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Segmental Dystonia

As many as 30 percent of patients with a primary focal dystonia experience dystonic spasms in areas next to the primary site. When dystonia affects two areas of the body that are next to each other, this is termed segmental dystonia. Dystonic spasms affect at least 2 adjacent areas of the body, such as facial and neck muscles ; muscle groups of the neck and upper arm; or trunk and leg muscles.

One common segmental dystonia involves muscles of the eyelids, jaw, mouth, and lower face. Known as Meige syndrome, the condition is characterized by periodic or sustained closure of the eyelids (blepharospasm). Eyelid closure is accompanied by forceful spasms of jaw opening or closure, clenching or grinding of the teeth, sideways displacement of the jaw, lip tightening and pursing, and tongue protrusion . In addition, this form of segmental dystonia may spread to neck muscles or other muscle groups. Meige syndrome more frequently affects women than men and typically becomes apparent during the sixth decade of life.