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

Definition

Trigeminal neuralgia is a disorder of the trigeminal nerve (the fifth cranial nerve) that causes episodes of sharp, stabbing pain in the cheek, lips, gums, or chin on one side of the face.

Description

The trigeminal nerve, which is divided into three branches, is responsible for chewing, for producing saliva and tears, and for sending facial sensations to the brain. When this nerve breaks down for some reason, it can trigger brief but agonizing sizzles of pain on one side of the face.

This condition is unusual in those under age 50 and more often occurs after 70. Women are three times more likely to have the condition than are men. When trigeminal neuralgia does occur in younger people, it is often associated with multiple sclerosis.

The pain, while brief, is so severe that the sufferer often can't do anything else while the attack lasts. People with this pain often wince or twitch, which is where trigeminal neuralgia gets its French nickname tic douloureux, meaning "painful twitch."

Causes & symptoms

The origin of trigeminal neuralgia is not certain, but scientists believe it may be caused by degeneration, pressure, or irritation of the trigeminal nerve. Some doctors believe the pain may be triggered by pressure from a nearby abnormally-formed artery lying too close to the nerve.

Any part of the three branches of the trigeminal nerve may be affected. Neuralgia of the first branch leads to pain around the eyes and over the forehead; the second branch causes pain in the upper lip, nose and cheek; the third branch causes pain on the side of the tongue and lower lip.

The first episodes are usually fairly mild and brief, and it may be minutes, hours, or weeks before the next attack. However, attacks tend to occur in clumps that may last for weeks at a time. As the sufferer ages, the episodes become more frequent and painful, until the person begins to live in constant fear of the next one.

The momentary bursts of pain usually begin from the same spot on the face each time. The pain can be triggered by touching the area, washing, shaving, eating, drinking, or even talking. Even a cool breeze across the face can set off an attack. Pain is more severe at the ends of the affected nerve, especially over the lip, chin, nostrils, or teeth.

Diagnosis

Diagnosis is usually made by eliminating other problems that could cause similar pain in teeth, jaw, head, or sinuses. Because patients with the condition tend to avoid trigger points, avoiding chewing, shaving, touching or washing their faces can be a clue to diagnosis of trigeminal neuralgia.

Treatment

It is not easy to treat trigeminal neuralgia. Pain can be suppressed by a range of medicines, including the anti-epilepsy medicines carbamazepine (Tegretol) or phenytoin (Dilantin). These drugs slow down the nerve signals at certain nerve terminals, which eases the pain. However, these drugs cause a wide range of side effects, including nausea, dizziness, drowsiness, liver problems, and skin allergies. Some people develop resistance to the drugs or they can't tolerate the high dosage needed to control the discomfort. If the medicines are stopped, the pain usually returns.

If drug treatment fails, surgical treatment to block pain signals from the nerve may be effective. Radio-frequency waves, gamma rays, or glycerol injections can deaden the nerve (and hence the pain). An operation that frees the nerve from whatever is compressing it (blood vessel or tumor) can permanently relieve pain, but this major neurosurgical procedure carries its own risks and complications. Alternatively, a new procedure seeks to place a cushioning sponge between the nerve and a pulsating artery wrapping around it to soothe the irritated nerve.

Prognosis

Although the pain is momentarily incapacitating, it's not life-threatening. As the person ages, the attacks can be expected to occur more and more frequently.

Prevention

While the condition itself can't be prevented, there are a number of things patients can do to avoid triggering attacks:

  • Wash with cotton pads and warm water over the face.
  • Rinse the mouth with water after eating, if toothbrushing triggers pain.
  • Eat and drink food and beverages at room temperature.
  • Chew on the unaffected side.
  • Eat soft foods, if eating is becoming a problem.

Key Terms

Multiple sclerosis
A progressive disease of the central nervous system in which the coverings of nerves in the brain and spinal cord are destroyed.

Further Reading

For Your Information

    Books

  • Greenberg, David, Michael Aminoff, and Roger Simon. Clinical Neurology. Norwalk, CT: Appleton & Lange, 1993.

    Organizations

  • Chronic Pain Outreach. 822 Wycliff Ct., Manassas, VA 22110. (703) 368-7357.
  • National Chronic Pain Outreach Association, Inc. 4922 Hampden Lane, Bethesda, MD 20814. (301) 652-4948.
  • National Institute of Neurological Disorders and Stroke. National Institutes of Health, 31 Center Drive, MSC 2540, Bldg. 31, Rm. 8A06, Bethesda, MD 20892. (800) 352-9424. http://www.ninds.nih.gov.
  • Trigeminal Neuralgia Association. PO Box 785, Barnegat Light, NJ 08006. (609) 361-1014.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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