title: Susac syndrome: a report of four cases and a review of the literature.
abstract: In Susac syndrome, occlusions of pre-capillary arterioles of the brain, retina, and cochlea lead to the classical clinical triad of subacute encephalopathy, visual disturbances due to branch retinal artery occlusions and sensorineural hearing impairment. Its pathogenesis is still obscure, but it is presumed to be mediated by an autoimmune response to an as yet unknown antigen. The syndrome is considered a rare but important differential diagnosis in various neurological, psychiatric, ophthalmological, and ear-nose-throat disorders. Brain magnetic resonance imaging, retinal fluorescein angiography, and audiometry findings enable diagnosis. Early therapy may reduce relapses and improve recovery. The features of four cases of this syndrome are presented, illustrating that cooperation among different medical specialists is essential, and that treatment may be best guided by an immunologist or rheumatologist as a case manager.
authors.affiliation: Department of Rheumatology, Haga Hospital, The Hague, the Netherlands, Departments of Radiology, Neurology, Ophthalmology, Rheumatology, Leiden University Medical Centre, Leiden, the Netherlands, *corresponding author: email: s.m.vanderkooij@hagaziekenhuis.nl.
authors.forename: S M
authors.initials: SM
authors.lastname: van der Kooij
authors.forename: M A
authors.initials: MA
authors.lastname: van Buchem
authors.forename: O M
authors.initials: OM
authors.lastname: Overbeek
authors.forename: G
authors.initials: G
authors.lastname: Dijkman
authors.forename: T W J
authors.initials: TW
authors.lastname: Huizinga
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology; D002533:Cerebral Angiography / Q000379:methods*; D003937:Diagnosis, Differential; D005260:Female; D005451:Fluorescein Angiography / Q000379:methods*; D005654:Fundus Oculi; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D055955:Susac Syndrome / Q000000981:diagnostic imaging*; D055815:Young Adult