Susac syndrome: clinical characteristics, diagnostic findings and treatment in 19 Cases

Publication date: Available online 27 May 2019Source: Multiple Sclerosis and Related DisordersAuthor(s): S. Zengin Karahan, C. Boz, S. Saip, N. Kale, S. Demirkaya, Y. Celik, S. Demir, A. Tuncer, S. Erer Ozbek, M. TerziAbstractSusac's Syndrome (SS), which was first described in 1979, is a rare and presumably autoimmune disorder characterized by encephalopathy, hearing loss, and visual disturbance resulting from branch retinal artery occlusion (BRAO). This sudy reports 19 SS patients’ clinical characteristics, MRI features, CSF analysis, treatment strategies and outcomes. At initial presentation, only three of 19 patients demonstrated the complete clinical triad. Clinic presentation varied from isolated hemiparesis to the full triad (encephalopathy, hearing loss and visual disturbances). Corpus callosum (CC) involvement was noted in the MRI of 18 patients (97%) and BRAO was detected in 17 (95%) patients. All patients were treated with intravenous methylprednisolone after the initial assesment. This case series is presented to emphasize the differences in clinical presentation of SS and the importance of MRI and FFA in diagnosis.
Source: Multiple Sclerosis and Related Disorders - Category: Neurology Source Type: research