Current Neurology and Neuroscience Reports (2018) 18:69

https://doi.org/10.1007/s11910-018-0880-0

HEADACHE (R B HALKER, SECTION EDITOR)

The Neuralgias
Danielle Wilhour 1 & Stephanie J. Nahas 1

# Springer Science+Business Media, LLC, part of Springer Nature 2018

Abstract
Purpose of Review Neuralgias are characterized by pain in the distribution of a cranial or cervical nerve. Typically, they are brief,
paroxysmal, painful attacks, although continuous neuropathic pain may occur. The most commonly encountered conditions are
trigeminal, postherpetic, and occipital neuralgia. Less common neuralgias include glossopharyngeal, superior laryngeal,
auriculotemporal, and nervus intermedius neuralgia, among others. The approach to diagnosis and treatment of this group of
disorders is reviewed.
Recent Findings Recent guidelines of medication administration, the use of botulinum toxin, and more targeted procedures have
improved treatment of neuralgias.
Summary Patients who present with neuralgias should have imaging studies to investigate for structural abnormalities unless the
etiology is apparent. Management of both common and rare neuralgias can be challenging and is best guided by the most recent
available evidence.

Keywords Neuralgia . Cranial neuralgia . Trigeminal neuralgia . Postherpetic neuralgia . Glossopharyngeal neuralgia . Occipital
neuralgia . Auriculotemporal neuralgia . Ophthalmoplegic neuropathy . Tolosa-Hunt . Raeder . Neck-tongue . Burning mouth

Introduction abnormalities, such as neoplasm, granulomatous disease, de-

myelinating disease, or vascular malformation/compression,
The neuralgias are characterized by paroxysmal, intense pain unless the etiology is clearly apparent (e.g., herpes zoster) [3].
within the distribution of a particular nerve. The pain is often This is especially true if there is evidence of neuronal injury
described as sharp, stabbing, or lancinating. Neuralgias refer- (e.g., true sensory loss) [2].
able to the head, face, and neck are often associated with trig-
gers such as brushing teeth, shaving, or chewing. Paroxysms of
pain are generally followed by pain-free refractory periods. The
Anatomy
clinical course may be monophasic, relapsing and remitting, or
chronic. Patients with neuralgia may have a primary (classical)
Nociception is the result of irritation or injury to nerves
or secondary (symptomatic) cause [1, 2]. It is crucial to distin-
subserving the scalp, face, or deeper cranial structures.
guish between the primary and secondary neuralgias based on
Neuropathic pain, which occurs in the absence of ongoing
clinical clues in order to develop an appropriate diagnostic and
tissue irritation or injury, also can arise from any of the sensory
therapeutic plan. In general, patients who present with a cranial
nerves that innervate these structures. The majority of cases of
neuralgia should have neuroimaging to evaluate for structural
cranial neuralgia involve one or more of the divisions of the
trigeminal nerve (ophthalmic, maxillary, and mandibular).
This article is part of the Topical Collection on Headache
The sensory branches of the facial, glossopharyngeal, and
second cervical nerves also transmit pain.
* Danielle Wilhour
danielle.wilhour@jefferson.edu

Stephanie J. Nahas
stephanie.nahas@jefferson.edu
Trigeminal Neuralgia
1
Jefferson Headache Center, Department of Neurology, Thomas Trigeminal neuralgia (TN), also known as tic douloureux, is
Jefferson University Hospital, Philadelphia, PA, USA the most common cranial neuralgia. It is uncommon before
69 Page 2 of 8
the age of 40. The incidence is approximately 0.2/100,000/
year. Incidence increases with advancing age, and after the age
of 80, it is approximately 25.9/100,000/year [4, 5]. Women are
affected 1.7 times more often than men [4]. Patients with
multiple sclerosis (MS) have a 20-fold increased risk of TN
compared with the general population, with a prevalence be-
tween 2 and 5% [6].
TN is defined as a “disorder characterized by recurrent
unilateral brief electric shock-like pains, abrupt in onset and
termination, limited to the distribution of one or more divi-
sions of the trigeminal nerve, and triggered by innocuous
stimuli.” [7] The pain of TN is usually in a unilateral maxillary
or mandibular distribution; the ophthalmic division alone is
involved in under 5% of cases [8]. The majority of patients
have one or more cutaneous trigger zones, usually in the cen-
tral part of the face near the nares, lips, gum line, or tongue.
Routine activities involving these zones, such as brushing
teeth, flossing, shaving, speaking, eating, drinking, or smiling,
can trigger excruciating pain. Wind, particularly cold wind, is
another commonly reported trigger. Attacks may also occur
spontaneously. The paroxysms of pain last seconds to minutes
followed by a refractory period (i.e., repeated stimulation fails
to elicit pan) lasting up to several minutes. Facial spasm or
grimacing may accompany the pain, hence the moniker tic
douloureux. These attacks may occur for weeks to months,
though some patients experience no remissions. Interictal pain
may be present, especially in severe cases. On physical exam-
ination, palpation of trigger zones may precipitate an attack,
and 25% of patients will have some degree of sensory loss.
TN in MS is characterized by a younger age of onset, bilateral
symptoms, lack of triggered pain, absence of a refractory pe-
riod, and an abnormal neurologic exam [9].
The majority of cases are classic (or primary) and result
from neurovascular irritation or compression of the trigeminal
nerve in the prepontine cistern [9]. An alternative secondary
cause is found in approximately 15% of cases of TN [9]. A
patient who presents with newly diagnosed TN should under-
go gadolinium-enhanced MRI of the brain with thin, heavily
T2-weighted cuts through the trigeminal nerves (i.e., vascular
loop study). The pathophysiology is believed to be focal de-
myelination of primary afferents near the entry of the trigem-
inal root into the pons [10]. Secondary etiologies include in-
vasive squamous cell carcinoma of the face, meningioma,
vestibular schwannoma, epidermoid cyst, skull base tumors,
Chiari malformation, saccular aneurysm, and arteriovenous
malformation compressing the trigeminal nerve root [3].
Carbamazepine is first-line of therapy in the treatment of
TN and carries Level A recommendation based on the
strength of available evidence. It should be started at a low
dose (e.g., 100 mg BID) and titrated by 100 to 200 mg every
few days as tolerated to a target dose of 400–800 mg per day.
Oxcarbazepine (target of 900–1800 mg/day) is also effective
with a more favorable side effect profile. Weaker evidence
Curr Neurol Neurosci Rep (2018) 18:69
exists for baclofen and lamotrigine, garnering a Level C rec-
ommendation [9]. Gabapentin, phenytoin, and fosphenytoin
may be useful in some patients [11–13]. Smaller studies/series
suggest that intradermal injection of onabotulinumtoxinA
may be effective without major adverse effects [14, 15].
Pimozide, while helpful for some patients, is not widely used
because of potential cardiac toxicity [16].
For patients with severe, debilitating pain refractory to
medical therapy, surgical options should be considered. The
evidence for microvascular decompression, gasserian gangli-
on percutaneous techniques, and gamma knife radiation ther-
apy supports a Level C recommendation [9]. Microvascular
decompression is a major surgical procedure, but potentially,
it is definitively curative. Up to 90% of patients achieve im-
mediate pain relief, which may be sustained at 1 year in 80%
of patients and at 5 years in 73% [10]. Complications include
CSF leak, infarction, hematoma, aseptic meningitis, and hear-
ing loss [17•]. In percutaneous ablative approaches, an elec-
trode is inserted to the trigeminal ganglion using fluoroscopic
guidance. When on target, ablation can be attempted by tech-
niques including thermocoagulation by radiofrequency, chem-
ical lesion by injection of high-concentration glycerol, or me-
chanical compression by balloon inflation. These techniques
provide immediate relief in a high percentage of cases; follow-
up studies report varying degrees of relief persisting in about
70% at 1 year and 50% at 5 years [10]. Trigeminal sensory
deficits occur transiently with balloon compression and glyc-
erol injection and are longer lasting after radiofrequency.
Stereotactic radiosurgery (gamma knife) is a more recent in-
tervention with increasing favorable evidence. Unlike other
types of intervention, the pain-relieving effects are not imme-
diate and can take up to 8 weeks. After 1 year, close to 70% of
patients remain pain-free, but this falls to about 50% at 3 years
[10]. Facial numbness and paresthesias are commonly report-
ed [17•]. The evidence for comparative efficacy and durability
of these surgical procedures is lacking, as they have not been
subjected to randomized trials or well-designed long-term
studies [18].
Patients with MS-related TN often do not respond suffi-
ciently to pharmacologic treatment, resulting in the use of high
doses and consequently intolerable side effects. Thus, surgical
interventions are often considered. Positive outcomes are re-
ported with multiple types of intervention, including micro-
vascular decompression, percutaneous ganglion lesions, and
gamma knife [10]. In these patients, however, the benefit
yielded by any type of surgical treatment tends to be shorter
in duration than in classic TN [6].
The overall clinical course is relapsing-remitting in nature
with about 50% of patients reporting spontaneous remissions
for at least 6 months. The number of episodes varies from one
single episode to numerous, and the length of each episode
ranges from a single day to several years [19]. The disorder
often becomes medication-refractory for patients with
Curr Neurol Neurosci Rep (2018) 18:69
recurrent episodes or no remissions, leading to surgical inter-
vention in about 50% of cases [8].
Postherpetic Neuralgia
Varicella-zoster virus (VZV), the herpes virus responsible for
varicella (chickenpox), enters a stage of dormancy in sensory
ganglia, including trigeminal. VZV may reactivate decades
later as herpes zoster (shingles) in older adults or in persons
who have diminished cell-mediated immunity (e.g., HIV, ma-
lignancy, chemotherapy, corticosteroid use, and emotional
stress). The incidence of herpes zoster sharply increases with
age; over 70% of cases occur after the age of 50 [20]. It is
slightly more common in women, and the lifetime risk is es-
timated at 30% [20]. It presents with a painful, vesicular, cu-
taneous eruption typically in the distribution of a single der-
matome. Pain may precede the emergence of rash by several
days. The syndrome generally resolves within a few weeks.
Postherpetic neuralgia (PHN) is the most frequent chronic
complication of herpes zoster. It is defined as dermatomal pain
persisting at least 90 days after acute herpes zoster rash. We
consider this within the family of cranial neuralgias when one
or more cranial nerve branches is affected. It is believed to be a
direct consequence of the response to peripheral nerve damage
sustained during the herpes zoster attack [21] and affects about
15% of people who have otherwise recovered [22••]. As with
zoster, the incidence of PHN increases with age—18% in
those aged 50 years and 33% in those aged 80 years or more
[20]. Overall, 80% of cases occur after the age of 50. Those
with more severe prodrome, rash, and pain during the acute
phase are more likely to develop it [23].
For acute herpes zoster, antiviral drugs, such as
valacyclovir or famciclovir, have been shown to accelerate
healing of the rash and shorten the duration of pain [24, 25].
However, strong evidence indicates that antiviral agents given
for acute herpes zoster do not reduce the incidence of PHN
[26]. Randomized trials have shown that the addition of oral
glucocorticoids to antiviral drugs during the acute phase of
herpes zoster does not reduce the incidence either [27, 28].
In one placebo-controlled trial, amitriptyline 25 mg daily giv-
en for 90 days upon the diagnosis of herpes zoster significant-
ly reduced the incidence of pain at 6 months [29].
Herpes zoster, and consequently PHN, can involve any of
the cranial nerves. In 10 to 20% of cases of zoster, the trigem-
inal nerve is affected [3]. Among those, about 80% manifest in
the ophthalmic division known as herpes zoster ophthalmicus
(HZO) [3]. This is in contrast to TN where the ophthalmic
division is involved in under 5% of cases. PHN occurs in
36.6% of patients over the age of 60 and in 47.5% over the
age of 70 in HZO [30]. In addition to pain, HZO can cause
ptosis, keratitis, uveitis, iritis, conjunctivitis, or acute retinal
necrosis.
Page 3 of 8 69
Treatment of PHN is primarily symptomatic. Pain may
persist for years or even for life; thus, medication is often
required for prolonged periods. Topical therapy alone is rea-
sonable as first-line therapy. It is often used in combination
with systemic drugs for moderate or severe pain. Patches con-
taining 5% lidocaine are approved for treatment of PHN in the
USA. Capsaicin 0.075% cream may be helpful, though its use
is limited due to the need for four times daily use and short-
term burning and erythema when applied [21]. Evidence sup-
ports the use of tricyclic antidepressants and the antiepileptic
drugs gabapentin and pregabalin. Meta-analysis regarding tri-
cyclic antidepressants estimates the number needed to treat
with amitriptyline, desipramine, or nortriptyline as 3, while
the number needed to harm is 16 [31, 32]. Meta-analysis re-
garding gabapentin and pregabalin estimates the number
needed to treat as 3 to 8 and the number needed to harm is 7
to 32 [31]. A recent Cochrane review concluded, there was not
a convincing benefit to treating this disorder with oxycodone
[33]. Opioids, including tramadol, should be used as third-line
agents for PHN, if at all. It appears that lower dose combina-
tion therapy is more effective and better tolerated than the use
of a higher dose of a single drug [21]. Peripheral or sympa-
thetic nerve blocks, cryotherapy, acupuncture, biofeedback,
and transcutaneous electrical stimulation are other options that
may complement conventional treatment or serve as an
alternative.
The only well-established method of preventing PHN is
prevention of herpes zoster. A live attenuated VZV vaccine
became available in 2006. It was originally licensed for im-
munocompetent individuals 60 years of age or older, but now
is approved for people 50 years or older. The live herpes zoster
vaccine administered to adults older than 60 years of age is
51% effective for prevention of zoster and 66% effective for
prevention of PHN [3, 34]. In 2017, a recombinant vaccine
was licensed for individuals over the age of 50 years admin-
istered in two doses 2–6 months apart. It is now recommended
to immunocompetent adults aged 50 years or older [35]. It has
also shown to have an efficacy of 97.2% [36•].
Occipital Neuralgia
Occipital neuralgia is defined a paroxysmal shooting or stab-
bing pain in the dermatomes of the greater or lesser occipital
nerve [7]. The greater occipital nerve originates from the dor-
sal ramus of C2, and the lesser occipital nerve originates from
C2 and C3 in the cervical plexus [37]. The pain is unilateral in
85% of patients [12]. Distribution of the pain follows the
greater occipital nerve in 90%, lesser occipital nerve in 10%,
and both nerves in 9% of cases [38]. Patients present with
paroxysms of lancinating pain lasting seconds to minutes that
can be associated with dysesthesia or allodynia in the posterior
scalp [39]. Pain may radiate to the ipsilateral fronto-orbital
69 Page 4 of 8
region due to interneuronal connections in the trigeminal spi-
nal nuclei. A dull aching pain may persist between paroxysms.
Palpation over the affected nerve branches elicits the pain.
Differential diagnosis includes migraine, C2 neuralgia,
cervicogenic headache, tumor, infection, and congenital
anomalies [39].
Initial conservative treatment includes warm or cold com-
press, massage, and physical therapy directed to improve pos-
ture. Nonsteroidal anti-inflammatory medications, muscle re-
laxants, tricyclic antidepressants, and anticonvulsants (e.g.,
carbamazepine, gabapentin, and pregabalin) have been report-
ed to be helpful [40]. Local anesthetic injection can both con-
firm the diagnosis and provide temporary pain relief [41].
Bedside sonography can be used to identify the location and
degree of occipital nerve entrapment and enhance the preci-
sion of nerve blocks [42]. Two small case series showed mod-
est improvement with onabotulinumtoxinA [43, 44••].
Limited evidence suggests that pulsed radiofrequency and im-
planted occipital nerve stimulators may be effective in some
patients refractory to standard treatments [40, 41].
Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia (GN) is similar in many ways to
TN but with different location (e.g., mouth, throat, and ear)
[44••]. It is characterized by clusters of attacks of pain; de-
scribed as sharp, stabbing, shooting, or lancinating; and locat-
ed at the posterior region of the tongue, tonsils, oropharynx,
larynx, auditory canal, middle ear, angle of the mandible, or
retro-molar region [45]. These painful attacks are provoked by
talking, swallowing, yawning, and coughing. The paroxysms
can remit and relapse for months to years, and between at-
tacks, patients are usually completely pain-free. GN is further
classified by whether the location is otalgic with pain deep in
or near the ear, or oropharyngeal with pain in the pharynx,
tonsil, soft palate, or posterior tongue [46]. Approximately
10% of attacks are associated with autonomic symptoms re-
ferable to vagal dysfunction including bradycardia, hypoten-
sion, syncope, seizures, and even cardiac arrest. [45]
The incidence of GN is 0.8 per 100,000 person-years. It is
less common than TN, though the two neuralgias may coexist
[47]. The majority of GN cases are idiopathic [45]. Secondary
GN can occur due to compression or injury of the
glossopharyngeal nerve by vascular structures, regional tu-
mors, neck trauma, and carcinomas, among others. MS
plaques involving the root entry zone may also cause it
[44••]. A high-resolution MRI brain with vascular loop study
is recommended to evaluate for secondary causes.
Pharmacologic treatment is similar to that for TN. GN is
usually responsive to sodium channel anticonvulsants, but re-
sponse rates may decline over time [36•]. Microvascular de-
compression is considered an effective and safe treatment
Curr Neurol Neurosci Rep (2018) 18:69
option with pharmacologic treatment failure [48, 49].
Rhizotomy is a preferred alternative if microvascular decom-
pression is technically difficult [48].
Superior Laryngeal Neuralgia
Superior laryngeal neuralgia is characterized by sharp, stab-
bing paroxysms of pain lasting seconds to minutes in the an-
terolateral neck. The pain is unilateral and can radiate from the
thyroid cartilage to the angle of the mandible and occasionally
the ear [44••]. The pain can be elicited by palpation of the
superior laryngeal nerve through the thyrohyoid membrane.
Attacks can also be precipitated by talking, swallowing,
coughing, straining the voice, or head turning [50]. Pain on
swallowing can cause decreased nutritional intake leading to
weight loss [51]. Hoarseness and cough can be associated.
[52, 53] This condition is very rare. It is believed to represent
between 1.3 and 3% of all cranial neuralgias [44••]. It is seen
in both sexes equally [44••]. Causes include trauma, surgery,
inflammation, upper respiratory infections, or viral infections
[54]. MRI or CT of the neck should be performed to rule out
pathologic conditions [54]. Treatment recommendations are
based on case reports. Carbamazepine, gabapentin, and ami-
triptyline have reportedly been effective [55]. Nerve blocks
with local anesthetic have been reported to provide long-
term pain relief [55, 56].
Nervus Intermedius Neuralgia
Also known as geniculate neuralgia, nervus intermedius neu-
ralgia is another uncommon facial neuralgia in which pain
arises from the sensory portion of the nervus intermedius
branch of the facial nerve. This branch innervates the external
auditory meatus, pinna, and retroauricular region [44••].
Patients present with intermittent unilateral stabbing pain in
the auditory canal triggered by sensory or mechanical stimuli.
The pain may be associated with impaired lacrimation, saliva-
tion, or taste [57]. Non-neurologic causes of otalgias should be
excluded. An MRI of the brain with thin cuts of the
cerebellopontine angle should be performed to assess for a
vascular loop compressing the nervus intermedius [57].
Medical treatment is similar to TN with carbamazepine
being first line [57]. Gabapentin, lamotrigine, and tricyclic
drugs have also been reported to be effective [3]. Regional
nerve blockade may also be helpful, and relief may persist
well after the initial conduction blockade has passed. If med-
ical treatment fails, then surgery may be considered [57]. For
patients in whom medical therapy has failed, anecdotal reports
of successful surgical treatments include transection or micro-
vascular decompression of the nervus intermedius [58].
Curr Neurol Neurosci Rep (2018) 18:69
Auriculotemporal Neuralgia
Auriculotemporal neuralgia manifests with pain in the temple,
ear, preauricular area, temporomandibular joint, or parotid ar-
ea. All described cases are unilateral with moderate to severe
paroxysms of stabbing pain. Background pain may also be
present. Pain can be triggered by pressure on the preauricular
area. Prevalence is reported to be 0.2 to 0.4% of patients treat-
ed at tertiary headache centers, most commonly affecting
middle-aged women [59, 60]. A speculative cause is mechan-
ical in nature, such as entrapment through the lateral pterygoid
muscle or compression through other structures including the
infratemporal fossa [44••, 60]. Clinicians must rule out tem-
poromandibular joint pathology as well as TN and primary
headache disorders. MRI of this region also should be obtain-
ed to rule out secondary causes. Because of the rarity of this
condition, no high-quality studies exist. Evidence for treat-
ment is based on case series showing response to local anes-
thetic blockade of the auriculotemporal nerve with or without
steroid. Auriculotemporal nerve blocks may be considered
both diagnostic and therapeutic [61]. Pharmacological thera-
pies such as carbamazepine and gabapentin may also be ef-
fective [59, 60].
Recurrent Painful Ophthalmoplegic
Neuropathy
Recurrent painful ophthalmologic neuropathy (RPON) is a
rare condition that is characterized by repeated attacks of
one or more ocular cranial nerve palsies with ipsilateral head-
ache [62]. RPON was formerly termed “ophthalmoplegic mi-
graine,” but in the ICHD-3, is reclassified as a cranial
neurlagia [7, 63]. It may occur as a single event or as recurrent
episodes of ophthalmoplegia [64]. The headache is typically
unilateral, severe, and ipsilateral to the side of the
ophthalmoplegia. It is described as migrainous in 68% of
cases [65]. The oculomotor nerve is the one most frequently
involved [64, 65]. Most cases appear in childhood or early
adulthood with a slight predominance in girls/women [65]. It
is recommended that MRI brain with gadolinium as well as
MRA head and lumbar puncture are obtained to evaluate for
secondary causes. Enhancement or thickening of the involved
cranial nerve can be seen on MRI in RPON. Attacks of
ophthalmoplegia appear and then resolve spontaneously.
Symptomatic treatment with corticosteroids may hasten re-
covery [64].
Tolosa-Hunt Syndrome
Tolosa-Hunt syndrome (THS) manifests from idiopathic gran-
ulomatous inflammation in the cavernous sinus or superior
Page 5 of 8 69
orbital fissure. It presents with retro-orbital pain and
ophthalmoplegia affecting one or more of the third, fourth,
and sixth cranial nerves. It has been reported in all age groups
[66]. Contrast-enhanced MRI along with blood and cerebro-
spinal fluid examination are required to exclude other condi-
tions [66]. In almost 50% of THS cases, MRI may show
abnormalities of the orbit, cavernous sinus, or superior orbital
fissure. THS is treated with prednisone 80 to 100 mg daily for
3 days and then a gradual taper in 2-week intervals [67]. The
prognosis for most patients is favorable. However, some pa-
tients follow a relapsing-remitting course requiring prolonged
corticosteroid or other immunosuppressive therapy [68].
Serial MR imaging is recommended in following cases to
complete resolution [69].
While both RPON and THS can present with ipsilateral
headache and ophthalmoplegia, there are notable differences
between the two diseases. With RPON, younger individuals
are typically affected, headache can precede ophthalmoplegia
by 14 days, cranial nerve III most commonly involved (80%
of cases), pupillary involvement is absent, and resolution typ-
ically occurs within 3 weeks [62]. In contrast, THS typically
affects older individuals (mean age of onset is 56 years), head-
ache can precede ophthalmoplegia by 2–3 days, cranial nerves
IV and VI are frequently involved in addition to cranial nerve
III, pupillary involvement is noted in > 20% of cases, and
mean recovery time is 2 months [62].
Paratrigeminal Oculosympathetic Syndrome
(Raeder Syndrome)
Paratrigeminal oculosympathetic syndrome consists of con-
stant unilateral pain in the distribution of the ophthalmic divi-
sion of the trigeminal nerve in addition to ipsilateral ptosis and
miosis [10]. The pain is worsened by eye movement and may
extend also to the maxillary division. This clinical presenta-
tion can be indicative of a lesion in the carotid artery or middle
cranial fossa [70, 71]. MRI/MRA or CT/CTA should be ob-
tained promptly for this reason. Hemicrania continua, a tri-
geminal autonomic cephalalgia responsive to indomethacin,
should be considered in the differential diagnosis.
Neck-Tongue Syndrome
Neck-tongue syndrome (NTS) is characterized by paroxysmal
neck or occipital pain, or both, as well as ipsilateral tongue
paresthesia [72]. It is triggered by rotation of the neck. NTS
typically has pediatric or adolescent onset [73•]. It may be
associated with pathologic findings in the region of the first
two cervical vertebrae [72]. In the absence of pathologic find-
ings requiring specific therapy, the disorder appears to be be-
nign, and conservative management is usually effective [72].
69 Page 6 of 8
Burning Mouth Syndrome
Burning mouth syndrome is characterized as a burning sensa-
tion of the oral mucosa in patients who have a clinically nor-
mal mucosal examination. It most commonly affects the ante-
rior two thirds of the tongue and is often accompanied by
dysguesia and xerostomia [74]. It is seen frequently in post-
menopausal women with a female to male ratio of 7:1. The
etiology of this disorder is poorly understood, though evi-
dence for a neuropathic etiology is emerging [75]. Burning
mouth syndrome may present as an idiopathic condition or a
secondary one from nutritional deficiencies, hormonal chang-
es associated with menopause, primary xerostomia, hypersen-
sitivity reactions, or medications [75]. If an underlying cause
cannot be found, treatments include B-complex vitamins, tri-
cyclic antidepressants, gabapentin, oral clonazepam, and top-
ical clonazepam. Psychotherapy and biofeedback can be of
benefit to some patients [75].
Conclusion
Cranial neuralgias are recognized by their characteristic clin-
ical presentations, including lancinating pain or other symp-
toms referable to a specific nerve. Although many cases are
primary, secondary etiologies are not uncommon.
Medications, chiefly anticonvulsant and tricyclic drugs, re-
main the first-line treatment for many of the neuralgias.
Nerve blocks, surgery, and other procedures may be necessary
in refractory cases.
Compliance with Ethical Standards
Conflict of Interest Danielle Wilhour declares no conflict of interest.
Stephanie J. Nahas has received personal fees from Allergan, Amgen,
Avanir, electroCore, Ely Lilly, and Supernus.
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.
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