[Susac syndrome: a report of two cases].

Zhonghua Nei Ke Za Zhi

Department of Neurology, the First Affiliated Hospital, Chongqing University of Medical Sciences, Chongqing 400016, China.

Published: December 2003

Objective: To investigate the clinic characters and diagnosis of Susac syndrome.

Methods: Two cases were systematically studied by the authors.

Results: All the two cases were young women. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid-to-low frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2-weighted brain magnetic resonance imaging.

Conclusions: This rare syndrome often can be identified at an early stage with a careful history and physical examination. MRI, SPECT, retinal fluorescein angiography and audiometry will contribute to diagnosis.

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