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NEUROLOGY 1987;37:112
© 1987 American Academy of Neurology

The search for a physiologic marker of Machado-Joseph disease

John R. Hotson, Elizabeth B. Langston, Anne A. Louis and Roger N. Rosenberg

Department of Neurology, Stanford University School of Medicine and the Institute for Medical Research at the Santa Clara Valley Medical Center, Stanford and San Jose, C A (Drs. Hotson, Langston. and Louis), and the University of Texas Health Sciences Center, Southwestern Medical School, Dallas, TX (Dr. Rosenberg).

Machado-Joseph disease is a dominantly inherited, multisystem, degenerative disorder that lacks a proven genetic marker. Peripheral nerve conduction-refractory period, sensory evoked potentials, and quantified oculomotor recordings were studied in nine patients affected with this disease to look for a potential physiologic marker. Only the oculomotor measurements of saccade and smooth pursuit gain were consistently abnormal in all patients. Identical eye movement recordings in 12 asymptomatic individuals at risk for Machado-Joseph disease revealed findings typical of affected patients in only 1 individual. Quantified oculomotor studies may contribute to the early confirmation of the disease, primarily in individuals at risk with minor or equivocal neurologic signs.

Address correspondence and reprint requests to Dr. Hotson, Department of Neurology, Santa Clara Valley Medical Center, 751 South Bascom Avenue, San Jose, CA 95128.

Supported by NEI grant EY03387; a grant from the International Joseph Disease Foundation, Livermore. CA; and a grant from the Pimley Fund at the Stanford University School of Medicine.

Received December 29, 1985. Accepted for publication April 14, 1986.






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