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Trigeminal neuralgia

Trigeminal neuralgia, or Tic Douloureux, is a neuropathic disorder of the trigeminal nerve that causes episodes of intense pain in the eyes, lips, nose, scalp, forehead, and jaw. Trigeminal neuralgia is considered by many to be among the most painful of conditions and has been labeled the "suicide disease," due to the significant numbers of people taking their own lives because they were unable to have their pain controlled with medications or surgery. more...

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An estimated one in 15,000 people suffers from trigeminal neuralgia, although numbers may be significantly higher due to frequent misdiagnosis. It usually develops after the age of 40 and affects women in a 2:1 ratio.

Pathophysiology

The trigeminal nerve is the fifth cranial nerve, a mixed cranial nerve responsible for sensory data such as tactition (pressure), thermoception (temperature), and nociception (pain) originating from the face above the jawline; it is also responsible for the motor function of the muscles of mastication, the muscles involved in chewing but not facial expression. Several theories exist to explain the possible causes of this pain syndrome. Among the structural causes, damage to the myelin sheath of this nerve causes the electrical impulses traveling along it to be erratic or excessive, activating pain regions or deactivating pain inhibitory regions in the brain. The damage may be caused by an aneurysm (an outpouching of a blood vessel) or abnormally coursing artery compressing the nerve, most frequently at the area of its cerebellopontine nerve root; the superior cerebellar artery has been an oft-cited culprit. Two to 4% of patients with TN, usually younger, have evidence of multiple sclerosis, which may damage either the trigeminal nerve or other related parts of the brain. Trigeminal Neuralgia may also be caused by a tumor or a traumatic event such as a car accident. When there is no structural cause, the syndrome is called idiopathic. Postherpetic Neuralgia, which occurs after shingles, may cause similar symptoms if the trigeminal nerve is affected.

Symptoms

The episodes of pain occur paroxysmally, or suddenly, sometimes triggered by common activities or cold exposure, and are said to feel like stabbing electric shocks. Individual attacks affect one side of the face at a time, last several seconds, and may come and go throughout the day, or for periods as long as several months. Three to 5% of cases are bilateral, and attacks may increase in frequency or severity over time. Although trigeminal neuralgia is not fatal, successive recurrences may be incapacitating, and the fear of provoking an attack may make sufferers reluctant to engage in normal activities.

There is a variant of trigeminal neuralgia called, "atypical trigeminal neuralgia." In some cases of atypical trigeminal neuralgia, the sufferer experiences a severe, relentless underlying pain similar to a migraine in addition to the stabbing pains. In other cases, the pain is stabbing and intense, but may feel like burning or prickling, rather than a shock. Sometimes, the pain is a combination of the zaps, the migraine-like pain, and the burning/prickly pain.

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Neuralgia
From Gale Encyclopedia of Medicine, 4/6/01 by Carol A. Turkington

Definition

Neuralgia is defined as an intense burning or stabbing pain caused by irritation of or damage to a nerve. The pain is usually brief but may be severe. It often feels as if it is shooting along the course of the affected nerve.

Description

Different types of neuralgia occur depending on the reason the nerve has been irritated. Neuralgia can be triggered by a variety of causes, including tooth decay, eye strain, or shingles (an infection caused by the herpes zoster virus). Pain is usually felt in the part of the body that is supplied by the irritated nerve.

Causes & symptoms

Neuralgia is caused by irritation or nerve damage from systemic disease, inflammation, infection, and compression or physical irritation of a nerve. The location of the pain depends on the underlying condition that is irritating the nerve or the location of the particular nerve that is being irritated.

Neuralgia can result from tooth decay, poor diet, eye strain, nose infections, or exposure to damp and cold. Postherpetic neuralgia is an intense debilitating pain felt at the site of a previous attack of shingles. Trigeminal neuralgia (also called tic douloureux, the most common type of neuralgia), causes a brief, searing pain along the trigeminal nerve, which supplies sensation to the face. The facial pain of migraine neuralgia lasts between 30 minutes and an hour and occurs at the same time on successive days. The cause is not known.

Glossopharyngeal neuralgia is an intense pain felt at the back of the tongue, in the throat, and in the ear--all areas served by the glossopharyngeal nerve. The pain may occur spontaneously, or it can be triggered by talking, eating, or swallowing (especially cold foods such as ice cream). Its cause is not known.

Occipital neuralgia is caused by a pinched occipital nerve. There are two occipital nerves, each located at the back of the neck, each supplying feeling to the skin over half of the back of the head. These nerves can be pinched due to factors ranging from arthritis to injury, but the result is the same: numbness, pain, or tingling over half the base of the skull.

Diagnosis

Neuralgia is a symptom of an underlying disorder; its diagnosis depends on finding the cause of the condition creating the pain.

To diagnose occipital neuralgia, a doctor can inject a small amount of anesthetic into the region of the occipital nerve. If the pain temporarily disappears, and there are no other physical reasons for the pain, the doctor may recommend surgery to deal with the pinched nerve.

Treatment

Glossopharyngeal, trigeminal, and postherpetic neuralgias sometimes respond to anticonvulsant drugs, such as carbamazepine or phenytoin, or to painkillers, such as acetaminophen. Trigeminal neuralgia may also be relieved by surgery in which the nerve is cut or decompressed. In some cases, compression neuralgia (including occipital neuralgia) can be relieved by surgery.

People with shingles should see a doctor within three days of developing the rash, since aggressive treatment of the blisters that appear with the rash can ease the severity of the infection and minimize the risk of developing postherpetic neuralgia. However, it is not clear whether the treatment can prevent postherpetic neuralgia.

If postherpetic neuralgia develops, a variety of treatments can be tried, since their effectiveness varies from person-to-person.

  • Antidepressants such as amitriptyline (Elavil)
  • Anticonvulsants (phenytoin, valproate, or carbamazepine)
  • Capsaicin (Xostrix), the only medication approved by the FDA for treatment of postherpetic neuralgia
  • Topical painkillers
  • Desensitization
  • TENS (transcutaneous electrical nerve stimulation)
  • Dorsal root zone (DREZ) surgery (a treatment of last resort).

Alternative treatment

B-complex vitamins, primarily given by intramuscular injection, can be an effective treatment. A whole foods diet with adequate protein, carbohydrates, and fats that also includes yeast, liver, wheat germ, and foods that are high in B vitamins may be helpful. Acupuncture is a very effective treatment, especially for postherpetic neuralgia. Homeopathic treatment can also be very effective when the correct remedy is used. Some botanical medicines may also be useful. For example, black cohosh (Cimicifuga racemosa) appears to have anti-inflammatory properties based on recent research.

Prognosis

The effectiveness of the treatment depends on the cause of the neuralgia, but many cases respond to pain relief.

Trigeminal neuralgia tends to come and go, but successive attacks may be disabling. Although neuralgia is not fatal, the patient's fear of being in pain can seriously interfere with daily life.

Some people with postherpetic neuralgia respond completely to treatment. Most people, however, experience some pain after treatment, and a few receive no relief at all. Some people live with this type of neuralgia for the rest of their lives, but for most, the condition gradually fades away within five years.

Key Terms

Desensitization
A technique of pain reduction in which the painful area is stimulated with whatever is causing the pain.
Dorsal root entry zone (DREZ)
A type of nerve surgery for postherpetic neuralgia that is occasionally used when the patient can get no other pain relief. The surgery destroys the area where damaged nerves join the central nervous system, thereby interfering with inappropriate pain messages from nerves to the brain.
Glossopharyngeal neuralgia
Sharp recurrent pain deep in the throat that extends to the area around the tonsils and possibly the ear. It is triggered by swallowing or chewing.
Migraine neuralgia
A variant of migraine pain, also called cluster headache, in which severe attacks of pain affect the eye and forehead on one side of the face.

Occipital neuralgia
Pain on one side of the back of the head caused by entrapment or pinching of an occipital nerve.
Postherpetic neuralgia
Persistent pain that occurs as a complication of a herpes zoster infection. Although the pain can be treated, the response is variable.
Shingles
A painful rash with blisters that appears along the course of a nerve. It is caused by infection with herpes zoster virus.
TENS
The abbreviation for transcutaneous electrical nerve stimulation, a technique used to control chronic pain. Electrodes placed over the painful area deliver a mild electrical impulse to nearby nerve pathways, thereby easing pain.
Trigeminal neuralgia
Brief episodes of severe shooting pain on one side of the face caused by inflammation of the root of the trigeminal nerve. Also referred to as tic douloureux.

Further Reading

For Your Information

    Books

  • Loeser, J. "Cranial Neuralgias." In The Management of Pain. 2nd ed. Philadelphia: Lea & Febiger, 1990.

    Periodicals

  • Fields, H. "Treatment of Trigeminal Neuralgia." The New England Journal of Medicine 334(April 1996): 1125-1126.

    Organizations

  • American Chronic Pain Association. PO Box 850, Rocklin, CA 95677. (916) 632-0922.
  • National Chronic Pain Outreach. PO Box 274, Millboro, VA 24460. (540) 997-5004.
  • Trigeminal Neuralgia/Tic Douloureux Association. PO Box 340, Barnegat Light, NJ 08006. (609) 361-1014.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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