Susac's syndrome: Clinical course and epidemiology in a Central European population.

Related Articles

Susac's syndrome: Clinical course and epidemiology in a Central European population.

Int J Neurosci. 2016 Oct 27;:1-16

Authors: Seifert-Held T, Langner-Wegscheider BJ, Komposch M, Simschitz P, Franta C, Teuchner B, Offenbacher H, Otto F, Sellner J, Rauschka H, Fazekas F

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 is 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(st) August 2010 and 1(st) 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: 10 patients with Susac's syndrome were identified, 8 of them were newly diagnosed within the 5-year timeframe. Minimum 5-year period prevalence of the disease is 0.148/100,000 (95% 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 MRI 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.

PMID: 27788613 [PubMed - as supplied by publisher]

http://ift.tt/2fjgcNX