The International Classification of Headache Disorders 2nd Edition


CRANIAL NEURALGIAS AND CENTRAL CAUSES OF FACIAL PAIN



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13. CRANIAL NEURALGIAS AND CENTRAL CAUSES OF FACIAL PAIN


13.1 Trigeminal neuralgia

13.1.1 Classical trigeminal neuralgia

13.1.2 Symptomatic trigeminal neuralgia

13.2 Glossopharyngeal neuralgia

13.2.1 Classical glossopharyngeal neuralgia

13.2.2 Symptomatic glossopharyngeal neuralgia

13.3 Nervus intermedius neuralgia

13.4 Superior laryngeal neuralgia

13.5 Nasociliary neuralgia

13.6 Supraorbital neuralgia

13.7 Other terminal branch neuralgias

13.8 Occipital neuralgia

13.9 Neck-tongue syndrome

13.10 External compression headache

13.11 Cold-stimulus headache

13.11.1 Headache attributed to external application of a cold stimulus

13.11.2 Headache attributed to ingestion or inhalation of a cold stimulus

13.12 Constant pain caused by compression, irritation or distortion of cranial nerves or upper cervical roots by structural lesions

13.13 Optic neuritis

13.14 Ocular diabetic neuropathy

13.15 Head or facial pain attributed to herpes zoster

13.15.1 Head or facial pain attributed to acute herpes zoster

13.15.2 Post-herpetic neuralgia

13.16 Tolosa-Hunt syndrome

13.17 Ophthalmoplegic “migraine”

13.18 Central causes of facial pain

13.18.1 Anaesthesia dolorosa

13.18.2 Central post-stroke pain

13.18.3 Facial pain attributed to multiple sclerosis

13.18.4 Persistent idiopathic facial pain

13.18.5 Burning mouth syndrome

13.19 Other cranial neuralgia or other centrally mediated facial pain



Introduction


Pain in the head and neck is mediated by afferent fibres in the trigeminal nerve, nervus intermedius, glossopharyngeal and vagus nerves and the upper cervical roots via the occipital nerves. Stimulation of these nerves by compression, distortion, exposure to cold or other forms of irritation or by a lesion in central pathways may give rise to stabbing or constant pain felt in the area innervated.

The cause may be clear, such as infection by herpes zoster or a structural abnormality demonstrated by imaging, but in some cases there may be no cause apparent for neuralgic pain.

Trigeminal and glossopharyngeal neuralgias present a problem of terminology. When pain is found to result from compression of the nerve by a vascular loop at operation, the neuralgia should strictly be regarded as secondary. Since many patients do not come to operation, it remains uncertain as to whether they have primary or secondary neuralgias. For this reason the term classical rather than primary has been applied to those patients with a typical history even though a vascular source of compression may be discovered during its course. The term secondary can then be reserved for those patients in whom a neuroma or similar lesion is demonstrated.

Another difficulty arises with the condition that used to be known as atypical facial pain (an inappropriate term since many cases conform to a pattern). The fact that some cases follow surgery or injury to the face, teeth or gums suggests the possibility of an infectious or traumatic cause. Until more is known of the condition, persistent idiopathic facial pain seems a preferable non-committal title.


13.1 Trigeminal neuralgia

Previously used term:

Tic douloureux

13.1.1 Classical trigeminal neuralgia

Description:

Trigeminal neuralgia is a unilateral disorder characterised by brief electric shock-like pains, abrupt in onset and termination, limited to the distribution of one or more divisions of the trigeminal nerve. Pain is commonly evoked by trivial stimuli including washing, shaving, smoking, talking and/or brushing the teeth (trigger factors) and frequently occurs spontaneously. Small areas in the nasolabial fold and/or chin may be particularly susceptible to the precipitation of pain (trigger areas). The pains usually remit for variable periods.
Diagnostic criteria:

A. Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, affecting one or more divisions of the trigeminal nerve and fulfilling criteria B and C

B. Pain has at least one of the following characteristics:

1. intense, sharp, superficial or stabbing

2. precipitated from trigger areas or by trigger factors

C. Attacks are stereotyped in the individual patient

D. There is no clinically evident neurological deficit

E. Not attributed to another disorder

Comments:

Classical trigeminal neuralgia usually starts in the second or third divisions, affecting the cheek or the chin. In <5% of patients the first division is affected. The pain never crosses to the opposite side but it may rarely occur bilaterally, in which case a central cause such as multiple sclerosis must be considered. Between paroxysms the patient is usually asymptomatic but a dull background pain may persist in some long-standing cases. Following a painful paroxysm there is usually a refractory period during which pain cannot be triggered. In some cases a paroxysm may be triggered from somatosensory stimuli outside the trigeminal area, such as a limb, or by other sensory stimulation such as bright lights, loud noises or tastes.

The pain often evokes spasm of the muscle of the face on the affected side (tic douloureux).

The increasing frequency of posterior fossa exploration and magnetic resonance imaging has demonstrated that many, possibly most, patients with this condition have compression of the trigeminal root by tortuous or aberrant vessels.

Classical trigeminal neuralgia is usually responsive, at least initially, to pharmacotherapy.


13.1.2 Symptomatic trigeminal neuralgia

Description:

Pain indistinguishable from 13.1.1 Classical trigeminal neuralgia but caused by a demonstrable structural lesion other than vascular compression.
Diagnostic criteria:

A. Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, with or without persistence of aching between paroxysms, affecting one or more divisions of the trigeminal nerve and fulfilling criteria B and C

B. Pain has at least one of the following characteristics:

1. intense, sharp, superficial or stabbing

2. precipitated from trigger areas or by trigger factors

C. Attacks are stereotyped in the individual patient

D. A causative lesion, other than vascular compression, has been demonstrated by special investigations and/or posterior fossa exploration



Comment:

There may be sensory impairment in the distribution of the appropriate trigeminal division. 13.1.2 Symptomatic trigeminal neuralgia demonstrates no refractory period after a paroxysm, unlike 13.1.1 Classical trigeminal neuralgia.

13.2 Glossopharyngeal neuralgia

13.2.1. Classical glossopharyngeal neuralgia

Description:

Glossopharyngeal neuralgia is a severe transient stabbing pain experienced in the ear, base of the tongue, tonsillar fossa or beneath the angle of the jaw. The pain is therefore felt in the distributions of the auricular and pharyngeal branches of the vagus nerve as well as of the glossopharyngeal nerve. It is commonly provoked by swallowing, talking or coughing and may remit and relapse in the fashion of trigeminal neuralgia.
Diagnostic criteria:

A. Paroxysmal attacks of facial pain lasting from a fraction of a second to 2 minutes and fulfilling criteria B and C

B. Pain has all of the following characteristics:

1. unilateral location

2. distribution within the posterior part of the tongue, tonsillar fossa, pharynx or beneath the angle of the lower jaw and/or in the ear

3. sharp, stabbing and severe

4. precipitated by swallowing, chewing, talking, coughing and/or yawning

C. Attacks are stereotyped in the individual patient

D. There is no clinically evident neurological deficit

E. Not attributed to another disorder1

Note:

1. Other causes have been ruled out by history, physical examination and/or special investigations.

13.2.2 Symptomatic glossopharyngeal neuralgia

Description:

Pain as in 13.2.1 Classical glossopharyngeal neuralgia with the proviso that aching pain may persist between paroxysms and sensory impairment may be found in the distribution of the glossopharyngeal nerve.
Diagnostic criteria:

A. Paroxysmal attacks of facial pain lasting from a fraction of a second to 2 minutes, with or without persistence of aching between paroxysms, and fulfilling criteria B and C

B. Pain has all of the following characteristics:

1. unilateral location

2. distribution within the posterior part of the tongue, tonsillar fossa, pharynx or beneath the angle of the lower jaw and/or in the ear

3. sharp, stabbing and severe

4. precipitated by swallowing, chewing, talking, coughing and/or yawning

C. Attacks are stereotyped in the individual patient

D. A causative lesion has been demonstrated by special investigations and/or surgery



13.3 Nervus intermedius neuralgia

Description:

A rare disorder characterised by brief paroxysms of pain felt deeply in the auditory canal.
Diagnostic criteria:

A. Pain paroxysms of intermittent occurrence, lasting for seconds or minutes, in the depth of the ear

B. Presence of a trigger area in the posterior wall of the auditory canal

C. Not attributed to another disorder1

Note:

1. Other causes, in particular a structural lesion, have been ruled out by history, physical examination and special investigations.
Comment:

Disorders of lacrimation, salivation and/or taste sometimes accompany the pain. There is a common association with herpes zoster. In view of the sparse innervation of the affected area by the nervus intermedius some patients may have an otalgic variant of glossopharyngeal neuralgia.

13.4 Superior laryngeal neuralgia

Description:

A rare disorder characterised by severe pain in the lateral aspect of the throat, submandibular region and underneath the ear, precipitated by swallowing, shouting or turning the head.
Diagnostic criteria:

A. Pain paroxysms lasting for seconds or minutes in the throat, submandibular region and/or under the ear and fulfilling criteria B-D

B. Paroxysms are triggered by swallowing, straining the voice or head turning

C. A trigger point is present on the lateral aspect of the throat overlying the hypothyroid membrane

D. The condition is relieved by local anaesthetic block and cured by section of the superior laryngeal nerve

E. Not attributed to another disorder1

Note:

1. Other causes, in particular a structural lesion, have been ruled out by history, physical examination and special investigations.

13.5 Nasociliary neuralgia

Previously used term:

Charlin’s neuralgia
Description:

A rare condition in which touching the outer aspect of one nostril causes a lancinating pain radiating to the medial frontal region.
Diagnostic criteria:

A. Stabbing pain lasting seconds to hours in one side of the nose, radiating upwards to the medial frontal region and fulfilling criteria B and C

B. Pain is precipitated by touching the lateral aspect of the ipsilateral nostril

C. Pain is abolished by block or section of the nasociliary nerve, or by the application of cocaine to the nostril on the affected side

13.6 Supraorbital neuralgia

Description:

An uncommon disorder characterised by pain in the region of the supraorbital notch and medial aspect of the forehead in the area supplied by the supraorbital nerve.
Diagnostic criteria:

A. Paroxysmal or constant pain in the region of the supraorbital notch and medial aspect of the forehead in the area supplied by the supraorbital nerve

B. Tenderness over the nerve in the supraorbital notch

C. Pain is abolished by local anaesthetic blockade or ablation of the supraorbital nerve

13.7 Other terminal branch neuralgias

Description:

Injury or entrapment of peripheral branches of the trigeminal nerve other than the nasociliary and supraorbital nerves may give rise to pain referred to the area innervated by the branch affected. Examples are neuralgias of the infraorbital, lingual, alveolar and mental nerves.
Diagnostic criteria:

A. Pain in the distribution of a peripheral branch of the trigeminal nerve other than the nasociliary or supraorbital nerves

B. Tenderness over the affected nerve

C. Pain is abolished by local anaesthetic blockade or ablation of the nerve

Comment:

A13.7.1 Nummular headache, described in the appendix, is probably a localised terminal branch neuralgia of the trigeminal nerve.

13.8 Occipital neuralgia

Description:

Occipital neuralgia is a paroxysmal jabbing pain in the distribution of the greater or lesser occipital nerves or of the third occipital nerve, sometimes accompanied by diminished sensation or dysaesthesia in the affected area. It is commonly associated with tenderness over the nerve concerned.
Diagnostic criteria:

A. Paroxysmal stabbing pain, with or without persistent aching between paroxysms, in the distribution(s) of the greater, lesser and/or third occipital nerves

B. Tenderness over the affected nerve

C. Pain is eased temporarily by local anaesthetic block of the nerve

Comment:

Occipital neuralgia must be distinguished from occipital referral of pain from the atlantoaxial or upper zygapophyseal joints or from tender trigger points in neck muscles or their insertions.

13.9 Neck-tongue syndrome

Description:

The sudden onset of pain in the occiput or upper neck associated with abnormal sensation in the same side of the tongue.
Diagnostic criteria:

A. Pain lasting seconds or minutes, with or without simultaneous dysaesthesia, in the area of distribution of the lingual nerve and second cervical root and fulfilling criteria B and C

B. Pain has acute onset

C. Pain is commonly precipitated by sudden turning of the head

Comment:

Proprioceptive fibres from the tongue enter the central nervous system through the second cervical dorsal root via connections between lingual and hypoglossal nerves and between the latter and the second cervical root. There is clinical and surgical evidence that the C2 root is compromised by sudden rotation of the neck, which is particularly likely when subluxation of the atlantoaxial joint occurs. The abnormal sensation in the ipsilateral side of the tongue may be numbness, paraesthesia or the sensation of involuntary movement.

13.10 External compression headache

Description:

Headache resulting from continued stimulation of cutaneous nerves by the application of pressure, for example by a band around the head, a tight hat or goggles worn to protect the eyes during swimming.
Diagnostic criteria:

A. Headache with all of the following characteristics and fulfilling criteria C and D:

1. non-pulsating

2. increasing over minutes

3. no accompanying symptoms

B. Continuing application of external pressure to the forehead or scalp

C. Headache develops during and is maximal at the site of pressure

D. Headache resolves after pressure is relieved

Comment:

External compression may lead to a more severe migrainous headache if the stimulus is prolonged.

13.11 Cold-stimulus headache

13.11.1 Headache attributed to external application of a cold stimulus

Description:

Generalised headache following exposure of the unprotected head to a low environmental temperature as in very cold weather or in diving into cold water.
Diagnostic criteria:

A. Diffuse and/or non-pulsating headache fulfilling criteria C and D:

B. Presence of external cold stimulus to the head

C. Headache develops during cold stimulus

D. Headache resolves after removal of cold stimulus



13.11.2 Headache attributed to ingestion or inhalation of a cold stimulus

Previously used term:

Ice-cream headache
Description:

Short-lasting pain, which may be severe, induced in susceptible individuals by the passage of cold material (solid, liquid or gaseous) over the palate and/or posterior pharyngeal wall.
Diagnostic criteria:

A. Acute frontal1 non-pulsatile headache fulfilling criteria C and D

B. Cold stimulus to palate and/or posterior pharyngeal wall due to ingestion of cold food or drink or to inhalation of cold air

C. Headache develops immediately, and only, after cold stimulus

D. Headache resolves within 5 minutes after removal of cold stimulus



Note:

1. In migrainous patients, the headache may be referred to the usual site of migraine headache.

13.12 Constant pain caused by compression, irritation or distortion of cranial nerves or upper cervical roots by structural lesions

Description:

Constant headache or facial pain caused by a lesion directly compromising afferent fibres in nerves mediating pain sensation from the head and/or neck. Sensory deficit may be detected within the appropriate distribution.
Diagnostic criteria:

A. Constant and/or jabbing pain in the territory supplied by a cranial sensory nerve, fulfilling criteria C and D

B. Evidence of compression, irritation or distortion of the appropriate cranial nerve

C. Pain and compression, irritation or distortion occur simultaneously and correspond in location

D. Pain is relieved by removal of the cause of compression, irritation or distortion



Comments:

Structural lesions may be space-occupying (eg, tumour or aneurysm) or contained within anatomical boundaries (eg, osteomyelitis of the cranial bones). If there is no sensory deficit or supporting imaging evidence, the diagnosis is doubtful.

Facial pain around the ear or temple may result from invasion of the vagus nerve by lung carcinoma.


13.13 Optic neuritis

Description:

Pain behind one or both eyes accompanied by impairment of central vision caused by demyelination of the optic nerve.
Diagnostic criteria:

A. Dull pain behind one or both eyes, worsened by eye movement and fulfilling criteria C and D

B. Visual impairment due to a central or paracentral scotoma

C. Onset of pain and onset of visual impairment separated by <4 weeks1

D. Pain resolves within 4 weeks

E. A compressive lesion has been ruled out

Note:

1. Pain precedes impairment of vision by <4 weeks. During this time, criterion B is not fulfilled and the diagnosis is Probable optic neuritis.
Comments:

Vision usually improves within 4 weeks.

Optic neuritis is often a manifestation of multiple sclerosis.


13.14 Ocular diabetic neuropathy

Description:

Pain around the eye and forehead associated with paresis of one or more ocular cranial nerves (usually the third cranial nerve) in a patient with diabetes mellitus.
Diagnostic criteria:

A. Pain, in a patient with diabetes mellitus, developing over a few hours around one eye

B. Third cranial nerve palsy, often with sparing of pupillary function, and/or paresis of the fourth and/or sixth cranial nerves

C. Neuropathy develops within 7 days of onset of pain1

D. Not attributed to another disorder



Note:

1. Pain precedes signs of neuropathy by <7 days. During this time, criterion B is not fulfilled and the diagnosis is Probable ocular diabetic neuropathy.

13.15 Head or facial pain attributed to herpes zoster

13.15.1 Head or facial pain attributed to acute herpes zoster

Description:

Head or facial pain caused by herpes zoster.
Diagnostic criteria:

A. Head or facial pain in the distribution of a nerve or nerve division and fulfilling criteria C and D

B. Herpetic eruption in the territory of the same nerve

C. Pain precedes herpetic eruption by <7 days1

D. Pain resolves within 3 months



Note:

1. Pain precedes herpetic eruption by <7 days. During this time, criterion B is not fulfilled and the diagnosis is Head or facial pain probably attributed to acute herpes zoster.
Comments:

Herpes zoster affects the trigeminal ganglion in 10-15% of patients with the disease, and the ophthalmic division is singled out in some 80% of those patients. Herpes zoster may also involve the geniculate ganglion, causing an eruption in the external auditory meatus. The soft palate or areas of distribution of upper cervical roots may be involved in some patients.

Ophthalmic herpes may be associated with third, fourth and/or sixth cranial nerve palsies and geniculate herpes with facial palsy and/or acoustic symptoms. Zoster occurs in about 10% of patients with lymphoma and 25% of patients with Hodgkin’s disease.


13.15.2 Post-herpetic neuralgia

Description:

Facial pain persisting or recurring 3 months after the onset of herpes zoster.
Diagnostic criteria:

A. Head or facial pain in the distribution of a nerve or nerve division

B. Herpetic eruption in the territory of the same nerve

C. Pain preceded herpetic eruption by <7 days

D. Pain persists after 3 months



Comment:

Post-herpetic neuralgia is more often a sequel of herpes zoster as age advances, afflicting 50% of patients contracting zoster over the age of 60 years. Hypaesthesia or hyperalgesia and/or allodynia are usually present in the territory involved.

13.16 Tolosa-Hunt syndrome

Description:

Episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but tends to relapse and remit.
Diagnostic criteria:

A. One or more episodes of unilateral orbital pain persisting for weeks if untreated

B. Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granuloma by MRI or biopsy

C. Paresis coincides with the onset of pain or follows it within 2 weeks

D. Pain and paresis resolve within 72 hours when treated adequately with corticosteroids

E. Other causes have been excluded by appropriate investigations1

Note:

1. Other causes of painful ophthalmoplegia include tumours, vasculitis, basal meningitis, sarcoid, diabetes mellitus and ophthalmoplegic “migraine”.
Comments:

Some reported cases of Tolosa-Hunt syndrome had additional involvement of the trigeminal nerve (commonly the first division) or optic, facial or acoustic nerves. Sympathetic innervation of the pupil is occasionally affected.

The syndrome has been caused by granulomatous material in the cavernous sinus, superior orbital fissure or orbit in some biopsied cases.

Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia.

13.17 Ophthalmoplegic “migraine”

Description:

Recurrent attacks of headache with migrainous characteristics associated with paresis of one or more ocular cranial nerves (commonly the third nerve) in the absence of any demonstrable intracranial lesion other than MRI changes within the affected nerve.
Diagnostic criteria:

A. At least 2 attacks fulfilling criterion B

B. Migraine-like headache accompanied or followed within 4 days of its onset by paresis of one or more of the third, fourth and/or sixth cranial nerves

C. Parasellar, orbital fissure and posterior fossa lesions ruled out by appropriate investigations

Comment:

This condition is very rare. It is unlikely that 13.17 Ophthalmoplegic “migraine” is a variant of migraine since the headache often lasts for a week or more and there is a latent period of up to 4 days from the onset of headache to the onset of ophthalmoplegia. Furthermore, in some cases MRI shows gadolinium uptake in the cisternal part of the affected cranial nerve which suggests that the condition may be a recurrent demyelinating neuropathy.

13.18 Central causes of facial pain

13.18.1 Anaesthesia dolorosa

Description:

Persistent and painful anaesthesia or hypaesthesia in the distribution of the trigeminal nerve or one of its divisions or of the occipital nerves.
Diagnostic criteria:

A. Persistent pain and dysaesthesia within the area of distribution of one or more divisions of the trigeminal nerve or of the occipital nerves

B. Diminished sensation to pin-prick and sometimes other sensory loss over the affected area

C. There is a lesion of the relevant nerve or its central connections

Comment:

Anaesthesia dolorosa is often related to surgical trauma of the occipital nerves or trigeminal ganglion, evoked most frequently after rhizotomy or thermocoagulation has been performed for treatment of 13.1.1 Classical trigeminal neuralgia.

13.18.2 Central post-stroke pain

Description:

Unilateral pain and dysaesthesia associated with impaired sensation involving part or the whole of the face, not explicable by a lesion of the trigeminal nerve. It is attributed to a lesion of the quintothalamic (trigeminothalamic) pathway, thalamus or thalamocortical projection. Symptoms may also involve the trunk and/or limbs of the affected or contralateral side.
Diagnostic criteria:

A. Pain and dysaesthesia in one half of the face, associated with loss of sensation to pin-prick, temperature and/or touch, fulfilling criteria C and D

B. One or both of the following:

1. history of sudden onset suggesting a vascular lesion (stroke)

2. demonstration by CT or MRI of a vascular lesion in an appropriate site

C. Pain and dysaesthesia develop within 6 months after stroke

D. Not explicable by a lesion of the trigeminal nerve



Comment:

Facial pain following a thalamic lesion is part of a hemisyndrome. With lateral medullary lesions hemifacial pain may occur in isolation, but it is more often accompanied by crossed hemidysaesthesia.

The pain and dysaesthesia are usually persistent.


13.18.3 Facial pain attributed to multiple sclerosis

Coded elsewhere:

Pain attributed to optic neuritis occurring as a manifestation of multiple sclerosis is coded as 13.13 Optic neuritis.
Description:

Unilateral or bilateral facial pain, with or without dysaesthesia, attributed to a demyelinating lesion of the central connections of the trigeminal nerve, which commonly remits and relapses.
Diagnostic criteria:

A. Pain, with or without dysaesthesia, in one or both sides of the face

B. Evidence that the patient has multiple sclerosis

C. Pain and dysaesthesia develop in close temporal relation to, and with MRI demonstration of, a demyelinating lesion in the pons or quintothalamic (trigeminothalamic) pathway

D. Other causes have been ruled out



Comment:

Pain may be tic-like, as in 13.1 Trigeminal neuralgia, or continuous. Trigeminal neuralgia occurring in young people or affecting one and then the other side should arouse the suspicion of multiple sclerosis.

13.18.4 Persistent idiopathic facial pain

Previously used term:

Atypical facial pain
Description:

Persistent facial pain that does not have the characteristics of the cranial neuralgias described above and is not attributed to another disorder.
Diagnostic criteria:

A. Pain in the face, present daily and persisting for all or most of the day, fulfilling criteria B and C

B. Pain is confined at onset to a limited area on one side of the face1, and is deep and poorly localised

C. Pain is not associated with sensory loss or other physical signs

D. Investigations including x-ray of face and jaws do not demonstrate any relevant abnormality



Note:

1. Pain at onset is commonly in the nasolabial fold or side of the chin, and may spread to the upper or lower jaw or a wider area of the face and neck.
Comments:

Pain may be initiated by surgery or injury to the face, teeth or gums but persists without any demonstrable local cause.

Facial pain around the ear or temple may precede the detection of an ipsilateral lung carcinoma causing referred pain by invasion of the vagus nerve.

The term atypical odontalgia has been applied to a continuous pain in the teeth or in a tooth socket after extraction in the absence of any identifiable dental cause.

13.18.5 Burning mouth syndrome

Description:

An intraoral burning sensation for which no medical or dental cause can be found.
Diagnostic criteria:

A. Pain in the mouth present daily and persisting for most of the day

B. Oral mucosa is of normal appearance

C. Local and systemic diseases have been excluded

Comment:

Pain may be confined to the tongue (glossodynia). Subjective dryness of the mouth, paraesthesia and altered taste may be associated symptoms.

13.19 Other cranial neuralgia or other centrally-mediated facial pain


Vail’s Vidian neuralgia and Sluder’s sphenopalatine neuralgia are not sufficiently validated. The recognition of Eagle’s syndrome (Montalbetti et al, 1995) as a distinct entity awaits clarification.

Bibliography and reference

13.1 Trigeminal neuralgia

Barker FG II, Jannetta PJ, Bissonette DJ, Larkins MV, Jho HD. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med 1997; 334: 1077-1083.

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Zakrzewska JM. Trigeminal neuralgia. In: Zakrzewska JM, Harrison SD, eds. Assessment and management of orofacial pain. Pain Research and Clinical Management, Amsterdam, Elsevier 2002; 14: 263-366.



13.2 Glossopharyngeal neuralgia

Ekbom KA, Westerberg CE. Carbamazepine in glossopharyngeal neuralgia. Arch Neurol 1966; 14: 595-596.

Laha RK, Jannetta PJ. Glossopharyngeal neuralgia. J Neurosurg 1977; 47: 316-320.

Minagor A, Sheremata WA. Glossopharyngeal neuralgia and MS. Neurology 2000; 54: 1368-1370.

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13.3 Nervus intermedius neuralgia

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13.4 Superior laryngeal neuralgia

Bruyn GW. Superior laryngeal neuralgia. In : Rose FC ed. Headache. Handbook of Clinical Neurology. Amsterdam : Elsevier 1986; 4(48): 495-500.

13.5 Nasociliary neuralgia

Bruyn GW. Charlin’s neuralgia. In: Rose FC ed. Headache. Handbook of Clinical Neurology, Amsterdam : Elsevier 1986; 4(48): 483-486.

13.6 Supraorbital neuralgia

Sjaastad O, Stolt-Nielsen A, Pareja JA, Vincent M. Supraorbital neuralgia. The clinical manifestation and a possible therapeutic approach. Headache 1999; 39: 204-212.

13.7 Infraorbital neuralgia

de Vries N, Smelt WL. Local anaesthetic block of posttraumatic neuralgia of the infraorbital nerve. Rhinology 1990; 28: 103-106.

13.8 Occipital neuralgia

Bogduk N. Greater occipital neuralgia. In : Long DM ed. Current Therapy in Neurological Surgery. Toronto, Philadelphia : BC Decker Inc 1985: 175-180.

Hammond SR, Danta A. Occipital neuralgia. Clin Exp Neurol 1978; 15: 258-270.



13.9 Neck-tongue syndrome

Bertoft ES, Westerberg CE. Further observations on the neck-tongue syndrome. Cephalalgia 1985; 5 (Suppl 3): 312-313.

Bogduk N. An anatomical explanation for the neck-tongue syndrome. J Neurol Neurosurg Psychiat 1981; 44: 202-208.

Lance JW, Anthony M. Neck-tongue syndrome on sudden turning of the head. J Neurol Neurosurg Psychiat 1980; 43: 97-101.

13.10 External compression headache

Pestronk A, Pestronk S. Goggle migraine. N Engl J Med 1983; 308: 226.

13.11 Cold stimulus headache

Drummond PD, Lance JW. Neurovascular disturbances in headache patients. Clin Exp Neurol 1984; 20: 93-99.

Odell-Smith R. Ice cream headache. In: Vinken PJ, Bruyn GW eds Handbook of Clinical Neurology. Amsterdam : Elsevier 1968; 5: 188-191.

Raskin NH. Ice cream, ice-pick and chemical headaches. In: Rose FC ed Headache. Handbook of Clinical Neurology. Amsterdam: Elsevier 1986; 4 (48): 441-448.

Raskin NH, Knittle SC. Ice cream headache and orthostatic symptoms in patients with migraine. Headache 1976; 16: 222-225.

Wolf S, Hardy JD. Studies on pain. Observations on pain due to local cooling and on factors involved in the “cold pressor” effect. J Clin Invest 1941; 20: 521-533.

13.12 Constant pain caused by compression, irritation or distortion of cranial nerves

Adams RD, Victor M, Ropper AH. Principles of Neurology 6th ed. New York : McGraw-Hill 1997: 187-193, 1370-85.

Capobianco DJ. Facial pain as a symptom of nonmetastatic lung cancer. Headache 1995; 35: 581-585.

Dalessio DJ ed. Wolff's Headache and other head pain. 6th edn New York : Oxford University Press 1993: 345-364.

Mokri B. Raeder's paratrigeminal syndrome. Arch Neurol 1982; 39: 395-399.

Schoenen J, Broux R, Moonen G. Unilateral facial pain as the first symptom of lung cancer : are there diagnostic clues? Cephalalgia 1992; 12: 178-9.

13.13 Optic neuritis

Adams RD, Victor M, Ropper AH. Principles of Neurology 6th edn. New York, McGraw-Hill 1997: 910-911.

Kaufman DI, Trobe JD, Eggenberger ER, Whitaker JN. Practice parameter : the role of corticosteroids in the management of acute monosymptomatic optic neuritis. Neurology 2000; 54: 2039-2044.

Shults WT. Diseases of the visual pathways I. In Swash M, Oxbury J, eds. Clinical Neurology. Edinburgh, Churchill Livingstone 1991: 410-413.

13.14 Ocular diabetic neuropathy

Kennard C. Disorders of eye movements I. In Swash M, Oxbury J, eds. Clinical Neurology. Edinburgh. Churchill Livingstone 1991: 446-447.

13.15 Head or facial pain attributed to herpes zoster

Bowsher D. The management of postherpetic neuralgia. Postgrad Med J 1997; 73: 623-9.

Dworkin RH, Portenoy RK. Pain and its persistence in herpes zoster. Pain 1996; 67: 241-252.

Ragozzino MW, Melton LJ, Kerland LT, Chu CP, Perry HO. Population-based study of Herpes Zoster and its sequelae. Medicine 1982; 61: 310-316.

13.16 Tolosa-Hunt syndrome

de Arcaya AA, Cerezal L, Canga A, Polo JM, Berciano J, Pascual J. Neuroimaging diagnosis of Tolosa-Hunt syndrome. MRI contribution. Headache 1999; 39: 321-325.

Forderreuther S, Straube A. The criteria of the International Headache Society for Tolosa-Hunt syndrome need to be revised. J Neurol 1999 ; 246: 371-377.

Goadsby PJ, Lance JW. Clinicopathological correlation in a case of painful ophthalmoplegia : Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiat 1989 ; 52: 1290-1293.

Hannerz J. Pain characteristics of painful ophthalmoplegia (the Tolosa-Hunt syndrome). Cephalalgia 1985 ; 5: 103-106.

Hunt WE. Tolosa-Hunt syndrome : one cause of painful ophthalmoplegia. J Neurosurg 1976; 44: 544-549.

Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954; 17: 300-302.



13.17 Ophthalmoplegic “migraine”

Lance JW, Zagami AS. Ophthalmoplegic migraine : a recurrent demyelinating neuropathy? Cephalalgia 2001 ; 21: 84-89.

Mark AS, Casselman J, Brown D, Sanchez J, Kolsky M, Larsen TC III, Lavin P, Ferraraccio B. Ophthalmoplegic migraine : reversible enhancement and thickening of the cisternal segment of the oculomotor nerve on contrast-enhanced MR images. Am J Neuroradiol 1998 ; 19: 1887-1891.



13.18.1 Anaesthesia dolorosa

Illingworth R. Trigeminal neuralgia : surgical aspects. In : Rose RD ed Headache. Handbook of Clinical Neurology. Amsterdam : Elsevier 1986 ; 4 (48): 449-458.

Pagni CA. Central and painful anaesthesia. Pathophysiology and treatment of sensory deprivation syndromes due to central and peripheral nervous system lesions. Progr Neurol Surg 1977; 2: 132-257.



13.18.2 Central post-stroke pain

Bowsher D. Central pain : clinical and physiological characteristics. J Neurol Neurosurg Psychiat 1996; 61: 62-9.

Bowsher D, Leijon G, Thuomas KA. Central poststroke pain. Correlation of MRI with clinical pain characteristics and sensory abnormalities. Neurology 1998; 51: 1352-8.

Fitzek S, Baumgartner U, Fitzek C et al. Mechanisms and Predictors of chronic facial pain in lateral medullary infarction. Ann Neurol 2001; 49: 493-500.

13.18.3 Facial pain attributed to multiple sclerosis

Jensen TS, Rasmussen P, Reske-Nielsen E. Association of trigeminal neuralgia with multiple sclerosis: clinical pathological features. Acta Neurol Scand 1982; 65: 182-189.

13.18.4 Persistent idiopathic facial pain

Boivie J, Casey KL. Central pain in the face and head. In Olesen J, Tfelt-Hansen P, Welch KMA eds. The Headaches, 2nd ed. Philadelphia, Lippincott, Williams & Wilkins 2000: 939-945.

Gouda JJ, Brown JA. Atypical facial pain and other pain syndromes. Differential diagnosis and treatment. Neurosurgery Clinics of North America 1997; 1: 87-99.

Harrison SD. Atypical facial pain and atypical odontalgia in Zakrzewska JM, Harrison SD, eds. Assessment and management of orofacial pain. Pain Research and Clinical Management, Amsterdam : Elsevier 2002; 14: 251-262.

13.18.5 Burning mouth syndrome

Zakrzewska J. Burning mouth. In Zakrzewska JM, Harrison SD, eds. Assessment and management of orofacial pain. Pain Research and Clinical Management. Amsterdam. Elsevier 2002; 14: 367-380.

13.19 Other cranial neuralgia or other centrally mediated facial pain

Montalbetti L, Ferrandi D, Pergami P, Savoldi F. Elongated styloid process and Eagle’s syndrome. Cephalalgia 1995; 15: 80-93.




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