The spectrum of Susac's syndrome.
We report a series of four patients with Susac's syndrome, which is characterized by the triad of visual loss due to branch retinal artery occlusions, sensorineural hearing loss due to cochlear involvement, and encephalopathy due to cerebral microangiopathy. However, as we describe in this series, the clinical triad may not be apparent for years, resulting in delays in diagnosis. We also report the variable cerebrospinal fluid and brain magnetic resonance imaging findings, and treatment using a combination of steroids and intravenous immunoglobulin, followed by mycophenolate mofetil.
Pawate
Siddharama
S
Multiple Sclerosis Center, Department of Neurology, Vanderbilt University Medical Center, 2201 Childrens Way Room 1222, Nashville, TN 37211, USA. siddharama.pawate@vanderbilt.edu
Agarwal
Anita
A
Moses
Harold
H
Sriram
Subramaniam
S
D000328:Adult; D001921:Brain / Q000098:blood supply / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology / Q000503:physiopathology; D002561:Cerebrovascular Disorders / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D003051:Cochlea / Q000098:blood supply / Q000503:physiopathology; D018450:Disease Progression; D042783:Endothelial Cells / Q000473:pathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D012161:Retinal Artery / Q000473:pathology / Q000503:physiopathology; D015356:Retinal Artery Occlusion / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D013256:Steroids / Q000627:therapeutic use; D013577:Syndrome; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D013256:Steroids