Recurrent bilateral branch retinal artery occlusion with hearing loss and encephalopathy: the first case report of Susac syndrome in Korea

• Published in: Journal of Korean medical science Vol. 26; no. 11; pp. 1518 - 1521
• Main Authors: Joe, Soo Geun, Kim, June-Gone, Kwon, Sun Uck, Lee, Choong Wook, Lim, Hyun Woo, Yoon, Young Hee
• Format: Journal Article
• Language: English
• Published: Korea (South) The Korean Academy of Medical Sciences 01-11-2011; 대한의학회
• Subjects: Autoimmune Diseases - diagnosis; Autoimmune Diseases - drug therapy; Azathioprine - administration & dosage; Azathioprine - therapeutic use; Brain - blood supply; Brain - pathology; Case Report; Female; Hearing Loss; Humans; Immunotherapy; Magnetic Resonance Imaging; Republic of Korea; Retinal Artery Occlusion - diagnosis; Retinal Artery Occlusion - drug therapy; Retinal Artery Occlusion - pathology; Susac Syndrome - diagnosis; Susac Syndrome - drug therapy; Susac Syndrome - pathology; Young Adult; 의학일반; Republic of Korea; Korean; Branch Retinal Artery Occlusion; Susac Syndrome; Encephalopathy; Hearing Loss
• ISSN: 1011-8934; 1598-6357
• DOI: 10.3346/jkms.2011.26.11.1518

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.