J Korean Med Sci.  2011 Nov;26(11):1518-1521. 10.3346/jkms.2011.26.11.1518.

Recurrent Bilateral Branch Retinal Artery Occlusion with Hearing Loss and Encephalopathy: The First Case Report of Susac Syndrome in Korea

• Joe SG
• Kim JG
• Kwon SU
• Lee CW
• Lim HW
• Yoon YH

• KMID: 1123456
• DOI: http://doi.org/10.3346/jkms.2011.26.11.1518

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.

Keyword

Susac Syndrome; Korean; Branch Retinal Artery Occlusion; Hearing Loss; Encephalopathy

MeSH Terms

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

Figure

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  Fig. 1 Fundus photo showing edematous lesions in the supra-temporal area (A). Fluorescein angiography, showing a hyperfluorescent arterial wall proximal to the obstructed branch retinal artery (B).

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  Fig. 2 Visual field test results, showing an infranasal field defect of the left eye corresponding to the obstructed lesion and a small area of visual field defect in the right eye.

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  Fig. 3 Pure tone audiometry, showing decreased sensitivity to low-frequency sounds in the left ear.

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  Fig. 4 Magnetic resonance image of the brain. (A, B) Diffusion weighted images, showing a diffusion restricted lesion in the corpus callosum and left fornix. (C) Sagittal Fluid-attenuated inversion recovery (FLAIR) image showing a focal signal change in the corpus callosum.

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  Fig. 5 Fluorescein angiography showing hyperfluorescent vasculitis lesions (arrows) (A-D) and an obstructed branch retinal artery (arrowheads) (D).

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