Curr Neurol Neurosci Rep (2014) 14:459
DOI 10.1007/s11910-014-0459-3
HEADACHE (RB HALKER, SECTION EDITOR)
The Neuralgias: Diagnosis and Management
Paul M. Gadient & Jonathan H. Smith
Published online: 6 May 2014
# Springer Science+Business Media New York 2014
Abstract The neuralgias are characterized by pain in the
distribution of a cranial or cervical nerve. While most often
brief, severe, and paroxysmal, continuous neuropathic pain
may occur. The most commonly encountered entities include
trigeminal, postherpetic, glossopharyngeal, and occipital neu-
ralgia. More unusual cranial neuralgias may occur in
periorbital (eg, supraorbital neuralgia) and auricular (eg,
nervus intermedius neuralgia) distributions. These disorders
may be mimicked by structural and inflammatory/infectious
neurologic disease, along with other primary headache disor-
ders (eg, primary stabbing headache). The approach to diag-
nosis and treatment of this group of headache disorders is
reviewed.
Keywords Neuralgia . Cranial neuralgia . Trigeminal
neuralgia . Occipital neuralgia . Postherpetic neuralgia .
Glossopharyngeal neuralgia . Nervus intermedius neuralgia .
Auriculotemporal neuralgia
Introduction
The neuralgias are characterized by paroxysmal, brief, and
intense pains described as sharp, lancinating, stabbing, or
lightning-like within the distribution of a particular nerve.
They are often associated with triggers that may take the form
of trivial stimuli, such as brushing teeth or shaving. The
paroxysms of pain often have characteristic triggers and are
generally followed by pain-free periods known as refractory
This article is part of the Topical Collection on Headache
P. M. Gadient : J. H. Smith (*)
Department of Neurology, University of Kentucky,
740 S. Limestone, L445, Lexington, KY 40536, USA
e-mail: jonathan.smith@uky.edu
periods. Tenderness over the involved nerve is typical as is
abolishment of pain by local anesthetic blockade. The clinical
course may be monophasic, relapsing and remitting, or chron-
ic in nature. Each neuralgia may be classified as primary
(classical) or secondary (symptomatic) and distinguishing
between the 2 is critical for developing an appropriate diag-
nostic and therapeutic plan.
An important distinction in terminology is between neural-
gia, which refers to pain in the distribution of a nerve and
neuropathy, implying neuronal injury. Patients with neuralgia
may or may not have a secondary cause. However, evidence
of neuronal injury, often in the form of sensory loss, necessi-
tates a neurodiagnostic evaluation to identify secondary etiol-
ogies [1]. Highlighting the importance of this is the “numb-
chin” sign of trigeminal branch mental neuropathy—associat-
ed with a high mortality because of a frequent association with
malignancy [1].
The International Classification of Headache Disorders
(ICHD) recognizes these disorders in the recently published
third edition under section 13, “Painful cranial neuropathies
and other facial pains” [2•]. The specific diagnoses recognized
by the ICHD-3 beta version include trigeminal neuralgia
(TN), postherpetic neuralgia (PHN), glossopharyngeal neural-
gia (GN), nervus intermedius neuralgia (NIN), and occipital
neuralgia (ON). The International Association for the Study of
Pain (IASP) Classification of Chronic Pain. 2nd Edition addi-
tionally recognizes superior laryngeal neuralgia [3]. Other
neuralgias (eg, lacrimal neuralgia) are not yet considered
sufficiently validated entities but will still be discussed here
as they may be encountered clinically. Our goal is to provide a
clinical overview of the most commonly encountered neural-
gias as organized by diagnosis for relatively common entities
(eg, TN) and then by topography (eg, periorbital and
auricular) for the more unusual neuralgias. Detailed dis-
cussion of cranial neuralgia pathophysiology can be found
elsewhere [4, 5].
459, Page 2 of 8
The Neuralgias
The most commonly encountered neuralgias are trigeminal,
postherpetic, glossopharyngeal, and occipital neuralgia. Al-
though the epidemiology of these conditions is incompletely
defined, there appears to be an age-related increase in inci-
dence [6]. Overall incidence estimates for these conditions are
as follows: 4.3/100,000/year for TN [7], 3.3/100,000/year for
PHN [8], 0.7/100,000/year for GN [9], and 3.2/100,000/y for
ON [10]. These neuralgias differ in their pathophysiology,
topography, complications, and treatment approach, necessi-
tating awareness of their individual presentations.
Trigeminal Neuralgia
TN is uncommon before the age of 40 (overall incidence of
0.2/100,000/year), and increases in incidence with advancing
age, occurring in 25.9/100,000/year in individuals after the
age of 80 [7]. In general, the disorder appears to be slightly
more common among women. The disorder has both classical
and symptomatic (~15 % of cases) subtypes with the former
most often associated with neurovascular compression of the
trigeminal nerve in the prepontine cistern [11]. When the
disorder is associated with a young age of onset bilateral
symptoms, lack of triggered pain, absence of a refractory
period, or an abnormal neurologic exam (eg, trigeminal sen-
sory loss), secondary causes such as multiple sclerosis should
be suspected [11]. When the classical form is seen in younger
patients, venous neurovascular contact is more frequently
observed [12]. Bilaterality may be seen in 5 % of classical
cases, but even in these cases, synchronous pain is not ob-
served. Patients with bilateral TN often have a positive family
history [13].
The pain of TN is most commonly felt in V2 and V3,
involving V1 alone in less than 5 % of cases [14]. Brief,
intense, electrical pain is characteristically triggered by
chewing, talking, and light touch (eg, wind). Interictal pain
may be seen, especially in severe cases with negligible
amounts of pain-free intervals. Interestingly, patients may
present days to years prior to the onset of TN with prodromal
side-locked intra-oral achy pain, which can be carbamazepine-
responsive [15]. The overall clinical course has a relapsing-
remitting nature with at least 50 % of patients reporting at least
a 6-month remission during their clinical course [16]. This
provides the rationale for periodically tapering therapy to
avoid over-treatment. Unfortunately, the disorder often be-
comes medication-refractory, requiring surgical intervention
in an estimated 50 % of cases [14].
The initial treatment of TN always involves pharmacother-
apy with carbamazepine (target=400–800 mg/d) generally
regarded as the first-line treatment of choice. Based on 4
clinical trials with carbamazepine, a greater than 50 % reduc-
tion in attacks has been observed in at least 88 % of patients,
Curr Neurol Neurosci Rep (2014) 14:459
giving a number needed to treat of less than 2 [11]. Carba-
mazepine can be increased by 200 mg every 3 days, and given
up to 4 times daily in an effort to minimize dose-related side-
effects, and target pain-predominant times of the day (eg,
nocturnal attacks). Oxcarbazepine (target=900–1800 mg/d)
is likely equally efficacious, generally with a better side-
effect profile but with less flexibility with titration [11]. Other
medications to consider, often as add-on therapies, include
phenytoin (target=300–500 mg/d), baclofen (target=40–
80 mg/d), clonazepam (target=1.5–8 mg/d), lamotrigine (tar-
get=150–400 mg/d), gabapentin (target=900–2400 mg/d),
and pimozide (target=4–12 mg/d) [14]. A 2013 Cochrane
review of nonantiepileptic drugs for TN concluded that
pimozide was more effective than carbamazepine and that
0.5 % proparacaine hydrochloride eye drops had no benefit
over placebo [17]. Pimozide is not widely used due to poten-
tial cardiac and neurologic toxicity [18]. The clinical utility of
certain agents, especially lamotrigine, is limited by the re-
quirement for a slow titration.
Among medication-refractory cases, botulinum toxin type
A (BTX) has been shown in 5 prospective studies and 1
double-blind, randomized, placebo-controlled study to be a
generally effective treatment without major adverse events
[19, 20]. The optimal injection strategy is yet to be deter-
mined, but subdermal trigger zones are most typically injected
in published studies [20]. Opioids are sometimes used during
acute exacerbations, although empiric data is lacking. Finally,
rescue intravenous infusion of either fosphenytoin or lido-
caine may provide transient relief while awaiting intervention-
al procedures [21, 22].
Neurosurgical interventions for refractory TN include ab-
lative (destructive) (eg, peripheral neurectomy, glycerol
rhizolysis, sensory rhizotomy, and gamma-knife radiosurgery)
and nonablative (nondestructive) (eg, microvascular decom-
pression (MVD)) interventions [23]. These procedures gener-
ally provide rapid relief with the exception of radiosurgery,
which often requires at least 6 weeks to see a peak benefit
[23]. Major concerns with use of ablative procedures surround
the development of anesthesia in the V1 distribution poten-
tially resulting in neurotrophic keratopathy and/or anesthesia
dolorosa in the face. Although there has never been a random-
ized trial, Dr. Peter Jannetta has reported on the outcomes of
MVD in 1185 patients, where 75 % reported complete relief
following the procedure, and 64 % continued to report excel-
lent results at 10 years [24]. The recurrence rate was estimated
to be between 1 %–2 % per year. In another series, 29 patients
with persistent or recurrent TN underwent repeat posterior
fossa exploration, where MVD was performed in 18 patients
and partial nerve section in 11 [25]. Overall, excellent results
were seen with 75 % being pain and medication-free at 3 years.
Two of the patients who underwent nerve section developed
anesthesia dolorosa. Therefore, repeat posterior fossa explo-
ration should be a consideration prior to ablative procedures in
Curr Neurol Neurosci Rep (2014) 14:459
patients with recurrent or persistent pain after a prior MVD.
Gamma knife radiosurgery targeting 70–90 Gy to the proxi-
mal root and/or dorsal root entry zone is thought to provide
pain relief to the majority of patients but carries a higher risk
of recurrence than MVD [26, 27]. Gamma knife does not
carry the risks associated with general anesthesia and craniot-
omy. Although trigeminal sensory dysfunction is relatively
common after radiosurgery, anesthesia dolorosa is very rarely
encountered [26, 27]. For the estimated 3 %–17 % of cases
without root compression, the optimal treatment approach
becomes more uncertain with partial sensory rhizotomy
thought to impose a high remission rate of 88 % with 28 %
recurrence after 5 years [28].
Patients with TN secondary to multiple sclerosis often have
more difficult to control pain and are less likely to have
neurovascular trigeminal contact on imaging [29]. Unfortu-
nately, even when neurovascular contact is present, the out-
comes of MVD are very modest compared with that seen in
classical TN [30, 31]. In patients with multiple sclerosis
ablative procedures, such as gamma knife radiosurgery should
be given [32].
Postherpetic Neuralgia
PHN is the most common complication of herpes zoster
(also known as shingles), which results from reactivation
of latent varicella zoster virus [33]. PHN is defined as
pain persisting more than 4 months after the onset of rash
in the area affected by herpes zoster. Herpes zoster
ophthalmicus is estimated to occur in 10 %–25 % of cases
[34]. PHN is more common among women, and its inci-
dence increases with age, occurring in about 18 % at age
50 and 33 % at age 80 [33]. While most cases will be
readily diagnosed by a history of the classic dermatomal
rash, the syndrome of zoster sine herpete may be encoun-
tered [35]. In these cases, ancillary laboratory evaluation
may be pursued to confirm diagnosis. A 2005 clinical trial
demonstrated a reduced incidence of both zoster and PHN
following administration of a zoster vaccine to immuno-
competent hosts age 60 or older [36]. More recent clinical
trial data suggests that these results may be extended to
individuals 50–59 years old [37]. However, it is a live
vaccine and, therefore, is contraindicated in immunocom-
promised patients. Oral acyclovir is not thought to reduce
the incidence of PHN [38].
As recommended by a 2004 American Academy of Neu-
rology practice parameter, first line therapies for PHN include
tricyclic antidepressants (target=25–150 mg/d), gabapentin
(target=1800–3600 mg/d), pregabalin (target=150–600 mg/
d), and topical lidocaine 5 % patch [39]. Divalproex sodium
1000 mg per day was shown to be beneficial in a randomized
double-blind placebo-controlled study [40]. In refractory
cases or in patients intolerant of first line agents, opioids and
Page 3 of 8, 459
capsaicin may be considered. Combining the lidocaine patch
with other agents has also proven effective. Intravenous acy-
clovir for 2 weeks followed by oral valacyclovir was reported
as helpful in a small open label series, following the concept
that latent ganglionitis is present and may contribute to pain
[41].
Glossopharyngeal Neuralgia
GN (also known as vagoglossopharyngeal neuralgia) is less
common than TN and may be associated with relatively less
severe attacks [9]. The 2 disorders may occasionally co-exist.
It is rarely seen in children and is most common in females
and patients over the age of 50 [42]. Most cases of GN are
idiopathic, where neurovascular compression may also be
seen. GN is less commonly associated with MS than is TN
[43].
GN is characterized by severe, transient, stabbing, uni-
lateral pain in the ear, tongue base, tonsillar fossa and/or
beneath the angle of the jaw [2•]. Although defined as
unilateral by current diagnostic criteria, side switching
and bilaterality have been described [44, 45]. Common
triggers include talking, yawning, coughing, and
swallowing. The latter may be severe enough to cause
weight loss. It may also be triggered by touching the
external auditory canal, the side of the neck, and the skin
anterior to the ear [44]. The course may be relapsing and
remitting similar to that of classical TN. GN may involve
branches of the vagal nerve leading to bradycardia and
syncope that may necessitate pacemaker placement. Al-
though not recognized by the current diagnostic criteria
for GN, vagally-mediated symptoms may rarely occur in
patients without a history of neuralgic pain [46].
Management of GN mirrors that of TN. Pharmacotherapy
should be tried initially with the preferred agents being the
same as those used in TN (Table 1).
Occipital Neuralgia
ON is a neuralgia with pathology related to cervical rather
than cranial nerves. The characteristic clinical presentation
consists of brief, sharp shooting pain in the distribution of
the occipital nerves, which is often associated with dysesthesia
and allodynia. Due to connections between the C2 dorsal root
and the nucleus trigeminal subnucleus pars caudalis, pain may
also be felt retro-orbitally [47]. A 1978 study found that 90 %
of ON was in the GON distribution, 10 % in LON, and 8.7 %
combined GON and LON [48]. The lesser occipital nerve
arises from the dorsal rami of C2 and/or C3 spinal nerves.
This study may have neglected the possibility of third ON,
which has been suggested to comprise the majority of occip-
ital headaches due to trauma [49]. While often post-traumatic
or idiopathic, diverse vascular (eg, giant cell arteritis),
459, Page 4 of 8 Curr Neurol Neurosci Rep (2014) 14:459

Table 1 Commonly used medications in the treatment of cranial neuralgia

Medication Starting dose Rate of titration Target dose Common side effects

Carbamazepine 200 mg/d 200 mg/d 400–800 mg/d ataxia (15 %), dizziness (44 %), drowsy (32 %), nausea (29 %), vomiting (18 %),
headache (22 %)
Oxcarbazepine 150 mg/d 150 mg/3 d 750–1800 mg/d dizziness (30 %–50 %), diplopia (30 %–50 %), headache (26 %–30 %)
Gabapentin 300 mg/d 300 mg/3–7 d 900–2400 mg/d somnolence (16 %–20 %), fatigue (11 %–15 %), ataxia (11 %–15 %), dizziness
(16 %–20 %),
Pregabalin 150 mg/d 150 mg/wk 600–1200 mg/d dizziness (21 %), somnolence (12 %), peripheral edema (9 %),
xerostomia (8 %), euphoria (2 %),
Lamotrigine 25 mg/d 25 mg/wk 100–400 mg/d rash, dizziness (38 %), ataxia (21 %), diplopia (26 %–30 %)
blurred vision (16 %–20 %), rhinitis (11 %–15 %)
Topiramate 25 mg/d 25 mg/wk 100–400 mg/d dizziness, diplopia, language impairment, weight loss, glaucoma, kidney stones
Baclofen 5–10 mg/d 5 mg/3 ds 50–75 mg TID drowsy (10 %–63 %), dizziness (5 %–15 %), nausea (4 %–12 %),
confusion (1 %–11 %)
Amitripyline 10–25 mg/d 10–25 mg/wk 75–300 mg/d fatigue, confusion, arrhythmias, orthostatic hypotension, urinary retention, seizure

neurogenic (eg, C2 schwannoma), muscular/tendinous, and
osteogenic mechanisms may underlie the nerve root irritation
[50]. Further, the syndrome may rarely be mimicked by upper
cervical myelitis [51].
Nerve block with a local anesthetic and corticosteroid may
provide temporary relief (Table 2), while a neuropathic pain
medication (eg, gabapentin) and physical therapy are initiated.
If this conservative treatment is ineffective, pulsed radiofre-
quency ablation, occipital nerve stimulation, or a trial of BTX
may be considered [50].
Periorbital and Auricular Neuralgias: Topographic
Diagnoses
When confronted with a short-duration headache with neural-
gic features, knowledge of peripheral nerve dermatomes can
be helpful in establishing a diagnosis based on the topography
of pain (Fig. 1). The primary regions where this becomes
important are in the periorbital and auricular distributions.
Cranial neuralgias which can cause periorbital pain include:
supraorbital/supratrochlear neuralgia, infraorbital neuralgia,

Table 2 Peripheral nerve blocks for cranial neuralgias

Peripheral nerve Landmarks Needle Suggested Suggested injectate Potential complications

gauge volume

Greater occipital A line from the occipital protuberance to the 25–30 1.5–3 mL 1 %–2 % lidocaine and alopecia, pain, infection,
mastoid process and moving 1/3 of the bupivacaine 0.25 %–0.5 % hematoma, lightheadedness
way laterally in 1–1 volume ratio with or
without triamcinolone
20 mg
Lesser occipital Same line used to localize the GON, but 25–30 1–2 mL same as above alopecia, pain, infection,
by moving 2/3 of the way laterally hematoma, lightheadedness
from the occipital protuberance
Supratrochlear Superomedial corner of orbit, at or just 30 0.2–1 mL 1 %–2 % lidocaine and pain, infection, bleeding,
nerve above eyebrow bupivacaine 0.25 %–0.5 % intravascular injection
in 1–1 volume ratio
Supraorbital Directly above supraorbital notch a 30 0.1–1 mL same as above pain, infection, bleeding,
nerve intravascular injection
Infraorbital nerve At the level of the infraorbital foramen, 30 0.5–1 mL same as above pain, infection, peri-orbital
direct the needle superomedially taking hematoma or ecchymosis,
care not to enter the foramen while intravascular injection
applying gentle pressure to the lower
eyelid and infraorbital tissue
Auriculotemporal Above posterior part of zygoma anterior to 30 0.5–1.0 mLc same as above pain, infection, bleeding,
nerve tragus, 2–3 mm anterior to superficial intravascular injection
temporal artery b
a
Alternatively, at or just above eyebrow at midpupillary line; or after supratrochlear block, redirect needle 2 cm laterally
b
Additional injections may be made superiorly to block temporal area branches
c
0.25 mL for each additional injection
Curr Neurol Neurosci Rep (2014) 14:459 Page 5 of 8, 459

Fig. 1 Sensory innervation of the

scalp. Lateral view of the head
depicting the course of superficial
trigeminal and cervical nerve
branches. Reproduced under the
terms of the Creative Commons
Attribution License, which
permits unrestricted use,
distribution, and reproduction in
any medium, provided the
original author and source are
credited. (From Kemp WJ III,
Tubbs RS, Cohen-Gadol AA. The
innervation of the scalp: a
comprehensive review including
anatomy, pathology, and
neurosurgical correlates. Surg
Neurol Int. 2011;2:178 [52])

and lacrimal neuralgia. While these disorders represent tri-
geminal branch neuralgias, they are considered distinct from
TN, which invariably spreads to involve other dermatomes
over time. The differential diagnosis of auricular pain is even
more challenging given the complex sensory innervation of
the ear [53]. The differential diagnosis of deep neuralgic ear
pain includes primarily glossopharyngeal, vagal (Arnold’s),
and nervus intermedius neuralgia. Superficial neuralgic
otalgia can be caused by ON, as well as auriculotemporal
neuralgia. These disorders will be discussed as follows, with
the exception of glossopharyngeal and ON, which are
discussed above.
Periorbital Neuralgias
Supraorbital/Supratrochlear Neuralgia
The supraorbital and supratrochlear nerves are the 2 branches
of the frontal nerve, which is in turn a branch of the V1
division of the trigeminal nerve. They supply sensation to
the forehead and upper eyelid, which are the affected areas
in these conditions. Due to their superficial location, most
cases are secondary to trauma, which may be minor in nature
(eg, wearing a tight hat). The disorders have female predom-
inance, and can have either a spontaneously remitting or
continuous phenotype, neither of which is associated with
autonomic features [54, 55]. Although trigger points are ab-
sent, tenderness is often noted over the supraorbital notch.
Though the syndrome is generally thought of as benign,
associated pain has led to suicidal thoughts among some
reported individuals [56].
Infraorbital Neuralgia
The infraorbital nerve is a division of the maxillary branch of
the trigeminal nerve. Like the other periorbital nerves, its
superficial location makes it prone to trauma. Cases are rarely
reported, and little is known about this condition [57, 58].
Once secondary causes have been ruled out, the diagnosis can
be confirmed with an infraorbital nerve block.
Lacrimal Neuralgia
The medial branch of the lacrimal nerve provides sensory
innervation to a small portion of the anterior temple as well
as the lateral eyelid. Lacrimal neuralgia is a recently reported
pain syndrome involving continuous pain in the lacrimal
nerve distribution, where tenderness can be observed at the
superoexternal edge of the orbit [59, 60]. Intra-orbital injec-
tion may be complicated by blindness, making blockade of the
nerve at the point of exit a preferable approach [60, 61].
Neuralgic Otalgia
Auriculotemporal Neuralgia
Auriculotemporal neuralgia (ATN) is an uncommon syn-
drome characterized by paroxysms of lancinating pain anteri-
or to the tragus, which may be accompanied by facial sweat-
ing and flushing (eg, Frey’s syndrome or gustatory sweating)
[62]. Frey’s syndrome is not uncommon following
parotidectomy or trauma to the area and responds well to
BTX administration [63, 64]. A recent case report
459, Page 6 of 8
documented symptomatic ATN secondary to a temporoman-
dibular joint synovial cyst [65].
Nervus Intermedius (Geniculate) Neuralgia
Nervus intermedius neuralgia (NIN) (also known as genicu-
late neuralgia) is a rare disorder characterized by brief parox-
ysms of pain felt deeply in the auditory canal. The pain lasts
seconds to minutes and may have a trigger area in the posterior
wall of the auditory canal. Although previously required as a
diagnostic criterion, triggers have been noted to be commonly
absent among surgically-validated cases [66]. This condition
may be accompanied by disorders of lacrimation, salivation,
and/or taste and has been associated with herpes zoster. The
diagnosis of NIN is challenging due to the complex sensory
innervation of the ear and may easily be misdiagnosed as GN,
which can present as deep ear pain alone [53, 66]. Cocainiza-
tion of the tonsillar fossa to block the glossopharyngeal nerve
can serve as a useful diagnostic tool in differentiating the
disorders.
Differential Diagnosis of Cranial Neuralgias
Structural Lesions
Although many cases of cranial neuralgias are primary in
nature, imaging should be pursued to rule out an underlying
structural cause. Cranial neuralgias may be secondary to neo-
plasms, demyelinating disease, arterovenous malformations,
brainstem infarction, synringobulbia, or inflammatory dis-
eases [14]. Imaging of the brain (upper cervical spine in the
case of ON) should be pursued, especially in treatment-
refractory cases or if sensory loss is present.
Primary Stabbing Headache
Primary stabbing headache (PSH) is recognized by the ICHD-
3 beta as a distinct entity [2•]. It is defined by head pain in the
form of a single stab or series of stabs, each lasting up to
several seconds and recurring irregularly up to many times per
day. The pain location may move but occurs in a fixed location
in up to 57 % of patients [67]. It may appear as an isolated
entity or be comorbid with other primary headaches, most
commonly migraine [2•]. PSH most commonly occurs in a V1
distribution but can be occipital, parietal, temporal, or nuchal
in location [68]. Cutaneous triggers and autonomic symptoms
are absent. Unlike the cranial neuralgias, PSH is often respon-
sive to indomethacin and other COX-2 inhibitors. Gabapentin,
nifedipine, and melatonin are other reported treatment options
[69–71]. Paracetamol has been used successfully in children
and adolescents [72].
Curr Neurol Neurosci Rep (2014) 14:459
Short Lasting Unilateral Neuralgiform Headache
with Conjunctival Tearing and Injection (SUNCT)
Short-lasting unilateral neuralgiform headache attacks with
conjunctival injection and tearing (SUNCT) and short-
lasting unilateral neuralgiform headache attacks with cranial
autonomic symptoms (SUNA) are classified as trigeminal
autonomic cephalalgias but share certain clinical symptom-
atology with first division TN. Diagnostic criteria for SUNCT
require the presence of 3 to 200 daily attacks, each lasting 5–
240 seconds, associated with ipsilateral conjunctival injection
and lacrimation [2•]. Although overlap does exist, the median
attack duration of first division TN (4 seconds) is much less
than that noted in patients with SUNCT (40 seconds) [73].
Further, although autonomic signs may be seen in first divi-
sion TN, they are much less pronounced than in patients with
SUNCT [74]. Refractory periods have traditionally been
thought to be absent in SUNCT, although recently cases have
been reported with refractory period preservation [75, 76].
However, SUNCT commonly is associated with cutaneous
trigger zones, creating further diagnostic confusion [77]. The-
se diagnoses exist on a pathophysiologic continuum, and
single patients with both disorders are reported [78, 79].
Despite certain clinical similarities, patients with SUNCT
often do not respond well to carbamazepine, although this can
be tried [80]. Lamotrigine (100–300 mg per day) is considered
to be the treatment of choice with topiramate (50–300 mg per
day) and gabapentin (800–2700 mg per day) being other
options [81]. Intravenous lidocaine infusion can be used for
acute treatment of SUNCT status [81]. For intractable cases,
surgical options range from occipital nerve stimulation to
MVD [82, 83].
Conclusions
The neuralgias represent painful conditions of the head occur-
ring in a specific nerve dermatome. These disorders may be
recognized by their characteristic clinical presentations, in-
cluding pain in a restricted topography. Although many cases
are classical (primary), secondary etiologies are not uncom-
mon. Pharmacologic agents remain the first line treatment for
many of the neuralgias, but nerve blocks, surgery, and other
procedures may be necessary in refractory cases.
Compliance with Ethics Guidelines
Conflict of Interest Paul M. Gadient and Jonathan H. Smith declare
that they have no conflict of interest.
Human and Animal Rights and Informed Consent This article does
not contain any studies with human or animal subjects performed by any
of the authors.
Curr Neurol Neurosci Rep (2014) 14:459
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