General Information
• Cluster Headache
• Geniculate Neuralgia
• Spheno-
palatine Neuralgia

• Paratrigeminal Neuralgia of

• Anesthesia Dolorosa
• Atypical Facial

• Occipital

• Tinnitus and

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     The other disorders discussed here include a variety of cranio-facial pain disorders or cephalic neuralgias. They are often named according to the nerve responsible for the pain. Examples discussed in this section are Cluster Headache (also known as Petrosal Neuralgia or Migrainous Neuralgia), Geniculate Neuralgia, Sphenopalatine Neuralgia (Sluder's Neuralgia), Paratrigeminal Neuralgia of Raeder and Occipital Neuralgia. Other facial pain disorders include Anesthesia Dolorosa and Atypical Facial Pain.

     Other cranial nerve disorders include balance difficulty or dizziness (Vertigo) and ringing in the ears (Tinnitus).

Migrainous Neuralgia (Cluster Headache)

     Cluster headache is characterized by severe pain on one side of the face in the frontal and/or eye (retro-orbital) regions. The pain, described as stabbing or shooting, lasts anywhere from a few minutes to hours, and may awake the sufferer from sleep. These attacks are usually "clustered" over several days to weeks and are followed by remissions lasting weeks to months.

     Men are more often affected than women (at a ratio of 8:1, men to women), and the most usual age of onset is in the 20's or 30's. Associated symptoms include involvement of the autonomic system. During or following an attack, sufferers may experience tearing, nasal stuffiness or running, facial swelling or flushing, and eyelid edema, all on the affected side. A diagnosis of cluster headache is based upon normal findings in radiological and laboratory tests including CT and MRI scans.

     Other names for cluster headache include migrainous neuralgia, petrosal neuralgia, Horton's histamine cephalgia, red migraine, erythromelalgia of the head and paroxysmal nocturnal cephalgia. About one-fifth of sufferers develop daily and relentless symptoms in a condition known as chronic cluster headache. A rare variation of the disease includes symptoms of trigeminal neuralgia in what is termed cluster-tic syndrome.

Treatments: In the majority of sufferers medical therapy is the most effective treatment for Cluster Headache. The mainstay of medical treatments include methysergid, Ergotamine, Verapamil, Flunarizine, Valproic acid, and Lithium carbonate. Corticosteroids (including Prednisone) can also control cluster headache, and generally take affect within a few days. Treatment of newly diagnosed cluster headache may be initiated with a prescription of subcutaneous Sumatriptine. Oxygen may also be used to control acute attacks of pain, and is effective in two-thirds of sufferers.

     However, approximately one-tenth of sufferers develop intolerable side effects or severe pain refractory to medications. In such cases a number of surgical procedures may be performed, most of which are directed at the trigeminal nerve and/or nervus intermedius (geniculate systems). Destructive interventions that involve harming the petrosal nerve, include percutaneous radiofrequency or glycerol rhizotomy. These surgeries are identical to those for trigeminal neuralgia. Between 50 and 70 percent achieve immediate pain relief and 10 to 30 percent are capable of improved pain control following radiofrequency rhizotomy.

     Alliteratively, microvascular decompression of the trigeminal nerve with or without partial cutting of the nervus intermedius offers a non-destructive surgical treatment for cluster headache. Approximately half of cluster headache sufferers attain good long-term control of their headache following this procedure.

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Geniculate Neuralgia

     Geniculate Neuralgia (GN), also called nervus intermedius neuralgia, is a rare disorder that involves severe pain deep in the ear, that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.

     The pain of GN is sharp, shooting or burning and can last for hours. Painful attacks can be triggered by cold, noise, swallowing or touch, but triggers are usually very unique to the sufferer. Other related symptoms that may be experienced include increased salivation, bitter taste, tinnitus and vertigo. This disorder usually occurs in young to middle-aged adults, and more commonly in women.

     Geniculate Neuralgia may be caused by neurovascular compression of the fifth, ninth and/or tenth cranial nerves. In sufferers of GN, signals sent along these nerves are altered and interpreted by the geniculate ganglion (a structure in the brain) as GN pain. GN may also develop following herpes zoster oticus (Ramsey Hunt syndrome), where cold sores occur on the ear drum or ear. This may also be associated with facial paresis (weakness), tinnitus, vertigo and deafness.

Treatments: GN usually cannot be treated with medications. A variety of surgeries have been performed including microvascular decompression of the fifth, ninth, and tenth nerves, as well as partial cutting of the nervus intermedius, geniculate ganglion, chorda tympani and/or the ninth and tenth cranial nerves.

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Sphenopalatine Neuralgia

     Also known as Sluder's neuralgia, this facial pain disorder is characterized by unilateral headache behind the eyes with pain in the upper jaw or soft palate, with occasional aching in the back of the nose, the teeth, the temple, the occiput, or the neck. The pain is associated with nasal and/or sinus congestion, swelling or redness of nasal mucous membranes, tearing and redness of the face. Sphenopalatine neuralgia must be distinguished from cluster headache, although both are characterized by similar symptoms. Sluder's neuralgia, however, involves pain that is longer in duration, with inflamed nasal mucosa on the involved side. This disorder is more common in women (2:1, women to men) and appears to be caused by an irritation of the sphenopalatine ganglion from intranasal infection, deformity or scarring.

Treatment: Medical therapy for sinus decongestion can alleviate symptoms. Ganglion blocks are also effective for pain control, either by intranasal application or direct injection. The underlying cause of Sluder's neuralgia can also be targeted if apparent.

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Paratrigeminal Neuralgia of Raeder

     This syndrome consists of headaches in the upper face associated with eye and skin changes (oculosympathetic palsy) on one side of the face. The pain is described as intense or throbbing, and there may also be drooping of the eyelid and contraction of the pupil (miosis). Symptoms are frequent in the morning, and attacks can last between a few days and a few weeks. Some sufferers experience an unpleasant taste (dysageusia), possibly due to involvement the chorda tympani (a branch of the VII cranial nerve).

     Paratrigeminal neuralgia of Raeder is separated into two groups. Sufferers with symptomatic paratrigeminal neuralgia usually have parasellar cranial nerve defects, including tumors and lesions, and a neuropathic quality of pain. The migranous form with oclulosympathetic palsy is of unknown cause, but may be associated with upper respiratory infection, pneumonia, otitis media, chronic sinusitis, and dental abscess.

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Anesthesia Dolorosa

     Following injury to the trigeminal nerve, a painful area of numbness may develop that is diagnosed as anesthesia dolorosa. This pain is severe and constant and described as burning, gnawing, or stinging. The most common cause is from destructive interventions for trigeminal neuralgia.

Treatments: Treatment of anesthesia dolorosa is often ineffective, and medications will often not relieve the pain. Surgical interventions that have been tried with limited success include focused injury to the brain stem (tractotomy of the nucleus caudalis), deep brain stimulation and pre-motor cortex stimulation. In general, additional destructive interventions are not effective.

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Atypical Facial Pain

     Atypical facial pain (or idiopathic facial pain) is characterized by deep, achy, constant, pulling or crushing pain that involves diffuse areas of the face and head. The pain fluctuates in intensity and severity. Trigger points on the face cannot be found, the pain is often worse at night, and may be aggravated by activity.

      In the majority of cases, only one side of the face is affected, but pain on both sides is also possible. The symptoms may initially appear similar to trigeminal neuralgia, although progress to an atypical facial pain pattern. The diagnosis is made when the origin of pain is undetermined or when symptoms do not correlate with any other facial pain syndrome. Atypical facial pain affects more woman than men, and is often accompanied by depression. However, this disorder is not of psychogenic origin.

Treatments: Unfortunately, atypical facial pain is typically difficult to treat, and surgery is generally avoided. While radiofrequency rhizotomy has been performed, such destructive interventions may actually worsen the pain. Tricyclic antidepressant medications can provide sufferers with modest relief of their symptoms. Conventional analgesic drugs, including opioids, can also be effective in selected individuals, often under the direction of a comprehensive pain management program.

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Occipital Neuralgia

     Occipital neuralgia is characterized by jabbing pain radiating from the neck to the back of the head on one side (distributions of the occipital nerve). The pain may also radiate to the forehead and eye, and there is usually some nerve tenderness and numbness in the affected area. There may be a continuous aching or throbbing pain upon which jabbing pains are superimposed. Attacks can be intensified or provoked by physical or emotional stress.

     This disease is typically caused by acute or chronic trauma, entrapment, or inflammation at any point along the course of the C2 and C3 nerves. Many sufferers will have had a previous "whiplash" injury to the head, but lesions are not apparent in radiological imaging. Other disorders may be confused with occipital neuralgia and include migraine or myofascial pain, C1-C2 arthroses, temporal arteritis, inflammatory
disorders, post-herpetic neuralgia, occipital adenopathy and neurosyphillis. Occipital neuralgia is similar to glossopharyngeal, trigeminal, and geniculate neuralgias, but is differentiated according to the location of pain.

Treatments: Carbamazepine or Neurontin can reduce occipital neuralgia pain. Injections of local anesthetic (diagnostic blocks) may temporarily relieve occipital neuralgia. Repeated injections can be combined with steroids for longer lasting pain control in some individuals.

    For severe and intractable pain, a variety of nerve injury procedures may be considered. These include C2 ganglionectomy by surgery or radiofrequency lesions, peripheral neurectomy, and intradural rhizotomy. As with all destructive interventions (rhizotomies) for neuralgic pain, long-term pain relief is not guaranteed and complications including weakness and numbness may occur. Microvascular decompression of the C2 root and ganglion at the neural foramen has been described.

     A ventrolateral partial rhizotomy of the posterior rootlets of C1, C2 and C3 is a potentially curative procedure. Good long-term pain relief may be achieved with reduced risks of developing post-operative vertigo (dizziness), scalp anesthesia (numbness), that are often encountered following non-selective rhizotomies. Another treatment option recently described is placement of an occipital nerve stimulator. This device is permanently implanted like a pacemaker and applies an electrical current to the occipital nerves that may result in continued pain relief.

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Tinnitus and Vertigo

     Disorders of the vestibular cochlear nerve (VIII) can result in tinnitus (ringing noise in the ear), vertigo (sensation of spinning), dizziness, nausea or hearing loss. There are many possible causes for these various symptoms, which are generally investigated by specialists in Otolaryngology. In rare instances, neurovascular compression involving the vestibular cochlear nerve may be implicated as the cause of such symptoms. In such cases,microvascular decompression surgery has been performed to cure patients of these symptoms.

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Prepared by A. M. Kaufmann & M. Patel
© 2001 Centre for Cranial Nerve Disorders, Winnipeg, University of Manitoba, Health Sciences Centre. The information provided on this web-site is intended for educational purposes only, and should not be used to diagnose or treat a disease or disorder. This information is not intended to substitute, supplement, or in any way qualify the services or advice provided by a qualified health care professional. Please consult with a certified health care professional before pursuing any form of medical action. Duplication in any part or form of this document is strictly prohibited. All rights reserved. For further information please read our disclaimer. Inquiries can be directed to the Information Provider of this web-site.