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A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions

Departments of Neurological Surgery (Dr. Monteiro), Neurology (Drs. Monteiro, Swanson, Cuneo. and Prusiner), Ophthalmology (Dr. Monteiro), and Pathology (Dr. DeArmond), University of California—San Francisco, School of Medicine, San Francisco, CA; and the Department of Ophthalmology (Dr. Coppeto). University of Connecticut Health Center, Farmington, CT.

A syndrome consisting of a subacute encephalopathy, sensorineural hearing loss, and retinal arteriolar occlusions is described in two women. Laboratory investigations did not reveal any svstemic vasculitis. CT and cerebral ang-iography showed no abnormalities, but magnetic resonance imaging revealed small, discrete lesions in the white matter. Biopsy of cortical brain from one patient showed disseminated microinfarcts in the gray matter as well as sclerosis of small vessels. This syndrome is characterized as an occlusive vasculopathy rather than vasculitis, and should be considered in evaluations of young women presenting with encephalopathy and hearing loss.

Address correspondence and reprint requests to Dr. Prusiner, Department of Neurology, 781 Health Sciences East, University of California, San Francisco, CA 94143.

Dr. Monteiro was supported by a grant from the Rotary Foundation of the Rotary International.

Accepted for publication November 16, 1984.

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