Recurrent Bilateral Branch Retinal Artery Occlusion with Hearing Loss and Encephalopathy: The First Case Report of Susac Syndrome in Korea.
10.3346/jkms.2011.26.11.1518
- Author:
Soo Geun JOE
1
;
June Gone KIM
;
Sun Uck KWON
;
Choong Wook LEE
;
Hyun Woo LIM
;
Young Hee YOON
Author Information
1. Department of Ophthalmology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea. yhyoon@amc.seoul.kr
- Publication Type:Case Reports
- Keywords:
Susac Syndrome;
Korean;
Branch Retinal Artery Occlusion;
Hearing Loss;
Encephalopathy
- MeSH:
Autoimmune Diseases/diagnosis/drug therapy;
Azathioprine/administration & dosage/*therapeutic use;
Brain/blood supply/pathology;
Female;
Hearing Loss;
Humans;
Immunotherapy;
Magnetic Resonance Imaging;
Republic of Korea;
Retinal Artery Occlusion/diagnosis/drug therapy/pathology;
Susac Syndrome/*diagnosis/*drug therapy/pathology;
Young Adult
- From:Journal of Korean Medical Science
2011;26(11):1518-1521
- CountryRepublic of Korea
- Language:English
-
Abstract:
We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.
