Clinical features of Meige's disease (idiopathic orofacial dystonia): a report of 17 cases

Abstract

Seventeen patients with prominent orofacial dystonia of unknown cause (idiopathic orofacial dystonia: Meige's disease) were examined and several clinical features seen that, to my knowledge, had previously not been recognized. These include a family history of dystonia or other extrapyramidal disorders, a high incidence of depression, and frequent extension of spasms beyond the orofacial muscles. The course of the muscle spasms varies: rapid progression (eg, two months) to maximal disability occurred in some patients, and clear improvement after years of severe disability was observed in others. In addition to the muscle spasms, neurological abnormalities that suggest dysfunction of the basal ganglia were frequently present. The "spasm facial median" of Meige may be a distinct dystonic disorder, unrelated to idiopathic torsion dystonia.