Meige's Syndrome (original) (raw)

by
George W. Paulson, M.D.
Kurtz Professor of Neurology
The Ohio State University Medical Center
Columbus, Ohio

Web page created on December 13, 1997

Henri Meige described in 1904 what is now commonly called oral facial dystonia. There is some variation in what has been described since in what has been called Meige's syndrome, but in all descriptions there is blinking and chin thrusting. Some patients have lip pursing or tongue movements and, for a few, the movements spread into the shoulders. The cause remains obscure, treatment less than ideal, and frustration is a major factor as it is with blepharospasm. Many physicians, even regarding the more common essential blepharospasm, may have had no experience with Meige's syndrome and the patient may be told the process is psychologic. It is not.

There are some similar conditions related to medication effects, for example, the mouth movement seen when excessive levodopa is present in patients with Parkinson's disease. Tardive dyskinesia, the movements that can follow prolonged use of major tranquilizers, can be associated with mouth and tongue movements. Some normal, elderly individuals develop spontaneous mouthing or chewing movements.

Meige is more than and different from any of these other mouth movements. Blinking forcefully is often a part of it as is grimacing and the chin thrusting forward. At times there is a joint interactive movement between the oral movements and the eye movements. The patients are more likely to be women than men and usually at middle age or beyond. There is, as in all neurologic conditions, some variation with stress, but the movement is present at rest and with activity and when with others or alone. As with almost all movement disorders, it disappears in sleep.

Most movement disorders can be inhibited voluntarily to some degree but not completely. The same is true of Meige's syndrome. Patients may chew gum, whistle or touch their face in an effort to lessen the movements.

Diagnostic studies such as magnetic resonance imaging (MRI) are not usually of any particular value in Meige's syndrome, but it is entirely proper to do an MRI one time to rule out structural disease. Observation is how the diagnosis is made, not by the laboratory.

It is unlikely that medication is the cause of Meige's syndrome but, in occasional cases, it may be hard to distinguish Meige's from tardive dyskinesia. Most people with tardive dyskinesia do not have blinking and are more likely to have a tongue that writhes around in the mouth than one that intermittently protrudes as with Meige's syndrome.

There is, unfortunately, no cure but, occasionally, patients will improve with time. BOTOX injections may help with the blepharospasm and can be used to suppress mouth movements but it is no cure. Some patients are benefited by anticholinergics such as Artane (trihexphenidyl) or Cogentin (benztropine) and a few are benefited by muscle relaxants such as Lioresal (baclofen). Anti-convulsants such as Tegretol (carbamazepine) have also been employed with sporadic benefit.

Support groups can and do help, particularly with learning to live with this disorder. Support groups have also led the way in educating doctors about Meige's syndrome.

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