Meige Syndrome

Disease Overview

Meige syndrome is a rare neurological movement disorder characterized by involuntary and often forceful contractions of the muscles of the jaw and tongue (oromandibular dystonia) and involuntary muscle spasms and contractions of the muscles around the eyes (blepharospasm). The specific symptoms and their severity vary from case to case.

Meige syndrome belongs to a group of disorders known as dystonia. Dystonia is a group of movement disorders that vary in their symptoms, causes, progression, and treatments. This group of neurological conditions is generally characterized by involuntary muscle contractions that force the body into abnormal, sometimes painful, movements and positions (postures). The exact cause of Meige syndrome is unknown.

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Synonyms

• Brueghel Syndrome
• Idiopathic Blepharospasm-Oromandibular Dystonia Syndrome
• Segmental Cranial Dystonia

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Signs & Symptoms

Meige syndrome is characterized by the combination of blepharospasm and oromandibular dystonia. The severity of these conditions varies from case to case. Meige syndrome most often affects middle-aged individuals.

Blepharospasm is characterized by frequent or forced blinking and eye irritation that often occurs as a result of specific stimuli including bright lights, fatigue, emotional tension, and environmental factors such as wind or air pollution. The frequency of muscle spasms and contractions may increase causing narrowing of the opening between the eyelids or involuntary closure of the eyelids. It may become progressively harder for affected individuals to keep their eyes open. Blepharospasm may originally affect one eye (unilateral), but usually becomes (bilateral). Some individuals with Meige syndrome may experience abnormally dry eyes.

Oromandibular dystonia is characterized by involuntary, forceful contractions of the jaw and tongue, often making it difficult to open or close the mouth. Some individuals may also experience clenching or grinding of the teeth, displacement of the jaw, grimacing, chin thrusting, or repeated pursing of the lips. Eyelid and facial muscle tone may gradually decline.

Some people with Meige syndrome may also experience spasms of the tongue and throat, resulting in repeated protrusion of the tongue from the mouth and difficulty swallowing. Muscle spasms of the respiratory tract may lead to breathing difficulties (dyspnea). In some cases, muscles in the neck, arms, legs or other muscle groups may become affected.

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Causes

The cause of Meige syndrome is unknown. Researchers speculate that the cause of Meige syndrome may be multifactorial (e.g., caused by the interaction of certain genetic and environmental factors).

Malfunctioning of a region of the brain known as the basal ganglia may play a role in the development of Meige syndrome. The basal ganglia is a structure composed of nerve cells located at the base of the brain. The basal ganglia is involved in the regulation of motor and learning functions. The exact problem(s) associated with the basal ganglia in individuals with Meige syndrome is unknown.

Some cases of oromandibular dystonia occur in association with or secondary to another disorder such as tardive dyskinesia, Wilson disease, and Parkinson disease.

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Affected populations

Meige syndrome affects women more often than men. Symptoms typically begin in middle-age between 40-70 years, although cases have been reported in individuals much younger. The disorder was first described in detail in the medical literature in 1910 by French neurologist Henry Meige.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Meige syndrome. Comparisons may be useful for a differential diagnosis.

Dystonia is a group of neurological movement disorders characterized by involuntary muscle contractions. Dystonia may be focal (affecting an isolated body part), segmental (affecting adjacent body areas, or generalized (affecting many major muscle groups simultaneously). Dystonia may result in abnormal, often painful movements or postures. There are many different causes for dystonia. Genetic as well as non-genetic factors contribute to all forms of dystonia. (For more information on this disorder, choose “dystonia” as your search term in the Rare Disease Database.)

Temporomandibular joint (TMJ) dysfunction is a general term for a group of conditions that affect the temporomandibular joint. The TMJs are small joints that connect the lower jaw (mandible) to the temporal bone of the skull. TMJ dysfunction is characterized by pain of the jaw joint that is made worse during or after eating or yawning. It can cause limited jaw movement and clicks and pops during chewing. In severe cases, pain can radiate into the neck, shoulders and back. There are many suspected causes of TMJ dysfunction. The only definite cause is trauma to the jaw as from a heavy blow to the mouth. (For more information on this disorder, choose “temporomandibular joint dysfunction” as your search term in the Rare Disease Database.)

Benign essential blepharospasm (BEB) is a rare neurological disorder in which affected individuals experience involuntary muscle spasms and contractions of the muscles around the eye. These spasms come and go (intermittent). Symptoms may begin as eye twitching, blinking and/or irritation. Eventually, BEB causes involuntary closure of the eyes. The exact cause of BEB is unknown. The disorder is one of a group of disorders collectively known as dystonia. (For more information on this disorder, choose “blepharospasm” as your search term in the Rare Disease Database.)

Tardive dyskinesia (TD) is an involuntary neurological movement disorder caused by the use of neuroleptic drugs that are prescribed to treat certain psychiatric or gastrointestinal conditions. Long-term use of these drugs may produce biochemical abnormalities in the area of the brain known as the striatum. The reasons that some people who take these drugs may develop tardive dyskinesia, and some people do not, is unknown. tardive dystonia is believed to be the more severe form of tardive dyskinesia. (For more information on this disorder, choose “tardive dyskinesia” as your search term in the Rare Disease Database.)

Hemifacial spasm, which is characterized by contractions on one side of the face, is technically not a form of dystonia. The initial symptom of hemifacial spasm may be twitching of the eyelids that eventually results in forced closure of the eyelid. Hemifacial spasm may be caused by inflammation of irritation to a facial nerve. (For more information on this disorder, choose “Hemifacial Spasm” as your search term in the Rare Disease Database.)

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Diagnosis

No tests exist to diagnose Meige syndrome. A diagnosis is made based upon a thorough clinical evaluation, a detailed patient history and identification of characteristic symptoms.

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Standard Therapies

Treatment

The treatment of Meige syndrome is directed toward the specific symptoms that are apparent in each individual. Treatment consists of drug therapy and botulinum A toxin (Botox) injections used separately or in combination.

Approximately one-third of affected individuals are treated with oral medications (drug therapy). Unfortunately, the results of these drug treatments are usually moderate or unsatisfactory and often temporary. No drugs appear to be uniformly effective. Drugs that have been used to treat Meige syndrome include clonazepam, trihexyphenidyl, diazepam, and baclofen.

The orphan drug botulinum A toxin (BOTOX) has been approved by the Food and Drug Administration (FDA) as a treatment for blepharospasm and has become the primary form of treatment. The technique of injecting small amounts of botulinum toxin into the orbicularis oculi paralyzes these muscles for several months, after which time the procedure must be repeated. Botulinum toxin injections have been helpful for many individuals with blepharospasm, but some people do not respond well. The drug is distributed by Allergan, Inc. For more information patients should ask their physician to contact: Allergan Inc., 2525 Dupont Drive, Irvine, CA 92713-9534.

BOTOX is also used to treat muscle spasms associated with oromandibular dysontia. Approximately 70 percent of individuals experience some reduction of spasm and improvement in chewing and swallowing following injection with BOTOX.

In some cases, individuals may experience relief of symptoms by engaging in specific movements sometimes referred to as "sensory tricks." Such movements include biting on a toothpick, chewing gums, talking, or lightly touching the lips or chin. Speech and swallowing therapy may lessen spasms, improve range of motion, and strengthened unaffected muscles.

According to the medical literature, some individuals with blepharospasm and/or oromandibular dystonia have improved without treatment (spontaneous remission).

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

According to the medical literature, the use of stereotactic brain surgery for cases of dystonia that do not respond to other treatment methods (refractory dystonia) is increasing. Deep brain stimulation has been used successfully as a treatment for Meige syndrome in individual cases. More research is necessary to determine the long-term safety and effectiveness of the treatment option for individuals with Meige syndrome.

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References

TEXTBOOKS

DeLong MR. Oromandibular Dystonia and Meige Syndrome. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:615-6.

JOURNAL ARTICLES

Castelbuono A, Miller NR. Spontaneous remission in patients with essential blepharospasm and Meige syndrome. Am J Ophthalmol. 1998;126:432-5.

Houser M, Waltz T. Meige syndrome and pallidal deep brain stimulation. Mov Disord. 2005;20:1203-5.

Maureillo JA Jr., Dhillon S, Leone T, et al., Treatment selections of 239 patients with blepharospasm and Meige syndrome over 11 years. Br J Ophthalmo. 1996;80:1073-6.

Paleacu D, Giladi N, Moore O, Stern A, Honigman S, Badarny S. Clin Neuropharmacol. 2004;27:230-3.

Tsubota K, Fujihara T, Minako K, et al., Dry eye and Meige’s syndrome. Br J Ophthalmol. 1997;81:439-442.

Zesiewicz TA, Louis ED, Sullivan KL, et al., Substantial improvement in a Meige’s syndrome patient with levetiracetam treatment. Mov Disord. 2004;19:1518-21.

FROM THE INTERNET

WE MOVE Web site. Segmental Dystonia. Last Updated: January 19, 2005. Available at: https://www.wemove.org/dys/dys_seg.html

Lam BL. Eyelid Myokymia. emedicine. Last Updated: August 4, 2005. 9pp. Available at: https://www.emedicine.com/oph/topic607.htm

Paulson GW. Meige’s Syndrome. BEBRF Blepharospasm Pages. Benign Essential Blepharospasm Research Foundation. Last Updated: December 13, 1997. Available at: https://www.blepharospasm.org/index.html#A4

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Programs & Resources

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