Increased Incidence Of Susac Syndrome: A Case Series Study

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.

Diagnosis, differential diagnosis and misdiagnosis of Susac syndrome

“…It is characterized by the clinical triad of encephalopathy, branch retinal artery occlusions (BRAOs) and sensorineural hearing loss (SNHL), usually affecting low‐ to mid‐frequencies [4,5]. The annual incidence of Susac syndrome is estimated to be in the range 0.024–0.13 per 100,000 [6,7], with 60% of individuals aged between 21 and 35 years at the time of diagnosis and a reported male:female ratio of 1:3.5 [4].…”

“…The annual incidence of Susac syndrome is estimated to be in the range 0.024-0.13 per 100,000 [6,7], with 60% of individuals aged between 21 and 35 years at the time of diagnosis and a reported male:female ratio of 1:3.5 [4].…”

Susac Syndrome: Collaboration is Required for a Diagnosis

“…With only about 400 cases of SUSAC reported worldwide, this uncommon but possibly underdiagnosed disease can present with a typical triad of-neurologic signs, branch retinal artery occlusion and sensorineural hearing loss [1][2][3]. However, less than 15%-20% of cases have all three symptoms at presentation [2][3][4]. Our patient presented with only 2 associated almost daily episodes of visual disturbances, paresthesia of the lips and intermittent paresthesia of the extremities with increasing fatigue.…”

Susac syndrome and pregnancy: a review of published cases and considerations for patient management

“…We searched the literature (Pubmed) and internet (Google) for published case reports, case series and review articles for descriptions of SuS patients during pregnancy, postpartum or after termination of pregnancy (search terms: Susac, pregnancy, postpartum; search until August 2020) and selected 27 reports describing a total of 33 SuS patients. 11 – 37 All cases are listed in Table 1 . The mean age at pregnancy was 28.6 years.…”