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 CASE REPORT
Year : 2017  |  Volume : 12  |  Issue : 4  |  Page : 374-377

Susac’s syndrome (retinocochleocerebral vasculopathy): Follow-up of a pediatric patient


1 Department of Pediatric Neurology, Ankara Children’s Hematology Oncology Research and Training Hospital, Ankara, Turkey
2 Department of Opthalmology, Ulucanlar Eyes Training and Research Hospital, Ankara, Turkey
3 Department of Pediatric Neurology, Ondokuz Mayis University School of Medicine, Samsun, Turkey

Correspondence Address:
Mrs. Zeynep Selen Karalok
Department of Pediatric Neurology, Ankara Children’s Hematology Oncology, Training and Research Hospital, Diskapi, Ankara 06110, Turkey

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JPN.JPN_128_17

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Susac’s syndrome (SS) is a triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss as a result of microvascular occlusions of the brain, retina, and inner ear. It is also a disorder of autoimmune endotheliopathy. SS usually affects young women between the age of 20 and 40 years. SS can be misdiagnosed as multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM) because of similar findings. A 15-year-old girl presented in June 2015 with vomiting and severe headache. Cerebral magnetic resonance imaging revealed multiple lesions in the corpus callosum. Cerebrospinal fluid findings gave normal results. The initial diagnosis was MS and steroid (1000mg/day) was given. She started to describe hallucinations and became paraplegic. She then underwent plasmapheresis five times without response. Her electroencephalogram was diffusely slow with 2–3 Hz delta rhythm at the frontal regions. Audiological examination showed that she had sensorineural hearing loss in her left ear. Ophthalmologic evaluation revealed BRAO in both eyes. On the basis of these findings, she was diagnosed with SS and treated with intravenous immunoglobulin (IVIG) and aspirin. After monthly treatment with IVIG for 6 months, the patient has almost fully recovered. SS should be kept in mind in the differential diagnosis of MS and ADEM.






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