characteristics.

HEADACHE
181
PRIMARY AND SECONDARY HEADACHE

Typically, these headaches develop soon after Trigeminal Neuralgia


the spinal tap, although they may be less precip-
itous after an injury. Generally, they occur during
Clinical Vignette
the waking hours and worsen when the patient
assumes the upright posture, clearing almost A 48-year-old woman developed electrical shock-
completely with recumbence. Often, the like attacks of intense pain in the left cheek. They usu-
ally came without warning but were often precipitated
headache is associated with nausea, vomiting,
by talking, chewing, or washing her face or applying
and neck pain, which all clear on recumbence. lipstick to the left upper lip. Between the attacks, she
Usually, the headache subsides with bed rest in a was free of pain. Neurologic examination was normal.
few days, but it can persist. A blood patch in- Treatment with 600 mg/d carbamazepine was initi-
jected at the lumbar puncture site, sealing the ated. This initially helped; however, a relapse of pain re-
quired increasing dosages to 1000 mg/d. Although her
leak, almost universally improves post–lumbar pain was now controlled, she became lethargic and
puncture headaches, providing credence to the forgetful with these higher medication levels.
theory that they are secondary to a CSF leak. Al- A neurosurgeon was consulted, and microvascular
though leptomeningeal enhancement is classi- decompression was advised. At craniotomy, an ectatic
cally seen with either a malignancy or infectious loop of the superior cerebellar artery was found
infiltration, the relatively smooth characteristics markedly compressing the trigeminal posterior root.
The artery was gently displaced from the vicinity of the
of the findings in the preceding vignette were nerve and a cushion of Teflon placed between the
typical for a benign dural leak. A diagnosis of pre- artery and the nerve. Postoperatively, she experienced
sumed traumatic leptomeningeal tear with CSF significant relief, stopped carbamazepine, and re-
leak and subsequent low-pressure syndrome gained her former quality of life.
was made. A therapeutic trial of an autologous Five years later, her pain recurred. Although initially
blood dural patch was successful, with relief of mild, it again became severe. After neurosurgical con-
sultation, a percutaneous radiofrequency lesion was
all symptoms within 1 week. performed. This provided pain relief with an accompa-
The chronic type of low CSF pressure headache nying mild sensory loss in the left cheek and lips. Her
usually has a less obvious and ill-defined initiating lasting relief of pain was extremely welcome.
event. Operative spinal procedures, a Valsalva
A careful history is the key to the diagnosis of
test, or even coughing are known to precipitate
this uncommon but eminently treatable facial neu-
this condition. Unfortunately, many of the provok- ralgia. Trigeminal neuralgia, also called tic
ing events seem minor to the patient and are for- douloureux, is a disabling, lancinating, or electrical-
gotten. like facial pain that occurs in the trigeminal nerve
MRI with gadolinium demonstrates diffuse distribution (Figure 18-7). It is one of the worst
pachymeningeal enhancement in most low CSF pains humans experience. This condition is not
pressure headaches. This can be striking and defined by any test, requires that the clinician
may be confused with leptomeningeal inflam- recognize the entity, and is diagnosed by its pri-
matory or neoplastic processes. Bed rest is the mary historical attributes. The patient, almost al-
initial treatment. Caffeine may help. A search for ways an adult and often a senior citizen, experi-
a CSF leak and its specific treatment is some- ences paroxysmal and frequently provokable
times necessary. Sometimes, a blood patch in intermittent unilateral pain that rarely occurs dur-
the lumbar spine can provide relief in these indi- ing sleep. It primarily involves the second or third
viduals, despite no identifiable source. Occa- divisions of the trigeminal nerve and occasion-
sionally, such headaches are resistant to therapy ally the first division. Characteristically, it is pro-
and become disabling. voked by talking, chewing, shaving, drinking hot
or cold liquids, or any form of sensory facial stim-
Cranial Neuralgias ulation. The pain is invariably unilateral; when it
This group of patients experience brief but ex- affects both sides of the face, it does not do so
ceedingly severe paroxysms of head pain in the concomitantly.
distribution of a specific cranial nerve, particu- In perhaps the majority of individuals, an idio-
larly the trigeminal. pathic loss of myelin insulation within the poste-

HEADACHE
182
PRIMARY AND SECONDARY HEADACHE

Figure 18-7 Cutaneous Nerves of Head and Neck

From ophthalmic division Auricular branch


of trigeminal nerve (V1) of vagus nerve (X)

Supraorbital nerve
Supratrochlear nerve
Medial branches
Palpebral branch of of dorsal rami
lacrimal nerve of cervical spinal
Infratrochlear nerve
External nasal branch of Greater occipital
anterior ethmoidal nerve nerve (C2)
3rd occipital
nerve (C3)
From 4th, 5th
From maxillary division 6th, and 7th
of trigeminal nerve (V2) nerves in
Infraorbital nerve succession
below
Zygomaticofacial nerve

Zygomaticotemporal nerve
Branches from
cervical plexus
Lesser occipital
From mandibular division nerve (C2, 3)
of trigeminal nerve (V3) Great auricular
Mental nerve nerve (C2, 3)
Transverse cervical
Buccal nerve nerve (C2, 3)
Auriculotemporal nerve Supraclavicular
nerves (C3, 4)

Dorsal rami of
Ophthalmic nerve (V1) cervical spinal
nerves

Note: Auricular branch


of vagus nerve to
Trigeminal external acoustic
nerve (V) Maxillary nerve (V2)
meatus and small
area on posteromedial

Branches from
Mandibular nerve (V3) cervical plexus

HEADACHE
183
PRIMARY AND SECONDARY HEADACHE

Figure 18-8 Suboccipital Triangle


Epicranial aponeurosis (galea aponeurotica) Rectus capitis posterior minor muscle

Occipital belly (occipitalis) of Rectus capitis posterior major muscle


occipitofrontalis muscle
Semispinalis capitis muscle
(cut and reflected)
Greater occipital nerve (dorsal
ramus of C2 spinal nerve) Vertebral artery (atlantic part)

Obliquus capitis superior


muscle
Occipital artery Suboccipital nerve
(dorsal ramus of C1
spinal nerve)
Posterior arch of
3rd (least) occipital atlas (C1 vertebra)
nerve (dorsal ramus of
C3 spinal nerve)
Occipital artery

Obliquus capitis
inferior muscle
Semispinalis capitis
and splenius capitis Greater occipital
muscles in posterior nerve (dorsal ramus
triangle of neck of C2 spinal nerve)

Splenius capitis
Posterior muscle (cut and
auricular reflected)
artery
3rd (least) occipital
nerve (dorsal ramus
Great auricular nerve of C3 spinal nerve)
(cervical plexus C2, 3)

Lesser occipital nerve


(cervical plexus C2, 3) Longissimus capitis muscle

Splenius cervicis muscle


Sternocleidomastoid
muscle
Semispinalis cervicis muscle

Trapezius muscle

Semispinalis capitis muscle (cut)


Posterior cutaneous branches
of dorsal rami of C4, 5, 6
spinal nerves Splenius capitis muscle (cut)

HEADACHE
184
PRIMARY AND SECONDARY HEADACHE

rior root of the trigeminal nerve causes the pain. in the trigeminal zone, including pain over the
When it occurs in a young adult, the primary de- top of the head, on the side of the neck, and be-
myelination of multiple sclerosis is often the hind the ear are not trigeminal neuralgia and will
mechanism. Another cause is an artery that has not be effectively treated by these procedures.
lengthened during adult life and become tortu- Knowledge of the anatomy of the trigeminal
ous. An ectatic loop of such an artery, usually a nerve and its facial distribution and an apprecia-
branch of the superior cerebellar artery, com- tion of the paroxysmal, provokable, and unilat-
presses the trigeminal posterior root causing the eral character of trigeminal neuralgia is essential
pain. Rarely, trigeminal neuralgia results from for accurate diagnosis and initiation of therapy.
other conditions causing pressure on the trigem-
inal nerve, including acoustic neuroma, menin- Glossopharyngeal Neuralgia
gioma, arteriovenous malformation, or an Unlike trigeminal neuralgia, glossopharyngeal
aneurysm pressing on the trigeminal posterior neuralgia is rare. The pain is paroxysmal, recur-
root. These various etiologies suggest that exter- rent, severe, and generally located in the tonsillar
nal pressure on the root or a primary or sec- area, deep within the throat in the sensory distri-
ondary form of demyelination (eg, multiple scle- bution of CN-IX. Swallowing is usually the pri-
rosis) may cause trigeminal neuralgia. Mass mary triggering mechanism. Occasionally, pa-
lesions of the gasserian (trigeminal) ganglion tients experience bradycardia during the pain
need to be excluded via MRI. paroxysms, sometimes causing loss of con-
Several treatments are available. Most pa- sciousness. Medical treatment is similar to that
tients respond initially to the use of carba- for trigeminal neuralgia. Rhizotomy or decom-
mazepine, which stabilizes cell membranes and pression of CN-IX may be necessary in severe,
raises the threshold of neural stimulation. Pheny- medically intractable cases.
toin, gabapentin, oxcarbazepine, and baclofen
may be useful. In many individuals, adverse ef- Occipital Neuralgia
fects of these therapies—lethargy, loss of mental Occipital neuralgia is the least common of the
acuity, drowsiness, hematologic effects and oth- cranial neuralgias. Similar in pain characteristics
ers—are troublesome. Several surgical ap- to trigeminal neuralgia, it is differentiated by lo-
proaches are available for patients who cannot cation to the unilateral posterior scalp inner-
tolerate medical therapy: trigeminal radiofre- vated by the second cervical dermatome (C2)
quency lesioning by percutaneous electrode (Figure 18-8).
technique, microvascular decompression in the
posterior cranial fossa (which can often relieve Infectious Mechanisms
the tic pain with less sensory loss than that of the Meningitis is an infectious disease process
radiofrequency lesion), open trigeminal rhizo- that must be considered in anyone having an
tomy in the posterior cranial fossa, and a form of acute-onset headache. Typically, these individu-
focused beam radiation by gamma knife ther- als have a concomitant fever and stiff neck
apy. (meningismus) as detailed in chapter 63.
Trigeminal neuralgia can recur after any pro- Herpes zoster (shingles) is a viral process that
cedure and does so at a lifetime rate of 15% to is a “reactivation” of the virus within the gasser-
20%. Recurrences can be treated with further or ian ganglion. It is a relatively rare etiology for an
subsequent radiofrequency ablation. An ipsilat- acute-onset severe headache. Typically, these
eral hearing loss is the occasional adverse effect patients report a severe, sometimes excruciat-
of the decompressive surgery, relating to the del- ing, boring head pain or neuralgia. Usually, the
icate anatomic relationship between the audi- rash precedes the onset of the headache, but, in
tory and trigeminal nerves. The operation carries other instances, intense head pain can antedate
a 1% risk of death or stroke. the skin lesions by a few days. The classic vesi-
Accurate diagnosis is essential to successful cles can sometimes vary from very few easily
surgical relief. Steady pain, posttraumatic pain, overlooked lesions to an extensive vesicular
pain after dental procedures, and pain that is not rash. After the dermatologic changes appear,

HEADACHE
185
PRIMARY AND SECONDARY HEADACHE

treatment with acyclovir is needed. There is an cus to later provide a source for bacterial menin-
increased incidence in patients who are im- gitis. Diagnosis depends on CT, MRI, or both.
munosuppressed, those with AIDS or receiving Appropriate antibiotic treatment is usually ef-
immunosuppressive medications, and those fective.
with underlying neoplasms, particularly lym-
phoma. Dental Infection
An abscessed tooth, primarily in the upper
Contiguous Structure Headaches jaw, is a relatively common cause of facial and
The last group of head pain etiologies is not head pain. Although the diagnosis is usually ob-
secondary to inherent brain, vascular, and lep- vious to patients, on occasion they may not ini-
tomeningeal structures as discussed in the sec- tially seek a dental opinion but come to a physi-
tions on tumors, stroke, or neuralgias. Con- cian first. Sometimes, the evaluation by the
tiguous anatomical structures such as the nasal dentist does not uncover the diseased tooth.
sinuses or teeth may be responsible. Therefore, the neurologist must consider a pri-
mary dental source as an unusual cause for
Nasal Sinus Infection some instances of head and facial pain. A careful
Often, a patient with headache presents with dental evaluation may be diagnostic when no
a self-diagnosis of “sinus headaches.” This entity other mechanism is identified by the neurologic
must be considered in each patient presenting physician.
with headache. Most “sinus headaches” repre-
sent a variety of tension headache. REFERENCES
The typical patient with active nasal sinus in- Cady R, Dodick DW. Diagnosis and treatment of migraine.
Mayo Clin Proc. 2002;77:266-261.
fection experiences a deep boring discomfort in Headache Classification Committee of the International
the maxillary or ethmoid facial region, or both. Headache Society. Classification and diagnostic criteria
With acute infections, there is often percussion for headache disorders, cranial neuralgia, and facial pain.
tenderness, a purulent nasal discharge, and, if Cephalalgia. 1988;8(suppl 7):1-96.
Olesen J, Tfelt-Hansen P, Welch KMA. The Headaches. 2nd
the infection is severe, fever. In contrast, sphe-
ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2000.
noid sinus infection is more easily masked, pre- Silberstein SD, Lipton RB, Dalessio DJ. Wolff’s Headache and
senting with only a deep-seated headache, and Other Head Pain. 7th ed. Oxford, England: Oxford Uni-
poses the greatest risk for a parameningeal fo- versity Press; 2001.

HEADACHE
186
Chapter 19

Arterial Supply to the


Brain and Meninges
Aaron C. Heide and Claudia J. Chaves

THE CAROTID ARTERY SYSTEM


The brain and meninges are supplied by arteries derived from the common carotid artery (CCA)
and vertebrobasilar system. The right CCA usually originates from the brachiocephalic trunk, while the
left CCA originates directly from the aortic arch. Both vertebral arteries (VAs) originate from the sub-
clavian arteries. The morphologic variants of the CCA and VAs usually are not clinically significant.
The CCA bifurcates at approximately the level headaches in the elderly because, if it goes un-
of the sixth cervical vertebrae into the external treated, it may lead to blindness (chapter 18).
and internal carotid arteries. The external carotid The maxillary artery supplies the face and
artery (ECA) supplies the neck, face, and scalp. through its middle meningeal branch provides
The internal carotid artery (ICA) and its branches most of the blood supply to the dura mater cov-
are mostly responsible for the arterial supply of ering the brain. The middle meningeal artery is
the anterior two thirds of the brain (anterior cir- often implicated in the formation of epidural
culation). hematomas in patients with skull fracture.
The vertebrobasilar and posterior cerebral ar- The ECA occasionally has an important role by
teries (PCAs) supply blood to the brainstem, supplying collateral flow in ICA occlusive dis-
cerebellum, occipital lobes, and posterior por- ease. Variable anastomoses develop between its
tions of the temporal and parietal lobes (poste- branches (facial, maxillary, and superficial tem-
rior circulation) (Figure 19-1). poral arteries) and the ophthalmic artery.

External Carotid Artery Internal Carotid Artery


At its origin, the ECA deviates anteriorly and There are 4 ICA segments: cervical, petrous, cav-
medially in relation to the ICA in the neck, pro- ernous, and supraclinoid. The cervical segment as-
viding many branches to the neck (superior thy- cends vertically in the neck, posterior and slightly
roid, ascending pharyngeal arteries) and face medial to the ECA. Significant atherosclerotic dis-
(lingual and facial arteries). As the artery as- ease is usually located at the ICA origin, with po-
cends, occipital and posterior auricular branches tential for artery-to-artery embolism, stenosis with
supply the scalp in the occipital region and be- eventual occlusion, or both. This segment does not
hind the ear, respectively. The occipital artery have branches, unlike the ECA, allowing differenti-
also has several meningeal branches that supply ation between those vessels on imaging scans.
the meninges of the posterior fossa and dura. The ICA enters the skull through the carotid
Within the substance of the parotid gland, the canal within the petrous bone. This petrous seg-
ECA divides into its 2 terminal branches: the su- ment ICA has 2 small branches, the caroticotym-
perficial temporal and maxillary arteries. panic and pterygoid branches, which are usually
The superficial temporal artery is the main sup- clinically irrelevant.
ply to the scalp over the frontoparietal convexity The cavernous segment, usually called the
and its underlying muscles. The more proximal carotid siphon because of its shape, is the por-
branches also supply the masseter muscle. The su- tion of the ICA within the cavernous sinus. Of its
perficial temporal artery is commonly involved in many branches, the ophthalmic artery is the
giant cell arteritis, an important consideration for most significant. The ophthalmic artery arises

CEREBROVASCULAR DISORDERS
188
ARTERIAL SUPPLY TO THE BRAIN AND MENINGES

Figure 19-1 Arteries to Brain and Meninges

L. middle meningeal a. R. and I. middle cerebral aa.


R. and I. post. cerebral aa. R. and I. ant. cerebral aa.
R. and I. sup. cerebellar aa. Ant. communicating a.

Basilar a.
R. ophthalmic a.
Mastoid
branch of
R. post.
I. occipital a.
communicating a.
L. int.
Cavernous
auditory
sinus
(laby-
rinthine) a.

Post.
meningeal
branch of I. R. deep
ascending temporal a.
pharyngeal a. R.
maxillary a.
R. and I. ant. inf.
cerebellar aa. R. middle
meningeal a.
R. and I. post.
inf. cerebellar aa. R. superficial
temporal a.
Post. meningeal branches
R. ext.
of r. and I. vertebral aa.
carotid a.
Ant. meningeal branch R. facial a.
of r. vertebral a.
R. lingual a.
R. post. auricular a.
Carotid body
R. occipital a. R. sup. laryngeal a.
R. int. carotid a.
Thyroid cartilage
R. ascending
pharyngeal a. R. sup. thyroid a.
R. carotid sinus
R. common carotid a.
R. vertebral a.
R. inf. thyroid a.
Transverse process of C6
R. int. thoracic a.
R. deep cervical a.
Brachiocephalic trunk
R. thyrocervical trunk

R. costocervical trunk

R. subclavian a.

CEREBROVASCULAR DISORDERS
189
ARTERIAL SUPPLY TO THE BRAIN AND MENINGES

from the ICA just before it emerges from the cav- which connects both A1 segments and consti-
ernous sinus and passes through the optic canal tutes an important collateral pathway in carotid
into the orbit just below and lateral to the optic artery occlusive disease. It provides an opportu-
nerve. It supplies the globe and orbital contents nity for cross-flow from the opposite side toward
through its 3 major branches: the ocular (central the hypoperfused hemisphere. Occasionally,
retinal and ciliary arteries), orbital, and extraor- only a single A1 exists where one ICA supplies
bital branches. The ophthalmic artery forms ex- both ACAs, termed an azygous ACA. The recur-
tensive anastomoses with branches of the ECA, rent artery of Heubner is the most important
which become an important source of collateral branch of the A1 segment, which supplies the
flow in ICA occlusive disease. anteroinferior portion of the head of the caudate
The supraclinoid segment is the last portion of nucleus, the putamen, and the anterior limb of
the ICA. It begins when this segment penetrates the internal capsule.
the dura. The posterior communicating artery The ACA continues as the A2 segment, where
(P-com) and the anterior choroidal artery are the orbitofrontal branch arises and travels
the 2 important branches originating at this level. around the genu of the corpus callosum to the
The ICA then bifurcates into the anterior cere- orbital and medial surface of the frontal lobe,
bral artery (ACA) and middle cerebral artery and the frontopolar branch supplies the rest of
(MCA). the medial surface of the frontal lobe. The ACA
The P-com is often hypoplastic. When present, then gives off its 2 major branches, the perical-
it travels posteriorly to communicate with the losal artery that runs just above the corpus callo-
posterior circulation at the level of the PCA. The sum and the callosomarginal artery that outlines
P-com also provides the thalamoperforate the cingulate gyrus. These 2 arteries supply the
branches that supply the anterior and medial mesial portions of the frontal and parietal lobes.
thalamus, parts of the cerebral peduncles, and One of the major fail-safe systems within the
important collateral pathways in extensive verte- cerebral circulation is the circle of Willis, formed
brobasilar disease or carotid artery occlusive le- by the confluence of both ACAs, the anterior
sions allowing collateral flow from the anterior to communicating artery, the cavernous carotid,
the posterior circulation or vice versa. the P-coms, and both PCAs. When a single ves-
The anterior choroidal artery arises from the sel becomes significantly diseased even with to-
posterior surface of the ICA just above the P-com tal occlusion, ie, 1 cervical ICA, this vascular net-
origin. This artery supplies an extensive cerebral work sometimes provides a viable option for
area, including the visual system (optic tract, por- ongoing perfusion without development of a
tions of the lateral geniculate body, optic radia- cerebral infarction. Each of these vessels, at their
tions), genu and posterior limb of the internal respective junctions and origins, are primary
capsule, basal ganglia (medial globus pallidus, sites for the origin of a berry aneurysm, the ma-
tail of the caudate), the diencephalon (portions jor source for development of subarachnoid he-
of the lateral thalamus and subthalamus), the morrhage.
midbrain (substantia nigra and portions of the The MCA originates from the supraclinoid
cerebral peduncle), the medial temporal lobe carotid stem and is a classic site for large-sized
(uncus, pyriform cortex, amygdala), and the cerebral artery emboli to lodge, sometimes
choroidal plexus of the temporal horn and amenable to emergent intraarterial thrombolytic
atrium. therapy. Subsequently, it travels laterally as the
The ACA travels medially and anteriorly to- main-stem M1 segment, giving off lenticulostri-
ward the interhemispheric fissure. It supplies the ate branches to the basal ganglia. As the MCA
anterior portions of the basal ganglia and inter- approaches the sylvian fissure, it usually divides
nal capsule and most of the mesial portions of into 2 large trunks, the superior and inferior divi-
the frontal and parietal lobes. sions. Occasionally, the MCA trifurcates, and a
The first segment of the ACA, the A1 segment, middle trunk is also present. Different branches
begins at the carotid bifurcation, terminating at supply the frontal (orbitofrontal, ascending
the level of the anterior communicating artery, frontal, precentral, and central branches), pari-

CEREBROVASCULAR DISORDERS
190
ARTERIAL SUPPLY TO THE BRAIN AND MENINGES

etal (anterior and posterior parietal and angular spinal arteries arise from the posterior-inferior
branches), and temporal (anterior and posterior cerebellar arteries or intracranial VAs and run
temporal) lobes. The orbitofrontal, ascending downward along the posterior and lateral aspect
frontal, precentral, and central branches usually of the spinal cord, supplying these cord areas.
arise from the superior division of the MCA, Often, asymmetry between VAs is seen, with
while the angular, anterior, and posterior tempo- deviation of the basilar artery toward the hy-
ral branches arise from the inferior division. The poplastic side. The basilar artery courses ros-
anterior and posterior parietal branches can trally on the anterior surface of the pons, and
arise from either division (Figures 19-2 ). along the clivus. It is particularly prone to ather-
osclerotic deposition throughout its length,
VERTEBROBASILAR ARTERIES which, at its extreme can cause formation of a
The VAs originate from the subclavian arteries fusiform aneurysm. When the basilar artery
(Figure 19-2). They have 2 portions, extracranial reaches the level of the cerebral peduncles, it ter-
and intracranial. After their origin, the extracra- minates by dividing into the 2 PCAs. At this site,
nial VAs travel posteriorly and enter the trans- the basilar is most likely to be occluded by an
verse foramen of the cervical vertebrae, usually embolus leading to the classic “top of the basi-
through C6 and extending superiorly to C1. This lar” syndrome. Similarly, this is the most com-
area is the most likely site of VA dissection. At the mon site for berry aneurysms within the verte-
atlas, the VAs turn sharply posteriorly before brobasilar system.
traveling rostrally, piercing the posterior atlanto- The most important branches of the basilar
occipital membrane and dura mater to enter the artery are the anterior inferior cerebellar arter-
intracranial cavity through the foramen mag- ies, superior cerebellar arteries (SCAs), and the
num; this segment is occasionally affected by paramedian arteries. The anterior inferior cere-
temporal arteritis. bellar arteries usually arise from the midportion
The intracranial VAs course anteriorly, first lat- of the basilar artery and supply the brachium
eral to the medulla, then ventral to the hypoglos- pontis, lateral pontine tegmentum, flocculus,
sal nerve to the midline base of the brainstem. At and anteroinferior portions of the cerebellum.
the pontomedullary junction, the confluence of Often, the internal auditory artery arises from
the 2 VAs forms the basilar artery (Figure 19-3). the anterior inferior cerebellar arteries and sup-
The lateral medullary branches, posterior-in- plies the vestibular and cochlear structures. The
ferior cerebellar arteries, and anterior and pos- SCAs arise from the distal portion of the basilar
terior spinal arteries are the most important VA artery before it bifurcates into the PCAs. During
branches. The medullary arteries supply the lat- their course around the midbrain, the SCAs pro-
eral portions of the medulla. The posterior-infe- vide branches that supply the superior lateral
rior cerebellar arteries primarily supply the pos- pontine tegmentum and the tectum of the mid-
terior and inferior regions of the cerebellum but brain. Then, the SCAs course toward the cere-
also a small portion of most of the dorsum of the bellum, supplying the superior vermis and lateral
medulla. It is the most common site for embolic portion of the cerebellar hemispheres. The SCA
occlusion within this group of vessels, resulting also irrigates most of the cerebellar nuclei and
in classic Wallenberg syndrome. the majority of the cerebellar white matter.
The anterior spinal artery is often a single ves- The PCA supplies portions of the temporal,
sel running the full length of the spinal cord in the parietal, and occipital lobes; and the thalamus,
anteromedial sulcus. It also supplies the medial midbrain, and other deep structures, including
portions of the medulla; however, adequate col- the choroid plexus and ependyma of the third
lateral circulation in this anatomic substrate must and lateral ventricles.
exist, because primary clinically apparent medial
medullary infarction is rare. In contrast, the ante- CEREBRAL SINUSES AND VEINS
rior spinal artery within the cord is crucial to Surrounded by dura, cerebral sinuses and
spinal cord function; its occlusion leads to the veins are the venous structures of the brain. They
anterior spinal artery syndrome. The posterior typically contain inpouchings of arachnoid cells,

CEREBROVASCULAR DISORDERS
191
ARTERIAL SUPPLY TO THE BRAIN AND MENINGES

Figure 19-2 Arteries of Brain (Lateral and Medial Views)

Ant. parietal branch Post. parietal branch


Central (rolandic) branch Angular branch

Precentral (prerolandic) branch Terminal


cortical
Ascending frontal branches of
(candelabra) branch I. posterior
cerebral a.
Terminal cortical
branches of ant.
cerebral aa.

Lateral
orbitofrontal a.

L. middle
cerebral artery

L. anterior
cerebral artery

Ant. communicating a.
Post. temporal branches
R. anterior cerebral artery
Ant. temporal branches
L. internal carotid a.

Pericallosal a. Paracentral a.

Posterior Precuneal a.
Int. frontal
branches Middle
Anterior R. posterior cerebral artery

Post. pericallosal a.

Callosomarginal a. Parietooccipital branch

Frontopolar a. Calcarine branch

R. anterior
cerebral
artery

Medial
orbito-
frontal a.

Ant. communicating a.
Post. temporal branch
Recurrent a. (of Heubner)
Ant. temporal branch
R. internal carotid a. Post. communicating a.

CEREBROVASCULAR DISORDERS
192
ARTERIAL SUPPLY TO THE BRAIN AND MENINGES

Figure 19-3 Arteries of Brain: Inferior Views


Medial frontobasal (orbitofrontal) artery
Anterior communicating artery
Anterior cerebral artery
Distal medial striate artery
(recurrent artery of Heubner)
Internal carotid artery
Anterolateral central
(lenticulostriate) arteries
Middle cerebral artery
Lateral frontobasal
(orbitofrontal) artery
Prefrontal artery
Anterior choroidal artery
Posterior communicating artery
Posterior cerebral artery
Superior cerebellar artery
Basilar artery
Pontine arteries
Labyrinthine (internal
acoustic) artery
Anterior inferior cerebellar artery
Vertebral artery
Anterior spinal artery

Posterior inferior cerebellar


artery (PICA) (cut)
Posterior spinal artery
Circle of Willis
Distal medial striate artery
(recurrent artery of Heubner)
Anterior communicating