Increased incidence of Susac syndrome: a case series study
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RESEARCH ARTICLE
Open Access
Increased incidence of Susac syndrome: a case series study A. Wilf-Yarkoni1* , O. Elkayam2,3, O. Aizenstein4, Y. Oron5, V. Furer2,3, D. Zur3,6, M. Goldstein3,6, D. Barequet3,6, H. Hallevi7, A. Karni3,7,8, Z. Habot-Wilner3,6 and K. Regev7
Abstract Background: Susac syndrome (SuS) is a rare condition characterized by a clinical triad of sensorineural hearing loss, branch artery occlusion and encephalopathy. This study reports an increased incidence of SuS in Israel. We describe the clinical characteristics of these patients, diagnostic procedures and the use and subsequent outcomes of newly published treatment guidelines. Methods: This is a single center retrospective study. Patients who were diagnosed with SuS between July 2017 and August 2018 were enrolled in this study. Results: Seven patients were diagnosed with SuS according to the diagnostic criteria in a time period of 13 months. The annual incidence was recently evaluated in Austria to be 0.024/100000, therefore, our case series represent at least a 5.4- fold increase in the annual incidence of SuS expected in Israel and a 7-fold increase in the annual incidence expected in our medical center. Mean time from the onset of the symptoms to diagnosis was three weeks and follow-up time was twenty four months. Recent exposure to cytomegalovirus was serologically evident in three patients and one patient had high titer of anti-streptolysin antibody. All patients underwent brain MRI, fluorescein angiography and audiometry. All patients were treated according to the newly recommended guidelines. All patients achieved clinical and radiological stability. Conclusions: We report of an increased incidence of SuS in Israel. Infectious serological findings may imply a post infectious mechanism. The use of the recommended diagnostic procedures reduced the time to diagnosis. Newly published treatment guidelines led to favorable clinical outcomes. Keywords: Susac syndrome, Treatment, cmv post infectious, Branch retinal artery occlusion
Background Susac syndrome (SuS) is a rare immune-mediated occlusive microvascular disease. It is characterized by a typical clinical triad of encephalopathy, visual disturbances and hearing loss [1–5]. However, there is a great variability in clinical manifestations and the complete triad is present in less than 20% of patients at disease onset [4– * Correspondence: [email protected] 1 Neuro-Immunology Service and Department of Neurology Rabin Medical Center, 4941492 Petach Tikva, Israel Full list of author information is available at the end of the article
9]. Treatment of SuS is particularly challenging, owning to its rarity, and the great variability in presentation, there are no randomized control trials to evaluate treatment strategies. Optimal outcome requires rapid and aggressive treatment, and acute treatments limited to glucocorticoids and/or IVIg appears to be insufficient to halt the progression of disease [10–15]. Recent treatment guidelines have been published based on disease severity [10]. The av
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