TY - JOUR
T1 - Susac's syndrome
T2 - clinical course and epidemiology in a Central European population
AU - Seifert-Held, Thomas
AU - Langner-Wegscheider, Beate J
AU - Komposch, Martina
AU - Simschitz, Philipp
AU - Franta, Claudia
AU - Teuchner, Barbara
AU - Offenbacher, Hans
AU - Otto, Ferdinand
AU - Sellner, Johann
AU - Rauschka, Helmut
AU - Fazekas, Franz
N1 - Publisher Copyright:
© 2016 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2017/9/2
Y1 - 2017/9/2
N2 - 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.
AB - 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.
KW - Adult
KW - Aged
KW - Austria/epidemiology
KW - Community Health Planning
KW - Female
KW - Humans
KW - Incidence
KW - Magnetic Resonance Imaging
KW - Male
KW - Middle Aged
KW - Prevalence
KW - Retrospective Studies
KW - Susac Syndrome/diagnostic imaging
KW - Young Adult
UR - http://www.scopus.com/inward/record.url?scp=84994846430&partnerID=8YFLogxK
U2 - 10.1080/00207454.2016.1254631
DO - 10.1080/00207454.2016.1254631
M3 - Journal article
C2 - 27788613
SN - 0020-7454
VL - 127
SP - 776
EP - 780
JO - International Journal of Neuroscience
JF - International Journal of Neuroscience
IS - 9
ER -