ePoster 

Die hochgeladenen ePoster sind nur für eingeloggte Teilnehmer ersichtlich. 

Bitte loggen Sie sich ein, um zu den ePoster zu gelangen. 

Hier geht es zum Log- in

 

Detaillierte Beitrags-Information

Zurück zur Liste

Beitragstitel Susac syndrome: a case series
Beitragscode P75
Autor:innen
  1. Florence Hoogewoud Hopital Ophtalmique Jules Gonin, UNIL Präsentierende:r
  2. François-Xavier Borruat Hôpital Ophtalmique Jules Gonin, Université de Lausanne
  3. Aki Kawasaki Hôpital ophtalmique Jules-Gonin, Fondation Asile des Aveugles
  4. Yan Guex-Crosier Hôpital ophtalmique Jules-Gonin, Fondation Asile des Aveugles
Präsentationsform ePoster
Themengebiete
  • Uveitis / Intraocular Inflammation
Abstract-Text Purpose: Susac syndrome is an immune-mediated occlusive microvascular endotheliopathy. It is characterized by a triad of branch retinal artery occlusion, encephalopathy and low-frequency sensorineural hearing loss.

Methods: Retrospective case series of patients seen at our institution for Susac syndrome between 2005 and 2020. Demographics, clinical characteristics, treatment and outcome were reported.

Results: Four patients (2 males) were included in the study. According to the newly published diagnostic criteria, 3 patients had a definite and one patient had a probable Susac syndrome (distinctive ophthalmological and ear involvement without cerebral involvement). Mean age at onset was 29 years old (range 24-33). The first symptoms were neurological in 3 patients and ophthalmological in 1 patient (visual field impairment). All four patients complained also of hearing loss. Initial ophthalmological status revealed a normal (10/10) visual acuity (VA) in all patients. One patient presented a unilateral quadrantanopsia. There was neither anterior chamber nor vitreous inflammation. Gass plaques (defined as yellow-white plaques found in the arteriolar wall away from arterial bifurcations) were present on fundus examination in all patients. Fluorescein angiography revealed arteriolar vascular leakage in the absence of other signs of intraocular inflammation in all patients. Initial treatment consisted in intravenous corticosteroid (CS) pulse therapy in the 3 definite Susac and of high dose oral CS in the probable Susac. Additional therapy consisted of azathioprine in two patients and intravenous immunoglobulins plus mycophenolate mofetil in the two others. The last two patients additionally needed cyclophosphamide pulse therapy in order to control the disease. Remission without treatment was achieved in three patients after a mean time of 7 years (2, 7.5 and 11.5 years respectively).

Conclusion: Susac syndrome is a rare disease with characteristic ophthalmological manifestation. Gass plaques should be looked for in young patients presenting with acute neurological symptoms and/or hearing loss. Prompt recognition of the disease and treatment can avoid long term disabilities.