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Department of Neurology, University of Medicine and Dentistry of New Jersey/Robert Wood Johnson Medical School (Drs. Lepore and Duvoisin), New Brunswick, NJ; the Veterans Administration Office (Dr. Steele), US Naval Hospital (Dr. McDarby), Guam; the Department of Ophthalmology (Dr. Cox and Ms. Tillson) and the Department of Medicine, Division of Neurology (Dr. Calne), University of British Columbia, Vancouver, BC, Canada; and the Neuroepidemiology Branch (Dr. Lavine), National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD.
We found abnormal supranuclear ocular or lid motility in all of 37 patients with Lytico-Bodig (amyotrophic lateral sclerosis/parkinsonism-dementia complex). Twenty-one patients had pursuit paresis, 18 abnormal vestibulo-ocular reflex (VOR) cancellation, 15 abnormal convergence, 13 abnormal optokinetic nystagmus (OKN), 12 conjugate gaze limitation, nine nystagmus, nine saccadic paresis, and six abnormal fixation. Lid abnormalities included glabellar hyperreflexia in 21, involuntary levator inhibition in three, and blepharospasm in two. Earlier reports have indicated infrequent ocular disturbances in Lytico-Bodig, but we now find supranuclear eye and lid deficits are universal and sometimes very prominent.
Address correspondence and reprint requests to Dr. Lepore, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, Academic Health Science Center, CN 19, New Brunswick, NJ 08903.
Presented in part at the fortieth annual meeting of the American Academy of Neurology, Cincinnati, OH, April 1988. Received May 9, 1988. Accepted for publication in final form June 27, 1988.
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