Korean J Ophthalmol.  2013 Oct;27(5):381-383. 10.3341/kjo.2013.27.5.381.

A Case of Susac Syndrome

• Cho HJ
• Kim CG
• Cho SW
• Kim JW

• KMID: 1707294
• DOI: http://doi.org/10.3341/kjo.2013.27.5.381

Abstract

The purpose of this article is to report on the first known Korean case of Susac syndrome. An 18-year-old female came to our clinic reporting blurred vision of the left eye for 2 days. She also complained of decreased hearing with tinnitus of the right ear and mild headache. She was previously healthy and had no remarkable medical history. Best-corrected visual acuity was 20 / 50 in the left eye and 20 / 20 in the right eye. An axiomatic triad of ocular, cochlear, and neurologic involvement was observed in the patient. Fluorescein angiography showed branched retinal arterial occlusions in the left eye. A sudden right sensorineural hearing loss was observed on audimetry. Magnetic resonance images showed a hyperintense lesion in the white matter around the corpus callosum. The patient was treated with high doses of systemic corticosteroids, and no neuropsychological sequelae were observed. This is the first case report of Susac syndrome in Korea. In cases of retinal arterial occlusion with hearing loss or neuropsychological symptoms, Susac syndrome should be suspected.

Keyword

Encephalopathy; Hearing loss; Retinal artery occlusion; Susac syndrome

MeSH Terms

Adolescent
Diagnosis, Differential
Female
Fluorescein Angiography
Fundus Oculi
Hearing
Hearing Loss, Sensorineural/*diagnosis/physiopathology
Humans
Magnetic Resonance Imaging
Retinal Artery Occlusion/*diagnosis/physiopathology
Susac Syndrome/*diagnosis/physiopathology
Visual Acuity

Figure

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  Fig. 1 (A) Fundus photography of the left eye showing ischemic retinal edema inferotemporally and narrowed branch artery superonasally. (B) Fluorescein angiography of the same patient showing arterial wall hyperfluorescence and branch retinal artery obstruction.

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  Fig. 2 (A) Mid sagittal T1-weighted magnetic resonance imaging (MRI) of the same patient showing punctuate hypointense foci within the corpus callosum (arrow). (B) Transverse, T2-weighted MRI showing a hyperintense foci in the mid white matter around the corpus callosum (arrow).

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