Review Article

Cranial Neuralgias
Address correspondence to
Dr William P. Cheshire Jr,
Mayo Clinic, 4500 San Pablo
Road, Jacksonville, FL 32224, William P. Cheshire Jr, MD, FAAN
Relationship Disclosure:
Dr Cheshire has received
personal compensation
for manuscript preparation ABSTRACT
from Turner White Purpose of Review: Pain arising from cranial neuralgias represents a significant health
Communications, Inc.
Unlabeled Use of
burden. Successful treatment depends on accurate diagnosis, which requires knowledge
Products/Investigational of neuroanatomy and pathophysiology as well as familiarity with the varied clinical
Use Disclosure: presentations encountered in neurologic practice. This article delineates the relevant
Dr Cheshire discusses the
unlabeled/investigational use
anatomy, clinical features, and management of the most common primary and secondary
of oxcarbazepine, baclofen, cranial neuralgias.
phenytoin, fosphenytoin, Recent Findings: Trigeminal neuralgia, which can result from neurovascular compres-
gabapentin, botulinum toxin,
tizanidine, pimozide, and
sion or demyelination, is a particularly severe form of facial pain. Herpes zoster virus is a
motor cortex stimulation for common cause of neuralgia that causes herpes zoster ophthalmicus acutely and post-
the treatment of trigeminal herpetic neuralgia chronically. Rarer facial pain syndromes arising from a single nerve
neuralgia; tricyclics, pregabalin,
opioids, tramadol, and capsaicin
include glossopharyngeal neuralgia, nervus intermedius neuralgia, and paratrigeminal
for the treatment of postherpetic oculosympathetic syndrome.
neuralgia; carbamazepine, Summary: In patients presenting with a cranial neuralgia, unless the etiology is
gabapentin, lamotrigine, and
tricyclics for the treatment of
apparent (eg, herpes zoster), cranial imaging studies should be undertaken to look
nervus intermedius neuralgia; for structural abnormalities such as neoplasm, granulomatous disease, demyelin-
and nonsteroidal anti-inflammatory ating disease, or vascular malformations. Management of both common and rare
drugs, muscle relaxants, tricyclics,
gabapentin, and occipital nerve
cranial neuralgias is often challenging and is best guided by the most recent
stimulators for the treatment available evidence.
of occipital neuralgia.
* 2015, American Academy Continuum (Minneap Minn) 2015;21(4):1072–1085.
of Neurology.

INTRODUCTION neuralgias involve the trigeminal nerve,
Cranial neuralgias encompass some of which comprises ophthalmic, maxillary,
the most debilitating forms of neuro- and mandibular divisions (Figure 9-11).
pathic pain that come to the attention The trigeminal nerve also mediates pain
of neurologists. One reason may be that arising from co-innervated structures, such
the face, which is disproportionately rep- as the optic nerve and ocular motor mus-
resented on the homunculus of the pri- cles. The sensory branches of the facial
mary somatosensory cortex, is the main and glossopharyngeal nerves and the
portal through which the nervous sys- second cervical nerve can also transmit
tem encounters the world. Facial pain pain (Table 9-12).
also impacts patients psychologically and
socially, as the face is integral to inter-
personal communication and one’s sense
of personal identity.
The clinical evaluation of the patient
NEUROANATOMICAL BASIS with facial pain requires an organized ap-
Nociceptive pain can arise from any area proach beginning with localization. Is
of the scalp, face, or deeper cranial struc- the pain unilateral or bilateral? Can it be
tures in response to injury. Neuropathic traced to a single cranial nerve, or is
pain, which occurs in the absence of it more broadly distributed, which may
an ongoing tissue injury, can arise from indicate a central or multiple cranial
any of the sensory nerves that innervate nerve pain syndrome? Are colocalizing
these structures. The majority of cranial neurologic deficits present?
1072 August 2015

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

however. aching.ContinuumJournal. h The pain of trigeminal neuralgia is paroxysmal FIGURE 9-1 Sensory innervation of the face by the trigeminal nerve. mal or continuous? How long do episodes The International Headache Society last? What factors trigger. or electrical in quality? Are there mandibular branches are most often af- autonomic accompaniments? What types fected at about equal frequency. or re. or electrical in quality. such as trauma. prickling. its prev- a viral rash. maxillary. Trigeminal neuralgia is also the ogy. often after the age of 50. although on the same Continuum (Minneap Minn) 2015. (IHS) defines trigeminal neuralgia as a lieve the pain? Does the pain correlate ‘‘disorder characterized by recurrent uni- with the menstrual cycle. The trigeminal nerve root and classically sharp exits the pons and gives rise to the trigeminal (gasserian or semilunar) ganglion. KEY POINTS h Most cranial neuralgias involve the trigeminal nerve. or certain types of physical in onset and termination. h An organized clinical approach is key to reaching the correct diagnosis in patients with facial pain. nerve V). The most common cranial neuralgia Patients may notice a sensitive trig- involves the trigeminal nerve (cranial ger zone. Women are narrow the diagnosis. and its incidence and associated features are also helpful to increases with advancing age.7 times more often than men.21(4):1072–1085 www. Unauthorized reproduction of this article is prohibited. or a structural intracranial lesion? 100. Painful attacks may be followed by TRIGEMINAL NEURALGIA a brief refractory period. time of day. to an identifiable insult. the sensory branches of the facial and glossopharyngeal nerves and the second cervical nerve can also transmit 1073 Copyright © American Academy of Neurology. Lea & Febiger.1 The next question to address is etiol. quality of pain. the trigeminal nerve and triggered by bing.3Y6 Onset is most The temporal profile. . Also known as tic douloureux. sharp. Can the onset of the pain be traced most intense of the cranial neuralgias. limited to the stimulation of the painful area or remote distribution of one or more divisions of facial areas? Is the pain dull. affected 1. abrupt cific activities. worsen. innocuous stimuli. throb. an infectious or inflammatory alence is estimated at between 5 and 29 per process. pulsing. stinging. lateral brief electric shock-like pains. Reprinted from Gray H. which.’’2 The maxillary and burning. and mandibular divisions of the trigeminal nerve. spe. which divides into ophthalmic. Is the pain paroxys.000 person-years. The sharp of medications have been effective? Are or electrical paroxysms last seconds to imaging abnormalities present? minutes and may occur in rapid succes- sion. h Trigeminal neuralgia is the most intensely painful of the cranial neuralgias.

comitant persistent facial pain. For most August 2015 Copyright © American Academy of Neurology. Headache attributed to ischemic oculomotor nerve palsy IV. neuralgia is usually normal or may dis- ence spontaneous remissions. with the location of pain. Neurologic examination in trigeminal Although many patients will experi. or tongue. geminal neuralgia is active. ‘‘classical trigeminal neuralgia with con- cruciating pain (Case 9-1). contin- routine daily activities. slightest touch of the trigger zone during although in a minority of cases. posttraumatic Painful trigeminal neuropathy attributed to space-occupying lesion Persistent idiopathic facial pain Burning mouth syndrome Cranial nerve VII Facial nerve Nervus intermedius neuralgia Cranial nerve IX Glossopharyngeal nerve Glossopharyngeal neuralgia Cervical nerve 2 Occipital nerve Occipital neuralgia CNS Central neuropathic pain Painful trigeminal neuropathy attributed to multiple sclerosis a Data from Headache Classification Committee of the International Headache Society (IHS).org/ _downloads/mixed/International-Headache-Classification-III-ICHD-III-2013-Beta. gum line. close a subtle trigeminal sensory def- and severe pain may return suddenly icit. trochlear.ihs-classification. patients are or along the lip. side of the face. continuous component is presumably ing. not all do. The usually asymptomatic between attacks. tient indoors. shaving. will unleash ex. purely paroxysmal Classical trigeminal neuralgia with concomitant persistent facial pain Trigeminal autonomic cephalalgias Painful trigeminal neuropathy attributed to acute herpes zoster Painful trigeminal neuropathy attributed to postherpetic neuralgia Painful trigeminal neuropathy. Pain triggered by exposure to wind owing to central sensitization and may or a cool breeze may confine the pa. In severe cases. does not always coincide months or years later. When tri- fold. The patient presenting with newly 1074 www. The trigger pain frequency and intensity progres- zone may be located near the nasolabial sively increase over time.pdf. Cephalalgia. Cranial Neuralgias TABLE 9-1 International Headache Society Classification of Cranial Neuralgias Organized Neuroanatomicallya International Headache Level Nerve Society Classification Cranial nerve II Optic nerve Headache associated with optic neuritis Cranial nerves III. such as brushing uous dull or burning background pain or flossing the teeth.’’2 The patients may lose weight from not eat. VI and abducens nerves Cranial nerve V Trigeminal nerve Classical trigeminal neuralgia. Oculomotor. washing may precede or linger after the more the face. smil. on the lateral aspect of the nares. applying facial cosmetics. or eating. severe paroxysms. The IHS terms this ing. respond poorly to treatment. .2 www. speaking.ContinuumJournal. Unauthorized reproduction of this article is prohibited.

or if other cranial nerve or focal other skull base tumor.7 When unmistakable or ralgia includes invasive squamous cell MRI of the brain with progressive facial sensory loss is detected. Although the majority of patients with trigeminal neuralgia will have normal cranial imaging results. although he had no sinus discharge. carbamazepine at 200 mg 3 times a day had almost eliminated it. whereas at the same level. The mass encases the cisternal portion of the right trigeminal nerve.7 FIGURE 9-2 Axial brain MRI disclosing a large nonenhancing T1 hypointense (A) and T2 hyperintense (B) prepontine epidermoid cyst causing posterior displacement of the brainstem and deforming the anterior aspect of the right brachium pontis. He had been thoroughly evaluated by his dentist. causing him to wince and withdraw. Episodes of pain were easily triggered by the slightest breeze against his face. which is not visualized. search should be undertaken for an KEY POINT dergo gadolinium-enhanced MRI of the underlying tumor. who assured him that the pain was not coming from his teeth. except that touching a wisp of cotton to his right nasolabial fold provoked the patient’s pain. Case 9-1 A 67-year-old man presented with a 2-year history of paroxysmal pain affecting his right cheek. or saccular aneurysm or arteriovenous Continuum (Minneap Minn) 2015. Whereas treatment with ibuprofen and oxycodone failed to prevent or reduce the pain. Unauthorized reproduction of this article is prohibited. surgery. because the pain felt like an electric shock shooting through his face. with newly diagnosed trigeminal neuralgia inal nerves. and at times by speaking or eating. occurring numerous times during the day. MRI of the brain disclosed a large prepontine mass (Figure 9-2). cranial imaging with attention to the en. the left trigeminal nerve root is seen as a linear structure just lateral to the basilar artery. fine cuts through the unless explained by prior trigeminal nerve tibular schwannoma. and although each episode lasted only a few seconds. A structural cause may be tire course of the trigeminal nerve. Chiari malforma- neurologic deficits are present. The diagnosis was trigeminal neuralgia affecting the right maxillary division secondary to a prepontine epidermoid cyst. epidermoid cyst or trigeminal nerves. a structural cause may be found in up to 15% of cases of trigeminal neuralgia.21(4):1072–1085 www. diagnosed trigeminal neuralgia should un. . Comment. carcinoma of the face. ves. he described the pain as excruciating and rated it as 10 times more severe than the pain he had experienced from a kidney stone years earlier. a careful tion. The first time he experienced this pain was while shaving. differential diagnosis for trigeminal neu- gadolinium-enhanced inal 1075 Copyright © American Academy of Neurology. by brushing his teeth. meningioma.ContinuumJournal. The should undergo found in up to 15% of cases of trigem. The pain had increased in frequency. and he wondered if his electric razor had a short circuit. Sneezing was particularly painful. including repeated h The patient presenting brain with fine cuts through the trigem. Neurologic examination was normal.

Un- suggest a diagnosis of tion induces a focus of demyelination.20 Limited.13 ing neurovascular pathology.7 The tients. pain. Cur. which ignites a pain. Unauthorized reproduction of this article is prohibited.22 Level C evidence exists for always (97%) unilateral. distinguished from other types of facial for pain in most cases of if not the majority. . For patients with severe. particularly if the subjected to randomized trials or well- patient is relatively young or has other designed long-term studies. Ultrastruc.25 1076 www. in the proximal centrally myelinated por. whom may have dizziness. over time larger doses are multiple sclerosis. Resolution of pain within ropathies. fortunately. low- high-resolution imaging.8 A low dose. term outcomes.24. when trigeminal microvascular decompression.23 neurologic signs or symptoms.11. be visualized in some cases by trigeminal neuralgia. cerebellar hematoma or con- a few days of beginning carbamaze.10 some patients. gamma knife radiation therapy. impinges on tients with multiple sclerosis. or CSF leakage. surgical options should be considered. or im- younger patients may rior fossa. meningitis. quality evidence suggests that tiza- structive interference in steady state (CISS) nidine and pimozide may be helpful in coronal imaging. pulsatile indenta. and may be bilateral in up to 30% of pa. Over time. as they have not been trigeminal neuralgia. Ac.8. result from trigem. most often the minimize side effects. for acute crisis. including con. some of occurs bilaterally or in the trigeminal nerve root in the poste. with the aim of reversing. level of evidence for comparative effi- sider a diagnosis of multiple sclerosis cacy and durability of these surgical pro- in the patient who presents with bilateral cedures is low.9 Neurovascular compression may.17. ataxia. phe- ful crescendo of maximal discharge. geminal neuralgia. paired coordination at baseline. 100 mg 2 to 3 times trigeminal neuralgia. tortuous vascular loop.12 Whereas idiopathic or neu. not be effective. may be effective in the treatment of however. it ganglion percutaneous techniques.11 Thus. the underly- tion of the trigeminal August 2015 Copyright © American Academy of Neurology.19. 20-fold increased risk of developing tri. which is nerve root. Microvascular decompression is the tural studies have shown demyelination one procedure that uniquely addresses. a redundant or lowest effective dose is preferable to standard MRI.8. debilitat- patients with multiple sclerosis have a ing pain refractory to medical therapy. Cranial Neuralgias KEY POINTS h Neurovascular malformation compressing the trigeminal pine is considered one of the diagnos- compression. as the usually not visible by cording to this model. and it The first line of therapy in the treat. it is important to con.8 tic features of trigeminal neuralgia as believed to be the basis Many cases of trigeminal neuralgia. routine MRI techniques lack the in some patients. This procedure carries epine. may be effective initially. is inal neurovascular compression.18 Several small short- resolution to identify neurovascular com. which carries Level A evidence potential complications of cranial neu- (Table 9-214Y16). particularly in pa- h Trigeminal neuralgia that superior cerebellar artery. and Level C evidence within the trigeminal nerve reaches a exists for baclofen and lamotrigine.ContinuumJournal. gasserian neuralgia occurs in multiple sclerosis. and eventually carbamazepine may of first choice in treating to normally innocuous afferent traffic. nytoin or fosphenytoin may be useful rently. daily. Gabapentin or. dermal injection of onabotulinumtoxinA cificity. Level B evidence exists trigeminal neuralgia. term studies have indicated that intra- pression with sufficient sensitivity or spe. Ephaptic spillover to adjacent fibers for oxcarbazepine.21 Compared to the general population. appears to lead to the most enduring long- ment of trigeminal neuralgia is carbamaz. on the neurosurgeon’s preference and rovascular trigeminal neuralgia is almost experience. with a prevalence of The choice of procedure often depends 2% to 5%.7 critical threshold. tusion. which leads to aberrant discharge of the typically required to maintain pain con- h Carbamazepine is the drug nerve spontaneously or in response trol.

Hyponatremia. Stevens-Johnson 3 times/d 3 times/d fatigue. . nystagmus. suicidal thoughtsc B Oxcarbazepined. suicidal thoughts Continued on page 1078 Continuum (Minneap Minn) 2015. if stopped abruptly dizziness Gabapentind 100 mg 100Y900 mg Peripheral edema. erythema 2 times/d 2 times/d nausea. neuroleptic malignant syndrome. Percutaneous procedures reach the niques include radiofrequency thermoco- gasserian ganglion via the foramen ovale agulation. Stevens- drowsiness. bone ataxia. drowsiness. bone marrow suppression. Available tech. tremor. hypotension. epidermal necrolysis. fatigue. anaphylaxis. nausea. abdominal pain. hypotonia or muscle drug withdrawal seizures weakness. nystagmus. fatigue. alcohol or glycerol. disseminated intravascular dysmenorrhea coagulation. Stevens-Johnson dizziness. Erythema multiforme. blurry vision including aplastic anemia or agranulocytosis. pancreatitis. blurry vision. pneumonia. hemorrhage. toxic diplopia. nephrotoxicity. angioedema C Baclofen 5 mg 10Y20 mg Drowsiness. Stevens-Johnson syndrome. toxic epidermal necrolysis. suicidal dizziness. nausea. insomnia.e 300 mg 600Y1200 mg Dizziness. injection of neurotoxins such as and induce a partial injury. multiforme. 2 times/d 3 times/d nausea. ataxia. anemia. hyponatremia. nausea. hypocalcemia. ataxia thoughts Lamotrigined 25 mg every 50Y200 mg Rash. syndrome. angioedema. multiorgan hypersensitivity. rhinitis. angioedema.ContinuumJournal. drug hypersensitivity syndrome. other day 2 times/d diarrhea. hepatotoxicity. or compression of the a TABLE 9-2 Pharmacologic Treatment of Trigeminal Neuralgia Level of Common Adverse Serious Adverse Evidenceb Drug Initial Dose Typical Dose Effectsc Effectsc A Carbamazepined. Gastrointestinal 3 times/d 3 times/d constipation. 1077 Copyright © American Academy of Neurology. fatigue. syndrome. Unauthorized reproduction of this article is prohibited. depression. aseptic meningitis. marrow suppression drowsiness. constipation. headache. suicidal thoughts. blurry vision Johnson syndrome. leukopenia.e 100 mg 100Y200 mg Dizziness. ataxia. eosinophilia. toxic epidermal necrosis. Atrioventricular block. hepatotoxicity.21(4):1072–1085 www.

22.26 radiation facial paresthesia and dyses- All result in some degree of facial numb. toxic epidermal nightly dosing refers nystagmus. vitamin D marrow suppression deficiency. systemic lupus to the extended slurred speech. syndrome. the level of the pons with ionizing radia. treatment (test. Reported outcomes have varied treating trigeminal neuralgia in patients greatly. . paroxysmal pain of trigeminal neuralgia. erythematosus. Stevens-Johnson 3 times/d bedtime (single ataxia.) C = Possibly effective. (Level B rating requires at least one Class I study or two consistent Class II studies.28 The procedure in- therapy may be an appropriate inter. ineffective or harmful (or probably useful/predictive or not useful/predictive) for the given condition in the specified population. the physician should inform the patient of the possible increased risk of suicidality and assess the overall benefits versus risks of the drug. (Level A rating requires at least two consistent Class I studies. which is tients. aching.ContinuumJournal. the same pharmacologic strategy may be utilized when treating glossopharyngeal neuralgia and nervus intermedius neuralgia. 2) the magnitude of effect is large [relative rate improved outcome 95 and the lower limit of the confidence interval is 92].) U = Data inadequate or conflicting. (Level C rating requires at least one Class II study or two consistent Class III studies. Unauthorized reproduction of this article is prohibited. Cranial Neuralgias a TABLE 9-2 Pharmacologic Treatment of Trigeminal Neuralgia Continued from page 1077 Level of Common Adverse Serious Adverse Evidenceb Drug Initial Dose Typical Dose Effectsc Effectsc C Phenytoind 50 mg 300Y400 mg at Rash. bone hirsutism. unlike the brief others with less risk of corneal anes. Uncertainty whether those studies controlled adequately for relevant comorbidities has caused some to question the strength of that association. necrolysis. The role of surgical intervention in tion. suicidal thoughts a In general.) (In exceptional cases. ineffective or harmful (or established as useful/predictive or not useful/predictive) for the given condition in the specified population. thesia exists.27 ness or paresthesia and. dizziness. blurred including agranulocytosis vision/double vision or aplastic August 2015 Copyright © American Academy of Neurology. with severe medically intractable trigemi- loon compression technique appears nal deafferentation pain. Motor cortex stimulation has shown ing multiple procedures. b A = Established as effective. especially follow. The bal. 1078 www. hepatotoxicity. e The risk of serious and sometimes fatal dermatologic reactions to carbamazepine may be tenfold higher in patients of Asian ancestry who have the HLA-B*1502 genotype. c Lists of potential adverse affects are incomplete and the prescribing physician should refer to the Physician’s Desk Reference or its equivalent for a complete list. predictor) is unproven. volves an open craniotomy and implan- vention for patients unable to tolerate a tation of a strip of electrodes over the surgical procedure. can potentially initial promise in treating selected pa- cause deafferentation pain. given current knowledge. This technique targets motor cortex. ineffective or harmful (or possibly useful predictive or not useful/predictive) for the given condition in the specified population. or Gamma knife stereotactic radiation burning in quality. drowsiness. d In 2008. one convincing Class I study may suffice for an ‘‘A’’ recommendation if 1) all criteria are met. and a high incidence of post.26 is typically continuous and dull. not with trigeminal neuralgia but termed anesthesia dolorosa. ganglion with an inflatable balloon. with multiple sclerosis is less certain. bullous release form) confusion. connected to a subcutane- the trigeminal nerve root entry zone at ously tunneled stimulator. dermatosis. nephrotoxicity. the US Food and Drug Administration (FDA) issued an alert warning that antiepileptic drugs in general carry an increased risk for suicidality. thesia or anesthesia dolorosa. nausea. which can be to have efficacy similar to that of the quite severe and. gingival hyperplasia.14Y16 Since trigeminal neuralgia itself carries an increased risk for suicide.) B = Probably effective. when prescribing antiepileptic drugs for cranial neuralgia. on the basis of a meta-analysis.

h Herpes zoster frequently along with ipsilateral ptosis and miosis sents with a painful vesicular cutaneous involves the trigeminal (Horner syndrome). respectively.39 sponded to microvascular decompres. nearly 7%. iritis.2. with burning.35 The inci- Similar in quality to the pain of trigem. than 5% of cases. VZV may reactivate or syncope.29. HERPES ZOSTER h Unilateral eye pain pression is not demonstrated. dence increases with age: 70% to 80% inal neuralgia but less severe.8 per 100. OCULOSYMPATHETIC SYNDROME infection in children.2 per 1000 person-years for women and neuralgia is 0.5. glossopha. prick- in some patients. or acute retinal necrosis with the In some cases. mediated immunity or in immunocom. Some pa. which re. involves the ophthalmic division in less tients will also manifest 1079 Copyright © American Academy of Neurology. . which pre. syndrome can be a clue PARATRIGEMINAL virus that causes varicella (chickenpox) to carotid artery dissection.33 Recurrent refractory syncope may is typically multimodal. keratitis. tingling. swallowing and may pain in the distribution of the ophthal. and the lifetime risk of years. neuralgia causes Paratrigeminal oculosympathetic syn. conjunc- for trigeminal neuralgia.37 inal neuralgia. MRI has shown vagal potential for permanent visual loss in neurovascular compression. or tuberculosis.34 ling.37. or ear that is triggered by swal.21(4):1072–1085 www.32 Paroxysms of pain may cause contrast to trigeminal neuralgia. clinical presentation is indicative of a Occasionally. human immu- nerve (cranial nerve X) and branches of nodeficiency virus (HIV). The age-adjusted incidence the glossopharyngeal nerve (cranial nerve of herpes zoster is approximately 3. and itching components (Case 9-2).30 viral basis has included the detection of VZV DNA or intrathecal synthesis of anti- GLOSSOPHARYNGEAL VZV IgG in CSF and pathologic findings NEURALGIA of active viral ganglionitis. or hoarseness. rologist to the possibility of a carotid In such cases. trigger reflex bradycardia mic division of the trigeminal nerve promised patients.000 person- men. Pain may precede involving the ophthalmic tend also to the maxillary division. culty with swallowing. lymphoma. of herpes zoster occurs after the age of ryngeal neuralgia affects the auricular 50. absence of a rash (zoster sine herpete). tivitis. KEY POINTS especially when neurovascular com. This is in lowing. This the emergence of rash by several days. diffi. posterior aspect of the which occur in the ophthalmic division tongue. after which the h Glossopharyngeal (RAEDER SYNDROME) virus remains latent for decades in tri. which reflex bradycardia or syncope. as herpes zoster (shingles).8 Varicella-zoster virus (VZV) is a ubiq. herpes zoster eye involvement can lead Pharmacologic treatment is similar to that to ptosis. 80% of in the pharynx. The pain is wors- eruption typically in the distribution of nerve. cranial fossa and should alert the neu. The incidence of glossopharyngeal 3. dorsal root.36.9 and IX). accompanied by Horner uitous and highly neurotropic herpes. evidence in support of a artery dissection. 80% of the time ened by eye movement and may ex- a single dermatome.ContinuumJournal. geminal. Continuum (Minneap Minn) 2015. stabbing. uveitis.2 to 0. Unauthorized reproduction of this article is prohibited. require placement of a cardiac pacemaker shooting. aching. The pain of herpes zoster ophthalmicus sion. (herpes zoster ophthalmicus). and autonomic pharyngeal pain with drome consists of constant unilateral ganglia. herpes zoster may cause with the potential for lesion in the carotid artery or middle dermatomal neuropathic pain in the visual impairment. With age-related decline in cell.38 In addition to pain. Groups at particular risk are patients and pharyngeal branches of the vagus with transplanted organs. division of this nerve.31 It may coexist with trigem- herpes zoster is estimated at 30%.7. Herpes zoster affects the trigeminal Patients report unilateral pain deep nerve in 8% to 28% of cases.

or stimulation of the posterior by herpes zoster in one or more branches wall of the auditory canal. be difficult to treat. Neurologic examination showed 20/20 visual acuity in each eye.41 ficacy beyond 5 years is uncertain. or hard palate vus intermedius neuralgia is a very rare (Ramsay Hunt syndrome). forehead. Comment. administered to adults older than 60 years crease zoster-associated pain during the of age is 51% effective for prevention of first few weeks. anterior two. Herpes zos. have been shown to accel. and the headache intensified with sharp. Two days later. throbbing. which innervates the that follow the rash. She reported that she had been under some stress recently.35 external auditory meatus. Cutaneous allodynia is frequently pres. herpetic neuralgia defined as pain caused speaking. Facial sensory examination was abnormal with patchy hyperesthesia involving the ophthalmic division of the right trigeminal nerve. Herpes zoster vaccine antiviral agents have been shown to de. pinna. 60 years of age with acute herpes zoster Patients present with paroxysmal unilat- will experience chronic pain. ner- thirds of the tongue. August 2015 Copyright © American Academy of Neurology. Second. and tingling components. duration of pain and are US Food and and topical lidocaine. eyebrow. Her pain gradually subsided from 10/10 to 6/10 in intensity. as a rash may not be present initially. This is a typical presentation for herpes zoster ophthalmicus. although ef- the incidence of postherpetic neuralgia. but causes sharp pain felt was worsened by wearing reading glasses or by anything that touched the right deep in the ear canal. disorder in which pain arises from the ter can involve any of the other cranial sensory branch of the facial nerve (cra- nerves acutely or in the days and weeks nial nerve VII).and third- Drug Administration (FDA) approved for line treatments include opioids. .ContinuumJournal. and a More than 40% of patients older than small area of skin below the ear lobe. Cranial Neuralgias KEY POINTS h Postherpetic neuralgia occurs in more than Case 9-2 A 72-year-old woman sought neurologic evaluation for pain in the forehead. 40% of patients older Symptoms began 1 month previous with a burning sensation that she initially than 60 years of age thought was sunburn. or lateral aspect of the nose. but it affected only the right side.42 affected or neurologically complex pa.44 Geniculate zoster involving cranial nerve VII presents with ipsilateral facial NERVUS INTERMEDIUS palsy and sometimes painful vesicles in NEURALGIA the external auditory canal. First-line treatments erate healing of the rash and shorten the include tricyclics. Preventive efforts by vaccination are tients may require IV acyclovir. The pain may 1080 www.35 Whereas showing promise. Her past medical history was remarkable only for hypertension. She was seen at an urgent care clinic and h Nervus intermedius given valacyclovir 1 g orally 3 times a day for 1 week and hydrocodone 10 mg neuralgia typically 4 times a day. blisters following acute erupted in the same area. of the trigeminal nerve that persists or ent. treatment of acute herpes zoster. such as valacyclovir recurs for at least 3 months. Postherpetic eral sharp or stabbing pain deep in the trigeminal neuropathy is a form of post. Unauthorized reproduction of this article is prohibited. tramadol. It is important to consider herpes zoster ophthalmicus in the differential diagnosis of new-onset unilateral frontal or temporal headache in the elderly patient.2 This pain can or famciclovir. gabapentin. Severely and topical capsaicin (Table 9-3). Also known as geniculate neuralgia. caring for a terminally ill family member. herpes zoster. Antiviral drugs. auditory canal triggered by swallowing. zoster and 66% effective for prevention cates that antiviral agents do not reduce of postherpetic neuralgia.43.40 strong evidence indi. pregabalin.

Oral or topical. fatigue. predictor) is unproven. nasopharyngitis B Opioids (eg. titrate based on pruritus. dry creatine kinase. insomnia. prolonged QT interval methadone. dyspnea. pruritus. (Level A rating requires at least two consistent Class I studies. depression. respiratory depression. ineffective or harmful (or established as useful/predictive or not useful/predictive) for the given condition in the specified population. myocardial infarction. hydrocodone. pain response somnolence. Stevens-Johnson 3 times/d fatigue. (Level C rating requires at least one Class II study or two consistent Class III studies. hypotension. 2) the magnitude of effect is large [relative rate improved outcome 95 and the lower limit of the confidence interval is 92]. Continuum (Minneap Minn) 2015. dry mouth. blurry vision. syndrome. headache. . dizziness heart 1081 Copyright © American Academy of Neurology. seizure. blistering application of 8% patch every 3 months a A = Established as effective. Myocardial infarction. angioedema ataxia.TABLE 9-3 Pharmacologic Treatment of Postherpetic Neuralgia Level of Common Adverse Serious Adverse Evidencea Drug Typical Dose Effectsb Effectsb A Lidocaine 5% 1Y3 patches Paresthesia or skin Systemic effects such as applied topically irritation at the hypotension or bradycardia for up to 12 out application site are unlikely with appropriate of 24 hours use of the patch Nortriptyline or 10Y75 mg at Constipation. increased 2 times/d gain. ataxia thoughts Pregabalin 75Y300 mg Peripheral edema.21(4):1072–1085 www. ineffective or harmful (or probably useful/predictive or not useful/predictive) for the given condition in the specified population. hyponatremia. nausea Hypertension. dizziness. 4 times/d constipation. one convincing Class I study may suffice for an ‘‘A’’ recommendation if 1) all criteria are met. given current knowledge. Confusion. (Level B rating requires at least one Class I study or two consistent Class II studies. dizziness. suicidal mouth. paralytic ileus. serotonin syndrome (when combined with other drugs that increase serotonin levels) Tramadol 25Y100 mg Flushing. Prolonged QT interval. Unauthorized reproduction of this article is prohibited. weight Hepatotoxicity. headache. increased intraocular pressure. urinary retention Gabapentin 100Y900 mg Peripheral edema. b Lists of potential adverse affects are incomplete and the prescribing physician should refer to the Physician’s Desk Reference or its equivalent for a complete list. (particularly for tremor methadone). nausea. hydromorphone. oxycodone. bone marrow depression. nausea. suicidal dizziness. somnolence serotonin syndrome C Capsaicin Single 1-hour Erythema.) B = Probably effective. pancreatitis.ContinuumJournal. amitriptyline bedtime weight gain. constipation. pain. Constipation. nausea. dizziness. peripheral edema. treatment (test. ineffective or harmful (or possibly useful predictive or not useful/predictive) for the given condition in the specified population. morphine.) U = Data inadequate or conflicting.) C = Possibly effective.) (In exceptional cases. disturbance in thinking. thoughts. anaphylaxis. drowsiness. fentanyl) and side effects difficulty swallowing. urinary retention.

OPTIC NEURITIS cant facial trauma or dental procedures Pain behind one or both eyes. perior orbital fissure. which is caused by demy- be very difficult.2 Clinical management can optic neuritis. idiopathic. .000 population. which may be superior orbital fissure. diffuse. anecdotal reports of HEADACHE WITH successful surgical treatments include OPHTHALMOPLEGIA transection of the nervus intermedius. and CSF analysis. superficial. Cranial Neuralgias KEY POINTS h Persistent idiopathic also extend to the auricle. cavern- regional.38 Nervus intermedius in middle-aged and elderly women.49 The granulo- absence of a clinical neurologic deficit. oral candidiasis. IV.2. accompa- logical comorbidity and psychosocial nied by impaired visual acuity may signal disability. Gabapentin. persistent idiopathic facial pain refers to and may be the presenting symptom of the clinical syndrome of continuous or neurosarcoidosis. facial mus. have brought improvement to some pa. syndrome. carotid dissection. and tricyclics ficiency. Diagnostic evaluation includes dilated scleroderma. Treatment is similar to that for August 2015 Copyright © American Academy of Neurology. sclerosis51 or neuromyelitis optica.2. men- poorly localized.52 such as mixed connective tissue disorder. su- Previously termed atypical facial pain. or Sjögren syn. although some patients have inflammation of the orbit. and diabetes mellitus. deep. or taste may occur. elination of the optic nerve. or ous carotid aneurysm. ocular motor (cranial nerve III. well to corticosteroids. Impaired lacrimation. diagnosis also includes vitamin B12 de- with ophthalmoplegia. salivation. (TOLOSA-HUNT SYNDROME) microvascular decompression of the Unilateral orbital pain accompanied by nervus intermedius.47 psychological comorbidity. Lubricating oral rinses may be helpful. chronic hyposalivation. BURNING MOUTH SYNDROME facial pain (formerly culature. lamotrigine. Cranial imaging is required when brain and orbits. or cavernous sinus. Optic neuri- The evaluation of persistent idiopathic tis can occur as an isolated syndrome facial pain should assess for a possible or as a manifestation of either multiple underlying connective tissue disorder. Unauthorized reproduction of this article is prohibited. etiologies include orbital tumors. and extracranial in. as well as to the palate and pain is burning mouth or burning tongue pain) may be difficult to tongue.46 for a structural lesion. or aching.48 MRI may disclose a daily recurring facial or oral pain in the focal enhancing mass. vasculitis. This condition occurs in 5 to treat because of central sopharyngeal neuralgia is not triggered 10 per 100. matous inflammation typically responds Its presentations are highly variable. The differential with unilateral orbital pain of treatment being carbamazepine. Other potential The pain may be unilateral or bilateral. which may and frequently is associated with psycho- be worse with eye movement. induced by anticholinergic medication or cavernous sinus presents geminal neuralgia. funduscopic examination. nagging. MRI of the drome. carotid-cavernous fistula. For patients in whom medical therapy has failed. with the first line or Sjögren syndrome.ContinuumJournal. and lichen planus.50 that is dull. which is caused by granulo- FACIAL PAIN matous inflammation in the orbit. This is the char- acteristic presentation of Tolosa-Hunt PERSISTENT IDIOPATHIC syndrome.45. or VI) fratemporal division of the cutaneous paresis should arouse strong suspicion branches of the facial nerve. most commonly sensitization and by swallowing. burning. tients. sarcoidosis. Op- focal neurologic deficits are found. Patients may report pain ingitis. but unlike the pain of glos. tical coherence tomography is useful to 1082 www. and over the parieto-occipital Related to persistent idiopathic facial termed atypical facial region. This pain may follow minor or insignifi. neuralgia may develop as a complication Burning mouth syndrome is frequently h Granulomatous of herpes zoster.

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