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NEUROLOGY 1990;40:293
© 1990 American Academy of Neurology

Extrapyramidal involvement in Rett's syndrome

P. M. FitzGerald, MB, J. Jankovic, MD, D. G. Glaze, MD, R. Schultz, RN, PNP and A. K. Percy, MD

Departments of Neurology (Drs. FitzGerald and Jankovic) and Pediatrics (Dr. Glaze, R. Schultz, and Dr. Percy), Baylor College of Medicine, Houston, TX.

Extrapyramidal dysfunction is poorly characterized in Rett's syndrome, a neurodegenerative disorder in girls. We studied the motor and behavioral findings in 32 Rett's syndrome patients, 21 months to.30 years old. In addition to the typical stereotyped movements and scoliosis, other motor disturbances included bruxism, sialorrhea, ocular deviations, parkin-sonian findings, dystonia, myoclonus, and athetosis. The types of movement disorders seemed to be age-related, with the hyperkinetic disorders occurring in the younger patients and the bradykinetic disorders occurring more frequently in the older patients.

Address correspondence and reprint requests to Dr. Joseph Jankovic, Professor of Neurology, Department of Neurology, Baylor College of Medicine, 6550 Fannin, #1801, Houston, TX 77030.

Supported in part by the NIH/HDMR, grant number HD24234, and in part by a grant to the General Clinical Research Center, Texas Children's Hospital, NIH grant number M01RR00188.

Presented in part at the 41st annual meeting of the American Academy of Neurology, Chicago, IL, April 1989.

Received May 31, 1989. Accepted for publication in final form August 4, 1989.




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