Dana Barequet1, Yahav Oron2, Ori Elkayam3, Arnon Karni4,5, Adi Wilf-Yarkoni6, Keren Regev4, Zohar Habot-Wilner7. 1. Division of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 2. Department of Ear, Nose and Throat, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 3. Department of Rheumatology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 4. The Neuroimmunology and Multiple Sclerosis Unit, Neurology Division, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. 5. Sackler Faculty of Medicine and Sagol School of Neuroscience, Tel Aviv University, Tel Aviv, Israel. 6. Neuro-Immunology Service and Department of Neurology Rabin Medical Center, Petah Tikva, Israel. 7. Division of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. zwilner@hotmail.com.
Abstract
BACKGROUND: Susac's syndrome, a rare autoimmune vasculo-occlusive disease, may pose a diagnostic challenge and result in a devastating ocular and systemic outcome. Our study identifies a new retinal finding and evaluates disease outcome. We aimed to assess clinical and imaging findings, systemic manifestations and disease outcome in patients with ocular Susac's syndrome under immunosuppressive/immunomodulation therapies. METHODS: Retrospective tertiary center study including patients with a diagnosis of Susac's syndrome with >12 months follow up. Medical record review including ocular, neurological and auditory clinical and imaging findings, and treatment modalities. Main outcome measures were clinical manifestations and disease outcome. RESULTS: Seven patients (14 eyes) with a mean age of 34.1 years were included. Mean follow-up was 31.9 months (12.4-72.4). All had bilateral ocular disease. Retinal microaneurysms, a new ocular finding, were demonstrated in 5 patients and persisted at the final visit. In 5 eyes, they further extended during follow-up. All were treated with immunosuppressive drugs and 5/7 additional immunomodulation therapy. At last examination, best corrected visual acuity was >20/40 in all eyes, 1/10 eyes had visual field deterioration, no eye had active ocular disease, all patients achieved neurological stability, and 1 patient had auditory deterioration. CONCLUSION: Retinal microaneurysms, a new ocular finding in Susac's syndrome, were present in most of our patients, indicating ischemic retinal damage. Immunosuppressive and immunomodulation therapies seem to be highly effective in the control of disease activity.
BACKGROUND: Susac's syndrome, a rare autoimmune vasculo-occlusive disease, may pose a diagnostic challenge and result in a devastating ocular and systemic outcome. Our study identifies a new retinal finding and evaluates disease outcome. We aimed to assess clinical and imaging findings, systemic manifestations and disease outcome in patients with ocular Susac's syndrome under immunosuppressive/immunomodulation therapies. METHODS: Retrospective tertiary center study including patients with a diagnosis of Susac's syndrome with >12 months follow up. Medical record review including ocular, neurological and auditory clinical and imaging findings, and treatment modalities. Main outcome measures were clinical manifestations and disease outcome. RESULTS: Seven patients (14 eyes) with a mean age of 34.1 years were included. Mean follow-up was 31.9 months (12.4-72.4). All had bilateral ocular disease. Retinal microaneurysms, a new ocular finding, were demonstrated in 5 patients and persisted at the final visit. In 5 eyes, they further extended during follow-up. All were treated with immunosuppressive drugs and 5/7 additional immunomodulation therapy. At last examination, best corrected visual acuity was >20/40 in all eyes, 1/10 eyes had visual field deterioration, no eye had active ocular disease, all patients achieved neurological stability, and 1 patient had auditory deterioration. CONCLUSION: Retinal microaneurysms, a new ocular finding in Susac's syndrome, were present in most of our patients, indicating ischemic retinal damage. Immunosuppressive and immunomodulation therapies seem to be highly effective in the control of disease activity.
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Authors: Ilka Kleffner; Jan Dörr; Marius Ringelstein; Catharina C Gross; Yvonne Böckenfeld; Wolfram Schwindt; Benedikt Sundermann; Hubertus Lohmann; Heike Wersching; Julia Promesberger; Natascha von Königsmarck; Anne Alex; Rainer Guthoff; Catharina J M Frijns; L Jaap Kappelle; Sven Jarius; Brigitte Wildemann; Orhan Aktas; Friedemann Paul; Heinz Wiendl; Thomas Duning Journal: J Neurol Neurosurg Psychiatry Date: 2016-10-25 Impact factor: 10.154
Authors: Jan Dörr; Sarah Krautwald; Brigitte Wildemann; Sven Jarius; Marius Ringelstein; Thomas Duning; Orhan Aktas; Erich Bernd Ringelstein; Friedemann Paul; Ilka Kleffner Journal: Nat Rev Neurol Date: 2013-04-30 Impact factor: 42.937
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