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

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...

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Bibliographic Details
Published in:Journal of Korean medical science 2011, 26(11), 157, pp.1518-1521
Main Authors: Joe, Soo Geun, Kim, June-Gone, Kwon, Sun Uck, Lee, Choong Wook, Lim, Hyun Woo, Yoon, Young Hee
Format: Article
Language:English
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
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Summary: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.
ISSN:1011-8934
1598-6357
DOI:10.3346/jkms.2011.26.11.1518