A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.
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 systemic vasculitis. CT and cerebral angiography 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.
D000328:Adult; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D001160:Arterioles; D001927:Brain Diseases / Q000175:diagnosis / Q000473:pathology*; D002544:Cerebral Infarction / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006311:Hearing Disorders / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D011241:Prednisone / Q000627:therapeutic use; D012171:Retinal Vessels; D013577:Syndrome
D003520:Cyclophosphamide; D011241:Prednisone