Susac Syndrome – A Portuguese Experience

Introduction: Susac syndrome (SS) is characterized by microvascular occlusions in the brain, retina, and inner ear. Despite significant advances in understanding this disorder, it remains an under- and misdiagnosed entity in the clinical setting. Methods: This study was retrospective review of clinical records. Results: We present the cases of three young adults complaining of visual loss, hearing impairment, and headache. In all patients, the fundoscopy and fluorescein angiography revealed nonperfused arteriolar segments and multifocal arterial vasculitis. The brain magnetic resonance imaging demonstrated characteristic white matter brain lesions and the audiometry revealed low-frequency hearing loss. The diagnosis of SS was therefore established. We present and discuss the different treatment regimens applied and the course and outcomes of each case. Conclusion: SS is a rare and complex disease with possibly severe consequences that must be promptly diagnosed and treated. However, as no prospective or randomized controlled trials exist, empirical treatment strategies are still the norm, mainly based on the case reports and series.