pCHAPTER 40 - Neurosurgery

Julian T. Hoff
Michael F. olan!
GENERAL CONSIDERATIONS
A detailed history and a physical examination are the foundation of neurosurical
dianosis! "eadache# altered consciousness# memory impairment# speech difficulty#
$isual distur%ance# &ea'ness# paresthesia# and incoordination are some symptoms
suesti$e of central ner$ous system disease! "istorical details suest a cause (e!!#
traumatic# neoplastic# $ascular# infectious# deenerati$e# or meta%olic) &hile
neuroloic examination permits anatomic locali*ation of the lesion! +or patients &ith
ner$ous system disorders# an accurate history needs to %e ta'en once# %ut the
examination must %e repeated and recorded often to aue the course of the illness
and to ,ude the urency of other dianostic steps!
Dianostic Studies
Once a differential dianosis is formulated usin the information athered from the
history and the examination# dianostic studies are used to confirm the definiti$e
dianosis! Common studies include plain film radioraphy# myeloraphy#
arterioraphy# computed tomoraphy (CT)# manetic resonance imain (-RI)#
ultrasonoraphy (.SN)# electromyoraphy and ner$e conduction $elocity testin
(E-G/NC0)# e$o'ed potentials ($isual# auditory# and somatosensory)# positron
emission tomoraphy (1ET)# electroencephaloraphy (EEG)# and cere%rospinal fluid
(CS+) analysis!
1lain films# especially of the spine# are useful in trauma and deenerati$e disorders!
-yeloraphy# often com%ined &ith CT# is useful for e$aluatin spinal ner$e roots and
the spinal cord in trauma# tumor# and deenerati$e spine disease! CT and -RI pro$ide
detailed imain of %oth cranial and spinal contents! They are useful in com%ination
for $isuali*ation of %one and soft tissues! Arterioraphy pro$ides detailed information
reardin aneurysms# $ascular malformations# and atherosclerotic disease! It is an
essential tool in the e$aluation of cere%ral hemorrhae# em%olic and throm%otic
stro'e# and preoperati$e plannin for tumor surery! .SN is an important ad,unct in
the operatin room# pro$idin $isuali*ation of tumors# cysts# $ascular malformations#
and conenital anomalies lyin %eneath the exposed surface! .SN is also used to
$isuali*e the %rain and spinal cord in ne&%orns!
E-G/NC0 helps to assess peripheral ner$e and ner$e root lesions! It is also used to
monitor %rachial plexus and peripheral ner$e reco$ery follo&in traumatic in,ury!
0isual and auditory e$o'ed potentials can %e employed in comatose patients to
monitor the se$erity of head in,ury! Auditory potentials are useful in e$aluatin
cranial ner$e and %rainstem function! Somatosensory potentials continually monitor
spinal cord (dorsal column) function and are used often durin surical manipulation
of spinal fractures and tumors! 1ET scans play an important role in epilepsy surery#
preem%oli*ation assessment of s'ull %ase tumors in$ol$in the ca$ernous sinus# and
surically untreata%le $ascular anomalies! EEG helps in delineatin structural and
meta%olic disorders and can %e employed durin cere%ro$ascular surery to monitor
ade2uacy of cere%ral perfusion! CS+ analysis remains essential for many dianoses#
such as %acterial or $iral meninitis!
S1ECIAL SIT.ATIONS
Sei*ures
Sei*ures are useful clinical sins %ecause the aura# onset# and type of sei*ure and the
postictal state may locali*e the lesion! Sei*ures are particularly common in patients
&ith neoplasms! Repetiti$e sei*ures should %e treated $iorously! 1henytoin
(Dilantin) is the dru of choice3 456 to 7666 m may %e i$en I!0! o$er 7 h as a
loadin dose! Supplemental doses usually consist of 766 m i$en three or four times
a day! 1heno%ar%ital is also useful (89 to :5 m# three or four times a day)# %ut larer
doses may depress consciousness! Dia*epam (0alium) i$en intra$enously in di$ided
doses (76 to 56 m) is hihly effecti$e in the control of status epilepticus %ut is not a
ood lon;term anticon$ulsant!
Raised Intracranial 1ressure
Almost any space;occupyin intracranial lesion can raise intracranial pressure (IC1)!
Clinical indications of ele$ated IC1 are headache# stupor# diplopia# nausea# $omitin#
and nec' stiffness! Altered %lood pressure and heart rate are late sins3 typically the
%lood pressure is increased and the heart rate is decreased! Apnea may occur if IC1 is
$ery hih! Raised IC1 may %e pre$ented and treated %y the follo&in measures!
"yper$entilation
The 1aCO 9 should %e monitored and maintained at a%out 85 mm"! <rain s&ellin
de$elops &hen 1aCO 9 rises a%o$e normal (=6 mm")! A ood air&ay is essential to
maintain control of 1aCO 9!
"ypothermia
<ecause fe$er causes the %rain to s&ell# the patient>s temperature should %e controlled
%y alcohol sponin# antipyretics (aspirin or acetaminophen)# and hypothermia
%lan'ets! Chlorproma*ine (Thora*ine# 5 m I!0! e$ery 8 to = h) minimi*es shi$erin
durin these maneu$ers!
Osmotic Diuretics
-annitol (7!5 /'/9= h) causes shrin'ae of the %rain and reduction of IC1! It should
%e used only &hen the clinical situation is desperate3 its %eneficial effect is transient#
and the dru can se$erely alter serum electrolytes and osmolarity! .rea and lycerol
are other osmotic diuretics that may %e effecti$e!
Steroids
Dexamethasone (Decadron# = to : m e$ery = h I!0! or orally) or methylprednisolone
(Solu;-edrol# 795 to 956 m I!-! or I!0! t&ice daily) lo&ers IC1 %y reducin %rain
edema! The dru pro%a%ly acts to sta%ili*e the %lood;%rain %arrier!
Infections
CNS infections include meninitis# su%dural empyema# %rain a%scess# and epidural
a%scess! Anti%iotics penetrate the normal %lood;%rain %arrier $aria%ly3 ho&e$er# in the
presence of meninitis anti%iotics are a%le to penetrate the %lood;%rain %arrier %etter!
Specific anti%iotics should %e i$en for specific infections! Durin the 7 to 9 days
re2uired to identify the pathoenic oranism# %road;spectrum anti%iotics should %e
i$en if the infection is life;threatenin! 0ancomycin (7!6 I!0! e$ery 79 h) and
entamicin (45 m I!0! e$ery ? h) are the drus of choice &hile a&aitin culture
results!
Anti%iotics for pre$ention of CNS infection are rarely indicated! In patients &ith
persistent CS+ lea'ae# a %road;spectrum anti%iotic is sometimes used (ampicillin or a
cephalosporin)!
+luid <alance
Neurosurical patients should ha$e lo&;normal to normal inta'e and output of fluids
(9666 to 9566 mL/9= h for an adult)! +ree &ater (5@ dextrose in &ater) should %e
a$oided %ecause it causes %rain s&ellin! The preferred I!0! solution is 5@ dextrose in
6!5 normal saline &ith potassium supplements (=6 mE2/day)! +luid %alance should %e
monitored %y daily &eihts and periodic measurements of serum electrolytes!
+eedins %y a$ae may %e started early (up to 8 days after in,ury or operation)
pro$ided astrointestinal function is normal!
Some neurosurical patients ha$e profound distur%ances of fluid %alance!
Inappropriate antidiuretic hormone (AD") secretion# &hich most commonly occurs
after head trauma# causes retention of free &ater# resultin in lo& serum sodium# hih
urinary sodium# lo& serum osmolarity# and hih urinary specific ra$ity! Sei*ures and
coma may %e the first clinical sins of inappropriate AD" secretion! The %est
treatment is fluid restriction!
Dia%etes insipidus is also common in patients &ith neurosurical illnesses! .rine
$olume is hih and specific ra$ity is lo&3 serum sodium and osmolarity are hih!
Sei*ures and stupor may appear! The condition is treated %y administration of I!0! or
oral fluids! Sometimes antidiuretic hormone ($asopressin# 1itressin) is re2uired to
control urinary loss of &ater!
1atients &ho are i$en steroids# osmotic diuretics# anticon$ulsants# and hyperosmolar
feedins (typical neurosurical patients) are prone to de$elopin a hyperosmolar state#
sometimes leadin to hyperlycemic non'etotic coma! Conse2uently# careful
monitorin of fluid and electrolyte status of the neurosurical patient is essential!
Coma
Coma is a loss of consciousness from &hich the patient cannot %e aroused %y any
stimulus! Stupor implies that the patient can %e partially aroused %y loud command or
painful stimulus %ut promptly lapses into unconsciousness &hen the stimulus is
&ithdra&n!
Dianosis
The dianosis of coma re2uires a careful history (enerally from friends or relati$es
of the patient) and a complete physical examination# &ith specific attention to the
neuroloic examination! La%oratory and radioloic tests of $alue include serum
electrolytes# %lood lucose# and %lood urea nitroen assays# s'ull x;rays# and CS+
examination $ia lum%ar puncture! -edical consultation should %e souht promptly!
Coma may %e caused %y poisonin# such as alcohol# %ar%iturate# and narcotic
o$erdose3 cere%ral lesions# such as those caused %y trauma# $ascular accidents#
tumors# infections# and epilepsy3 meta%olic disorders# such as dia%etes mellitus#
hypolycemia# Addison>s disease# uremia# hepatic disease# and eclampsia3 and other
stresses# such as se$ere infection# shoc'# asphyxia# heatstro'e# and hypoxia!
Dianostic features of common types of coma are as follo&s!
Acute Alcoholic Intoxication
A history of alcohol a%use# alcoholic %reath# flushed face# slo& and stertorous
respirations# diminished reflexes# and a %lood alcohol le$el a%o$e =66 m/dL esta%lish
this dianosis!
Narcotic 1oisonin
E$en small doses of narcotics may cause respiratory depression and coma in patients
&ith li$er insufficiency# myxedema# emphysema# or head in,uries and in de%ilitated or
elderly patients! +indins include cold# clammy# cyanotic s'in# pinpoint pupils#
respiratory depression# and a fee%le and often irreular pulse!
Acute toxicity caused %y an o$erdose of a self;administered narcotic occurs
commonly in some localities! The type and purity of the dru are difficult to
determine# althouh a companion or ac2uaintance may 'no& the patient>s dru ha%its!
The examiner should loo' for needle mar's in the arms and les! La%oratory tests are
$alua%le to determine %ar%iturate# alcohol# or narcotic le$els!
Dia%etic Coma
Coma may %e precipitated in a dia%etic patient %y infection or %y failure to reulate
insulin dosae carefully! Dianostic features include the follo&inA history of
dia%etes# radual onset# &ith %lurred $ision and thirst# air huner or Bussmaul
respiration# dehydration (soft eye%alls)# acetone %reath (CfruityD odor on %reath)#
lycosuria# acetonuria# hyperlycemia# 'etonemia# and lo& plasma %icar%onate le$el!
"ypolycemia
"ypolycemic reactions in dia%etics may %e precipitated %y failure to eat# %y $iorous
exercise# or %y insulin o$erdose! -ental confusion and %i*arre %eha$ior precede coma
and con$ulsions! Tachycardia# s&eatin# tremors# and $omitin are other
manifestations! Lo& %lood lucose le$el confirms the dianosis!
-anaement
Emerency -easures
Identify and treat any life;threatenin condition immediately! Esta%lish and maintain
an air&ay to pro$ide oxyenation! Insert an endotracheal tu%e if the respiratory rate is
less than 76/min# if the 1aO 9 is %elo& 46 mm"# or if the 1aO 9 is reater than 56
mm" &ith the patient %reathin oxyen throuh a mas'! -onitor arterial %lood
ases fre2uently and treat the patient for shoc'! Ehen no cause for coma is
immediately o%$ious# o%tain %lood for lucose determination and toxicoloic analysis
and then administer each of the follo&inA 56 mL of 56@ dextrose in &ater for
possi%le hypolycemia# 7 mL (6!= m) naloxone for possi%le narcotic o$erdose# and
766 m thiamine I!0! for possi%le Eernic'e>s (alcoholic) encephalopathy!
General -easures
O%ser$e the patient fre2uently# record neuroloic and $ital sins at reular inter$als#
and chane the patient>s position often to a$oid postural pneumonia and decu%itus
ulcers! A lateral and slihtly head;do&n position is %est for patients &ho are li'ely to
$omit! A suction machine and an alert attendant near the %edside are essential!
-aintain $entilation and monitor urinary output throuh an ind&ellin catheter!
-aintain fluid# electrolyte# and caloric inta'e! Nasoastric feedin should %e started if
the coma lasts more than 9 to 8 days! A$oid administerin narcotics# sedati$es# and
other medications until the dianosis is esta%lished3 aitation can then %e treated %est
%y administration of parenteral dia*epam (9 to 5 m I!0! e$ery 9 h as needed)!
TRA.-A
Trauma is the sinle most common cause of death in children# adolescents# and youn
adults! The ma,ority of accidents in$ol$in motor $ehicles and falls include in,ury to
the %rain# spinal cord# and their supportin structures!
Scalp In,ury
Scalp in,ury may cause hemorrhae and su%se2uent shoc' if not promptly treated!
<leedin can usually %e controlled %y a pressure dressin or %y clamps applied to the
alea aponeurotica! Scalp &ounds should %e closed as soon as possi%le! Lacerations
that o$erlie a depressed fracture or a penetratin &ound of the s'ull re2uire
de%ridement and closure in the operatin room!
Simple scalp lacerations should %e de%rided# copiously irriated# and closed primarily#
ta'in care to approximate %oth the alea and the s'in! A ood aleal closure pro$ides
excellent hemostasis! Scalp a$ulsions typically include all layers of the scalp# sparin
the underlyin periosteum! If the a$ulsion is small# closure can often %e accomplished
primarily! Replacement of the a$ulsed part# employin microsurical techni2ue# is the
preferred method of repair for lare scalp a$ulsions# pro$ided the tissue has %een
preser$ed ade2uately and surery is not delayed!
If the in,ured scalp is not $ia%le %ut the periosteum is intact# split;thic'ness s'in rafts
can %e used to close the defect! The periosteum must %e 'ept moist %efore operation!
Ehen the periosteum is a%sent or desiccated# closure is more difficult# since it is
throuh the periosteal layer that the outer s'ull ta%le recei$es its %lood supply! In this
instance# closure can %e accomplished usin free myocutaneous flaps attached %y
microsurical $ascular anastomoses!
S'ull +racture
S'ull fractures are classified accordin to &hether the s'in o$erlyin the fracture is
intact (closed) or disrupted (open or compound)# &hether there is a sinle fracture line
(linear)# se$eral fractures radiatin from a central point (stellate)# or framentation of
%one (comminuted)# and/or &hether the edes of the fracture line ha$e %een dri$en
in&ard %elo& the le$el of the surroundin %one (depressed) or not (nondepressed)!
Simple s'ull fractures (linear# stellate# or comminuted nondepressed) re2uire no
specific treatment! They are# ho&e$er# potentially serious if they cross $ascular
channels in the s'ull# such as the middle menineal artery or the dural $enous sinuses!
If these structures are torn# an epidural or su%dural hematoma may form! A simple
s'ull fracture that extends into the accessory nasal sinuses or the mastoid air cells is
considered open# since it is in communication &ith air!
Depressed s'ull fractures often re2uire surical treatment to ele$ate the depressed
%one framents! If there are no unto&ard neuroloic sins and the fracture is closed#
repair may %e done electi$ely! Intraoperati$ely# the dura should %e inspected and
repaired!
Open s'ull fractures also re2uire surical inter$ention! Linear or stellate#
nondepressed open fractures can %e treated %y simple closure of the scalp after
thorouh cleansin! Open fractures &ith se$ere comminution of underlyin %one
should %e treated in the operatin room# &here thorouh de%ridement can %e carried
out! The dura should %e inspected to $erify that a laceration has not %een o$erloo'ed!
Dural tears should %e closed either primarily or &ith a fascial patch raft to reduce the
ris' of infection and pre$ent CS+ lea'ae! Depressed# open s'ull fractures should %e
de%rided# ele$ated# and closed in the operatin room after preparations ha$e %een
made for craniotomy# in case %roader exposure of the underlyin dura and/or %rain is
necessary!
<asal s'ull fractures in$ol$e the floor of the s'ull! <ruisin may occur a%out the eye
(raccoon sin) or %ehind the ear (<attle>s sin)# suestin a fracture in$ol$in either
the anterior or middle fossa# respecti$ely! Isolated cranial ner$e deficits can %e seen in
association &ith this fracture type %ecause the ner$es> exit foramina lie at the s'ull
%ase! The facial ner$e is fre2uently affected# &ith in,ury due to laceration or s&ellin!
-ost facial ner$e deficits resol$e spontaneously# so no specific therapy is &arranted!
On the other hand# complete transections of the facial ner$e are usually explored#
althouh the timin of exploration remains a matter of de%ate!
Any associated CS+ rhinorrhea or otorrhea should %e treated expectantly! Traumatic
CS+ lea's typically stop &ithin the first 4 to 76 days! If a lea' persists# lum%ar CS+
drainae can %e implemented to seal the lea' %y lo&erin CS+ $olume and IC1! If
drainae therapy fails# surical exploration is indicated and accomplished %y
o$erse&in the defect &ith an intradural fascial patch raft! +e&er than 5 percent of
patients re2uire surical repair! 1rophylactic anti%iotics are not used routinely %ecause
recent prospecti$e studies ha$e failed to demonstrate any %enefit from their use!
<rain In,ury
In,ury to the %rain is caused %y rapid deceleration# acceleration# rotation# or a
com%ination of these# associated &ith a %lo& to the head! The initial impact can
produce neuronal and axonal disruption# &hich constitutes the primary in,ury! Any
su%se2uent complication such as an intracranial hematoma# cere%ral edema# hypoxia#
hypotension# hydrocephalus# or endocrine distur%ance characteri*es secondary in,ury#
&hich compounds the initial insult!
-ild head in,ury is usually not associated &ith sinificant primary %rain in,ury# and
neuroloic deficits are limited to temporary loss of consciousness (concussion)!
-oderate to se$ere head in,ury# on the other hand# is typically associated &ith deficits
that may or may not %e re$ersi%le! -oreo$er# this deree of in,ury is usually
accompanied %y secondary in,ury!
Distortional forces causin the primary in,ury may %e reat enouh to tear
intraparenchymal capillaries# superficial su%dural %ridin $eins# or epidural arteries
and $eins# allo&in extra$asation of %lood and hematoma formation! Cere%ral edema
occurs in response to $asodilatation and %lood; %rain %arrier disruption! Ischemia
from hypotension or hypoxia can produce cell death and conse2uent cytotoxic edema!
Disruption of CS+ a%sorption %y contamination of CS+ &ith %lood may lead to
hydrocephalus! Inappropriate secretion of antidiuretic hormone or the de$elopment of
dia%etes insipidus can ara$ate cere%ral edema %y alterin fluid and electrolyte
%alance! These chanes# either separately or in com%ination# can result in ele$ation of
IC1!
Ele$ated IC1 contri%utes to secondary %rain in,ury %y reducin cere%ral perfusion
pressure (C11)# &hich is defined as the difference %et&een mean arterial %lood
pressure (-A<1) and cere%ral $enous pressure or IC1! Thus &hen IC1 increases and
-A<1 remains sta%le# C11 decreases! Ehen C11 falls %elo& 46 mm"# cere%ral
perfusion is compromised# producin cere%ral ischemia and compoundin the primary
%rain in,ury &ith a secondary insult!
Intracranial hypertension is one of the most important factors affectin outcome in
head in,ury! +or this reason# aressi$e manaement to circum$ent cere%ral %lood
flo& reduction and secondary in,ury is imperati$e! Early resuscitati$e therapy should
%e initiated at the scene of the accident if possi%le &ith air&ay control and
hyper$entilation! In the a%sence of hypotension# osmotherapy can also %e used early
to reduce %rain %ul'!
Rapid clinical assessment is essential! Althouh extensi$e neuroloic testin is limited
in uncooperati$e or unresponsi$e patients# certain features of the exam are critical!
The Glaso& Coma Scale (Ta%le =6;7)# esta%lished in 7F4=# uses a numerical score to
e$aluate eye openin and $er%al and motor %eha$ior# %oth spontaneously and in
response to stimulation! This scale is used to follo& the patient>s neuroloic status and
pro$ides information a%out the ultimate outcome of the patient!
The initial neuroloic exam determines &hether dianostic testin is indicated! It is
unli'ely that patients &ithout headache# lethary# or a focal neuroloic deficit &ill
suffer a secondary complication from their in,ury! Imain studies are enerally not
indicated in the asymptomatic patient! Con$ersely# symptomatic patients &ith or
&ithout a focal deficit should undero CT scannin of the head! If CT fails to disclose
a lesion despite hih clinical suspicion# carotid and/or cere%ral anioraphy may %e
helpful to identify a $ascular a%normality that cannot %e appreciated %y CT scan!
Treatment
After remo$al of any traumatic intracranial mass# the oals of manaement are
normali*ation of C11 and pre$ention of secondary in,ury to damaed %rain!
Intracranial pressure monitorin may %e indicated# especially in those patients &ith
mar'ed depression or deterioration in neuroloic function! Comatose patients &ho
re2uire emerency surery (a%dominal# thoracic# orthopaedic# etc!) should also ha$e
IC1 monitorin# since fre2uent neuroloic examinations are not possi%le durin
eneral anesthesia! 0entriculostomy to measure IC1 also allo&s drainae of CS+#
&hich can lo&er the pressure sinificantly! Ehen the $entricular system is collapsed
and $entriculostomy not feasi%le# intraparenchymal monitorin should %e esta%lished!
"ead ele$ation in the neutral position facilitates $enous drainae3 sedation reduces
posturin and com%ati$e acti$ity# %oth of &hich ele$ate IC13 hyper$entilation to 'eep
1aCO 9 around 85 mm" lo&ers cere%ral %lood $olume and IC13 prophylactic use of
anticon$ulsants pre$ents cere%ral in,ury from sei*ures3 mild dehydration &ith
,udicious sodium replacement and prompt treatment of SIAD" (syndrome of
inappropriate antidiuretic hormone secretion) protects the %rain from insult secondary
to fluid o$erload3 pre$ention of hypotension reduces the extension of ischemic in,ury#
and aressi$e treatment of hypertensi$e episodes reduces cere%ral %lood $olume and
further disruption of the %lood;%rain %arrier3 and treatment of hyperthermia a$oids an
increase in the %rain>s meta%olic demands! All of these manaement principles may %e
re2uired simultaneously to achie$e optimal homeostasis of the %rain!
If IC1 remains ele$ated despite these measures# mannitol (6!5 to 7!6 /' I!0!) and
furosemide (7!6 m/') can %e used to reduce cere%ral edema! Deep sedation &ith
narcotics and the use of paralytic aents such as pancuronium may %e helpful!
<ar%iturate coma# once popular in other&ise medically refractory IC1 ele$ation# does
not sinificantly chane ultimate outcome and thus is seldom used! Corticosteroids#
&hile occasionally used# ha$e no pro$ed %enefit for se$ere %rain in,ury!
Outcome after head in,ury depends on many factors! Increasin ae and preexistin
illness contri%ute to a poor pronosis! 1enetratin in,uries# particularly unshot
&ounds# are associated &ith poorer outcome compared &ith %lunt trauma! The
presence of an intracranial hemorrhae also implies a su%optimal result! Su%dural
hematoma has a poorer pronosis than epidural hematoma! Com%ined su%dural and
intracere%ral hemorrhae has the &orst pronosis of all se$ere head in,ury su%types!
Other important factors that influence outcome include delay in treatment# multiple
trauma# and systemic insults such as acidosis# hypoxia# and hypotension! 1redictors of
poor pronosis include e$idence of %rainstem dysfunction on the initial examination
and refractory intracranial hypertension &ithin the first fe& days of in,ury!
Diffuse Axonal In,ury
Diffuse &hite matter in,ury is associated &ith anatomic disruption of axons
throuhout %oth cere%ral hemispheres! This primary shearin in,ury is associated &ith
hih mortality and su%stantial neuroloic mor%idity! It results from rotational forces
that are perpendicular to the axis of the &hite matter# causin axonal transection!
Despite se$ere shearin in,ury# the %rain may appear rossly normal# %ut numerous
axonal transections are seen microscopically in &hite matter &ithin 9 &ee's of in,ury!
Classically# small hemorrhaes are present in the corpus callosum and cere%ral
peduncles on CT scan! +i%er tract demyelini*ation su%se2uently de$elops# extendin
throuhout the cere%ral hemispheres and into the %rainstem!
Epidural "ematoma
"emorrhae %et&een the inner ta%le of the s'ull and the dura mater most commonly
arises from a tear of the middle menineal artery or one of its %ranches! Arterial
%leedin strips the dura from the undersurface of the %one and produces a hematoma#
&hich may increase in si*e and compress the underlyin %rain!
An epidural hematoma can also arise from torn $enous channels in the %one at a point
of fracture or from lacerated ma,or dural $enous sinuses! Since $enous pressure is
lo&# $enous epidural hematomas usually form only &hen a depressed s'ull fracture
has stripped the dura from the %one and left a space &here the hematoma can de$elop!
Epidural hematoma classically follo&s a %lo& to the head that fractures the s'ull and
causes a %rief period of unconsciousness! After the patient reains consciousness#
there may %e a Clucid inter$alD durin &hich there are minimal symptoms or sins!
Ehen the hematoma enlares# hemispheric compression occurs! Eith time# the medial
portion of the temporal lo%e is forced o$er the ede of the tentorium# causin
compression of the oculomotor ner$e and dilation of the ipsilateral pupil! Similarly#
compression of the ipsilateral cere%ral peduncle causes contralateral hemiparesis#
&hich may proress to decere%rate posturin! Coma# fixed and dilated pupil(s)# and
decere%ration are a classic triad indicatin transtentorial herniation!
E$en thouh epidural hematomas are cura%le lesions# the mortality rate remains hih
%ecause the se$erity of in,ury is often not reconi*ed early! A patient may %e seen
durin the Clucid inter$alD and dischared! Later# the patient may %ecome unconscious
%ecause of proressi$e %rain compression %y the expandin hematoma!
<ecause of the daner of misdianosis# any patient &ith a history of a %lo& to the
head leadin to a period of unconsciousness should ha$e a CT scan (+i! =6;7)! If an
epidural hematoma is found# urent craniotomy is indicated! If the CT scan is normal
and the patient>s examination sho&s no neuroloic deficit# the patient may %e
dischared! An accompanyin person should %e instructed to a&a'en the patient
fre2uently o$er the next 9= h to %e certain that he or she remains arousa%le! Any
deterioration in consciousness should prompt ree$aluation!
Su%dural "ematoma
Su%dural hematomas may de$elop &hen $eins %ridin the cortex and the dura or
$enous sinuses are torn or &hen an intracere%ral hematoma extends into the su%dural
space! They can %e lare e$en thouh the %leedin is of $enous (lo&;pressure) oriin!
Acute su%dural hematomas are associated &ith se$ere head in,ury and arise from a
com%ination of torn %ridin $eins# disruption of cortical $essels# and laceration of the
cortex! The hematoma is %est seen &ith CT scannin! E$acuation of the clot may lead
to sinificant impro$ement# %ut often a ma,or neuroloic deficit remains %ecause of
the accompanyin &idespread parenchymal in,ury!
Su%acute su%dural hematomas %ecome apparent se$eral days after in,ury and are
associated &ith proressi$e lethary# confusion# hemiparesis# or other hemispheric
deficits! Remo$al of the hematoma usually produces stri'in impro$ement!
Chronic su%dural hematomas arise from tears in %ridin $eins# often after a minor
head in,ury! Initially# the hematoma is small! Later# it %ecomes encased in a fi%rous
mem%rane# li2uefies# then radually enlares! These lesions are more common in
infants and the elderly! Typical presentation includes proressi$e mental status
chanes# &ith or &ithout focal sins (hemiparesis# aphasia# etc!)! 1apilledema may %e
present! The dianosis is confirmed %y CT scannin! Treatment consists of hematoma
drainae %y trephination! Craniotomy may %e necessary if the fluid reaccumulates!
Spinal Cord In,ury
Traumatic in,ury of the spinal cord may result from $erte%ral fracture#
fracture/su%luxation# hyperextension of the cer$ical spine in the presence of a narro&
spinal canal# herniation of inter$erte%ral disc material into the canal# and penetratin
in,uries such as unshots or sta%%ins! Neuroloic in$ol$ement ranes from mild and
transient to se$ere and permanent! Spinal fracture and cord in,ury should %e suspected
in head;in,ured patients# &ith or &ithout coma# and in those patients &ith multiple
in,uries! It is %est to assume that the spine is unsta%le initially and immo%ili*e the
patient on a %ac'%oard &ith a hard cer$ical collar until careful examination and
dianostic testin are done!
Clinical findins of spinal or spinal cord in,ury include spinal tenderness# extremity
&ea'ness# num%ness or paresthesia# respiratory em%arrassment# and hypotension!
Spinal root in$ol$ement accounts for radiculopathy# characteri*ed %y motor and
sensory impairment in the correspondin myotome and dermatome (+i! =6;9)! Spinal
cord in$ol$ement produces myelopathy &ith $aria%le manifestations!
A complete lesion# clinically defined as total loss of motor and sensory function %elo&
the le$el of in,ury# is associated &ith anatomic or physioloic transection of the cord!
Acute transections are characteri*ed %y areflexia# flaccidity# anesthesia# and
autonomic paralysis %elo& the le$el of the lesion! Arterial hypotension is in$aria%ly
present &hen the transection is a%o$e T5 %ecause of the loss of sympathetic $ascular
tone! Common spinal cord syndromes are sho&n in +i! =6;8!
Incomplete lesions of the cord may result in the <ro&n;SG2uard syndrome#
manifested %y ipsilateral loss of motor function and position/$i%ratory sensation &ith
contralateral loss of pain and temperature sensation %elo& the le$el of in,ury!
Anatomically# this presentation is explained %y hemisection of the cord! The central
cord syndrome is characteri*ed %y %ilateral loss of motor function and pain and
temperature sensation in the upper extremities# &ith relati$e preser$ation of these
functions in the lo&er extremities! Typically# the distal upper extremities are more
se$erely affected %ecause the most medial portions of the corticospinal and
spinothalamic tracts su%ser$e these areas! The central cord syndrome is often seen
follo&in a hyperextension in,ury of the cer$ical spine# &ith or &ithout fracture! The
anterior spinal artery syndrome in$ol$es %ilateral loss of motor function and pain and
temperature sensation %elo& the le$el of the lesion# &ith sparin of position#
$i%ratory# and liht touch sensation! This incomplete lesion de$elops &hen the
anterior spinal artery is in,ured# renderin the cord ischemic &ithin the distri%ution of
the anterior spinal artery# affectin the anterior and lateral columns %ilaterally! A
common cause of anterior spinal artery syndrome is an acutely ruptured cer$ical disc!
Trauma to the lum%ar spine may produce sins and symptoms of cauda e2uina
compression! 1resentation consists of multiple lum%osacral radiculopathies of
$aria%le se$erity! Lo&er extremity motor# sensory# and reflex functions may %e
affected# producin $aria%le derees of &ea'ness# sensory loss (all modalities in the
specific distri%ution of the roots in$ol$ed)# and diminution or a%sence of reflexes!
<ladder distention from detrusor muscle paralysis# flaccidity of the anal sphincter# and
loss of perineal sensation are common in se$ere in,uries!
In addition to the neuroloic deficit# acute spinal cord in,ury is accompanied %y a
$ariety of systemic responses! If the spinal cord is damaed a%o$e C8# respiratory
efforts cease# accountin for this in,ury>s hih mortality at the scene of the accident!
Althouh spontaneous $entilatory efforts can %e initiated &ith in,uries in$ol$in C=H
C:# tidal $olumes are often insufficient# accountin for proressi$e hypoxia and
car%on dioxide retention! Air&ay o%struction# atelectasis# and pneumonia are common
complications! Assisted $entilation is often re2uired early after in,ury!
Ileus &ith astric distention is common# necessitatin nasoastric drainae! Similarly#
%ladder distention occurs %ecause the %ladder and pel$ic floor muscles are flaccid!
<ladder drainae &ill pre$ent o$erdistention# &hich may %e se$ere enouh to cause
compression of the inferior $ena ca$a and pel$ic $eins# impairin $enous return to the
heart and contri%utin to systemic hypotension!
<lood pressure is usually lo& if the cord in,ury is a%o$e the T5 le$el! This effecti$ely
dener$ates the sympathetic ner$ous system# &hich leads to increased $enous
capacitance and decreased $enous return! The resultin hypotension is controlled %y
the administration of intra$enous fluids! Colloid is preferred to reduce the threat of
$ascular o$erload and iatroenic pulmonary edema! 1ostural chanes that &ill
precipitously drop the %lood pressure# such as upper %ody ele$ation# should also %e
a$oided!
Tachycardia is a common compensatory response to hypotension# %ut %radycardia is
the rule &hen the cer$ical cord is damaed and the sympathetic input to the heart is
lost! This type of %radycardia does not re2uire treatment unless the patient is
symptomatic or at ris' for myocardial infarction or stro'e %ecause of ae or other
de%ilitatin illness! If necessary# treatment &ith atropine and fluids is effecti$e!
Once the patient is hemodynamically sta%le# spinal radioraphs are essential# %ut only
&hile the patient remains immo%ili*ed on a %ac'%oard in a hard cer$ical collar!
Standard $ie&s are o%tained# ensurin ood $isuali*ation of the cer$icothoracic
,unction (+i! =6;=)! Comatose and/or se$erely in,ured patients &ith multiple trauma
should ha$e ood plain;film imain of the complete spine! +ractured areas can %e
studied further &ith CT# usin %oth axial and saittal $ie&s (+i! =6;5)! If no
a%normality is found %y plain films or CT and a neuroloic deficit exists indicatin a
spinal cord le$el# -RI or myeloraphy follo&ed %y CT should %e utili*ed to identify
other causes of cord compromise# such as traumatic inter$erte%ral disc rupture or
spinal epidural hematoma!
Treatment
The o%,ecti$es of treatment are to correct spinal alinment# protect undamaed neural
tissue# restore function to re$ersi%ly damaed neural tissue# and achie$e permanent
spinal sta%ility! Reduction and immo%ili*ation of any fracture/dislocation must recei$e
top priority to meet these o%,ecti$es!
Cer$ical spine malalinment can almost al&ays %e reduced %y s'eletal traction in the
neutral position! Traction may %e applied usin s'ull tons or a halo apparatus! <oth
are seated percutaneously throuh the outer ta%le of the s'ull &hile the patient is 'ept
supine and immo%ili*ed! The patient is then transferred to a special %ed and traction
%eun! +re2uent lateral $ie& radioraphs are o%tained to document reduction and
pre$ent o$erdistraction# &hich can lead to further cord in,ury! Once the spinal in,ury
is reduced# traction should %e maintained! +re2uent follo&;up films are then ta'en to
confirm correct alinment!
Sometimes a cer$ical fracture cannot %e reduced %y traction alone &ithout
,eopardi*in spinal cord function! Open reduction# usually throuh a posterior
approach# com%ined &ith a fusion procedure may %e necessary in those instances!
This especially pertains to unilaterally or %ilaterally loc'ed facets!
1atients &ith thoracic and lum%ar spine fractures are also treated &ith immo%ili*ation
initially! Immo%ili*ation is less strict compared &ith that for cer$ical fractures# %ut the
principles are the same! 1atients are 'ept flat in %ed &ithout traction &hile flexion#
extension# lateral %endin# and rotational mo$ements are a$oided! They typically ha$e
fe&er systemic complications associated &ith their neuroloic in,ury %ut ne$ertheless
re2uire $iilance to pre$ent neuroloic deterioration and pro$ide the %est chance for
neuroloic reco$ery!
Indications for early operation in patients &ith spinal cord in,ury include ina%ility to
reduce the fracture/dislocation satisfactorily %y closed methods# neuroloic
deterioration in a patient &ith an incomplete cord lesion initially# se$ere compression
of the spinal cord %y an intraspinal mass sho&n %y myeloraphy or -RI# and a
penetratin in,ury &ith or &ithout a CS+ lea'! Open &ounds# such as those inflicted
%y sta%%ins or unshots# should %e de%rided and closed &hether the cord in,ury is
complete or incomplete! Early operation to sta%ili*e the spine is &arranted %ecause
this translates into early mo%ili*ation and reha%ilitation! Either the anterior or the
posterior approach may %e used# dependin on the nature of the spinal in,ury and the
deree of insta%ility!
If closed reduction is successful and the fracture is sta%le# external immo%ili*ation is
necessary for a minimum of 8 months to ensure proper healin! If surical reduction
and/or fixation is necessary# external immo%ili*ation is still indicated! +or the cer$ical
spine# this in$ol$es a halo $est! Certain exceptions include anterior and posterior
metal platin procedures in &hich a hard cer$ical collar may suffice (+i! =6;:)! The
thoracic and lum%ar spine usually re2uire a plastic %ody ,ac'et or plaster cast for a
minimum# once aain# of 8 months! 1lain films are used to follo& spinal alinment
and the extent of fusion durin the reco$ery period!
If any cord function is preser$ed immediately after in,ury# additional function usually
returns# pro$ided the cord and spine are protected from secondary in,ury! 1atients &ith
complete in,uries rarely reco$er function %elo& the le$el of the lesion! Reha%ilitation
for them is directed to&ard self; care and $ocational read,ustment! -ost persons &ith
these handicaps can e$entually achie$e independence! Life expectancy is shortened
slihtly in parapleics and sinificantly in 2uadripleics! Lon;term pro%lems
associated &ith s'in care and recurrent urinary tract infections account for the early
mortality rate!
1eripheral Ner$e In,ury
1eripheral ner$e in,uries may %e cateori*ed functionally! Neurapraxia is a temporary
loss of function &ithout axonal in,ury! Structural damae does not occur! The foot that
Coes to sleepD after crossin the les is an example of functional loss &ithout
patholoic chane! Axonotmesis is disruption of the axon &ith preser$ation of the
axon sheath! Eallerian deeneration of the distal axon frament occurs! Stretch or
proloned compression causes this functional and structural loss! Reeneration of the
proximal axon occurs# %ut functional reco$ery depends on associated in,uries# the
amount of healthy proximal axon remainin after in,ury# and the ae of the patient!
Neurotmesis is disruption of %oth the axon and axon sheath &ith correspondin loss
of function! Transection of a ner$e causes this phenomenon! Reeneration occurs# %ut
function rarely returns to normal!
Clinically# sensory and motor chanes correspond to the peripheral ner$e in$ol$ed! A
detailed history and a precise neuroloic examination can locali*e the site of in,ury
&ith reat accuracy! Sensory findins are usually apparent early and remain so until
reeneration is nearly complete! Compensatory motor function# often seen in the hand
months after in,ury# is rarely seen acutely! A crude %ut clinically helpful sin of
sensory reeneration is Tinel>s sin! 1ercussion of the s'in o$erlyin the lenth of the
in,ured ner$e elicits paresthesias at the site &here reeneration is occurrin!
Radioraphs of the in,ury site are helpful to loo' for fracture or forein %ody!
Electromyoraphy (E-G) is not useful &ithin the first 8 &ee's of in,ury# %ut this
dianostic aid %ecomes hihly effecti$e to follo& the state of the
deeneration/reeneration process occurrin later! -anaement decisions are often
made dependin on E-G findins &ee's to months after trauma!
Treatment
Treatment of a lacerated ner$e consists of primary repair &hen the &ound is clean and
uncomplicated# as in sta% &ounds# lacerations from lass# and surical incisions!
Secondary or delayed repair is indicated &hen the &ound is dirty or complicated# as in
unshot &ounds and a$ulsions# &hich disrupt tissue se$erely# ma'in primary repair
less successful! Secondary repair is %est accomplished a fe& &ee's after in,ury# &hen
tissue $ia%ility is o%$ious# the li'elihood of infection is reduced# and dissection planes
are distinct! If end;to;end anastomosis of ner$es is not possi%le %ecause of tissue loss#
ner$e raftin usin autoloous sural ner$e may %e done! Intraoperati$e factors such
as axial orientation of fascicles# proper coaptation# suture material# hemostasis# and
suture line tension determine the outcome!
Ner$e in,uries in continuity (i!e!# resultin from contusion or compression &ithout
laceration) are often explored if they do not impro$e &ithin : &ee's of in,ury#
&hether loss of function is complete or incomplete! Intraneural and extraneural scar
tissue at the site of the lesion may pre$ent axonal rero&th %y its constrictin effect!
Neurolysis releases the reeneratin ner$e fi%ers from the impinin scar and may
impro$e functional reco$ery!
1rompt institution of physical therapy is also indicated for impro$ement of muscle
function and maintenance of ,oint motion! It is the %est means of minimi*in the
complications of dener$ation! The dener$ated portion of the lim% is su%,ect to muscle
atrophy and fi%rosis# ,oint stiffness# motor endplate atrophy# and trophic s'in chanes!
The loner the dener$ation persists# the less li'ely &ill ood function result!
Reeneration in a peripheral ner$e occurs at 7 mm/day (rouhly 7 inch per month)# so
impro$ement may not %e o%$ious for many months! +actors that ad$ersely affect the
return of function include ad$anced ae of the patient# proximal ner$e in,ury#
extensi$e ner$e tissue loss# associated soft tissue in,ury# and presence of mixed
sensorimotor function deficits! .nfortunately# incomplete neuroloic reco$ery is often
the rule! The use of tendon transfers should %e considered to impro$e functional
outcome if neuroloic function is inade2uate after reco$ery has ceased!
NEO1LAS-S
Ner$ous system tumors represent almost 76 percent of all neoplasia! Of these# 75 to
96 percent occur in children! Nearly 46 percent of adult tumors are found a%o$e the
tentorium (supratentorial)# &hereas 46 percent of childhood tumors are found %elo&
(infratentorial)! CNS tumors are the most common solid tumors in children! Of all
pediatric cancers# they are second in incidence only to leu'emia!
The incidence of ner$ous system neoplasia decreases in the late teen years and %eins
to pea' aain %y middle ae! <y then# only 95 percent of intracranial tumors are
%enin! This percentae# ho&e$er# rises to 56 percent in the older patient %ecause of
the increasin incidence of meniniomas and sch&annomas! O$erall# there is a
slihtly reater incidence of tumors in men (55 percent)# %ut sch&annomas and
meniniomas are more common in &omen! Of all CNS neoplasms# spinal tumors
constitute approximately 75 to 96 percent!
Intracranial Tumors"emanio%lastoma
These %enin tumors are 2uite $ascular and usually occur in the cere%ellum! They are
uncommon and constitute only 7 percent of intracranial tumors! Typical presentation
is in the fourth decade! +ifteen percent of patients ha$e $on "ippel;Lindau disease# an
autosomal dominant disorder consistin of central ner$ous system
hemanio%lastomas# retinal aniomatosis# renal and pancreatic cysts# and renal cell
carcinoma! Irrespecti$e of this syndrome# many patients &ith hemanio%lastoma ha$e
polycythemia! Si*a%le neoplastic cysts are present in :6 percent of cases# often &ith
only a small mural nodule of tumor! Total surical remo$al is curati$e! Radiation
therapy is used &hen resection is not possi%le! Reoperation is recommended for
recurrences! The lon;term sur$i$al is excellent# &ith up to ?6 percent of patients
ali$e at 76 years!
-etastatic Tumor
T&enty;fi$e percent of all intracranial tumors are metastatic! -alinant cells in$ade
the central ner$ous system hematoenously and tend to lode at the ray and &hite
matter ,unction! -etastatic tumors occur sinly or multiply and may in$ol$e $irtually
any portion of the %rain or# less commonly# spinal cord! Althouh any malinancy has
the potential to metastasi*e# the most common primary sites are lun# %reast# 'idney#
testis# colon# and s'in! The presentin symptoms are determined %y the site(s) of the
metastases! In eneral# these commonly include headache# mental status chanes#
sei*ures# and hemiparesis!
-etastatic lesions are %est imaed &ith hih;resolution -RI# %ut they may %e
mimic'ed %y other lesions such as meniniomas# a%scesses# primary %rain tumors#
and e$en aneurysms! If a metastasis is suspected# a &or'up to find the primary source
is recommended! If the primary site is not identified# an excisional %iopsy is indicated
to esta%lish the dianosis!
In eneral# a symptomatic# solitary lesion that is surically accessi%le should %e
remo$ed! Surery# ho&e$er# should not %e underta'en for multiple lesions or in
patients &ho are se$erely afflicted %y their primary disease! Treatment should also
include preoperati$e dexamethasone# as in any %rain or spinal cord tumor# to reduce
ad,acent %rain edema! Ehole %rain irradiation is almost al&ays indicated! 1ronosis
depends on tumor type# &ith the median sur$i$al ranin from 7 to 9 years! Lon;
term sur$i$ors ha$e %een reported &ith surical remo$al of solitary %rain metastases!
Iuality of life is almost al&ays impro$ed! There is little e$idence that chemotherapy
plays a sinificant treatment role!
Tumor metastasis to the leptomenines (menineal carcinomatosis) is also 2uite
common# particularly in the childhood leu'emias and in adults &ith lymphoma# %reast
and lun cancers# and melanoma! 1atients may present &ith cranial ner$e palsies#
radiculopathies# or o%structi$e hydrocephalus! They often ha$e sins and symptoms
suesti$e of meninitis! Analysis of the CS+ is usually critical# often re$ealin an
increased openin pressure# an ele$ated &hite cell count and protein le$el# and a
decreased lucose le$el! There may or may not %e identifia%le malinant cells# %ut
cytoloic examination should al&ays %e done!
Treatment of menineal carcinomatosis usually in$ol$es radiation therapy and
intra$entricular chemotherapy! -ethotrexate is a common chemotherapeutic aent!
The outloo' for patients &ith leptomenineal tumor spread is enerally poor# %ut
aain a fe& lon;term sur$i$ors emere!
Spinal Tumors
Spinal tumors constitute approximately 96 percent of all CNS tumors! They are
classified as intradural or extradural! Of the intradural $ariety# ?= percent are outside
the spinal cord (extramedullary) and 7: percent are &ithin it (intramedullary)!
Intradural tumors are almost al&ays primary CNS tumors# &hereas the ma,ority of
extradural tumors are either metastatic or primary %one tumors! The ma,ority of
intradural spinal neoplasms are %enin and can often %e excised surically! Tumors
occurrin &ithin the cord (intradural# intramedullary) tend to produce &ea'ness#
spasticity# and sensory loss! Extramedullary lesions present &ith radicular pain from
ner$e root (lo&er motor neuron) compression as &ell as &ith lon tract (upper motor
neuron) sins from cord compression! 1atients &ith lesions in$ol$in the conus
medullaris reion may ha$e early loss of %ladder and %o&el function3 those &ith
lesions in the cauda e2uina present primarily &ith le pain and only later de$elop
sphincter distur%ances!
The definiti$e study for spinal tumors is -RI# althouh a%normalities on plain films
and myelorams may %e dianostic! 1lain films may sho& &idenin of the
interpeduncular distance# %ony erosion# enlarement of neural foramina# or a
paraspinous mass! -yeloraphy helps to determine the tumor>s relationship to the
spinal cord and dura! 1ostmyeloram CT can further define that relationship!
Neurilemoma and Neurofi%roma
Typically %enin# these are the most common spinal cord tumors# comprisin almost
86 percent of the total! They are usually intradural# extramedullary in location! Of
these# 78 percent ha$e extradural extension throuh an ad,acent foramen# producin
the classic Cdum%%ellD shape of the tumor! +ourteen percent are totally extradural! The
extradural component tends to enlare the in$ol$ed foramen! Treatment is surical
remo$al! -ultiple neurofi%romas are associated &ith $on Rec'linhausen>s
neurofi%romatosis! In these instances# only symptomatic tumors should %e remo$ed!
-eninioma
-eniniomas constitute 9: percent of spinal cord tumors# are %enin# and are usually
intradural# extramedullary (+i! =6;79)! +ifteen percent occur extradurally! T&o;thirds
arise in the thoracic spine# affectin &omen in their fourth throuh sixth decades in ?6
percent of cases! Surical excision is the treatment of choice!
Ependymoma
Arisin from the ependymal cells of the central canal of the cord# these intramedullary
tumors constitute 78 percent of all spinal cord tumors! They occur more fre2uently in
males! Nearly :6 percent are found in the conus medullaris reion! Ependymomas
should %e surically excised! Their distinct %orders often allo& complete resection3
&hen total remo$al is not possi%le# radiation therapy is usually employed!
Astrocytoma
These lial tumors are deri$ed from astrocytes and are often intramedullary! Their
incidence is a%out the same as that of spinal ependymomas! Total excision is rarely
possi%le due to their infiltrati$e nature! Lo&;rade astrocytomas# if recurrent# are
usually reoperated! Radiation therapy is reser$ed for the malinant astrocytomas# %ut
this is usually only palliati$e! Ehile the ro&th rate of spinal cord astrocytomas is
slo&# pronosis is enerally poor!
Lipoma
Lipomas constitute 76 percent of spinal tumors and are often associated &ith spina
%ifida and a su%cutaneous lipoma! Althouh %enin# they tend to %e intert&ined &ith
cord tissue and are usually only partially excised! These do not re2uire radiation# and
the mortality is lo&!
Dermoid
Dermoids are conenital lesions usually found in the lum%osacral area! They often
ha$e an associated sinus tract to the s'in surface and may present &ith infection! The
treatment is surical resection# includin the sinus tract! The resection of the portion
enterin the spinal cord is usually incomplete! The lon;term pronosis is ood!
-etastatic Tumor
.p to 95 percent of all spinal neoplasms are metastatic in oriin and most appear in an
extradural location (+i! =6;78)! Common primary sites include %reast# lun# prostate#
and 'idney! If the primary site is not 'no&n or if the neuroloic decline is rapid#
treatment is surical decompression &ith %iopsy! Other&ise# local radiation therapy is
the treatment of choice! Other extradural malinant tumors include lymphoma#
myeloma# plasmacytoma# chordoma# and osteoenic sarcoma! Ehen sinificant %one
destruction or the surical decompression renders the spine unsta%le# surical
sta%ili*ation throuh an anterior or posterior route is often necessary!
1eripheral Ner$e Tumors
The peripheral ner$ous system includes the peripheral and cranial ner$es# spinal roots#
and autonomic ner$ous system! Tumors can arise from any of these elements! The
more common tumors are discussed %elo&! -ore unusual tumors include
anlioliomas# neuro%lastomas# paraanliomas# chemodectomas# and
pheochromocytomas!
Sch&annoma
This tumor arises from the peripheral ner$e Sch&ann cells that pro$ide the myelin
sheaths for axons! Sch&annomas tend to displace the ner$e of oriin and thus usually
present as a painless mass! Eith continued ro&th# they can create pain in the
distri%ution of the ner$e! As they enlare# ner$e function deteriorates! They tend to
arise from sensory ner$es %ut may also %e found on motor ner$es! The treatment is
surical excision! The ner$e of oriin can usually %e preser$ed! At times# ho&e$er#
total excision may mean di$ision of the parent ner$e! If the ner$e ser$es a sinificant
function# it is prefera%le to lea$e a portion of the tumor in order to spare the ner$e!
This is ,ustified %ecause malinant transformation is rare!
Neurofi%roma
Neurofi%romas differ from sch&annomas in that they actually enulf the ner$e of
oriin# since they arise from the ner$e itself! They are often cutaneous# ma'in it
difficult to identify one specific ner$e of oriin! Ehen associated &ith $on
Rec'linhausen>s neurofi%romatosis# they are usually multiple! Ehen found sinly#
treatment is resection! Ehen multiple tumors are present# only the symptomatic ones
should %e resected! Remo$al re2uires sacrifice of the ner$e if that ner$e is
expenda%le! If the function of the ner$e is critical# a portion of tumor should %e left
attached to the ner$e! .nli'e in the case of sch&annomas# patients &ith
neurofi%romas should %e follo&ed closely# since these tumors ha$e a hiher incidence
of malinant transformation!
-alinant Ner$e Sheath Tumor
These tumors typically occur after the ae of thirty! The treatment of choice is radical
&ide resection! If there is e$idence of muscle or soft tissue in$asion# amputation of
the in$ol$ed extremity is recommended! These tumors are enerally resistant to
radiation therapy!
CERE<RO0ASC.LAR DISEASE
Cere%ro$ascular disease is the third most common cause of death in the .nited States
and is a sinificant cause of disa%ility! Death and disa%ility are due either to ischemia
causin focal or diffuse infarction or to hemorrhae causin compressi$e mass
lesions! Althouh discussed separately# cere%ro$ascular pro%lems producin infarction
may %ecome hemorrhaic# and hemorrhaic lesions may lead to infarction!
Ischemic 0ascular Disease (Stro'e)
Ischemia and su%se2uent infarction of the %rain can occur in the distri%ution of any of
the cere%ral $essels3 thus any portion of the cere%rum# %rainstem# or cere%ellum may
%e affected! <ecause the carotid circulation pro$ides the reatest %lood supply to the
%rain# ischemia and infarction &ithin its distri%ution are most common! Ischemia may
%e the result of diminished flo& secondary to stenosis or occlusion of ma,or arteries or
due to transient or permanent occlusion of smaller arterioles from intra$ascular
em%oli!
The most common cause of stenosis or occlusion of lare $essels is atherosclerosis!
This disease often de$elops extracranially at the oriin of the internal carotid artery in
the nec' %ut may occur in the carotid siphon (that portion of the artery &ithin the
ca$ernous sinus)# the distal internal carotid# or e$en the proximal middle cere%ral
artery (+i! =6;7=)!
Arterial em%oli usually oriinate either from atherosclerotic ulceration in the reion of
the carotid %ifurcation or from sources &ithin the heart! The heart is a common source
of em%oli &hen a mural throm%us forms after a myocardial infarction or as a result of
atrial fi%rillation! Other ris' factors for cere%ral ischemia include hypertension#
dia%etes# hypercholesterolemia# o%esity# smo'in# and family history of stro'e!
Since there is no effecti$e medical or surical therapy for a completed stro'e# the oal
of neurosurical inter$ention is to identify stro'e;prone patients and reduce their ris'
of cere%ral ischemia! These hih;ris' patients are %est identified %y a history of
transient ischemic attac's (TIAs)# &hich ta'e the form of either transient cere%ral
ischemia or amaurosis fuax! Transient cere%ral ischemia in the carotid circulation
usually consists of temporary hemianesthesia# hemiparesis# or aphasia! Amaurosis
fuax is transient loss of $ision in one eye! Ischemia in the $erte%ro%asilar system
may cause transient diplopia# di**iness# dysarthria# dysphaia# &ea'ness# num%ness#
loss of $ision# or e$en loss of memory!
-ost ischemic episodes last seconds to minutes and rarely loner than 86 min! As
lon as the neuroloic deficit resol$es &ithin 9= h# the episode is# %y definition# a
TIA! A re$ersi%le ischemic neuroloic deficit (RIND) is one that lasts 9= h to 8
&ee's! Ischemic deficits lastin loner are considered completed stro'es! Careful
2uestionin of indi$iduals &ith completed stro'es re$eals that :6 percent had a prior
history of TIAs# 96 percent presented in a slo&# step&ise fashion# and only 96 percent
&ere sudden in onset!
1atients &ith TIAs or slo&;onset stro'es are potential candidates for pre$enti$e
surical inter$ention! Surical procedures to pre$ent stro'e are directed to&ard either
remo$al of the source of em%oli or aumentation of %lood flo& to the %rain!
Operations for these conditions include carotid endarterectomy and micro$ascular
%ypass! 1otential candidates enerally undero a CT or -RI scan of the %rain to
e$aluate any deree of cere%ral infarction and to rule out other dianoses such as
tumor# su%dural hematoma# or su%arachnoid hemorrhae! 1atients then undero
anioraphy# includin the aortic arch and the carotid# $erte%ral# and cere%ral arteries!
Nonin$asi$e studies of the carotid circulation are less accurate# althouh they are
useful as screenin procedures %ecause of their lo& ris'!
Carotid endarterectomy is indicated &hen ipsilateral symptoms of cere%ral ischemia
or amaurosis fuax exist and anioraphy demonstrates either sinificant stenosis
(usually more than 45 percent) or ulceration in the accessi%le portion of the common
and/or proximal internal carotid arteries (+i! =6;75)! The procedure consists of
openin the affected portion of the carotid artery under systemic heparini*ation and
remo$in the atherosclerotic pla2ue! The mortality from carotid endarterectomy is
a%out 7 percent and the neuroloic mor%idity 5 percent in experienced hands!
A num%er of patients present &ith cere%ral ischemia ipsilateral to an occluded internal
carotid artery or &ith stenosis of the internal carotid or middle cere%ral artery that is
not surically accessi%le! +or these patients &ith inade2uate collateral cere%ral
circulation# a micro$ascular %ypass procedure is sometimes indicated! The most
common of these is the superficial temporal artery to middle cere%ral artery
anastomosis (STA; -CA)!
"n#racranial Aneurys$
Intracranial aneurysms are diseased dilatations of the cere%ral arteries# their &alls
consistin of %allooned;out tunicae intima# media# and ad$entitia &ith a $aria%le
deree of intraluminal or mural throm%us! -ost are conenital in oriin# e$ol$in and
de$elopin durin life! They may %ecome atherosclerotic! Aneurysms are typically
found at the %ifurcation of the ma,or $essels of the circle of Eillis! .p to 96 percent
of patients &ith aneurysms ha$e multiple aneurysms# and 7 percent demonstrate an
associated arterio$enous malformation (A0-)! If aneurysms are found more
peripherally in the cere%ral $asculature# secondary causation such as trauma or
infection should %e considered!
O$er ?5 percent of cere%ral aneurysms occur in the carotid or CanteriorD circulation!
Approximately 86 percent arise from the intracranial portion of the internal carotid
artery# usually at or near the oriin of the posterior communicatin artery! Another 86
percent occur in the reion of the anterior communicatin artery! A%out 95 percent
arise from the middle cere%ral artery# usually at its first ma,or %ranch point# &hich is
commonly a trifurcation! Aneurysms of the $erte%ro%asilar or CposteriorD circulation
are most fre2uently found at the tip of the %asilar artery %ut may occur more
proximally alon its trun'! The oriin of the posterior inferior cere%ellar artery is the
next most common location!
1atients &ith intracranial aneurysms most commonly present &ith sins and
symptoms of su%arachnoid hemorrhae (SA")! Eihty percent of nontraumatic
su%arachnoid hemorrhaes are caused %y aneurysm rupture! The patient notes a
sudden se$ere headache commonly follo&ed %y nec' stiffness and photopho%ia due to
associated menineal irritation caused %y the su%arachnoid %lood! Transient loss of
consciousness may occur! Some patients may de$elop a focal neuroloic deficit or
%ecome comatose as a result of the acute rise in intracranial pressure! The se$erity of
the SA" can %e raded# as sho&n in Ta%le =6;8! In eneral# the lo&er the rade# the
%etter the outcome!
Not all patients &ith aneurysms present &ith symptoms related to rupture! Throuh
mass effect# an internal carotid artery (ICA) aneurysm may compress the optic (IId)
ner$e (+i! =6;7: A# <)# causin monocular %lindness# or the oculomotor (IIId) ner$e#
producin a palsy characteri*ed %y diplopia# ptosis# and dilated pupil! An ICA
aneurysm &ithin the ca$ernous sinus may compress the a%ducens (0Ith) ner$e and
create diplopia! A iant aneurysm (larer than 95 mm in diameter) of the %asilar tip
may %loc' the cere%ral a2ueduct and create hydrocephalus! Rarely# an aneurysm may
%e lare enouh to %e mista'en for a tumor!
The dianosis of SA" is usually made clinically and confirmed either %y notin %lood
&ithin the su%arachnoid spaces on CT scan or %y findin %loody CS+ &ith
xanthochromia on a lum%ar puncture (L1)! The CT scan should %e o%tained first#
since it usually spares the patient an L1 and also eliminates the potential ris' of
%rainstem compression from herniation if an unsuspected mass lesion is present!
Complete cere%ral anioraphy is then employed to identify and delineate the
aneurysm and# at the same time# rule out multiple aneurysms or an associated A0-!
Once the dianosis of aneurysmal rupture is confirmed# the patient is placed on a
medical reimen to reduce the ris' of re%leedin! This includes strict %ed rest &ith the
head ele$ated! Stimulation is 'ept to a minimum! <lood pressure is tihtly controlled
to 'eep it %elo& 756 mm" systolic! Careful o%ser$ation is necessary to &atch for
sins of raised intracranial pressure &hich may %e attri%uta%le to delayed
hydrocephalus! Anticon$ulsants are started for sei*ure prophylaxis! Calcium channel
%loc'ers are used to reduce the ris' of $asospasm!
The ultimate treatment of aneurysms is microsurical dissection and o%literation#
usually %y placin a metallic clip across the aneurysms>s nec' $ia a craniotomy!
Timin of surery depends on the clinical rade of the patient (see Ta%le =6;8)! Good;
rade (I and II) patients should usually undero operation &ithin 49 hours of rupture!
1oor;rade (III and I0) patients should continue intensi$e medical manaement until
they impro$e to a lo&er rade# if possi%le# %ecause mortality rises &ith rade!
Surically accessi%le unruptured aneurysms should %e operated on electi$ely to
pre$ent rupture! Some inaccessi%le aneurysms can %e effecti$ely o%literated %y
em%oli*ation usin inter$entional neuroradioloic techni2ues!
Complications of aneurysmal rupture include a 86 percent re%leedin rate &ithin the
first ? &ee's if the lesion remains unrepaired# hydrocephalus from o%struction of the
arachnoid $illi %y su%arachnoid clot# $asospasm# intracere%ral hematomas# raised
intracranial pressure# and sei*ures! The most sinificant and least understood of these
is cere%ral $asospasm! This phenomenon occurs most fre2uently &ithin = to 4 days
after the hemorrhae and results in narro&in of ad,acent cere%ral arteries! 0asospasm
may %e noted on anioraphy &ithout any unto&ard clinical effects# or it may produce
profound and life;threatenin cere%ral ischemia in the distri%ution of the in$ol$ed
$essels!
Anioplasty done %y inter$entional neuroradioloic techni2ues can# in selected cases#
increase cere%ral %lood flo& in order to o$ercome the spasm! Impro$ed perfusion
throuh dilated spastic arterial sements com%ined &ith induced hypertension can
re$erse ischemic neuroloic deficits! The use of inotropic support and intra$ascular
$olume expansion# usually &ith colloid and red cell transfusion# is also %eneficial!
Cardio$ascular status is monitored continuously %y means of a S&an;Gan* catheter!
1atients &ho undero electi$e clippin of unruptured aneurysms ha$e %etter outcomes
than those &ith ruptured aneurysms %ecause the %rain has not %een in,ured %y the
su%arachnoid hemorrhae! In addition# aneurysms of the internal carotid artery carry
less ris' than those of the $erte%ro%asilar system# &ith the exception of complex
anterior communicatin artery aneurysms! In eneral# if the aneurysm can %e clipped
and $asospasm a$oided or effecti$ely o$ercome# most patients do &ell!
Ar#erio%enous Malfor$a#ion
Arterio$enous malformations (A0-s) occur &ithin the central ner$ous system as
conenital a%normalities that allo& %lood to %e shunted directly from arteries to $eins#
%ypassin the normally interconnectin capillary %ed! These malformations may %e
2uite small &ith only a sinle feedin artery# or they may encompass se$eral lo%es of
the %rain and arterial feeders from multiple sources! They may occur in $irtually any
portion of the %rain# includin the cere%ellum and %rainstem! In the cere%ral
parenchyma# &here they are most commonly located# the lesion ta'es on a conical
shape# &ith the apex deep# often reachin the lateral $entricle! Rarely# A0-s occur
&ithin the spinal cord# and they may exclusi$ely in$ol$e the dura either intracranially
or &ithin the spinal canal!
1atients &ith A0-s tend to de$elop symptoms %efore ae thirty! The most common
initial presentation is hemorrhae (56 percent of cases and 76 percent of all
intracere%ral hemorrhaes# second only to aneurysms)! <leedin usually occurs &ithin
the %rain su%stance %ut may occur &ithin the $entricular system or the su%arachnoid
space! The patient experiences a sudden headache# often associated &ith loss of
consciousness and/or a neuroloic deficit! The next most common presentin
symptom is a sei*ure! In a fe& cases# sei*ures may %e fre2uent and refractory to
medical therapy! A0-s may also present &ith the insidious onset of a focal
neuroloic deficit due to mass effect# increased $enous pressure# or $ascular steal
phenomenon! Occasionally# youn patients &ith se$ere unrelentin headaches are
found to har%or an A0-!
+rom the time of disco$ery of an unruptured A0-# the ris' of hemorrhae is a%out 7
percent per year cumulati$ely! Once an A0- has %led# the ris' of re%leedin increases
to 5 percent per year! Eith each hemorrhae# the ris' of dyin is approximately 76
percent and mor%idity at least 75 percent! Smaller A0-s are more li'ely to %leed than
larer ones! -ost A0-s remain sta%le in si*e# %ut some enlare &ith time! .p to 76
percent ha$e an associated aneurysm on a feedin artery! In these cases# hemorrhae
is usually due to rupture of the aneurysm!
An A0- can %e identified on contrast;enhanced CT scannin as a hyperdense mass#
part of &hich has a serpentine confiuration related to the presence of lare drainin
$eins! Its confiuration and extent are more easily delineated &ith -RI! After
hemorrhae# an unenhanced CT scan usually demonstrates intracere%ral or
su%arachnoid %lood! A0-s may %e too small to %e seen on CT# so careful
anioraphy may %e necessary to identify the source of hemorrhae! Lum%ar puncture
may %e necessary if su%arachnoid hemorrhae is suspected clinically %ut not $erified
%y the CT scan!
In all cases of suspected or pro$en A0-# complete cere%ral anioraphy must %e
underta'en to define carefully the extent of the malformation (+i! =6;74 A# <)! All
feedin arteries# includin any from the external carotid system as &ell as the drainin
$eins# must %e e$aluated! It is only from hih;resolution anioraphy that a treatment
decision can %e reached! If anioraphy fails to delineate a lesion despite suspicion#
-RI may identify the Canioraphically occultD a%normality!
The treatment of A0-s is dependent on the si*e and location of the lesion# the
presentin symptoms# and the ae and condition of the patient! <ecause of the ris' of
re%leedin# an A0- that has %led should %e surically excised if possi%le! The
treatment decision is more difficult in the patient &ho presents &ith sei*ures! If the
patient is youn and the malformation is readily accessi%le# surical resection is
usually recommended# especially &hen the sei*ures are medically refractory!
Operation in$ol$es the microsurical dissection and resection of the entire
malformation# rather than simple liation of feedin arteries! The results of operation
are related to the si*e and location of the malformation! O$erall# the operati$e
mortality is less than 5 percent and the mor%idity less than 76 percent!
Alternati$e or ad,uncti$e methods of treatment include intraarterial em%oli*ation and
radiation therapy! .sin inter$entional neuroradioloic techni2ues# particulate matter
or lues may %e introduced into A0-s $ia feedin $essels to occlude the $ascular
shunt nidus! It is rarely possi%le to o%literate these lesions completely &ith this
method# ho&e$er! This techni2ue ne$ertheless can reduce flo& throuh the A0- prior
to direct surical inter$ention!
Ioni*in radiation# on the other hand# has the capa%ility of completely o%literatin
selected small; to medium;si*ed A0-s! +ocused amma or proton %eam irradiation
has demonstrated efficacy# and occasional success &ith con$entional irradiation has
%een reported! Ioni*in radiation causes endothelial proliferation and may ta'e :
months to 9 years to o%literate the lesion! +ocused irradiation is recommended for
deep# surically inaccessi%le A0-s!
"n#racere&ral He$orrhage
Spontaneous hemorrhae is most commonly associated &ith systemic hypertension
and occurs in predicta%le locations# includin the putamen# thalamus# cere%ellum# and
pons! "emorrhae can also occur &ithin the $arious lo%es of the %rain!
Nonhypertensi$e causes of %rain hemorrhae ha$e %een discussed a%o$e# such as
rupture of A0-s and aneurysms and hemorrhae into areas of ischemia! Additional
causes include induced or endoenous coaulopathies# primary or metastatic %rain
tumors# and rare conditions such as amyloid aniopathy!
Chronic hypertension results in lipohyalinosis of the $essel &all# &hich sets the stae
for either $ascular occlusion or rupture! Occlusion results in infarction# and rupture
produces an intracere%ral hemorrhae! The shorter penetratin arteries of the %rain
appear to %e the most $ulnera%le! The lenticulostriate and thalamoperforatin $essels
are in$ol$ed in putaminal and thalamic hemorrhaes# and affected %asilar %ranches
contri%ute to pontine hemorrhae!
Althouh %rain hemorrhae is often de$astatin# it may %e surprisinly &ell tolerated!
The hematoma tends to dissect alon axonal planes# separatin rather than destroyin
$ital structures! If the resultant mass is tolerated %y the patient# the %lood is slo&ly
resor%ed %y macrophaes alon the periphery# lea$in only a hemosiderin;stained slit
in the %rain! 1atients may &orsen clinically anytime after the initial hemorrhae as a
result of associated edema formation!
"emorrhae into the putamen accounts for the ma,ority of hypertensi$e hematomas!
1resentation is characteri*ed %y the lac' of headache &ith radual de$elopment of
hemiparesis proressin to hemipleia! This may %e associated &ith a hemisensory
loss# aphasia# hemianopia# and/or ipsilateral de$iation of the eyes# dependin on the
si*e of the hematoma and its direction of dissection! The patient may# of course#
proress into coma if the lesion is lare! Similarly# thalamic hemorrhae presents
initially &ith a hemisensory loss and hemiparesis! Locali*in features include
do&n&ard eye de$iation &ith limitation of $ertical a*e and small# sluish pupils
due to in$ol$ement of the near%y mesencephalon! "eadache is uncommon!
Cere%ellar hemorrhae is sudden in onset and presents &ith headache! 0omitin#
ataxia# and di**iness are accompanyin features! This hemorrhae is extremely
danerous in that it may cause coma and ultimately death due to %rainstem
compression and acute hydrocephalus! <rainstem hemorrhae (usually pontine) is the
most de$astatin and often presents &ith 2uadriparesis# decere%rate posturin#
pinpoint pupils# and coma! -ost patients do not sur$i$e if the hematoma is larer than
7 cm in si*e! -oreo$er# those &ho do sur$i$e ha$e a hih deree of mor%idity!
Lo%ar hemorrhae is less li'ely to %e associated &ith hypertension and# in eneral# is
%etter tolerated %y the patient! The symptoms depend on the area of %rain in$ol$ed!
CT scannin has %ecome an in$alua%le tool in dianosin and definin %rain
hemorrhae! CT not only delineates the hemorrhae %ut also permits assessment of
$entricular si*e# the presence of edema# and often the cause of the hemorrhae (e!!#
A0-# tumor# aneurysm)! The hematoma appears hyperdense in the acute phase (+i!
=6;7?)! Eith time# as the %lood %rea's do&n# the clot proresses to a hypodense
lesion! At any time# from days to &ee's# the hematoma may demonstrate an
enhancin rin! If a $ascular lesion is suspected# careful anioraphy is indicated! In
all cases# appropriate coaulation studies should %e o%tained!
The treatment of %rain hemorrhae may %e medical or surical dependin on the si*e
of the lesion# its location# and the condition of the patient! Surical resection is
recommended if the patient is deterioratin neuroloically# no matter &hat the si*e of
the hematoma! Cere%ellar hematomas are particularly important to remo$e# since a
small chane in surroundin reacti$e edema may result in life;threatenin %rainstem
compression and/or hydrocephalus!
<ecause hematomas are mass lesions# medical manaement is directed to&ard
'eepin the intracranial pressure under control! If the hemorrhae renders the patient
unconscious# hyper$entilation and hyperosmolar aents may %e re2uired to control
IC1! IC1 monitorin may %e a helpful ad,unct to direct treatment! Steroids are useful
in controllin %rain edema if the patient has %led into a tumor# and coaulopathies
should %e corrected! Despite medical and/or surical therapy# mortality and mor%idity
remain hih from all types of %rain hemorrhae!
'E(ENERAT")E *P"NE '"*EA*E
Anatomy and 1athophysioloy
The spinal column is composed of 88 $erte%rae ma'in up those di$isions la%eled
cer$ical (4)# thoracic (79)# lum%ar (5)# sacral (5 fused)# and coccyeal (= fused)! Each
$erte%ra consists of a %ody# &hich %ears &eiht# and the posterior elements (pedicles#
laminae# spinous and trans$erse processes)# &hich pro$ide the flexi%ility and sta%ility
to the $erte%ral column! The spinal canal has an o$oid shape in the trans$erse plane
throuh the cer$ical and thoracic reions and assumes a more trianular shape in the
lum%ar reion! -ost spine mo$ement occurs in the cer$ical and lum%ar reions!
+lexion and extension are reatest in the lo&er cer$ical and lum%ar sements# and
maximum rotation occurs predominantly in the upper cer$ical and lum%ar sements!
The inter$erte%ral disc consists of t&o parts! The circumferential anulus fi%rosus#
ma'in up the outer portion# is composed of dense# fi%rous tissue! The central nucleus
pulposus consists of fi%rocartilae# &hich has little tensile strenth %ut su%stantial
elasticity! The fi%rocartilae may frament acutely or deenerate radually! It heals
poorly %ecause of limited %lood supply! The anulus heals &ell and is %uttressed %y
hea$y anterior and posterior lonitudinal liaments! Inter$erte%ral disc disease may
occur at any le$el from C7 to S7! The lo&er sements of the cer$ical and lum%ar areas
are affected most often! Thoracic disc disease is rare!
The spinal cord extends from the cer$icomedullary ,unction at the %ase of the s'ull to
the conus medullaris at the L7HL9 le$el! The cord is centrally placed &ithin the spinal
canal and mo$es rostrally and caudally a fe& millimeters durin spinal flexion and
extension! Lateral motion of the cord is restricted %y intradural dentate liaments! The
%lood supply is pro$ided %y radicular arteries# &hich arise from the $erte%ral arteries
and the thyrocer$ical trun's in the nec'# from the intercostal arteries in the thorax# and
from the lum%ar arteries in the lo& %ac'! An arterial confluens# the artery of
Adam'ie&ic*# is typically found in the T76HL9 reion# usually on the left side! It
supplies the lo&er thoracic cord and conus medullaris!
Three fi%er tracts of the spinal cord are important clinically (see +i! =6;8)! The
laterally positioned corticospinal tracts carry motor fi%ers from the cortical upper
motor neurons to the spinal lo&er motor neurons located in the $entral horns of the
spinal cord! These tracts cross the midline at the pyramidal decussation in the lo&er
medulla! The spinothalamic tract# also positioned laterally# transmits pain and
temperature sensation from the contralateral side of the %ody! Its axons cross throuh
the anterior commissure of the cord &ithin t&o or three sements of each dorsal root
entry *one and ascend to the ipsilateral thalamus! The dorsal columns carry sensory
fi%ers con$eyin position# $i%ratory# and liht touch sensation from the dorsal roots
rostrally on the same side# then cross to the opposite cere%ral cortex throuh a
decussation in the %rainstem!
Dorsal and $entral ner$e roots emere from the spinal cord separately and pass to
their respecti$e inter$erte%ral foramina# &here they exit from the spinal canal (see
+i! =6;9)! The roots ,oin to form a spinal ner$e &ithin the neural foramen! In the
cer$ical spine# the roots exit a%o$e the correspondin $erte%rae3 for instance# the C5
root exits a%o$e the C5 pedicle! Since there are eiht cer$ical roots# C4 exits a%o$e
the C4 pedicle and C? exits %elo& it! Conse2uently# all roots %elo& C? exit %elo& the
pedicle of their correspondin $erte%ra!
Lum%ar and sacral roots form the cauda e2uina %elo& the conus medullaris! The
sacral roots are more centrally located ad,acent to the filum terminale! <ecause a
lum%ar root (e!!# L=) passes laterally to&ard the neural foramen as it descends &ithin
the spinal canal# it crosses the ad,acent inter$erte%ral disc (e!!# L=HL5) at its extreme
lateral ede# huin the pedicle of the L= $erte%ra laterally! The ner$e root that
descends to the next lo&est foramen (e!!# L5) passes across the disc space (e!!# L=H
L5) more medially# ma'in that root more $ulnera%le to disease in$ol$in that disc
(+i! =6;7F)!
Inter$erte%ral Disc Disease
If the nucleus of an inter$erte%ral disc extrudes (herniates) throuh the anulus#
ad,acent neural structures may %e compressed! In the cer$ical and thoracic spine#
compression of the spinal cord may result in paraparesis or 2uadriparesis# dependin
on the spinal sement in$ol$ed! At all le$els# compression of a spinal root may cause
&ea'ness and sensory loss in structures inner$ated %y that root! The se$erity of the
clinical syndrome depends on the site and se$erity of compression %y the displaced
disc frament! In some cases the anulus and ad,acent liament hold# pre$entin
complete extrusion of the framented disc! The anulus may only stretch sufficiently to
allo& the disc to %ule into the spinal canal or foramina# accountin for %ac' and le
pain# %ut often &ithout neuroloic deficit!
Often the nucleus does not extrude# %ut simply framents in response to the forces
exerted on the spinal column! This is intensified %y the concomitant dehydration and
loss of elasticity of the disc as it aes! The disc space radually narro&s# the ,oint
%ecomes loose# and the cartilainous endplates of the ad,acent $erte%ral %odies a%ut
and &ear more 2uic'ly! <ony spurs (osteophytes) de$elop at the ,oint in reaction to
the increased mo%ility and decreased elasticity!
+ormation of osteophytes around the ,oints of $erte%rae# termed spondylosis# is a
common disorder that represents the normal process of ain! If an osteophyte forms
in a neural foramen# the ner$e root passin throuh may %e chronically irritated and
compressed! If the osteophyte de$elops &ithin the cer$ical or lum%ar canal# the cord
or cauda e2uina may %e compromised!
The onset of symptoms and sins of an extruded disc frament may %e acute or
chronic! Acute symptoms may or may not %e related to trauma! In disc disease of the
cer$ical spine# nec' and radicular discomfort occur simultaneously! Spinal cord
symptoms are rare! There is usually limitation of nec' motion# &ith loss of normal
cer$ical lordosis! Eith foraminal osteophytes# episodes of cer$ical discomfort recur
o$er many months or years %efore radicular symptoms appear! Interscapular achin#
su%occipital headaches# and e$en chest pain are common complaints!
Ner$e root compression produces radiculopathy# often characteri*ed %y pain and
hypoesthesia in the distri%ution of the in$ol$ed root! Associated loss of deep tendon
reflex &ith or &ithout &ea'ness may %e seen on examination! Cer$ical cord
compression causes myelopathy characteri*ed %y proressi$e spastic 2uadriparesis or
paraparesis# mild to moderate sensory chanes in the lo&er extremities and trun' &ith
cer$ical dermatomal sensory loss# &ea' upper extremities# hyperreflexia# and extensor
plantar response!
Cer%ical 'isc 'isease
Cer$ical disc disease must %e differentiated from other ailments! These include
inflammatory disease of the soft tissues and ,oints of the arm and shoulder# ner$e
entrapment syndromes# and neoplasms! The pain must %e distinuished from that
&hich accompanies cardiac disease! Spinal infections# conenital lesions# and
posttraumatic disorders are other important considerations!
1lain radioraphs typically demonstrate loss of the lordotic cur$e of the cer$ical
spine# &ith narro&in of one or more disc spaces! Osteophyte formation may %e seen!
In cer$ical spondylosis# there is usually radioloic e$idence of osteophytes and disc
space narro&in at multiple le$els! In most cases# the anterior;posterior diameter of
the cer$ical spinal canal is narro&ed! -yeloraphy &ith CT is $ery useful in the
dianostic &or'up of ner$e root compression! The use of intrathecal contrast medium
enhances the po&er of CT to delineate the lesion! -RI is suita%le for in$estiatin
myelopathies! In addition to definin the compressi$e lesion# -RI often sho&s
intrinsic cord a%normalities related to compression! Electromyoraphy may confirm
the dianosis and locali*e the lesion more specifically# particularly &hen
myeloraphic defects are multiple!
Treatment
1ainful cer$ical disc disease may %e treated medically as lon as there is no e$idence
of a proressi$e neuroloic deficit (motor loss and %o&el and %ladder dysfunction
%ein most important)! Ade2uate medical therapy includes immo%ili*ation of the nec'
&ith a soft or hard cer$ical collar# analesics# muscle relaxants# and local heat! These
methods# in association &ith a ood physical therapy proram# pro$ide relief under
most circumstances!
.p to 45 percent of patients &ith cer$ical disc disease impro$e follo&in an ade2uate
trial (76 to 7= days) of medical therapy! Some ha$e recurrence of radicular symptoms
on return to full acti$ity! In many cases# these patients can %e manaed for years &ith
intermittent cer$ical traction and a cer$ical collar# %ut some re2uire surical therapy!
+or the 95 percent &ho do not respond to conser$ati$e means# operation is often
helpful!
There are t&o approaches for the surical treatment of cer$ical disc disease!
Anteriorly# ner$e roots# spinal cord# or %oth may %e decompressed throuh discectomy
&ith or &ithout %one raft fusion! The other approach is posteriorly throuh a
laminectomy and/or foraminotomy! The choice of operati$e direction is %ased on
consideration of the patient>s anatomic lesion! Impro$ement follo&s operati$e
treatment of symptomatic cer$ical disc disease %y either approach in approximately
?6 percent of patients &ho fail to respond to medical treatment! Surical treatment of
cer$ical spondylotic myelopathy results in impro$ement in most cases! Arrest of the
proressi$e myelopathic deficit usually occurs!
Lum%ar Disc Disease
"erniated lum%ar discs often produce some deree of ner$e root compression! The
se$erity of the syndrome depends on the deree of root compression! Occasionally#
the entire cauda e2uina may %e in$ol$ed# resultin in loss of motor and sensory
function# includin %o&el and %ladder sphincter control! Sometimes disc rupture may
occur in the midline# compressin centrally positioned sacral roots preferentially#
&ithout in$ol$ement of laterally placed lum%ar roots!
+ramentation of a lum%ar disc may occur &ithout extrusion of the nucleus pulposus
as descri%ed a%o$e for cer$ical disc disease! <ecause of loss of elasticity &ithin the
disc# mo%ility of the inter$erte%ral ,oint is increased! The anulus fi%rosus may simply
%ule &ithout tearin! Eith time# osteophytes may form around the deenerated disc
and encroach on the spinal canal and neural foramina! This deenerati$e hypertrophy
in$ol$es the liamentous structures as &ell! Stenosis of the lum%ar spinal canal is the
e$entual result# a spondylotic condition common in the elderly!
In the lum%ar spine# o$er F6 percent of clinical pro%lems arise from the L=HL5 and
L5HS7 inter$erte%ral discs! 1ain is usually chronic# %ut its onset may %e acute &hen
associated &ith fran' herniation! There may %e %ac' pain# le pain# or %oth! Radiation
of lo& %ac' pain into the %uttoc'# posterior thih# and calf is usually the same &ith
disease at the L=HL5 and L5HS7 le$els! This radiatin pain may %e exacer%ated %y
couhin# snee*in# or strainin! <endin and sittin accentuate the discomfort# &hile
lyin do&n characteristically relie$es it! 1ain is typically descri%ed as achin %ut
fre2uently has a sharp or shootin element and is limited to one lo&er extremity! Eith
lum%ar stenosis# patients are una%le to extend their spine &ithout de$elopin pain#
num%ness# and/or &ea'ness# usually in %oth lo&er extremities! In the upriht posture#
either standin or &al'in# the cauda e2uina %ecomes relati$ely ischemic# producin
neuroenic claudication! Relief is o%tained %y sittin or flexin for&ard (+i! =6;96)!
1alpation usually re$eals tenderness o$er the sciatic notch# the popliteal fossa# or
%oth! 1ara$erte%ral muscles may %e in spasm! Eith true ner$e root compression#
straiht;le raisin produces le pain that is accentuated %y dorsiflexion of the foot!
Ipsilateral le pain produced %y contralateral straiht;le raisin is hihly suesti$e
of lum%ar disc herniation! Sensory loss# &ea'ness# and loss of tendon ,er's may occur
in a $ariety of com%inations and to $aria%le derees!
<ac' pain &ith radiation to the le has many causes %esides lum%ar disc disease! The
differential dianosis includes %ony a%normalities such as su%luxation# deenerati$e
facet fracture# and osteophyte formation3 primary and metastatic tumors of the cauda
e2uina# spine# and pel$is3 inflammatory disorders# includin a%scess# arachnoiditis#
an'ylosin spondylitis# and rheumatoid arthritis3 deenerati$e lesions of the spinal
cord3 peripheral neuropathies3 peripheral $ascular occlusi$e disease includin
a%dominal aortic aneurysm3 and ynecoloic pro%lems such as endometriosis!
1lain films of the lum%osacral spine can identify conenital or ac2uired %ony chanes!
Disc space narro&in is an unrelia%le sin of symptomatic disease# since narro&in of
the disc space may occur &ithout clinical symptoms! +lexion and extension lateral
$ie&s re$eal concomitant insta%ility! -yeloraphy can %e dianostic in symptomatic
lum%ar disc disease# %ut CT alone can delineate the lesion in most cases (+i! =6;97)!
-RI has replaced myeloraphy and CT at some centers in the &or'up of lum%ar
radiculopathy! Electromyoraphy may confirm the dianosis# especially &hen
physical examination is una%le to locali*e the in$ol$ed ner$e root!
Treatment
Initially medical treatment is indicated in all patients &ho do not ha$e neuroloic
deterioration! <ed rest# local heat# analesics# and s'eletal muscle relaxants are
usually effecti$e &ithin a fe& days! 1hysical therapy and limited exercise often help
&hen the acute episode passes! A %ac' %race partially immo%ili*es the patient and can
minimi*e muscle spasm! Eith aressi$e conser$ati$e manaement# most patients
impro$e sufficiently to return to full acti$ity! Recurrent symptoms may %e treated in a
similar fashion# often successfully! Surical treatment is reser$ed for the patient &ith
an acute or proressi$e neuroloic deficit# chronic disa%lin pain# or %oth! The acute
onset of &ea'ness or sphincter distur%ance constitutes an emerency# demandin
prompt dianosis and early operation!
Operation usually entails a unilateral laminotomy &ith remo$al of the offendin disc
frament! +oraminotomy may %e necessary in the presence of osteophyte formation!
Eith lum%ar stenosis# multile$el laminectomy is curati$e! Should plain films
demonstrate any insta%ility preoperati$ely# com%inin the laminectomy &ith posterior
fusion# either &ith or &ithout instrumentation# is enerally indicated! If the imain
studies demonstrate an extruded disc frament that accounts for the clinical sins and
symptoms# ?5 to F6 percent of patients reco$er &ith surical treatment! If the
syndrome is atypical# the myeloram e2ui$ocal# and the patient poorly moti$ated#
operation is less effecti$e! Emotional factors# psycholoic distur%ances# litiation# and
industrial in,ury play an important role in the e$entual outcome# &hether the treatment
is medical or surical! Alternati$es to the laminotomy approach include percutaneous
discectomy done %y endoscopic excision of the pro%lematic nucleus pulposus!
Clinical results for the alternati$e approaches are less predicta%le!
IN+ECTIONS
The central ner$ous system may %e infected %y $iruses# %acteria# funi# and parasites!
De$elopment of infection depends on the host>s resistance (i!e!# immune defenses) and
on the infectin aent>s $irulence! <one# %rain# spinal cord# menines# and
cere%rospinal fluid may %e in$ol$ed separately or in com%ination! The routes of
infection include hematoenous dissemination# local extension from a neih%orin
source# and direct contamination throuh an open &ound! The infection may %e
diffuse# as in meninitis# or focal# as in %rain a%scess!
The clinical spectrum of sins and symptoms of CNS infection $aries from
nonspecific (such as fe$er# confusion# and lethary) to hihly specific (such as
,ac'sonian epilepsy and focal neuroloic deficits)! Conse2uently# CNS infections
present difficult dianostic and therapeutic pro%lems! Early dianosis and treatment
are critical to achie$e a successful outcome!
<acterial
Su%aleal A%scess
Locali*ed infection %et&een the alea of the scalp and the pericranium constitutes
su%aleal a%scess! .sually# the process is initiated %y contamination of an open scalp
&ound %y staphylococci# streptococci# or anaero%ic cocci! Locali*ed scalp tenderness#
&armth# and s&ellin are sins of a%scess formation! Osteomyelitis of the s'ull may
occur secondarily! Su%aleal infections rarely extend intracranially# unless the s'ull
has %een penetrated! Treatment includes open drainae# de%ridement# and systemic
anti%iotics!
Osteomyelitis
Osteomyelitis may de$elop from extension of a locali*ed infection# such as sinusitis
or mastoiditis# from direct contamination at operation or after trauma# or# rarely# %y
hematoenous spread from a distant source such as the respiratory or urinary tract! An
esta%lished s'ull or spine infection may extend to the epidural space# producin a
locali*ed a%scess (+i! =6;99)! The usual osteomyelitis pathoens are staphylococci
and anaero%ic streptococci! Occasionally# ram;neati$e oranisms and funi are
responsi%le! Treatment consists of drainae# de%ridement of infected %one# and
appropriate anti%iotics for a proloned period# usually : &ee's!
Epidural A%scess
Spinal epidural a%scess is much more common than intracranial epidural a%scess! It is
characteri*ed %y fe$er# local spinal tenderness# and rapid proression of neuroloic
deficits# often constitutin a medical and surical emerency! Radicular pain and
impairment of cord function# &ith early motor and sensory deficits includin sphincter
distur%ances# occur &ithin a fe& days! -ost epidural a%scesses are caused %y local
extension of osteomyelitis or %y hematoenous spread from a distant suppurati$e
focus! The dianosis is suested %y the clinical presentation! The CS+ often has a
mar'edly ele$ated protein le$el &ith mild pleocytosis! -RI defines the extent of the
epidural mass! If the dura is intact# infection rarely extends across it!
The most common causati$e oranisms are Staphylococcus aureus and the
streptococci! Treatment should %e immediate# %einnin &ith %road anti%iotic
co$erae until the offendin aent is identified! Specific anti%iotic therapy should %e
continued for a proloned period# often up to : &ee's! Surical drainae is necessary
&hen neuroloic deficits proress despite aressi$e medical therapy! Corticosteroids
in the perioperati$e period are %eneficial in reducin locali*ed edema# althouh
proloned use can reduce the host immune response to the infection! Reco$ery of
neuroloic function is directly related to the duration and se$erity of impairment
%efore treatment!
Su%dural Empyema
Su%dural empyema is a purulent infection of the su%dural space! It accounts for
approximately 95 percent of all intracranial infections and is usually a complication of
sinusitis# meninitis# or open contamination of the su%dural space at operation or after
trauma!
Eith sinusitis# infection can spread intracranially %y transcranial emissary $ein
throm%ophle%itis! Staphylococci# streptococci# and anaero%ic cocci are commonly
responsi%le! Once the su%dural space is $iolated# infection can spread o$er the
con$exity of the %rain! The accumulation of purulent material may %e sufficient to
produce an intracranial mass# pro$o'in ad,acent %rain s&ellin! The clinical result is
rapid neuroloic deterioration# often &ith laterali*in sins# coma# and death!
Treatment includes craniotomy &ith de%ridement# drainae# and intra$enous
anti%iotics! The source of the infection must %e treated aressi$ely! A sinus or
mastoid drainae procedure is often re2uired if this is the source! -ortality from acute
fulminant su%dural empyema from a paranasal source remains a%out 95 percent!
The dianosis of intracranial su%dural empyema is made readily %y CT or -RI scan#
%ut it may %e difficult to distinuish from su%acute or chronic su%dural hematoma!
The mass itself may %e isodense# necessitatin the administration of intra$enous
contrast! Includin the sinuses on the scan may demonstrate the source of infection!
Lum%ar puncture to o%tain CS+ for analysis ris's transtentorial herniation3 thus L1
should %e a$oided if the scan sho&s sinificant mass effect!
Spinal su%dural empyema is rare! It usually de$elops from local extension
transdurally or throuh the arachnoid in the presence of meninitis! Spinal cord
compression and trans$erse myelitis may de$elop! Treatment is emerent# consistin
of surical drainae and proloned anti%iotic administration!
-eninitis
<acterial meninitis is an acute# purulent infection of the leptomenines! It is
manifested %y fe$er# lethary# headache# nausea# $omitin# and nuchal riidity!
Sei*ures occur in approximately 96 percent of patients and cranial ner$e palsies in
a%out 5 percent! Coma may de$elop in up to 76 percent of patients &ith missed
dianoses# heraldin a poor pronosis! .ntreated %acterial meninitis is almost al&ays
fatal!
A CS+ Gram stain may demonstrate the offendin oranism in 45 percent of cases!
Cultures pro$ide a dianosis F6 percent of the time! Ehen CS+ cultures are neati$e
despite hih clinical suspicion# as in a mild case or in an incompletely treated case of
meninitis# latex alutination studies are helpful! These immunoloic studies are
specifically directed at Streptococcus pneumoniae# "aemophilus influen*ae# and
Neisseria meninitidis and are hihly sensiti$e &hen an oranism is present# e$en
&ith neati$e cultures! <lood cultures may %e positi$e and there%y helpful in the
dianosis# particularly &ith infections caused %y S! pneumoniae and N! meninitidis!
CS+ pleocytosis &ith a preponderance of polymorphonuclear cells is typical of
untreated %acterial meninitis! CS+ lucose le$el is almost al&ays reduced# and the
protein content is typically increased!
-eninitis that de$elops after a penetratin &ound or a neurosurical procedure is
usually caused %y staphylococcal# streptococcal# or ram; neati$e oranisms!
-eninitis occurrin after closed head trauma &ith either a s'ull fracture or CS+
rhinorrhea is most often caused %y S! pneumoniae! 0entricular shunt and reser$oir
infections leadin to meninitis are more li'ely due to Staphylococcus epidermidis or
aureus!
The treatment for acute %acterial meninitis depends on the causati$e oranism# its
anti%iotic sensiti$ity# and the primary source of infection from &hich the menines
&ere contaminated! The presumed dianosis is made clinically# a sample of CS+ is
o%tained $ia lum%ar puncture# and %road; spectrum intra$enous anti%iotics are
immediately started! Once culture results are a$aila%le# the choice of anti%iotics is
chaned to an appropriate sinle aent! <acterial endocarditis# pneumonia# sinusitis#
concurrent su%dural empyema# and %rain a%scess are sometimes associated &ith
meninitis! Treatment should %e directed at %oth the meninitis and the primary
source!
The extent of anti%iotic penetration into the CNS $aries# dependin on the deree of
menineal inflammation! Intrathecal administration of those anti%iotics that do not
readily cross the %lood;CS+ %arrier may %e necessary (the commonly used intrathecal
preparations are entamicin and $ancomycin)! This is especially true &hen a forein
%ody# such as a $entricular shunt# is present! Shunt remo$al is often necessary despite
intra$enous and intrathecal anti%iotic administration! Rarely# therapy may also include
the use of steroids and/or osmotic diuretics if intracranial pressure is ele$ated as a
result of cere%ral edema or locali*ed %rain a%scess!
Complications of %acterial meninitis include communicatin hydrocephalus# %rain
a%scess# su%dural empyema# and su%dural effusions# particularly after "! influen*ae
meninitis in infants! The ris' of complications is sinificantly reduced %y prompt#
early treatment!
rain A&scess
<rain a%scess is a purulent lesion of %rain tissue# %einnin as a focal infection#
usually in the &hite matter# surrounded %y a typical inflammatory response! The
%lood;%rain %arrier %ecomes disrupted! Necrosis and li2uefaction follo& the acute
inflammatory stae! E$entually# either the process is encapsulated %y fi%rous
ranulation tissue or the infection spreads throuh the parenchyma to the
su%arachnoid spaces and the $entricular system!
<rain a%scess is usually secondary to focal infection else&here! A%scesses that
de$elop %y direct intracranial extension are usually solitary and are typically found in
the frontal and temporal lo%es near ad,acent nasal sinuses or mastoid processes &here
the infection %ean (Ta%le =6;=)! -ultiple %rain a%scesses that de$elop in the septic
patient are often related to %acterial endocarditis# pneumonia# and di$erticulitis!
Cyanotic conenital heart disease &ith concurrent infection is a fre2uent source!
Direct contamination of the %rain throuh a penetratin &ound# especially &hen
accompanied %y in;dri$en %one framents# is another cause of a%scess! A%scess
formation is fre2uent amon patients &ith compromised immunity either from an
underlyin illness such as "I0 infection or durin pharmacoloic immunosuppression
such as in oran transplantation!
Sins and symptoms of %rain a%scess are related to its mass effect! "eadache# focal#
neuroloic deficits# and impaired mentation are often noted! There may %e little or no
e$idence of systemic infection# and the patient may %e afe%rile! Con$ersely# the
patient may %e mori%und from %acteremia &ith fe$er# hypotension# and a mar'edly
ele$ated &hite %lood cell count! Sei*ures may occur! 1roressi$e mass effect leads to
%rain shifts follo&ed %y coma!
Contrast CT and -RI are hihly accurate in detectin %rain a%scess and should %e
done %efore CS+ is sampled (+i! =6;98)! The CS+ of patients &ith %rain a%scess may
%e entirely normal# %ut usually some pleocytosis is noted! The causati$e oranism can
%e identified and cultured from the a%scess itself in :6 to ?6 percent of cases#
pro$ided cultures are processed carefully for %oth aero%ic and anaero%ic oranisms!
<lood cultures are also helpful# particularly if the a%scess is secondary to systemic
infection!
In certain cases of early a%scess formation or hih surical ris'# medical therapy alone
&ith appropriate parenteral anti%iotics may %e sufficient! The most effecti$e therapy#
ho&e$er# is drainae of purulent material &ith simultaneous administration of
appropriate intra$enous anti%iotics! Althouh needle aspiration may %e successful#
craniotomy &ith e$acuation and remo$al of the a%scess &all may %e necessary!
Surical drainae reduces the mass effect# there%y reducin the most critical and
danerous aspect of the infection# and allo&s accurate %acterioloic analysis!
Results of treatment for %rain a%scess depend on the patient>s neuroloic status
initially# the efficacy of the anti%iotic used# the extent to &hich the intracranial mass is
controlled %y surery# and the effecti$e treatment of the primary source of the a%scess!
Despite aressi$e surical and medical manaement# mortality rates associated &ith
%rain a%scess approach =6 percent# especially in the malnourished# chronically
de%ilitated# or immunosuppressed patient!
1ostoperati$e Infection
Any or all of the pyoenic infections descri%ed earlier may de$elop after operation!
Once identified# characteri*ed# and treated &ith appropriate anti%iotics# the infection
&ill almost al&ays su%side! Commonly isolated oranisms include Staphylococcus
aureus and epidermidis! If a forein %ody such as prosthetic material or a $entricular
shunt is in$ol$ed# eradicatin the infection %ecomes more difficult# often re2uirin a
com%ination of intra$enous and intrathecal anti%iotics and remo$al of the forein
material! Occasionally# infections can %e treated satisfactorily in the presence of
retained forein %odies# such as a shunt# pro$ided the infection is indolent!
+unal
+uni may %ecome pathoenic as a result of depression of the host immune system#
proloned systemic anti%iotic therapy# or se$ere systemic illness! Ehen the CNS
%ecomes infected# it is usually associated &ith pulmonary funal infection and
depressed host resistance! The CNS in$ol$ement may %e a diffuse meninitis or a
focal a%scess! -ultiple a%scesses may %e present! Treatment re2uires lon;term
systemic antifunal chemotherapy! Surical inter$ention is reser$ed for drainae of
a%scesses and resection of symptomatic mass lesions! "ydrocephalus# a potential late
complication# is treated &ith a $entricular shunt!
1arasitic
Ehile relati$ely uncommon in North America and &estern Europe# parasitic diseases
of the CNS are a ma,or cause of neuroloic disa%ility and death &orld&ide! Control of
these diseases remains a pu%lic health pro%lem! A ma,or emphasis is placed on their
pre$ention# for once the CNS is infested# therapeutic options are limited! Treatment#
%oth medical and surical# is usually ineffecti$e or palliati$e at %est!
Cysticercosis
Taenia solium# the por' tape&orm# infests the human CNS %y transmission of its
lar$ae throuh the %lood follo&in inestion! It is most pre$alent in eastern Europe#
Latin America# China# 1a'istan# and India! Its presence may ta'e one or all of four
forms! -enineal cysticercosis is characteri*ed %y parasitic $esicles throuhout the
%asal cisterns and CS+ path&ays# usually &ith resultant hydrocephalus! 1arenchymal
cysticercosis diffusely in$ol$es the %rain# sometimes formin lare cysts! Sei*ures
and focal deficits are common! The $entricular $ariety resem%les the menineal form!
O%structi$e hydrocephalus is commonplace! Spinal cysticercosis may %e
intramedullary or extramedullary# producin either a trans$erse myelitis or a
compressi$e myelopathy!
The dianosis rests on seroloic and radioloic testin! The presence of intracranial
cysts and calcifications &ithin s'eletal muscle is often presumpti$e of the dianosis!
1ra*i2uantel# an anthelmintic aent# is effecti$e medical therapy for systemic
infestation! Anticon$ulsants# CS+ shuntin# and occasional remo$al of symptomatic
cysts are additional treatment options!
Echinococcosis
"ydatid disease is caused %y Echinococcus ranulosus# the do tape&orm! It is
pre$alent in southern South America# northern and eastern Europe# Australia# Africa#
China# and the -iddle East! "umans may ser$e as intermediate hosts %y inestin the
lar$ae! The li$er and luns are preferentially in$ol$ed throuh hematoenous
dissemination &ith su%se2uent formation of hydatid cysts! Ehen the CNS is in$ol$ed#
cysts are usually solitary# lare# and confined to &hite matter! There is a nelii%le
inflammatory response!
-ost cysts produce sins and symptoms related to their mass effect! Dianosis of the
infection is made seroloically! CT and -RI of the %rain and ultrasonoraphy of the
li$er and spleen may %e definiti$e! Chest x;ray often sho&s calcified pulmonary cysts!
Treatment consists of patient isolation from the source and surical remo$al of
symptomatic cysts! Care must %e ta'en to remo$e the intact cyst to a$oid seedin &ith
$ia%le lar$ae! "ydatid disease of the CNS is disa%lin# %ut rarely fatal# pro$ided cysts
are remo$ed &hen they %ecome symptomatic!
C+N(EN"TA, AN' 'E)E,+PMENTA, AN+RMA,"T"E*
Approximately 9 percent of ne&%orns possess some type of conenital a%normality!
Sixty percent of these in$ol$e the central ner$ous system# and o$er half of those are
related to defecti$e de$elopment or closure of the dorsal midline structures! -any
ha$e associated hydrocephalus! The commonly encountered neuroloic
malformations are listed in Ta%le =6;5!
Spinal Dysraphism
<et&een 7? and 9? days of em%ryonic de$elopment# the neural roo$e closes
posteriorly in the midline to form the neural tu%e! This tu%e is encircled %y %one
deri$ed from ad,acent somites and is co$ered superficially %y s'in deri$ed from
ectoderm! A%normal closure of the neural roo$e# failure of fusion of the ad,acent
%one# and/or malde$elopment of the o$erlyin ectoderm can lead to a $ariety of spinal
dysraphic states! Thus# dysraphism implies an a%normal fusion of normally united
parts!
+ailure of the %ony structures to close &ith normal closure of the neural roo$e is
called spina %ifida occulta! 1atients &ith this anomaly ha$e a normal spinal cord and
normal cord function! The a%normality usually is unnoticed unless seen on plain
radioraphs! If the menines fail to close# a meninocele de$elops# producin a
cutaneous a%normality! The underlyin neural structures# ho&e$er# de$elop normally#
so there is no compromise of neuroloic function!
+ailure of the underlyin neural tissue to fuse has %een called spina %ifida cystica# or
more recently# spina %ifida aperta! -yelomeninocele# the more common form#
in$ol$es incomplete closure of the neural roo$e# usually in the lum%ar reion# &ith
the a%normal# unfused neural tissue on the dorsal surface# exposed throuh an
associated defect in the spinal column! This may %e partially or totally co$ered &ith
epithelium! The accompanyin neuroloic deficit usually consists of complete
a%sence of motor and sensory function %elo& the le$el of spinal cord in$ol$ement!
The most se$ere form of spinal dysraphism is myeloschisis# &hich is much less
common than myelomeninocele! The spinal cord is unfused and presents directly on
the surface of the %ac' &ithout o$erlyin menines or epithelium! It usually occurs at
the thoracolum%ar reion and is $irtually al&ays associated &ith parapleia and
a%sence of %ladder function!
<oth myelomeninocele and myeloschisis are associated &ith hydrocephalus!
"ydrocephalus is caused %y a de$elopmental a%normality of the hind%rain called
Arnold;Chiari malformation# &hich is associated &ith the more se$ere forms of spinal
dysraphism! This malformation is composed of caudal displacement of the cere%ellar
tonsils# $ermis# inferior fourth $entricle# and medulla! There is a dorsal 'in' in the
cer$icomedullary ,unction and %rea'in of the 2uadrieminal plate! Associated
anomalies include aenesis of the corpus callosum and o%structi$e hydrocephalus!
The treatment of spinal dysraphism is surical! -eninoceles are excised and the s'in
is closed primarily after &atertiht closure of the posterior menineal defect!
-yelomeninoceles and myeloschises are closed as early as possi%le to reduce the
ris' of superficial infection and su%se2uent meninitis! The oal is to preser$e as
much neural tissue as possi%le# untether the spinal cord from surroundin soft tissue#
and fashion a dural closure to pre$ent CS+ lea'ae! Accompanyin hydrocephalus is
treated %y shuntin!
Sur$i$al of infants &ith these dysraphic states continues to impro$e! Those ne&%orns
&ith lo&er;le$el lesions ha$e %etter outcomes than those &ith hiher lesions o$erall!
The more se$ere the dysraphic state# the hiher the mor%idity and mortality! Ris' of
sepsis from %ladder infection is reduced &ith intermittent catheteri*ation &hen
indicated! Timely re$ision of failed shunts placed for hydrocephalus preser$es
potential for intellectual de$elopment!
Cranial Dysraphism
Cranial dysraphic states are one;tenth as common as their spinal counterparts!
Encephaloceles# althouh rare# are the most common manifestation! They consist of a
midline s'ull defect throuh &hich a small portion of %rain protrudes! -ost
encephaloceles are co$ered &ith s'in# and only some 85 percent ha$e associated
hydrocephalus! Once %elie$ed to arise from defects in the closure of the primiti$e
neural tu%e# they pro%a%ly de$elop %ecause of an o$erlyin mesodermal a%normality#
&ith su%se2uent pertur%ation of underlyin cere%ral tissue! In North America and
Europe# 46 percent of encephaloceles occur in the posterior cranial $ault3 the
remainder are found in the anterior cranial $ault! In southeast Asia# this distri%ution is
re$ersed for un'no&n reasons!
Surical repair in$ol$es early resection of malformed and de$itali*ed %rain and dural
closure! The mortality of patients &ith encephaloceles is extremely $aria%le!
1ronostic factors include the si*e and location of the anomaly# the extent of %rain
protrusion# and the presence of associated hydrocephalus# sei*ure disorder# or cere%ral
dysenesis! The smaller and more anterior defects enerally ha$e a %etter outcome! Of
those &ho sur$i$e# only 85 percent attain normal intellience!
"ydrocephalus
The term hydrocephalus implies an increase in the amount of CS+ &ithin the
$entricular system (+i! =6;9=)! This is almost al&ays due to a decrease in the
a%sorption of fluid# althouh there are rare cases of choroid plexus papillomas causin
hydrocephalus %y an increase in CS+ production! "ydrocephalus is traditionally
classified as communicatin and noncommunicatin! In the former# the $entricular
system continues to communicate &ith the su%arachnoid spaces outside the %rain
throuh the fourth $entricular foramina of Lusch'a and -aendie! In the
noncommunicatin $ariety (often termed o%structi$e)# it does not! The common
causes of hydrocephalus $ary &ith ae and are listed in Ta%le =6; :!
Infantile "ydrocephalus
"ydrocephalus occurs most fre2uently %et&een %irth and 9 years of ae and is most
commonly due to conenital a%normalities of the %rain! These a%normalities typically
produce noncommunicatin hydrocephalus! Stenosis of the cere%ral a2ueduct is one
such common conenital anomaly! Another is the Arnold;Chiari malformation#
descri%ed earlier! The Dandy;Eal'er malformation produces a mar'edly enlared
fourth $entricle as a result of conenital o%struction of CS+ outflo& from the fourth
$entricle# &ith resultant hydrocephalus! Other# less common conenital lesions
include arachnoid cysts# $ascular anomalies# and conenital tumors!
Ac2uired hydrocephalus in the infant is often the result of meninitis or intracranial
hemorrhae# %oth potentially causin o%struction of either the CS+ a%sorpti$e
mechanism or the intra$entricular path&ays! A2ueductal stenosis may de$elop &ell
after %irth %ecause of infection or hemorrhae and thus %e considered Cac2uired!D
Tumors may also o%struct the outflo& of CS+# resultin in noncommunicatin
hydrocephalus!
Infants &ith hydrocephalus usually# %ut not in$aria%ly# present &ith an enlarin head
circumference! They often ha$e a tense# %ulin anterior fontanelle &ith distended
scalp $eins and split cranial sutures! They may appear to ha$e Csun settinD of the
eyes# &ith only the tops of the irises $isi%le (1arinaud>s syndrome)! The head may
transilluminate %ecause of a lac' of cere%ral su%stance! "ydrocephalus usually does
not impair the infant initially %ecause the open cranial sutures allo& for cranial $ault
expansion! In the more chronic forms# or in older infants &ith closed sutures#
papilledema# optic atrophy# and 0Ith ner$e palsies may %e seen!
Childhood "ydrocephalus
"ydrocephalus in children o$er 9 years of ae may ha$e a more acute presentation
%ecause of the decreased a%ility of the more mature %rain and s'ull to accommodate
the increase in CS+! Conse2uently# raised intracranial pressure may cause headache#
nausea# $omitin# lethary# coma# and e$en death! Slo&er onset may result in
decreased mentation# %eha$ioral chanes# diminished performance in school# 0Ith
ner$e palsies# optic atrophy# paralysis of up&ard a*e# spastic le &ea'ness# and
endocrine (hypothalamic) disorders! Causes of hydrocephalus in this ae roup
include tumors# meninitis# intracranial hemorrhae (%oth spontaneous and
traumatic)# and a2ueductal stenosis! 0entricular shunt malfunction can cause acute
hydrocephalus in the shunt;dependent patient# reardless of the patient>s ae or the
underlyin cause of the hydrocephalus!
Adult "ydrocephalus
"ydrocephalus in adults may also result from o%structi$e tumors# meninitis# and
intracranial hemorrhae# %ut it may also %e more insidious in onset! An entity called
normal pressure hydrocephalus occurs in the older population# in$ol$in a
communicatin hydrocephalus &ith relati$ely normal intra$entricular pressure! The
cause remains un'no&n %ut is thouht to %e due to su%clinical hemorrhae or
infection in the patient>s remote past and mala%sorption of CS+! The classic symptom
triad of ataxia# urinary incontinence# and failin mentation suests the dianosis!
Treatment is %y $entricular fluid shuntin!
Reardless of the cause# the treatment of hydrocephalus is essentially the same! Either
the cause must %e remo$ed (e!!# tumor) or a shuntin procedure must %e performed
to di$ert accumulated CS+! Sometimes %oth measures are necessary! The most
commonly used procedure is a lateral $entricle to peritoneal ca$ity shunt &ith a one;
&ay pressure;reulatin $al$e in the system! If the peritoneal ca$ity is not suita%le for
shuntin# the distal catheter may %e placed in the riht atrium of the heart or in the
pleural ca$ity! In selected cases of communicatin hydrocephalus# a lum%ar
su%arachnoid to peritoneal shunt may %e used! Common complications of ind&ellin
shunts include shunt o%struction and infection!
Craniosynostosis
Craniosynostosis is the premature closure of one or more cranial sutures# typically
manifested &ithin the first : months of life! Since the %rain dou%les in si*e durin the
first : months of life and ro&s another 56 percent %y ae t&o# the cranial sutures
must remain open to allo& for s'ull expansion to accommodate this ro&th! .sually#
&hen one suture fuses prematurely# the %rain is not compressed sinificantly# %ut the
s'ull &ill then de$elop in a distinctly a%normal shape! If more than one suture is
fused# %rain ro&th may %e restricted!
The saittal suture fuses prematurely most often! The s'ull then de$elops an elonated
shape &ith a narro& %iparietal diameter# often referred to as scaphocephaly! The
supraor%ital ride may %e s2uare;shaped due to o$erexpansion of the open metopic
suture! Associated conenital anomalies are rare!
The next most common suture to %e in$ol$ed is the coronal# &hich may close
prematurely on one or %oth sides! .nilateral in$ol$ement produces an asymmetrically
shaped forehead &ith flattenin on the affected side and compensatory enlarement
on the opposite side! This is called plaiocephaly and is not usually associated &ith
other a%normalities! <ilateral coronal synostosis produces a more se$ere
foreshortenin of the entire anterior fossa and is often manifested %y shallo& or%its
&ith exophthalmos and hypertelorism! This entity is often associated &ith inherited
conenital disorders such as Crou*on>s disease and Apert>s and Carpenter>s
syndromes!
Less common forms of craniosynostosis include premature closure of the lam%doid
suture or the metopic suture! Eith unilateral synostosis of the lam%doid suture# the
s'ull appears flattened in the affected occipital area# &hich may %e confused &ith
%irth moldin! Eith premature closure of the metopic suture# the forehead assumes a
trianular shape (trionocephaly)! Neither of these forms is associated &ith other
conenital anomalies!
The treatment of craniosynostosis is surical and enerally in$ol$es the openin of
the affected suture alon its entire lenth! This should %e carried out as soon as
possi%le after the dianosis is made# since early surical inter$ention pro$ides the %est
cosmetic result! In cases of multiple suture in$ol$ement# prompt treatment pro$ides
early s'ull expansion to accommodate %rain ro&th!
NE-R+*-R("CA, MANA(EMENT +F PA"N
The ma,ority of neurosurical patients ha$e pain# either as their primary complaint or
as a secondary manifestation of their disease process! 1ainful conditions can %e
cateori*ed as acute processes# such as arm pain from a herniated cer$ical disc# or
chronic processes# such as extremity pain from an in$asi$e neoplasm! +or most acute
pain states# the cause can %e identified and treated# %ut for chronic pain there is often
no ready solution! In this section# the more common neurosurical procedures
a$aila%le to manae chronic pain &ill %e descri%ed!
At one time# the perception of pain &as thouht to in$ol$e a relati$ely simple system
of path&ays extendin from the peripheral receptors to the %rain! It has since %een
sho&n that this system is an extremely complex net&or' of path&ays# &ith a
considera%le amount of modification at multiple synaptic le$els! Impulses from pain
receptors reach the spinal cord $ia the dorsal root anlion and may %e sinificantly
modified in the $arious laminae of the dorsal horn! This information is then relayed to
the thalamus# %ut aain may undero considera%le modification in the area of the
%rainstem reticular formation! This sensory input is su%se2uently relayed to the cortex
for conscious interpretation! -odifiers in this complex system include the endoenous
su%stances endorphins# en'ephalins# and su%stance 1! In addition# the psycholoic
state of the patient influences the perception of painful stimuli!
Traditionally# neurosurical procedures for chronic pain ha$e %een a%lati$e or
destructi$e# %ut a $ariety of neuromodulatin or stimulatin procedures ha$e %een
de$eloped in recent years! These procedures are enerally reser$ed for those chronic
pain conditions that ha$e failed medical therapy!
Cere%rum
In eneral# fe& painful states &arrant procedures in$ol$in the cere%ral hemispheres
or deep %rain nuclei! <ilateral rostral cinulotomies ha$e %een performed for
treatment of intracta%le pain! This procedure disrupts the cinulum# a lare fasciculus
runnin deep to the cinulate yrus! It has pro$ed useful for affecti$e disorders
in$ol$in pain &hen performed %ilaterally!
As the primary relay station for pain impulses# the thalamus has %een the taret for
stereotaxic a%lati$e procedures! Thalamotomies are performed &ith considera%le
accuracy and ha$e %een used for thalamic pain disorders# phantom lim% pain# and pain
from in$asi$e tumors! -ore recently# deep %rain electrodes ha$e %een placed for
stimulation rather than a%lation of these structures! In eneral# thalamic stimulation
has pro$ed useful in patients sufferin from deafferentation pain# pain that typically
does not respond to opiates! Con$ersely# %rainstem stimulation is successful in
chronic painful states that are responsi$e to opiates! Lon;term success (control of
pain for a minimum of 9 years) is reported to %e a%out :6 and ?6 percent for the
respecti$e roups!
Cranial Ner%es
Trieminal neuralia (tic douloureux) is one of the more commonly occurrin
neuropathic painful conditions! It presents as an intermittent# shoc'li'e pain in one or
more di$isions of one trieminal ner$e! It most commonly in$ol$es the second
(maxillary) and/or third (mandi%ular) di$ision of the ner$e and is rarely %ilateral! The
pain usually lasts for seconds %ut is extremely se$ere and may %e incapacitatin! It is
often triered %y touchin the face# tal'in# or che&in! The pain may %e present for
&ee's or months# then spontaneously disappear# only to return &ith increased se$erity!
+or most patients the pain can %e controlled initially &ith phenytoin or
car%ama*epine# %ut e$entually many &ill re2uire surical inter$ention! A small
percentae of patients may ha$e a posterior fossa tumor causin the pain# so
e$aluation should include a CT or -RI scannin %efore therapy!
In the past# surical treatment in$ol$ed a%lation of the in$ol$ed %ranch(es) of the
trieminal ner$e! This could %e accomplished peripherally %y surical section or
alcohol a%lation of the supraor%ital# infraor%ital# or inferior al$eolar ner$es! 1ain
control throuh these neurectomies &as usually short;li$ed# ho&e$er! Experience
sho&ed that preanlionic lesions must %e made for more permanent relief!
Retroasserian rhi*otomy is no& carried out %y open surical approaches
su%temporally or throuh the posterior fossa# or percutaneously %y placin a
radiofre2uency electrode throuh the foramen o$ale into the anlion!
A nona%lati$e approach in$ol$es micro$ascular decompression of the trieminal
ner$e in the posterior fossa! The theory %ehind this approach is that trieminal
neuralia is caused %y external pressure on the ner$e %y $ascular structures (an artery
or $ein) near its entry into the %rainstem! Eith the use of the operatin microscope#
the offendin artery can %e mo$ed or the $ein a%lated# thus decompressin the ner$e!
This procedure has a hih success rate %ut carries more ris' than the percutaneous
method! An ad$antae is that the ner$e>s function remains intact!
Spinal Cord
Just li'e other surical procedures for chronic pain# those in$ol$in the spinal cord
ha$e traditionally %een a%lati$e! Cordotomy# desined to o%literate the spinothalamic
tract (see +i! =6;8)# can %e performed %y open operation or percutaneously!
Anterolateral cordotomy may pro$ide excellent temporary relief of pain for patients
&ith terminal malinancies# %ut it is rarely effecti$e for chronic %enin conditions
such as lo& %ac'# postherpetic# or phantom lim% pain!
+or selected cases of se$ere pain of peripheral ner$e oriin# such as %rachial plexus
in,ury# postherpetic neuralia# traumatic lim% amputation# and root a$ulsion# a%lati$e
lesions can %e made at the dorsal root entry *ones (DREK) of the spinal cord! These
lesions are made &ith a radiofre2uency lesion enerator or laser throuh an open
exposure of the cord $ia a laminectomy! Se$eral le$els are usually included! A%out 56
percent of patients o%tain ood relief from pain!
Chronic pain that de$elops in chest# flan'# or a%dominal incisions may &arrant an
a%lati$e procedure! Since these reions do not contain critical sensory areas# unilateral
dorsal ner$e roots may %e sectioned to depri$e the in$ol$ed area of sensation! It is
necessary to a%late at least three ad,acent le$els in order to dener$ate one dermatome
ade2uately %ecause of o$erlappin sensory distri%ution! Dorsal rhi*otomy may %e
carried out openly throuh a laminectomy or percutaneously throuh radiofre2uency
thermocoaulation! 1ain relief from rhi*otomy is ood initially# then less so as time
passes!
Intrathecal morphine can %e i$en temporarily or permanently %y infusion of small
%ut effecti$e doses! This ne&er method of pain control is particularly effecti$e in
de%ilitated patients &ith terminal illnesses! The procedure in$ol$es the su%cutaneous
implantation of a constant infusion pump that can %e rechared periodically!
In chronic painful states of nonmalinant spinal oriin# such as lo& %ac' and/or le
pain# a nona%lati$e neuromodulation techni2ue may play a role in therapy! This
in$ol$es transcutaneous excitatory ner$e stimulation (TENS)# &hich C%loc'sD ner$e
conduction of pain impulses! It is simple# safe# and relati$ely inexpensi$e! Electrodes
are taped to the s'in# usually o$er the reion of the pain or directly o$er the affected
ma,or ner$e# and then connected to a small# porta%le stimulatin de$ice! The de$ice
has a $aria%le pulse &idth# fre2uency# and amplitude# &hich the patient can ad,ust in
order to achie$e maximum pain relief! .se of this de$ice for a %rief time often
pro$ides lon;lastin relief &ith no unto&ard side effects! Direct stimulation of the
spinal cord %y surically implanted electrodes com%ined &ith percutaneous telemetry
can also pro$ide relief from chronic pain!
Peripheral Ner%e
1ain from a partial or complete ner$e in,ury usually in$ol$es the ner$e>s sensory
distri%ution %ut may include the &hole extremity! Chronic pain de$elopin after an
amputation may %e present in the remainin portion of the lim% at the site of the
amputation (Cstump painD) or in the nonexistent amputated portion (Cphantom painD)!
The causation of the pain may %e related to the sensory component of the ner$e or to
its associated sympathetic ner$e supply!
Eith partial or complete peripheral ner$e transection# a painful neuroma may form!
The usual treatment is excision of the neuroma# &ith pre$ention of recurrent
formation %y %uryin the ner$e end in %one or muscle or &rappin it in tantalum or
Silastic! Neuromodulation techni2ues may also %e applied in cases of painful
neuromas!
Chronic pain resultin from peripheral ner$e in,ury may %e sinificantly altered %y
interruption of the sympathetic ner$e supply to the affected extremity! The classic
example of this dysautonomic state is ma,or causalia! This term implies a partial
in,ury to a ma,or ner$e in an extremity! -inor causalia is reser$ed for an in,ury to a
more distal minor sensory ner$e# &hich may also %ecome a source of sinificant pain!
It is also &ell reconi*ed that a dysautonomic state may %e created %y ma,or or minor
trauma to an extremity that does not in$ol$e a peripheral ner$e! This has %een termed
ma,or or minor traumatic dystrophy! The entire collection of causalias and traumatic
dystrophies ma'es up a syndrome called reflex sympathetic dystrophy!
-a,or causalia is most commonly related to partial in,ury of the sciatic or the median
ner$e! Typically# symptoms %ein in the affected ner$e>s distri%ution %ut may proress
to in$ol$e the &hole extremity! The extremity first %ecomes s&ollen# &arm#
erythematous# and 2uite sensiti$e to touch! Eith time# it %ecomes cool and pale!
"yperhidrosis (excessi$e s&eatin) may follo&! Due to lac' of ,oint motion# the
normal flexion and extension creases disappear and the s'in %ecomes smooth and flat!
1lain radioraphs may demonstrate osteoporosis! E$entually the extremity may
%ecome completely useless! A constant# %urnin pain de$elops and persists throuhout
these $arious staes! It may %e exacer%ated %y touchin or mo$in the extremity!
E$en temperature chanes and emotional stress can trier &orsenin of pain! -inor
causalia and the traumatic dystrophies may %e accompanied %y similar# %ut less
se$ere symptoms!
The treatment of these dysautonomic states is complex! They may %e helped %y
disruption of the sympathetic ner$e supply to the extremity! Sympathetic dener$ation
may %e extremely re&ardin in ma,or causalia %ut is less so in minor causalia and
the traumatic dystrophies!
Less se$ere and more easily treated pain may arise from chronic compression of
selected peripheral ner$es! The most common are compression of the median ner$e at
the &rist (carpal tunnel syndrome) and compression of the ulnar ner$e at the el%o&!
Chronic compression may result in pain# paresthesias# num%ness# and e$entually
&ea'ness and atrophy of muscles in the distri%ution of the affected ner$e! These
compression syndromes are dianosed clinically and confirmed %y findin
dener$ation and slo&ed ner$e conduction on electromyoraphy and ner$e conduction
$elocity testin! Treatment is surical decompression of the in$ol$ed ner$e# &ith
prompt and lon;lastin relief in most cases!
EP",EP*. AN' M+)EMENT '"*+R'ER*
Epilepsy
That remo$al of specific areas of the %rain can cure epilepsy has %een 'no&n for
many years! Inade2uate dianostic tools and poorly defined indications for surery#
ho&e$er# precluded &idespread use of surery to treat the disease! A%out 9 million
people in the .nited States ha$e epilepsy# and most undero treatment for it! -ost
$arieties of epilepsy can %e cateori*ed into either enerali*ed (rand mal) sei*ures
characteri*ed %y loss of consciousness &ith tonic/clonic mo$ements# or partial
sei*ures manifested %y in$oluntary mo$ements %ut no loss of consciousness! Epilepsy
can %e controlled fairly &ell %y a$aila%le medications in a%out ?6 percent of patients!
Of the remainin 96 percent# nearly 766#666 may %enefit from a$aila%le surical
procedures! Interest in surical manaement of epilepsy has increased sinificantly in
recent years %ecause of the a$aila%ility of accurate physioloic locali*ation of sei*ure
foci and hih; 2uality imain of the %rain %y CT# -RI# and 1ET scannin!
Epilepsy has many etioloies! Conenital anomalies of the %rain are relati$ely
common causes in the pediatric ae roup! <irth in,ury is also a &ell;'no&n precursor
of sei*ure disorders in children! Neoplasms and $ascular anomalies# includin
arterio$enous malformations# are common sei*ure sources in adults! A lare num%er
of adults ha$e epilepsy %ecause of mesial temporal sclerosis# a condition that pro%a%ly
results from hypoxia and/or hypermeta%olic states durin early de$elopment of the
%rain! Some sei*ures ha$e no 'no&n cause!
1atients can %e tested effecti$ely in order to select those &ho can clearly %enefit from
surery! Electroencephaloraphy and -RI are initial monitorin tools! -ore in$asi$e
monitorin in$ol$es recordins from depth electrodes placed stereotactically and
su%dural electrodes placed %y open craniotomy! Specific foci can %e identified &ith
proloned monitorin usin $ideo recordin coupled &ith continuous EEG
monitorin! Areas of %rain that ser$e specific functions such as speech and
motor/sensory functions can also %e identified %y specific tests# includin 1ET
scannin and functional -RI!
Operation is planned once a focus has %een identified that is amena%le to surical
resection &ithout undue ris'! Often sei*ure surery is performed &ith the patient
under local anesthesia! Electrocorticoraphy# &hich is similar to
electroencephaloraphy except that it is performed directly from %rain cortex# can
further identify the focus of sei*ures at the time of operation! Stimulation of %rain is
also used to map the cortex and to identify reions that su%ser$e speech# $ision# and
sensory/motor functions!
Outcomes of sei*ure surery today are often ratifyin! -any patients &ith
uncontrolled epilepsy are not a%le to function in society %ecause of their sei*ures!
A%out half of those &ho ha$e uncontrolled sei*ures can %e made sei*ure;free %y
surical techni2ues! Another one;2uarter can ha$e sinificant reduction in %oth
sei*ure fre2uency and sei*ure intensity %y surical therapy!
-o$ement disorders
-o$ement disorders result from a $ariety of central ner$ous system diseases and are
manifested %y a%normal $olitional mo$ement! Typical examples are the restin (or
Cpill;rollinD) tremor of 1ar'inson>s disease and the intention tremor that accompanies
$olitional mo$ement as a conse2uence of cere%ellar disorders! -any treatments ha$e
%een used since these conditions &ere first reconi*ed# %ut most ha$e %een
ineffecti$e! <ecause of ad$ances in neurophysioloy# neuropharmacoloy#
neuropatholoy# and anatomic imain durin this century# the %alance %et&een
excitatory and inhi%itory effectors of mo$ement has come to %e %etter understood# and
the im%alance responsi%le for a%normal $olitional mo$ements %etter explained!
Impro$ements in %oth medical and surical treatments ha$e resulted!
The symptoms of 1ar'inson>s disease# characteri*ed %y restin tremor# co&heel
riidity# and %rady'inesia# &ere not effecti$ely controlled until it &as 'no&n that
there is a dopamine deficiency in the su%stantia nira resultin in inhi%ition of the
$oluntary initiation of mo$ement! Dopamine supplements &ere then de$eloped# and
symptoms &ere often impro$ed!
Surical treatment %ean in the 7F56s &ith a%lati$e lesions of the %asal anlia
desined to interrupt im%alanced inhi%itory/excitatory circuitry and its effect on
mo$ement coordination! An a%lati$e lesion in the lo%us pallidus# called pallidotomy#
&as not $ery effecti$e at first# thouh it did seem to help riidity and %rady'inesia!
Similarly# thalamotomy pro$ed to ha$e a modestly %eneficial effect on tremor! Neither
operation &as used extensi$ely %ecause medical treatment# particularly &ith
dopamineric aents# &as enerally effecti$e!
Stereotactic methods# particularly pallidotomy# ha$e %ecome popular %ecause medical
therapy has not remained effecti$e in many patients o$er the lon term! Escalatin
doses of medication ha$e often %een necessary to control par'insonism# resultin in
&idely fluctuatin symptoms and sins! 1atients &ho ha$e restin tremor &ithout
%rady'inesia are considered for thalamotomy# and those &ith riidity# %rady'inesia#
and a*e and %alance difficulties are candidates for pallidotomy!
Other mo$ement disorders that respond to a%lati$e lesions in the %asal anlia include
essential tremor# intention tremor of multiple sclerosis# and the choreiform
mo$ements of "untinton>s disease! "emi%allismus and hereditary dystonia can also
%e treated %y stereotactic lesions in the %asal anlia!
RA'"+*-R(ER.
Radiosurery is a hihly focused ioni*in radiation deri$ed from an external source
deli$ered to a stereotactically defined intracranial taret! Treatment of lesions in the
depths of the %rain %y focused radiosurery %ean a%out 96 years ao! The techni2ue#
usin the so;called Camma 'nife#D allo&s deli$ery of radiation to a specific taret
from a $ariety of sources# a%latin the taret &ithout sinificant ad$erse effects on
tissue surroundin it! In some instances# the techni2ue is deli$ered %y fixed co%alt
sources deli$erin photon radiation to the taret# aided %y a stereotactic frame
attached to the patient>s head and a computer proram couplin the radiation source to
the frame on the patient! Identification of the intracranial taret is made %y CT or -RI
scannin# usin a computer to relate the taret to the stereotactic frame and the patient
&ithin it! Other systems in$ol$e a similar principle# %ut the radiation source# deli$ered
%y linear accelerator# mo$es around a fixed taret!
Currently radiosurery is i$en in a sinle dose re2uirin little or no time in the
hospital! +ractionated treatments usin radiosurery are %ein de$eloped# reducin the
ris' of treatment and increasin its effecti$eness! Radiosurery is used for a $ariety of
conditions# includin arterio$enous malformations and neoplasms# and is particularly
adapta%le to deep intracere%ral lesions! It causes sclerosis of $ascular structures#
resultin in proressi$e occlusion of arteries# such as those &ithin arterio$enous
malformations# and cell necrosis in tumors! Radiosurery can radually a%late small
to medium;si*ed arterio$enous malformations &ith ?5 percent efficacy o$er a 9; to 8;
year follo&;up period!
Radiosurery is also used to treat neoplasms# includin intracranial sch&annomas and
meniniomas! In %oth instances the taret is usually &ell defined and ad,acent %rain is
not in$ol$ed! A tumoricidal dose can %e deli$ered in a sinle shot &ithout sinificant
ad$erse affects on surroundin tissue! Collimation of the %eam allo&s it to %e shaped
to fit the anatomic dimensions of the taret lesion!
The role of radiosurery in other tumors# such as metastatic tumors and liomas# is
uncertain at this time! It is pro%a%le that metastatic intracere%ral lesions &ill %e treated
more %y this techni2ue in the future %ecause it is relati$ely nonin$asi$e# and it is
pro%a%ly as effecti$e as surery for small lesions! Larer lesions are less &ell suited to
radiosurery %ecause they &ould re2uire radiation doses larer than can %e deli$ered
safely!

(<i%lioraphy omitted in 1alm $ersion)