title: Diagnosis and classification of Susac syndrome.
abstract: Susac syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and neuro-sensorial hearing loss; it is due to a microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear. SS is characterized by typical radiological features on magnetic resonance imaging (MRI) which, together with clinical symptoms, may permit a diagnosis. Branch retinal artery occlusions (BRAOs) are best evaluated using fluorescein angiography (FA) which may show the typical multifocal fluorescence. SS is an autoimmune endotheliopathy that requires treatment with immunosuppressive agents: steroids, azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide and intravenous immunoglobulin, usually in combination. Plasma exchange is also useful. In addition, antiplatelet agents may be a useful adjunct. Correct immunosuppressive therapy results in significant clinical and radiological improvement. An early diagnosis and treatment are important to delay the disease progression and prevent permanent disability.
authors.affiliation: Systemic Autoimmune Diseases Research Unit, HGR 36, Instituto Mexicano del Seguro Social, Puebla, Mexico; Department of Rheumatology and Immunology, Benemérita Universidad Autónoma de Puebla, Mexico.
authors.forename: Mario
authors.initials: M
authors.lastname: García-Carrasco
authors.affiliation: Systemic Autoimmune Diseases Research Unit, HGR 36, Instituto Mexicano del Seguro Social, Puebla, Mexico.
authors.forename: Claudia
authors.initials: C
authors.lastname: Mendoza-Pinto
authors.affiliation: Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain. Electronic address: rcervera@clinic.ub.es.
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
mesh_terms: D001921:Brain / Q000098:blood supply / Q000473:pathology; D034381:Hearing Loss / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D015356:Retinal Artery Occlusion / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D055955:Susac Syndrome / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology