Microangiopathy of brain, retina, and inner ear.
Microangiopathy of brain, retina, and inner ear is a rare syndrome manifesting as arteriolar occlusions of the brain, retina, and inner ear, with resultant encephalopathy, visual, and hearing loss. Despite exhaustive laboratory examinations in these patients, no evidence of a systemic disease can be found. We treated and followed an adolescent with this disorder who initially presented with a branch retinal artery occlusion. A unique finding in this case was retinal vessel wall hyperfluorescence noted five days prior to retinal infarction. The patient developed recurrent branch artery occlusions, sensorineural hearing loss, and central nervous system infarctions despite anticoagulation and immunosuppressive treatment.
Notis
C M
CM
Department of Ophthalmology, St. Luke's Roosevelt Hospital Center, New York, New York, USA.
Kitei
R A
RA
Cafferty
M S
MS
Odel
J G
JG
Mitchell
J P
JP
D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D001921:Brain / Q000098:blood supply / Q000473:pathology; D002544:Cerebral Infarction / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008833:Microcirculation; D012161:Retinal Artery / Q000473:pathology; D015356:Retinal Artery Occlusion / Q000175:diagnosis* / Q000188:drug therapy; D013577:Syndrome; D014012:Tinnitus / Q000175:diagnosis
D000925:Anticoagulants; D007166:Immunosuppressive Agents