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SHR Neuro Cancer Cardio Lipid

Seifert-Held, T; Langner-Wegscheider, BJ; Komposch, M; Simschitz, P; Franta, C; Teuchner, B; Offenbacher, H; Otto, F; Sellner, J; Rauschka, H; Fazekas, F.
Susac's syndrome: clinical course and epidemiology in a Central European population.
Int J Neurosci. 2017; 127(9):776-780
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Authors Med Uni Graz:
Fazekas Franz
Langner-Wegscheider Beate-Julia
Seifert-Held Thomas
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Abstract:
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. 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. 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. We provide for the first time population-based data about the clinical course, prevalence and incidence of Susac's syndrome.
Find related publications in this database (using NLM MeSH Indexing)
Adult -
Aged -
Austria - epidemiology
Community Health Planning -
Female -
Humans -
Incidence -
Magnetic Resonance Imaging -
Male -
Middle Aged -
Prevalence -
Retrospective Studies -
Susac Syndrome - diagnostic imaging
Susac Syndrome - epidemiology
Susac Syndrome - therapy
Young Adult -

Find related publications in this database (Keywords)
Susac's syndrome
retinal vasculitis
epidemiology
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