12
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Susac syndrome is a rare condition characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). The purpose of this study is to examine the demographics, clinical characteristics, treatment, and long-term prognosis of Susac syndrome. The data recorded for all Susac syndrome patients treated at the Sheba Medical Center between 1998 and 2014 included demographics, clinical signs at presentation and during the disease course, imaging findings, treatment, and prognosis.

          Susac syndrome was diagnosed in 10 patients (age range 30–45 years). Only 2 patients presented with the full triad and 7 patients developed the full triad during mean follow-up period of 35 months. The average time to full triad was 7 months. Based on our observations at presentation, we divided the disease course into suspected, incomplete, and complete Susac syndrome. All 10 patients were treated at diagnosis with a pulse of high-dose intravenous methylprednisolone. There was improvement in visual acuity and visual field at the end of follow-up compared to baseline, but it was not statistically significant ( P = 0.479 and P = 0.053, respectively). Five patients remained with neurological damage, and 5 patients had no improvement of their hearing loss at study closure. In conclusion, Susac syndrome is a rare condition that can mimic other disorders. The diagnosis is challenging because most patients do not initially present with the definitive triad. We suggest a clinical classification for the syndrome that may assist in early diagnosis.

          Related collections

          Most cited references28

          • Record: found
          • Abstract: found
          • Article: not found

          Characteristics of Susac syndrome: a review of all reported cases.

          In Susac syndrome, occlusions of microvessels--presumed to be mediated by an autoimmune response to an as yet unknown antigen--lead to a characteristic clinical triad of CNS dysfunction, branch retinal artery occlusions, and sensorineural hearing impairment. Susac syndrome is considered a rare but important differential diagnosis in numerous neurological, psychiatric, ophthalmological, and ear, nose and throat disorders. Improved understanding of this disorder is crucial, therefore, to ensure that patients receive appropriate treatment and care. Current knowledge on Susac syndrome is largely based on reports of single patients, small case series, and nonsystematic reviews. The aim of this Review is to extend these previous, primarily anecdotal findings by compiling data from all 304 cases of Susac syndrome that have been published worldwide, which were identified following a literature search with predefined search, inclusion and exclusion criteria. From this data, we present an overview of demographic, clinical and diagnostic data on Susac syndrome, providing a reliable basis for our current understanding of this rare disease. Where possible, we make recommendations for clinical diagnosis, differential diagnosis, and management of patients with suspected Susac syndrome.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Microangiopathy of the brain and retina.

            Two women (26 and 40 years old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal evidence of the known vasculitides, including systemic lupus erythematosus (SLE) and syphilis. Cerebral angiography suggested vasculitis in the younger patient. Brain biopsy in the older patient (after 3 months of steroid therapy) revealed a sclerosis of the small pial and cortical vessels that was consistent with a "healed" angitis. Both patients seemed to respond to steroid therapy.
              Bookmark
              • Record: found
              • Abstract: not found
              • Article: not found

              Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women.

              John Susac (1994)
                Bookmark

                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                October 2016
                28 October 2016
                : 95
                : 43
                : e5223
                Affiliations
                [a ]Goldschleger Eye Institute
                [b ]Department of Neurology, Sagol Neuroscience Center, Tel Hashomer
                [c ]Department of Ophthalmology, Sheba Medical Center, Tel Hshomer
                [d ]Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, are affiliated to the Sackler faculty of medicine Tel Aviv University, Israel.
                Author notes
                []Correspondence: Vicktoria Vishnevskia-Dai, Goldschleger Eye Institute; Department of Ophthalmology, Sheba Medical Center, Tel Hshomer, Tel Aviv University, Israel. 52621, Israel (e-mail: vivida65@ 123456gmail.com ).
                Article
                05223
                10.1097/MD.0000000000005223
                5089114
                27787385
                86a0bab8-d01d-4de0-92c6-0f171c39ce68
                Copyright © 2016 the Author(s). Published by Wolters Kluwer Health, Inc. All rights reserved.

                This is an open access article distributed under the Creative Commons Attribution-NoDerivatives License 4.0, which allows for redistribution, commercial and non-commercial, as long as it is passed along unchanged and in whole, with credit to the author. http://creativecommons.org/licenses/by-nd/4.0

                History
                : 4 November 2015
                : 1 October 2016
                : 4 October 2016
                Categories
                5800
                Research Article
                Observational Study
                Custom metadata
                TRUE

                branch retinal artery occlusions,cns dysfunction,sensorineural hearing impairment,susac syndrome

                Comments

                Comment on this article