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Limb dystonia in progressive supranuclear palsy

Division of Neurology, Roger Williams General Hospital and Brown University, Providence, RI.

Progressive limb dystonia contributed to disability in 8 of 30 patients with progressive supranuclear palsy (PSP). In five, it was present when the patients were on no medication. In four, it had been present before the distinctive ophthalmoplegia permitted a correct diagnosis. The severity of limb dystonia did not correlate with the severity of either ophthalmoplegia or neck dystonia. The importance of dystonia in the pathophysiology of PSP is emphasized, with regard to both the branchial dystonia that underlies several cardinal features of the disease, and to the frequent occurrence of limb dystonia as an early sign.

Address correspondence and reprint requests to Dr. Rafal, Roger Williams General Hospital, Division of Neurology, 825 Chalkstone Avenue, Providence, RI 02908.

Received June 9, 1986. Accepted for publication in final form December 30, 1986.

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