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April 1990

Cone Dysfunction in a Subgroup of Patients With Autosomal Dominant Cerebellar Ataxia

Author Affiliations

From the Division of Neurology, Department of Medicine (Drs Hamilton and Bird), Division of Electroencephalography and Clinical Neurophysiology, Departments of Laboratory Medicine and Neurological Surgery (Dr Chatrian), and the Department of Ophthalmology (Dr Mills and Kalina), University of Washington Medical Center, and the Veterans Administration Medical Center (Dr Bird), Seattle.

Arch Ophthalmol. 1990;108(4):551-556. doi:10.1001/archopht.1990.01070060099057

• Four patients with autosomal dominant cerebellar ataxia and retinal degeneration underwent neurologic and ophthalmologic examinations and computerassessed corneal electroretinography. Previous reports described progressive panretinal degeneration initially involving the cones and subsequently spreading to the rods. By contrast, all our patients displayed evidence of selective dysfunction of the cone system in one or two successive electroretinograms, irrespective of their age or duration of visual symptoms. Color vision testing, funduscopy, and fluorescein angiography suggested a cone dystrophy. Within the period of observation only one patient showed progression of cone dysfunction. In patients with hereditary ataxias, quantitative electroretinography provides an objective and sometimes early indication of retinal degeneration and helps to characterize it.

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