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Electro-oculographic findings in Machado-Joseph disease

Divisions of Neurology (Dr. Dawson) and Speech and Hearing (Mr. Feudo and Dr. Zubick), Brigham and Women's Hospital and Harvard Medical School, Boston, and the Neurology Service, Veterans Administration Medical Center, Hampton, VA and the Department of Neurology, Eastern Virginia Medical School, Norfolk, VA.

Electro-oculographic recordings (EOG) were made on 26 patients with Machado-Joseph disease or at genetic risk for that disease. All patients with clinically apparent disease (ataxia, dysarthria, spasticity, or ophthalmoparesis) had abnormal eye movements. Defects in caloric response, sinsuoidal tracking, opticokinetic nystagmus (OKN), refixation saccades, and presence of gaze paretic nystagmus were detected in that order of frequency. Fourteen subjects were clinically at risk but had normal neurologic examinations or minor equivocal signs. Nine of the fourteen had abnormal EOG, with sinusoidal tracking, calorics, refixation saccades, OKN, and gaze paretic nystagmus being abnormal in that order. EOG may be useful in early case detection and may contribute to genetic counseling.

Address correspondence and reprint requests to Dr. Dawson, Brigham and Women's Hospital, 721 Huntington Avenue, Boston, MA 02115.

Accepted for publication April 9, 1982.

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