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Hereditary dystonia-parkinsonism syndrome of juvenile onset

Department of Neurology, UMDNJ-Rutgers Medical School, New Brunswick, NJ.

We studied a family with an extrapyramidal disorder characterized by childhood onset of lower-limb and axial dystonia, followed by parkinsonism. Dramatic response to levodopa therapy and minimal progression in adult life was seen. The family included five generations of affected members of both sexes in an autosomal dominant pattern.

Address correspondence and reprint requests to Dr. Nygaard, Department of Neurology, College of Physicians and Surgeons, Columbia University, 710 West 168th Street, New York, NY 10032.

Supported by the James Parkinson Fund.

Presented in part at the thirty-seventh annual meeting of the American Academy of Neurology. Dallas, TX, May 1985.

Accepted for publication February 25. 1986.

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