title: Susac's syndrome: clinical course and epidemiology in a Central European population.
abstract: OBJECTIVE
  Susac's syndrome is characterized by inflammation and occlusion of pre-capillary arterioles with the clinical triad of branch retinal artery occlusion (BRAO), encephalopathy and hearing loss. No epidemiological data are available for the disease.
  
  
  METHODS
  All neurology departments in Austria were addressed to report adult patients who were on immunosuppressive treatment for a diagnosis of Susac's syndrome between 1 August 2010 and 1 August 2015. Clinical course, treatment regimens, period and point prevalence rates, and annual incidence of Susac's syndrome in Austria in people over 19 years of age are reported.
  
  
  RESULTS
  Ten patients with Susac's syndrome were identified, and eight of them were newly diagnosed within the five-year timeframe. Minimum five-year period prevalence of the disease is 0.148/100,000 (95% confidence interval (CI) 0.071-0.272), annual incidence is 0.024/100,000 (95% CI 0.010-0.047). Minimum point prevalence rates varied from 0.030/100,000 (95% CI 0.004-0.108) to 0.088/100,000 (95% CI 0.032-0.192). Of all 10 patients, 8 showed typical callosal or internal capsule magnetic resonance imaging lesions at first presentation, 7 presented with BRAO and 5 had hearing loss or tinnitus at the beginning of the disease. Four patients developed the complete clinical triad of Susac's syndrome during the observation period.
  
  
  CONCLUSIONS
  We provide for the first time population-based data about the clinical course, prevalence and incidence of Susac's syndrome.
authors: a Department of Neurology , Medical University of Graz , Graz , Austria.
authors: Thomas
authors: T
authors: Seifert-Held
authors: b Department of Ophthalmology , Medical University of Graz , Graz , Austria.
authors: Beate J
authors: BJ
authors: Langner-Wegscheider
authors: c Department of Neurology , Klinikum Klagenfurt , Klagenfurt , Austria.
authors: Martina
authors: M
authors: Komposch
authors: d Department of Neurology , Klinikum Klagenfurt , Klagenfurt , Austria.
authors: Philipp
authors: P
authors: Simschitz
authors: e Department of Neurology , University Hospital St. Pölten , St. Pölten , Austria.
authors: Claudia
authors: C
authors: Franta
authors: f Department of Ophthalmology , Medical University of Innsbruck , Innsbruck , Austria.
authors: Barbara
authors: B
authors: Teuchner
authors: g Department of Neurology , Landeskrankenhaus Judenburg-Knittelfeld , Knittelfeld , Austria.
authors: Hans
authors: H
authors: Offenbacher
authors: h Department of Neurology , Paracelsus Medical University , Salzburg , Austria.
authors: Ferdinand
authors: F
authors: Otto
authors: i Department of Neurology , Paracelsus Medical University , Salzburg , Austria.
authors: Johann
authors: J
authors: Sellner
authors: j Department of Neurology , Sozialmedizinisches Zentrum Ost , Wien , Austria.
authors: Helmut
authors: H
authors: Rauschka
authors: k Department of Neurology , Medical University of Graz , Graz , Austria.
authors: Franz
authors: F
authors: Fazekas
mesh_terms: D000328:Adult; D000368:Aged; D001317:Austria / Q000453:epidemiology; D018672:Community Health Planning; D005260:Female; D006801:Humans; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D055955:Susac Syndrome / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000628:therapy*; D055815:Young Adult
keywords: Susac's syndrome; epidemiology; retinal vasculitis