RT Journal Article
SR Electronic
T1 A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 1113
OP 1113
DO 10.1212/WNL.35.8.1113
VO 35
IS 8
A1 Monteiro, Mario L.R.
A1 Swanson, Raymond A.
A1 Coppeto, James R.
A1 Cuneo, Richard A.
A1 DeArmond, Stephen J.
A1 Prusiner, Stanley B.
YR 1985
UL http://n.neurology.org/content/35/8/1113.abstract
AB 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.