September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Susac's syndrome: Clinical characteristics, clinical classification and long term prognosis
Author Affiliations & Notes
  • Ofira Zloto
    Department of Ophthalmology, Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel
  • Joav Chapman
    Department of Neurology and the Sagol Neuroscience Center, Sheba Medical Center, Tel Hashomer, Israel
  • Roee Sheinfeld
    Department of Neurology and the Sagol Neuroscience Center, Sheba Medical Center, Tel Hashomer, Israel
  • Tal Sharon
    Department of Ophthalmology, Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel
  • Ruth Huna- Baron
    Department of Ophthalmology, Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel
  • Riri manor
    Department of Ophthalmology, Rabin Medical center, Petah Tiqva, Israel
  • Yehuda Shoenfeld
    Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Israel
  • Vicktoria Vishnevskia Dai
    Department of Ophthalmology, Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel
  • Footnotes
    Commercial Relationships   Ofira Zloto, None; Joav Chapman, None; Roee Sheinfeld, None; Tal Sharon, None; Ruth Huna- Baron, None; Riri manor, None; Yehuda Shoenfeld, None; Vicktoria Vishnevskia Dai, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 4124. doi:
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      Ofira Zloto, Joav Chapman, Roee Sheinfeld, Tal Sharon, Ruth Huna- Baron, Riri manor, Yehuda Shoenfeld, Vicktoria Vishnevskia Dai; Susac's syndrome: Clinical characteristics, clinical classification and long term prognosis. Invest. Ophthalmol. Vis. Sci. 2016;57(12):4124.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Susac`s syndrome (SUS) is a rare syndrome characterized by clinical triad of: CNS dysfunction, sensorineural hearing impairment and branch retinal artery occlusion (BRAO). The purpose of this study is to examine the demographic, clinical characteristics, treatment and long term prognosis of SUS.

Methods : The following data was recorded for all Susac's syndrome patients treated at Sheba Medical Center, between the years 1998 – 2014: demographic data, clinical signs at presentation and during the disease, imaging findings, treatment and prognosis.

Results : SUS was diagnosed in ten patients. Only two patients present with full triad. However, seven patients developed a full triad during the follow up period. The average time to full triad was seven months. Based on our observation at presentation we classified the disease into three courses: (1) Suspected SUS, (2) Incomplete SUS (3) Complete SUS.
All patients were treated at diagnosis with a pulse of high dose intravenous methylprednisolone. Improvement in visual acuity and visual field was noted at the end of the follow up time, yet not statically significant (p= 0.479, 0.053 respectively, matched pairs). Five patients (50%) had neurological damage at the end of the study. Five patients (50%) had no improvement in hearing loss.

Conclusions : SUS is a rare syndrome that can mimic other disorders. The diagnosis is challenging because most of the patients do not initially present with the tried. We suggest a clinical classification for the syndrome that may assist in early diagnosis of the syndrome. Early diagnosis may lead to better prognosis in those young patients.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

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