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Severe dystonia and myoglobinuria

Baylor College of Medicine (Dr. Jankovic), Houston, TX, and Columbia University College of Physicians and Surgeons (Dr. Penn), The Neurological Institute, New York, NY.

Myoglobinuria may follow extreme muscular exertion or disorders that cause muscle necrosis. Dystonia has not been implicated previously. We studied an 8-year-old boy of non-Jewish, Mexican-American descent with autosomal-dominant dystonia musculorum deformans who developed rapidly progressive and severe generalized dystonia, hyperpyrexia, myoglobinuria, and renal failure. Curarization was required. Transient improvement was achieved with tetrabenazine and baclofen, but bilateral thalamotomy was then performed. Patients with severe dystonia should be observed for evidence of myoglobinuria.

Address correspondence and reprint requests to Dr. Jankovic, Baylor College of Medicine, 6501 Fannin No. 302, Houston, TX 77030.

Supported by a Center Grant From the Muscular Dystrophy Association (Dr. Penn).

Accepted for publication March 30, 1982.

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