Susac's syndrome: Clinical characteristics, clinical classification and long term prognosis

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.