Susac syndrome: Study of five cases

Introduction. - Susac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss. Methods. - We report a series of five patients with Susac syndrome followed in our department from 1997 to 2007. Results. - There were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n = 1), ophthalmological (n = 1), auditory (n = 1) and clinical triad (n = 2). Neurologic symptoms included encephalopathy (n = 2), headache (n = 5), transient ischemic attacks (n = 1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n = 4], cylophosphamid [n = 3], intravenous immunoglobulins [n = 5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n = 2), led to dramatic clinical improvement (n = 5). Conclusion. - Due to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis. (C) 2008 Elsevier Masson SAS. All rights reserved.