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NEUROLOGY 1985;35:1212
© 1985 American Academy of Neurology

Syndrome of palatal myoclonus and progressive ataxia

Two cases with magnetic resonance imaging

Michael R. Sperling, MD and Christian Herrmann, Jr., MD

UCLA School of Medicine, Los Angeles, CA.

Two patients are reported with palatal myoclonus, progressive ataxia, and dysarthria, unresponsive to treatment with trihexyphenidyl or L-5-hydroxytryptophan. MRI showed enlargement of the inferior olives in one patient, consistent with the pathology usually associated with palatal myoclonus. The syndrome of progressive ataxia and palatal myoclonus should be distinguished from other ataxias and degenerations that affect the brainstem and cerebellum. Pathology and specificity of site of the lesions are distinctive.

Address correspondence and reprint requests to Dr. Sperling, Department of Neurology, Graduate Hospital, 19th & Lombard St., Philadelphia, PA 19146.

Accepted for publication December 4, 1984.




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