title: [Susac syndrome: an interdisciplinary challenge].
abstract: Susac syndrome, named after John Susac, the first to describe this condition, is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Although certainly a rare disease, Susac syndrome needs to be considered in the differential diagnosis of a broad variety of diseases. The pathogenesis is not yet clear. Autoimmune processes leading to damage and inflammation-related occlusion of the microvessels in brain, retina, and inner ear are thought to play a causal role. The diagnosis is based primarily on the clinical presentation, the documentation of branch retinal artery occlusion by fluorescence angiography, and characteristic findings on cerebral MRI. Usually, immunosuppressive therapy is required, though controlled therapy trials are missing so far. The intention of this review article is to raise awareness of this disease among neurologists, psychiatrists, ophthalmologists, and ENT specialists as a high number of unreported cases probably exists. Accordingly, the focus is on the clinical presentation and the diagnostic approach.
authors.affiliation: NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Deutschland. jan-markus.doerr@charite.de
authors.forename: J
authors.initials: J
authors.lastname: Dörr
authors.forename: S
authors.initials: S
authors.lastname: Jarius
authors.forename: B
authors.initials: B
authors.lastname: Wildemann
authors.forename: E B
authors.initials: EB
authors.lastname: Ringelstein
authors.forename: W
authors.initials: W
authors.lastname: Schwindt
authors.forename: M
authors.initials: M
authors.lastname: Deppe
authors.forename: K P
authors.initials: KP
authors.lastname: Wandinger
authors.forename: J
authors.initials: J
authors.lastname: Promesberger
authors.forename: F
authors.initials: F
authors.lastname: Paul
authors.forename: I
authors.initials: I
authors.lastname: Kleffner
mesh_terms: D003299:Cooperative Behavior; D003337:Corpus Callosum / Q000473:pathology; D003937:Diagnosis, Differential; D038524:Diffusion Magnetic Resonance Imaging; D005451:Fluorescein Angiography; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D033183:Interdisciplinary Communication; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D011379:Prognosis; D015356:Retinal Artery Occlusion / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D055955:Susac Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy
chemical_list: D007166:Immunosuppressive Agents