OROFACIAL PAIN

Craniofacial Neural Disorders: A Guide for

Diagnosis and Management
Steven R. Kilpatrick, D.D.S.

Abstract: The purpose of this article is to provide a succinct diagnosis and management regimen for
0886-9634/2204- neural disorders of the craniofacial region. This guide is an attempt to organize available data in a format
304$05.00/0, THE for use by the craniofacial pain practitioner. The management regimens are brief because the manage-
JOURNAL OF
CRANIOMANDIBULAR ment of many of these disorders may be outside the scope of dentistry. Also, the purpose of this guide
PRACTICE, is to be user-friendly and complete. Terminology is based on a literature review so individual disorders
Copyright © 2004
by CHROMA, Inc. may be researched more completely.

Manuscript received
December 22, 2003;
revised
manuscript received
March 29, 2004; accepted
March 31, 2004
Address for reprint
requests:
Dr. Steven R. Kilpatrick
2909 S. 74th Street
Fort Smith, AR 72903
E-mail: srkaacp@swbell.net

Dr. Steven R. Kilpatrick received his
D.D.S. degree in 1974 from the
University of Missouri at Kansas City
where he was an assistant clinical profes-
sor in oral medicine until 1975. He has
been in private practice since 1975 and
currently practices in Fort Smith,
Arkansas. He is a Diplomate of the
American Board of Craniofacial Pain, the
American Board of Orofacial Pain, and
the American Academy of Pain
Management. Dr. Kilpatrick is a Fellow
of the International College of Dentists,
the American Academy of Craniofacial
Pain and the Academy of General
Dentistry. He is president of the American
Academy of Craniofacial Pain and
President-elect of the Arkansas State
Dental Association.
N
euralgia is defined as pain in the distribution of a
nerve or nerves.l Common usage often implies a
paroxysmal quality, but neuralgia should not be
reserved for paroxysmal pains. There are also a number
of related terms for which the definitions are presented
for convenience:
¥ Neuritis: Inflammation of a nerve or nerves.
¥ Neurogenic pain: Pain initiated or caused by a pri-
mary lesion, dysfunction, or transitory perturbation
in the peripheral or central nervous system.
¥ Neuropathic pain: Pain initiated or caused by a pri-
mary lesion or dysfunction in the nervous system.
Peripheral neuropathic pain occurs when the lesion
or dysfunction affects the peripheral nervous system.
Central pain may be retained as the term when the
lesion or dysfunction affects the central nervous
system.
¥ Neuropathy: A disturbance of function or pathologi-
cal change in a nerve: in one nerve, mononeuropa-
thy; in several nerves, mononeuropathy multiplex; if
diffuse and bilateral, polyneuropathy.
The International Association for the Study of Pain
(IASP) has developed a classification system for neural-
gias of the head and face, which is presented in Table 1

304
KILPATRICK CRANIOFACIAL NEURAL DISORDERS

Table 1
IASP Classification System and Diagnosis Codes for Neuralgias of the Head and Face
Neuralgia Type Diagnosis Code
1. Trigeminal neuralgia (tic douloureux) 006.X8a
2. Secondary neuralgia (trigeminal) from central 006.X4 (tumor)
nervous system lesions 006.X0 (aneurysm)

Arnold-Chiari Syndrome (code only) 002.X2b (congenital)

3. Secondary trigeminal neuralgia from facial trauma 006.X1
4. Acute herpes zoster (trigeminal) 002.X2a
5. Postherpetic neuralgia (trigeminal) 003.X2b
6. Geniculate neuralgia (VIIth cranial nerve): Ramsey Hunt Syndrome 006.X2
7. Neuralgia of the nervus intermedius 006.X8c
8. Glossopharyngeal neuralgia (IXth cranial nerve) 006.X8b
9. Neuralgia of the superior laryngeal nerve (vagus nerve neuralgia) 006.X8e
10. Occipital neuralgia 004.X8 or
004.X1 (if subsequent to trauma)
11. Hypoglossal neuralgia (code only) 006.X8
12. Glossopharyngeal pain from trauma (code only) 003.X1a
13. Hypoglossal pain from trauma (code only) 003.X1b
14. Tolosa-Hunt Syndrome (painful ophthalmoplegia) 002.X3a
15. SUNCT Syndrome (shortlasting, unilateral, neuralgiform pain with
conjunctival injection and tearing) 006.X8j
16. Raeder’s Syndrome (Raeder’s Paratrigeminal Syndrome) Type I 002.X4 (tumor)
002.X1a (trauma)
002.X3b (inflammatory, etc.)
Type II 002.X8 (unknown)
Note: X = to be completed individually in each case (patient’s statement of intensity).

for reference. An attempt is made to provide a succinct
diagnosis and management regimen for these disorders
(Table 2).
Neuralgias of the Head and Face
seconds to minutes.3 A sustaining, deep, dull ache is often
present between acute paroxysms. The pain does not
cross to the other side but may be bilateral in 3-5% of the
cases.4,5 Pain is triggered by light mechanical contact

Trigeminal Neuralgia
Trigeminal neuralgia (TN) is sudden, usually unilat-
eral, severe, brief stabbing recurrent pain in the distribu-
Table 2
tion of one or more branches of the Vth cranial nerve. The
Conditions and Cases Per
pain is unilateral in 95% of the cases, with the right being
1,000,000 Population Per Year
the more frequently involved side. Prevalence is rela-
Cases per
tively rare in the US with an incidence of 2.7 men and 5.0 Condition 1,000,000 per year
women per 100,000 citizens per year. Age of onset is typ- Trigeminal neuralgia 27-50
ically the fifth to seventh decade. The second division is Secondary trigeminal .5-1
the most frequently affected, with approximately 44% of Glossopharyngeal neuralgia 5
reported cases. The third division is affected in 36% of Superior laryngeal neuralgia .5
cases and the first division in 19% of cases.2 Geniculate neuralgia .3
Sphenopalatine neuralgia Rare/no epidemiology
The key clinical features of trigeminal neuralgia
include brief, unilateral, lancinating pain, which is
described as bright, stimulating, shock-like pain lasting

OCTOBER 2004, VOL. 22, NO. 4 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 305
CRANIOFACIAL NEURAL DISORDERS
from a less restricted site, termed a trigger zone. The
paroxysms may occur at intervals and may be followed
by a refractory period. There may be remissions for days,
months or years, followed by recurrence. The pain is clas-
sified as extremely severe and probably one of the most
intense of all acute pains.
The location of the pain cannot be used to assign
origin. Pains involving the cranial nerves and cervical
spine may be referred to any other region of the head and
neck. Conversely, pain arising in the thorax or portions of
the esophagus or gastrum and innervated by the vagus
nerve can be referred to the ear and face.6
Clinical evidence supports a peripheral cause of TN.
Current theory suggests a focal demyelination of the fifth
cranial nerve, often from vascular compression just prior
to entry into the pons. Although not yet scientifically
proven, considerable evidence from the surgical treat-
ment of TN indicates that the pathophysiology of TN.
involves abnormal vascular cross compression of the root
entry zone of the trigeminal nerve.7 While this is the most
accepted theory, the pathophysiology is unclear. Multiple
sclerosis can produce pain indistinguishable from TN
Intracranial tumors, aneurysms, sphenoid sinusitis, verte-
brobasilar ectasia, and infiltrative disease have produced
painful paroxysms that may mimic TN. Some of the con-
troversy regarding the pathophysiology is whether focal
demyelination can be responsible for the symptoms of
TN or if there are coexisting factors that must be present.
Ratner and Bouquot have proposed that bony cavities
found in the alveolar bone may be the cause of TN.8-l0
There is a question that arises from the various patho-
physiologic theories: Why would such a variety of treat-
ments produce results if there were a single cause?
Medications, surgeries and mandibular maneuvers11,12 are
reported to provide relief.
The diagnosis of TN is made by history and clinical
features. The pain has characteristic clinical features and
distribution. The diagnosis is supported by the use of MR
imaging to rule out intracranial tumors, contrast enhanced
MR imaging to evaluate arterial and venous relationships
with the trigeminal nerve in the cerebellopontine
angle,7,13,l4 and diagnostic anesthetic blocks.5 Neuropathies
such as TN can cause tooth pains of nondental origin.
Patients presenting with pain that they perceive as a
toothache must be thoroughly evaluated. If a nonodonto-
genic origin is suspected, the dentist should refer for
appropriate evaluation.
Treatment of TN is fairly standard. The treatment
may be medical or surgical. The standard medical treat-
ment is likely to start with carbamazepine 300-1200
mg/day (See Table 3 for anti-epileptic medications/
dosages). In the past, a positive response to carba-
306 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE
KILPATRICK
mazepine was considered diagnostic.7 Other medications
that are likely to be effective are:
1. Phenytoin: 300-600 mg/day
2. Baclofen: 40-80 mg/day
3. Gabapentin: up to 3600 mg/day
4. Divalproex Sodium: 500-2000 mg/day
5. Clonazepan: 2-8 mg/day
6. Pimozide: 4-12 mg/day
7. Lamotrigine: 50-400 mg/day15
Approximately 70% of patients with TN are initially
controlled with medications. Mahan11 has reported that
remissions of TN have been achieved by anesthetic
blocks, mandibular maneuvers and by replacing inade-
quate dentures. However, when medical or dental treat-
ment is not adequate, surgical treatment is available.
There are a number of surgical remedies available for
TN. The procedures range from relatively minor to major
surgical procedures. The minor procedures include per-
cutaneous (i.e., performed through the skin), retrogasser-
ian glycerol rhizolysis, percutaneous radiofrequency
trigeminal gangliolysis,l6 invasive electrical stimulation
of the Gasserian (trigeminal) ganglion,17 percutaneous
trigeminal ganglion compression, and sterotactic radio-
surgery (i.e., gamma knife radiosurgery).l8,19 The choice
of procedure depends on the experience and preference of
the surgeon. The main criticism of these minor proce-
dures is that they do not address the primary pathophysi-
ology. Even so, these procedures are indicated for the
patients who are not candidates for major surgery.
The only surgery that directly addresses the presumed
etiology of vascular compression of the trigeminal nerve
at the root entry zone is microvascular decompression.
This procedure was developed by Jannetta. 20-22 The
surgery involves a craniotomy and posterior fossa surgery.
Once the area of vascular compression is located, the
Table 3
Anti-epileptic Drugs (AED) for
Treatment of Neuropathic Pain
Medication Dosage
Carbamazepine 300-1,200 mg/day in
divided doses
Gabapentin 900-3,600 mg/day in
divided doses
Lamotrigine 50-400 mg/day
OCTOBER 2004, VOL. 22, NO. 4
KILPATRICK
offending artery and/or vein is decompressed by padding
with muscle or Teflon (DuPont Corp.), using various
slings, gluing the artery to the dura with adhesive or
using a titanium bone fixation plate.23 Microvascular
decompression has a reported mortality rate of 1% and
associated morbidity rate consisting of 1% hearing loss,
1-2% rate of cerebrospinal fluid leakage and uncommon
transient or permanent cranial nerve deficits.24,25 Micro-
vascular decompression surgery can produce outstanding
results. One study of 420 consecutive patients undergo-
ing posterior fossa surgery reported a lessening of pain in
98% of patients, with pain completely resolved in 87%.
After a mean follow-up of 56.3 months, 93% reported
significant pain improvement and 72% continued to have
no pain. 26 The posterior fossa surgery reported by
Theodosopoulos, et al.7, also included partial sensory rhi-
zotomy (PSR). PSR has evolved as an adjunct to microvas-
cular decompression when a convincing neurovascular
impingement is not found.
Secondary Trigeminal Neuralgia
Secondary trigeminal neuralgia was previously termed
symptomatic trigeminal neuralgia. The symptoms of sec-
ondary TN are indistinguishable from TN. However, in
secondary TN, there is a distinct, structural, pathologi-
cal lesion, such as tumor, aneurysm, or other vascular
malformation.
Secondary TN may also arise from facial trauma.27
Tooth extraction or endodontic procedures may be the
cause of the trauma. The pain of secondary TN due to
trauma is described as a chronic, continuous throbbing
or burning pain with paroxysmal exacerbations. The
pain may be self-limiting due to nerve regeneration. The
pathophysiology of secondary TN is consistent with
deafferentation, (i.e., loss of normal afferent input into
the central nervous system). Treatment is the same as
for TN.
Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia (GN) is pain similar to TN
but in the distribution of the glossopharyngeal and vagus
nerves. The paroxysmal pain is in and around the throat,
jaw, ear, larynx, or tongue. The prevalence is very rare in
the US with an incidence of 0.5 persons per 100,000 pop-
ulation per year.1 Cardiac arrhythmia and syncope may
occur due to vagus involvement.27,28 The pain is often
triggered by swallowing, coughing, talking, yawning or
chewing. Cold or acidic liquids may also trigger the
episodes. The tonsillar and pharyngeal regions are trigger
zones.5 Deep, continuing pain may be present between
paroxysms. There may be numerous attacks daily. The
pain may awaken patients. Paroxysms of pain may last
OCTOBER 2004, VOL. 22, NO. 4
CRANIOFACIAL NEURAL DISORDERS
seconds to minutes. It may go into remission like trigem-
inal neuralgias.
Because the pain may be triggered by chewing or swal-
lowing, GN may be confused with pain emanating from
the temporomandibular joint or masticatory muscles.5
GN must also be differentiated from cerebellopontine
angle tumors, nasopharyngeal carcinoma, carotid
aneurysm, peritonsillar abscess, and EagleÕs syndrome.30,31
The diagnosis of GN may be confirmed by diagnostic
anesthetic blocks into the trigger zones. An endovascular
provocative test has been reported to demonstrate that
GN may be a vascular compression syndrome.32 Typically,
a complete history, clinical exam and diagnostic testing,
such as MRI, will confirm the diagnosis.
Treatment of GN follows the same course as TN. If
medical treatment fails, surgical treatment involves
intracranial sectioning of the GN nerve and the upper
rootlets of the vagus nerve at the jugular foramen or
microvascular decompression.33-35
Superior Laryngeal Neuralgia
Superior laryngeal neuralgia (SLN) is defined as
paroxysms of unilateral lancinating pain radiating from
the side of the thyroid cartilage or pyriform sinus to the
angle of the jaw and occasionally to the ear. The superior
laryngeal nerve is a branch of the vagus nerve. It inner-
vates the cricothyroid muscle of the larynx. This may be
a variant of GN, which has also been called vago-glos-
sopharyngeal neuralgia. The prevalence is rare. The
prevalence of vago-glossopharyngeal neuralgia is 0.5
persons per 1,000,000 population per year.
Pain may last seconds to minutes. The pain is usually
severe. It is precipitated by talking, swallowing, cough-
ing, yawning or stimulation of the nerve at its point of
entrance into the larynx. Mild forms do occur.36
Diagnosis is through history, clinical exam and diag-
nostic nerve blocks. The differential diagnosis would
include GN, carotidynia, neoplasms and neuritis.
Treatment is by using the same drugs indicated for TN,
repeated nerve blocks, or neurectomy.37,38
Geniculate Neuralgia
Geniculate neuralgia is characterized by severe sharp
or electrical pains lancinating in the ear, tympanic mem-
brane or skin between the ear and the mastoid process. It
is not distinct from glossopharyngeal neuralgia and may
be a variation. The prevalence of the condition in the US
is very rare, with an incidence of .03 persons per 100,000
population per year. Age of onset is typically in the fifth
to seventh decade. IASP states neuralgia of the nervus
intermedius is a tentative category because of contro-
versy. If the symptoms are produced subsequent to an
THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 307
CRANIOFACIAL NEURAL DISORDERS
acute attack of herpes zoster, it is called Ramsay Hunt
syndrome.39 The pain of both geniculate neuralgia and
Ramsay Hunt syndrome is in the distribution of the
nervus intermedius, a component of the seventh cranial
nerve, which contains primary sensory afferent fibers
with cell bodies located in the geniculate ganglion.
Differential diagnosis must separate geniculate neural-
gia due to herpes zoster from the idiopathic form and
from TN and GN as well.
Treatment should be started with medications after the
etiology is determined.40 If medications fail, there are sur-
gical alternatives, which include microvascular decom-
pression and/or neurectomy.41,42
Sphenopalatine Neuralgia
Sphenopalatine neuralgia is known by many names,
including vidian neuralgia, greater superficial petrosal
neuralgia, SluderÕs neuralgia, pterygopalatine neuralgia,
lower half headache,4 and others. It consists of paroxys-
mal pains that begin on the medial side of the nose or
medial canthus of the eye and radiate to the roof of the
mouth, retro-orbitally, or rarely, to the ipsilateral neck,
shoulder, and upper extremities.7 This clinical entity is
rare and is characterized by unilateral facial pain that
never extends to the ear, and no trigger point is found.2
During attacks of pain, vasomotor activity often results
in ipsilateral nasal drainage, eye irritation, and lacrima-
tion. 43 It is considered by some to be atypical facial
neuralgia.
Diagnosis is made by history, clinical presentation, and
diagnostic anesthetic blocks via injection or topical appli-
cation. When medications fail to control symptoms, there
are surgical alternatives, which include radiofrequency
thermocoagulation or neurectomy of the sphenopalatine
ganglion. 44-47 Differential diagnosis includes cluster
headache variants and paroxysmal hemicrania variants.
Occipital Neuralgia
Occipital neuralgia is a neuralgic-like syndrome
involving the greater occipital nerve. The International
Headache Society defines occipital neuralgia as a Òparox-
ysmal jabbing pain in the distribution of the greater or
lesser occipital nerves, accompanied by diminished sen-
sation or dysesthesia in the affect area.Ó Loeser2 classifies
occipital neuralgia as a cranial neuralgia and states,
ÒOccipital neuralgia is characterized by continuous
aching and throbbing pain on which shock-like jabs can
be superimposed. The pain starts in the suboccipital
region and radiates over the posterior scalp and some-
times across the lateral scalp. Retro-orbital pain is
common in a severe attack. The pain is not triggered, but
pressure over the occipital nerves can lead to an exacer-
308 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE
KILPATRICK
bation.Ó2 The IASP defines occipital neuralgia as Òpain,
usually deep and aching, in the distribution of the second
cervical dorsal root.Ó In their summary of essential fea-
tures and diagnostic criteria, they state, ÒIntermittent
episodes of deep, aching, and sometimes stabbing pain in
the suboccipital area on one side; marked tendency to
chronicity; often associated with tender posterior cervical
muscles; and can be bilateral.Ó1
The pathogenesis of occipital neuralgia is unknown. It
may be the result of trauma from whiplash injury, infec-
tion by herpes zoster or chronic compression of the
greater or lesser occipital nerves. Travell48 describes trig-
ger points in the trapezius, sternocleidomastoid and pos-
terior cervical muscles as producing pain in the same
distribution.
The diagnosis of occipital neuralgia is by history, clin-
ical features and local anesthetic blockade. 49 Neuro-
blockade in this area is nonspecific because the pain may
be arising from a variety of structures. According to the
IASP, the differential diagnosis includes cluster headache,
posterior fossa and high cervical tumor, herniated cervi-
cal disk, uncomplicated flexion-extension injury, and
metastatic neoplasm at the base of the skull. In addition,
myofascial syndromes must be considered when poste-
rior cervical musculature is painful with palpation.
Treatment is generally instituted with medications
and/or repetitive nerve blocking.50 The particular treat-
ment will depend on the suspected etiology and may also
include cervical collar, traction, myofascial release, spray
and stretch, massage or surgery. Surgery is generally a
last resort.51
Atypical Facial Pain
Atypical facial pain (AFP) generally reflects the pres-
ence of continuous, deep, often burning pain in the
absence of clear features defining the more delineated
facial pain syndromes.4 The IASP has no category for
AFP, and the International Headache Society (IHS) clas-
sifies AFP as Òpersistent idiopathic facial pain (13.18.4)Ñ
a persistent facial pain that does not have the characteristics
of the cranial neuralgias and is not attributed to another
disorder.Ó Elias, et al.7, state, ÒHistorically, atypical
facial pain or prosopalgia (trigeminal neuralgia), has
been the term most commonly used to describe diffuse,
nonanatomic orofacial pain of unknown pathophysiol-
ogy.Ó Madland and Feinmann52 state, ÒAtypical facial
pain is an unrecognized and unhelpful diagnosis but one
which describes chronic pains that do not fit the present
classification system.Ó Obviously, AFP is a wastebasket
term, which has been used in a wide variety of disorders.
There are certain symptom subsets that do not meet
particular criteria, and AFP fits that category.
OCTOBER 2004, VOL. 22, NO. 4
KILPATRICK
Elias and Burchiel7 offer the following: ÒIn the authorsÕ
opinion, the diagnosis of atypical facial pain should be
made only on the following basis: 1. When other etiolo-
gies for facial pain have been considered and evaluated;
2. When objective evidence for most of the facial pain
syndromes is lacking; and 3. When specific antecedent
psychologic or behavioral factors can be identified.Ó
Loeser2 states, ÒUnilateral atypical facial neuralgia repre-
sents a diverse group of facial pain problems that have
common symptoms and signs but varied causes.Ó Finally,
Wessely, et al.,53 postulate, Òthe existence of specific
somatic syndromes is largely an artifact of medical spe-
cialization. That is to say that the differentiation of spe-
cific syndromes reflects the tendency of specialists to
focus on only those symptoms pertinent to their specialty,
rather than any real differences between patients.Ó
The term atypical facial pain is not specific and has
little usefulness in classification of craniofacial pain dis-
orders. AFP has been considered to be a psychologically
induced pain syndrome by many.2,6,7,50-53 The dilemma for
the physician or craniofacial pain dentist is to avoid inap-
propriate treatment for these patients. Saper4 offers the
following considerations in diagnosis of AFP: 1. maxil-
lary and mandibular bone disease, which can give rise to
AFP from occult, infected bone cavities9; 2. phantom
tooth pain, which is a deafferentation disorder; 3. sphe-
noid sinusitis; 4. post-traumatic facial pain, which may
have a central mechanism; 5. hemicrania continua, which
is a continuous unilateral headache with moderate to
severe pain intensity, a jabbing, ice pick-like phenome-
non provoked by exertion and completely responsive to
indomethacin; 6. anesthesia dolorosa, which is a painful
anesthesia that arises from trauma to the trigeminal nerve
or other cranial nerve; 7. thalamic pain, which is a unilat-
eral facial pain with dysesthesia and usually follows
ischemic lesions in the thalamus; and 8. other causes. The
absence of an identifiable, objective cause should not
itself justify or constitute the presumption of psychogenic
origin. Okeson5 classifies AFP as a neurovascular vari-
ant. He offers the following guidelines: Neurovascular
variants: 1. usually have a characteristic pulsatile, throb-
bing quality; 2. follow the vascular arborization; 3. vary
in pain intensity from high to low; 4. are prone to occur in
episodes; 5. do not cause appreciable dysfunction; and 6.
frequently present characteristic autonomic effects, i.e.,
nasal congestion, lacrimation, conjunctiva injection and
edema of the eyelids and face on the affected side.5
Treatment of AFP is difficult. The initial treatment
consists of varying combinations of medications, which
may include tricyclic antidepressants (TCA), see Table 4
for medications/dosages), anticonvulsants, nonsteroidal
anti-inflammatory drugs (NSAIDs), muscle relaxants,
OCTOBER 2004, VOL. 22, NO. 4
CRANIOFACIAL NEURAL DISORDERS
opiods and migraine drugs. One study produced positive
results with a combination of dothiepin and counseling
sessions.54 Okeson5 recommends two mg of ergotamine
sublingually at the onset of the pain as a diagnostic/treat-
ment trial. Sphenopalatine ganglion block has also been
useful in the treatment of AFP.55
Postherpetic Neuralgia
Postherpetic neuralgia (PHN) is a continuous neuralgic
syndrome, resulting from a previous acute herpes zoster
eruption along a specific nerve distribution. It is a Òper-
sistent burning pain and hyperesthesia along the distribu-
tion of a cutaneous nerve following an attack of herpes
zoster; it may last for a few weeks or many months.Ó56
When the trigeminal nerve is involved, it usually affects
the distribution of the ophthalmic (first) division, but has
been reported to involve the maxillary or mandibular
division. The facial nerve may be involved. The occur-
rence of herpes zoster in the face is between 15% and
30% of reported cases.3
Postherpetic neuralgia follows the acute attack of
herpes zoster by days or weeks. PHN is more likely to
occur with more intense acute episodes of herpes zoster.
The prevalence is relatively infrequent. Onset is more
common with increasing age approaching a 50% inci-
dence in the 7th decade. 6 Of all patients afflicted by
herpes zoster, approximately 10% will develop PHN.2
The key clinical feature is pain following acute vesicular
eruption with skin changes. The quality of the pain is
burning, tearing, itching dysesthesia and crawling dyses-
thesia in the skin of the affected area. The pain may be
exacerbated by mechanical contact, which leads to inter-
ruption of normal activities, including sleep. The inten-
sity is usually moderate, but constancy and intractability
in many instances contribute to the intolerable nature of
complaint.1 PHN may last for years. There is spontaneous
improvement in most patients.
Table 4
Tricyclic Antidepressant Drugs for
Treatment of Neuropathic Pain
Medication Dosage
Amitriptyline 10-150 mg/day
Clomipramine 20-200 mg/day
Desipramine 10-300 mg/day
Doxepin 30-300 mg/day
Imipramine 10-150 mg/day
Nortriptyline 10-100 mg/day
THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 309
CRANIOFACIAL NEURAL DISORDERS
The pathophysiology of PHN is not proven. There is a
loss of many large fibers in the affected sensory nerve. It
may involve central deafferentation because injury to the
sensory nerve results in interruption of the involved
neuron. There is no single pathology that can possibly
explain the whole spectrum of pain-generating mecha-
nisms in PHN.57
Treatment of PHN is fairly straightforward. The first
line of treatment is a tricyclic antidepressant medication.
Treatment with amitriptyline has been the most studied
medication. When PHN lasts longer than six months, the
treatments that have been shown to be more effective
than placebo include tricyclic antidepressants, topical
capsaicin .075%, gabapentin and controlled-release oxy-
codone.57,58 Other treatments include other antiepileptic
drugs (AED) and, recently, lidocaine impregnated topical
patches (lidoderm patches).
Sympathetic nerve blocks have long been used for
patients with herpes zoster and PHN but their role remains
controversial.59 When PHN becomes intractable, other
treatments including intrathecal methylprednisolone and
gamma knife surgery have been advocated.60,61 The use of
antiviral agents during the acute phase of herpes zoster
has not been shown to reduce the incidence of PHN.62,63
Acute Herpes Zoster
Herpes zoster is an infection that may involve the
nerves of the head and face. The acute infection produces
a neuritis. The first division (ophthalmic) of the trigemi-
nal nerve is most commonly affected. 5 The pain is
described as burning or tingling with occasional lancinat-
ing components. The pain intensity may be severe and
will be in the distribution of the involved nerve. The
infection is self-limiting. Treatment regimens include
antiviral drugs, topical anesthetics or anesthetic blocks.
Tolosa-Hunt Syndrome
Tolosa-Hunt Syndrome is characterized by episodes of
unilateral pain in the ocular and periocular area combined
with ipsilateral paresis of oculomotor nerves (opthalmo-
plegia) and of the first branch of the fifth cranial nerve.1
The oculomotor symptoms may involve a combination of
the cranial nerves three, four or six.64 There might also be
involvement of cranial nerve five. The prevalence of
Tolosa-Hunt Syndrome is rare. The pathogenesis is gen-
erally considered to be granulomatous tissue located in or
near the cavernous sinus, although an inflammatory
process has been implicated.65
The differential diagnosis of Tolosa-Hunt is extensive.
The following must be considered: RaederÕs paratrigemi-
nal syndrome, ophthalmoplegic migraine, vascular
lesions, atypical cluster headache, neoplasms, inflamma-
310 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE
KILPATRICK
tory processes and injections.66-68 Treatment is with corti-
costeroids.69
SUNCT Syndrome
SUNCT syndrome is short lasting, unilateral neuralgi-
form pain with conjunctival injection and tearing. The
attacks are strictly unilateral, generally with the pain per-
sistently confined to the ocular/periocular area. The pain
usually lasts approximately one minute and is moderate
to severe in intensity. The pain is paroxysmal and
described as burning, stabbing or electrical in nature.70
SUNCT syndrome was first reported in the dental litera-
ture in 1998. 71 At that time, only 24 cases had been
reported. The attacks are usually during the day and may
occur as often as 30 attacks per hour.
Diagnosis is based on clinical features. The conjuncti-
val injection and tearing are diagnostic criteria. The dis-
order is considered rare and predominates in males
(male/female ratio is 4.25:1). The mean age of onset is
50.7 years. Pareja, et al.,72 present the following clinical
features that can be selected as diagnostic criteria:
1. strictly unilateral headache with attacks persistently
confined to the ocular/periocular area; 2. moderate to
severe but apparently not excruciatingly severe pain;
3. attacks regularly accompanied by prominent, ipsilat-
eral conjunctival injection and lacrimation; 4. precipita-
tion of attacks; 5. mean duration of paroxysms about one
minute; 6. during active periods, the usual frequency of
attacks may vary from a few attacks per day to more than
30 attacks per hour.
The differential diagnosis consists mainly of cluster
headache (CH), chronic paroxysmal hemicrania (CPH),
idiopathic stabbing headache (ISH) and trigeminal neu-
ralgia (TN). The precipitating factors differ in each con-
dition. CH cannot be precipitated mechanically, CH and
CPH do not have trigeminal trigger zones, and precipitat-
ing mechanisms are rare in ISH.
Treatment of SUNCT syndrome is difficult. No treat-
ment has been found to be uniformly effective. Lamotrigine
and gabapentin have been reported to be successful in
small numbers of patients.76 Unfortunately, some patients
remain refractory to treatment.77
Raeder’s Syndrome
RaederÕs syndrome, or RaederÕs paratrigeminal syn-
drome, is characterized by a steady aching pain in the
ocular and periocular area. There is complete or incom-
plete HornerÕs syndrome, (i.e., sinking in of the eyeball),
ptosis of the upper eyelid, slight elevation of the lower
lid, constriction of the pupil, narrowing of the palpebral
fissure, anhidrosis and flushing of the affected side of the
face, which is caused by paralysis of the cervical sympa-
OCTOBER 2004, VOL. 22, NO. 4
KILPATRICK
thetic nerves.56 Two types of pain result: a migrainous
variant with episodic and recurrent pain lasting hours or
days, and a more persistent type, often secondary to an
aneurysm or tumor in the region of the middle fossa or
cavernous sinus.l,7
Treatment is surgical if a cause is found. Otherwise,
analgesics may be beneficial. No specific therapy is
known at present.
Central Causes of Craniofacial Pain
The IASP defines central pain as pain initiated or
caused by a primary lesion or dysfunction in the central
nervous system (CNS).1 According to Tasker,78 ÒCentral
pain, like neural injury pain in general, is a process of
unknown etiology, which in certain individuals follows
partial, complete, or, in some cases, subclinical interrup-
tion of somatosensory pathways, particularly of the
spinothalamic tract, at any level in the central nervous
system.Ó Pain of spinal cord origin is usually caused by
trauma and that of brain origin by cerebrovascular acci-
dent. The quality of pain caused by lesions of the CNS
more closely resembles the deafferentation pain of the
peripheral nervous system than that of nociceptive pain.
Two central pains of craniofacial distribution are anesthe-
sia dolorosa and thalamic pain.
Anesthesia dolorosa is a painful area of anesthesia or
dysesthesia in a cranial nerve distribution that arises from
trauma or surgical treatment for neuralgia. It is primarily
a burning pain but is also described as an unpleasant tin-
gling, pins and needles or numbness. Treatment is diffi-
cult. Most therapies include tricyclic antidepressants,
anticonvulsants or antineuralgic therapies.
Thalamic pain may present as a unilateral facial pain
with dysesthesia. There may be decreased sensitivity to
pinprick or temperature. The pain is a result of a lesion of
the thalamus, usually following an ischemic event. There
is usually trunk and limb pain. Central post stroke pain
(CPSP) may be a more appropriate term.79 Treatment is
difficult and may include medications (i.e., TCAs, AEDs,
and occasional narcotics), electrical stimulation, or
surgery.80-82
Atypical Odontalgia
Atypical odontalgia (AO) is defined by the IASP as a
severe throbbing pain in the tooth without major pathol-
ogy. Dworkin and Burgess83 state, ÒThe pain of atypical
odontalgia is without organic cause, located in the teeth,
and is often referred to adjacent bony areas. In general,
AO is a tooth pain with no organic cause. Graff-Radford84
has proposed the following criteria: 1. continuous or
almost continuous pain in a tooth or surrounding alveolar
bone; 2. no obvious local cause; 3. no abnormality on
OCTOBER 2004, VOL. 22, NO. 4
CRANIOFACIAL NEURAL DISORDERS
radiograph; 4. pain present longer than four months;
5. associated hyperesthesia; and 6. equivocal somatic
block.
The most common area affected is the maxillary molar
or premolar region. There is a greater prevalence in
females in the fourth or fifth decade of life.5 The IASP
states the involved teeth are hypersensitive to heat and
cold, while Okeson5 states that local provocation of the
tooth or surrounding tissues does not alter the pain.5 The
diagnosis is difficult because one must eliminate all con-
ditions that can cause tooth pain in order to arrive at a
diagnosis of AO. This condition has been reviewed
extensively.85-90
Treatment is difficult. At this time, there is no suitable
medication that reliably reduces the pain of AO.5 There is
some evidence that low dose tricyclic antidepressants
may be of some benefit. Topical capsaicin has also been
shown to be effective.90
Sympathetically Maintained Pains/Complex Regional
Pain Syndromes
These conditions are covered together because of
overlap.
Sympathetically maintained pains (SMP) are pain con-
ditions that are maintained by the activity of the sympa-
thetic nervous system. 5 SMP is a general term that
includes spontaneous pain and pain evoked by mechani-
cal and thermal stimuli. Thus, SMP is now defined as a
symptom, not as a clinical entity.91 SMP is considered by
the IASP to be pain that is maintained by sympathetic
efferent innervation or by circulation catecholamines.
SMP may be found in association with complex regional
pain syndromes (CRPS), but they are not synonymous.
SMP may occur in some patients with CRPS but certainly
does not occur in all.92 CRPS replaces reflex sympathetic
dystrophy and causalgia.
The diagnosis of SMP/CRPS is based on a history of
tissue damage and characteristics of the pain. The pain
is frequently described as burning and continuous and
exacerbated by movement, continuous stimulation or
stress. The diagnostic criteria given by IASP is the pres-
ence of continuing pain, allodynea, or hyperalgesia with
which the pain is disproportionate to any inciting event
(CRPS Type I), occurs after a nerve injury and not neces-
sarily limited to the distribution of the injured nerve
(CRPS Type II). These pains may be craniofacial but are
classified as relatively generalized syndromes.92 These
conditions are rare in the craniofacial region and must be
differentiated from atypical facial pain and atypical
odontalgia.
The diagnosis of SMP in the craniofacial area is con-
firmed by stellate ganglion block with local anesthetic.
THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 311
CRANIOFACIAL NEURAL DISORDERS KILPATRICK

Treatment of SMP/CRPS is difficult. Clonidine, tri-
cyclic antidepressants, repetitive nerve blocks, peripheral
nerve stimulation, spinal cord stimulation, intrathecal
opioid infusion, and other modalities have been advo-
cated.95-96
References
1. Merskey H, Bogduck N: Classification of chronic pain. 2nd ed. IASP Press,
1994.
2. Loeser JD: Cranial neuralgias. In: Bonica JJ, ed. The management of pain.
2nd ed. Philadelphia: Lea and Febiger, 1990: 676-686.
3. Pertes RA, Gross SG: Clinical management of temporomandibular disorders
and orofacial pain. Chicago: Quintessence, 1995:329-341.
4. Saper JR, Silberstein S, Gordon CD, Hamel RL: Handbook of headache
management. Baltimore: Williams and Wilkins, 1993:122-137.
5. Okeson JP: Bell’s orofacial pains. 5th ed. Chicago: Quintessence, 1995:403-
455.
6. Saper JR, Silberstein S, Gordon CD, Hamel RL, Swidan S: Handbook of
headache management. 2nd ed. Baltimore: Lippincott, Williams and
Wilkins, 1999: 249-270.
7. Elias WJ, Burchiel KJ: Trigeminal neuralgia and other neuropathic pain syn-
dromes of the head and face. Current Pain and Headache Reports 2002;
6:115-124.
8. Ratner EJ, Person P, Kleinman DJ, Shklar G, Socransky SS: Jaw bone cavi-
ties and trigeminal and atypical facial neuralgias. Oral Surg 1979; 48:3-20.
9. Bouquot JE, Roberts AM, Person P, Christian J: Neuralgia-inducing cavita-
tional osteonecrosis (NICO), osteomyelitis in 224 jawbone samples from
patients with facial neuralgia. Oral Surg Oral Med Oral Pathol 1992;
74(3):348-350.
10. Bouquot JE, Christian J: Long-term effects of jawbone curettage on the pain
of facial neuralgia. J Oral Maxillofac Surg 1995; 53(4):387-97.
11. Mahan PE, Alling CC: Facial pain. 3rd ed. Philadelphia: Lea and Febiger,
1991: 287-291.
12. Okeson, JP: Orofacial pain - guidelines for assessment, diagnosis, and man-
agement. Chicago: Quintessence, 1996: 73-85.
13. Majoie CBLM, Hulsmans FJH, Verbeeten B, et al.: Trigeminal neuralgia:
comparison of two magnetic resonance imaging techniques in the demon-
stration of neurovascular contact. Radiol 1997; 204:455-460.
14. Meaney JFM, Eldridge PR, Dunn LT, et al.: Demonstration of neurovascular
compression in trigeminal neuralgia with magnetic resonance imaging.
J Neurosurg 1995; 83:799-805.
15. Lord SM, Bogduk N: Radio frequency procedures in chronic pain. Best
Practice and Research Clinical Anaesthesiology 2002; 16(4):597-617.
16. Backonja, MM: Use of anticonvulsants for treatment of neuropathic pain.
Neurol 2002; 59(5 Suppl 2):S14-17.
17. Holsheimer J: Electrical stimulation of the trigeminal tract in chronic,
intractable facial neuralgia. Archives of Physiology and Biochemistry 2001;
109(4):304-308.
18. Brisman R: Gamma knife radiosurgery for primary management of trigemi-
nal neuralgia. J Neurosurg 2000; 93(Suppl 3):159-161.
19. Chang JW, Chang JH, Park YG, et al.: Gamma knife radiosurgery for idio-
pathic and secondary trigeminal neuralgia. J Neurosurg 2000; 93(Suppl 3):
147-151.
20. Jannetta PJ: Arterial compression of the trigeminal nerve at the pons in
patients with trigeminal neuralgia. J Neurosurg 1967; 26:159-162.
21. Jannetta PJ: Treatment of trigeminal neuralgia by suboccipital and transtento-
rial cranial operations. Clin Neurosurg 1977; 24:538-549.
22. Jannetta PJ: Microsurgical management of trigeminal neuralgia. Arch Neurol
1985; 42:800.
23. Taki W, Matsushima S, Hori K, Mouri G, Ishida F: Repositioning of the ver-
tebral artery with titanium bone fixation plate for trigeminal neuralgia. Acta
Neurochir 2003; 145:55-61.
24. Barker FG, Jannetta PJ, Bissonette DJ, et al.: The long-term outcome of
microvascular decompression for trigeminal neuralgia. N Engl J Med 1996;
334:1077-1083.
25. McLaughlin MR, Jannetta PJ, Clyde BL, et al.: Microvascular decompression
of cranial nerves: lessons learned after 4400 operations. J Neurosurg 1990;
90:1-8.
26. Theodosopoulos PV, Marco E, Applebury C, Lamborn K, Wilson C:
Predictive model for pain recurrence after posterior fossa surgery for
trigeminal neuralgia. Arch Neurol 2002; 59:1297-1302.
27. Wallin BG, Westerberg CE, Sundlof G: Syncope induced by glossopharyn-
geal neuralgia: sympathetic outflow to muscle. Neurol 1984; 34(4):522-
524.
28. Ferrante L, Artico M, Nardacci B, Fraioli B, Cosentino F, Furtuna A:
Glossopharyngeal neuralgia with cardiac syncope. Neurosurg 1995;
36(1):58-63.
29. Kaplan AS, Assael LA: Temporomandibular disorders - diagnosis and treat-
ment. Philadelphia: WB Saunders, 1991:264-265.
30. Montalbetti L, Ferrandi D, Pergami P, Savoldi F: Elongated styloid process
and EagleÕs Syndrome. Cephal 1995; 15(2):80-93.
31. Soh KB: The glossopharyngeal nerve, glossopharyngeal neuralgia and the
EagleÕs syndromeÐcurrent concepts and management. Singapore Med J
1999; 40(10):659-665.
32. Matsushima T, Goto T, Ishioka H, Mihara F, Fukui M: Possible role of an
endovascular provocative test in the diagnosis of glossopharyngeal neural-
gia as a vascular compression syndrome. Acta Neurochir 1999; 141(11):
1229-1232.
33. Kondo A: Follow-up results of using microvascular decompression for treat-
ment of glossopharyngeal neuralgia. J Neurosurg 1998; 88(2):221-225.
34. Jarrahy R, Cha ST, Eby JB, Berci G, Shahinian HK: Fully endoscopic vascu-
lar decompression of the glossopharyngeal nerve. J Craniofac Surg 2002;
13(1):90-95.
35. Patel A, Kassam A, Horowitz M, Chang YF: Microvascular decompression
in the management of glossopharyngeal neuralgia: analysis of 217 cases.
Neurosurg 2002; 50(4):705-710.
36. Bruyn GW: Superior laryngeal neuralgia. Cephal 1983; 3(4):235-240.
37. Brownstone PK, Ballenger JJ, Vick NA: Bilateral superior laryngeal neural-
gia: its successful treatment with carbamazepine. Arch Neurol 1980;
37(8):525.
38. Taha JM, Tew JM: Long-term results of surgical treatment of idiopathic neu-
ralgias of the glossopharyngeal and vagal nerves. Neurosurg 1995;
36(5):926-930.
39. Kuhweide R, Vande Steene V, Vlaminck S, Casselman JW: Ramsay Hunt
Syndrome: pathophysiology of cochleovestibular symptoms. J Laryngol
Otol 2002; 116(10):844-848.
40. Sweeney CJ, Gilden DH: Ramsay Hunt Syndrome. J Neurol Neurosurg
Psych 2001; 71:149-154.
41. Lovely TJ, Jannetta PJ: Surgical management of geniculate neuralgia. Am J
Otol 1997; 18(4):512-517.
42. Pulec JL: Geniculate neuralgia: Long-term results of surgical treatment. Ear
Nose Throat J 2002; 81(1):30-33.
43. Pollock BE, Kondziolka D: Stereotactic radiosurgical treatment of sphenopala-
tine neuralgia, case report. J Neurosurg 1997; 87(3):450-453.
44. Kamal SA: Experience with the xylocaine test as a prognostic aid for surgery
in SluderÕs neuralgia. J Laryngol Otol 1995; 109(3):193-195.
45. Salar G, Ori C, Iob I, Fiore D: Percutaneous thermocoagulation for sphenopala-
tine ganglion neuralgia. Acta Neurochir 1987; 84(1-2):24-28.
46. Cepero R, Miller RH, Bressler KL: Long-term results of sphenopalatine gan-
glioneurectomy for facial pain. Am J Otolaryngol 1987; 8(3):171-174.
47. Day M: Sphenopalatine ganglion analgesia. Curr Rev Pain 1999; 3(5):342-
347.
48. Simons DG, Travell JG, Simons LS: Myofascial pain and dysfunction - the
trigger point manual. vol I, 2nd ed. Baltimore: Williams and Wilkins,
1999:278-328, 445-471.
49. Kuhn WF, Kuhn SC, Gilberstadt H: Occipital neuralgias: clinical recognition
of a complicated headache. A case series and literature review. J Orofac
Pain 1997; 11(2):158-165.
50. Anthony M: Headache and the greater occipital nerve. Clin Neurol Neurosurg
1992; 94(4):297-301.
51. Dubuisson D: Treatment of occipital neuralgia by partial posterior rhizotomy
at C1-3. J Neurosurg 1995; 82(4):581-586.
52. Madland G, Feinmann C: Chronic facial pain: a multidisciplinary problem. J
Neurol Neurosurg Psych 2001; 71:716-719.
53. Wessely S, Nimnuan C, Sharpe M: Functional somatic symptoms: one or
many? Lancet 1999; 354:936-939.
54. Feinmann C: The Long-term outcome of facial pain treatment. J Psychosom
Res 1993; 37:381-387.
55. Klein RW, Burk DT, Chase PF: Anatomically and physiologically based
guidelines for use of the sphenopalatine ganglion block versus the stellate
ganglion block to reduce atypical facial pain. J Craniomand Pract 2001;
19(1):48-55.
56. Dorland’s illustrated medical dictionary. 27th ed. Philadelphia: Saunders,
1988:1126, 1636.
57. Hansson PT, Fields HL, Hill RG, Marchettini P: Neuropathic pain: patho-
physiology and treatment. Seattle: IASP Press 2001:158.
58. Alper BS, Lewis PR: Treatment of postherpetic neuralgia: a systematic
review of the literature. J Fam Pract 2002; 51(2):121-128.
59. Bowsher D: Factors influencing the features of postherpetic neuralgia and
outcome when treated with tricyclics. Eur J Pain 2003; 7(1):1-7.
60. Wu CL, Marsh A, Dworkin RH: The role of sympathetic nerve blocks in

312 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE OCTOBER 2004, VOL. 22, NO. 4
 KILPATRICK CRANIOFACIAL NEURAL DISORDERS

herpes zoster and postherpetic neuralgia. Pain 2000; 87(2):121-129. Febiger 1990:264-283.
61. Kotani N, Kushikata T, Hashimolo H, et al.: Intrathecal methylprednisolone 79. Devulder J, Crombez E, Mortier E: Central pain: an overview. Acta Neurol
for intractable postherpic neuralgia. N Engl J Med 2000; 343(21):1514- Belg 2002; 102(3):97-103.
1519. 80. Vick PG, Lamer TJ: Treatment of central post-stroke pain with oral ketamine.
62. Urgosik D, Vymazal J, Vladyka V, Liscak R: Treatment of postherpetic neu- Pain 2001; 92(1-2):311-313.
ralgia with the gamma knife. J Neurosurg 2000; 93 [Suppl 3]:165-168. 81. Chen B, Stitik TP, Foye PM, et al.: Central post-stroke pain syndrome: yet
63. Tyring S, Barbarash RA, Nahlik JE, et al.: Famciclovir for the treatment of another use of gabapentin? Am J Phys Med Rehabil 2002; 81(9):718-720.
acute herpes zoster: effects on acute disease and post-herpetic neuralgia. 82. Helmchen C, Lindid M, Petersen D, Tronnier V: Disappearance of central
Ann Intern Med 1995; 123:89-96. thalamic pain syndrome after contralateral parietal love lesion: implications
64. Hannerz J: Recurrent Tolosa-Hunt Syndrome: a report of ten new cases. for therapeutic brain stimulation. Pain 2002; 98(3):98.
Cephal 1999; 19 [Suppl 25]:33-35. 83. Dworkin SF, Burgess JA: The management of pain. 2nd ed. Philadelphia: Lea
65. Tessitore E, Tessitore A: Tolosa-Hunt Syndrome preceded by facial palsy. and Febiger 1990: 784-792.
Headache 2000, 40(5):393-396. 84. Graff-Radford SB: In: Temporomandibular disorders and orofacial pain.
66. Bogduk N: Pain of cranial nerve and cranial nerve origin other than primary Chicago: Quintessence 1995:329-341.
neuralgias. In: Olesen J, Tfelt-Hansen P, Welch KMA, eds. The headaches. 85. Marbach JJ: Phantom tooth pain. J Endodont 1978; 4:362-372.
New York: Raven, 1993:765-772. 86. Rees RT, Harris M: Atypical odontalgia. Br J Oral Surg 1979; 16(3):212-
67. Cakirer S: MRI findings in the patients with the presumptive clinical diagno- 218.
sis of Tolosa-Hunt Syndrome. Eur Radiol 2003; 13(1):17-28. 87. Brooke RI: Atypical odontalgia. J Oral Surg 1980; 49:196-199.
68. Wasmeier C, Pfadenhauer K, Rosler A: Idiopathic inflammatory pseudotu- 88. Graff-Radford SB, Solberg WK: Atypical odontalgia. J Craniomandib
mor of the orbit and Tolosa-Hunt SyndromeÐare they the same disease? J Disord 1992; 6(4):260-265.
Neurol 2002; 249(9):1237-1241. 89. Woda A, Pionchon P: A unified concept of idiopathic orofacial pain: clinical
69. Gonzales GR: Pain in Tolosa-Hunt Syndrome. J Pain Symptom Manage features. J Orofac Pain 2000; 14(3):196-212.
1998; 16(3):199-204. 90. Vickers ER, Cousins MJ, Walkers, Chisolm K: Analysis of 50 patients with
70. Pareja JA, Caminero AB, Sjaastad O: SUNCT Syndrome, diagnosis and atypical odontalgia. A preliminary report on pharrnacological procedures
treatment. CNS Drugs 2002; 16(6):373-383. for diagnosis and treatment. Oral Surg Oral med Oral Pathol Oral Radiol
71. Benoliel R, Sharav T: SUNCT Syndrome: case report and literature review. Endod 1998; 85(1):24-32.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998; 85(2):158-161. 91. Janig W, Baron R: The role of the sympathetic nervous system in neuropathic
72. Pareja JA, Sjaastad O: SUNCT Syndrome: a clinical review. Headache 1997; pain: clinical observations and animal models. Progress in pain research
37:195-202. and management. Vol 21, Seattle: IASP Press 2001:125-149.
73. DÕAndrea G, Granella F, Ghiotto N, Nappi F: Lamotrigine in the treatment of 92. Merskey H, Bogduk N: Classification of chronic pain. 2nd ed. Seattle: ISAP
SUNCT Syndrome. Neurol 2001; 57(9):1723-1725. Press, 1994: 40-42.
74. Gutierrez-Garcia JM: SUNCT syndrome responsive to lamotrigine. Headache 93. Stanton-Hicks M: Reflex sympathetic dystrophy: a sympathetically mediated
2002; 42(8):823-825. pain syndrome or not? Curr Rev Pain 2000; 4(4):268-275.
75. Graff-Radford SB: SUNCT syndrome responsive to gabapentin (Neurontin). 94. Stanton-Hicks M: Complex regional pain syndrome (Type I, RSD; Type II,
Cephal 2000; 20(5):515-517. Causalgia): controversies. Clin J Pain 2000; 16 [2nd Suppl]:S33-40.
76. Hannerz J, Linderoth B: Neurosurgical treatment of short-lasting, unilateral, 95. Wasner G, Shattschneider J, Binder A, Baron R: Complex regional pain syn-
neuralgiform hemicrania with conjunctival injection and tearing. Headache dromeÑdiagnostic mechanisms, CNS involvement and therapy. Spinal
2003; 43(4):429. Cord 2003; 41(2):61-75.
77. Black DF, Dodick DW: Two cases of medically and surgically intractable 96. Ebel H, Balogh A, Volz M, Klug N: Augmentative treatment of chronic deaf-
SUNCT: a reason for caution and an argument for a central mechanism. ferentation pain syndromes after peripheral nerve lesions. Minim Invasive
Cephal 2002; 22(3):201-204. Neurosurg 2000; 43(1):44-50.
78. Tasker RR: In: The management of pain. Vol 1, 2nd ed. Philadelphia: Lea and

OCTOBER 2004, VOL. 22, NO. 4 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 313