Journal
NATURE REVIEWS NEUROLOGY
Volume 5, Issue 12, Pages 683-688Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nrneurol.2009.176
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- Deutsche Forschungsgemeinschaft (DFG) [SFB 650, SFB-TRR 43, Exc 257]
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Background. A 23 year-old female presented to a neurology department with a 3 year history of recurrent episodes involving hearing loss, encephalopathy, focal neurological deficits, and visual field deficits. In the 3 years before presentation, the patient had been treated with methylprednisolone for suspected acute demyelinating encephalomyelitis and peripheral otogenic dysfunction from which she made a complete recovery, and for a visual defect in both eyes caused by bilateral branch retinal arterial occlusion, from which she partially improved and commenced long-term treatment with acetylsalicylic acid. Investigations. Detailed history, clinical examination, extensive laboratory work-up, cerebrospinal fluid analysis, cerebral and spinal MRI, periventricular single-voxel H-1 magnetic resonance spectroscopy, retinal fluorescence angiography, optical coherence tomography, audiometry, neurophysiological work-up (EEG, evoked potentials). Diagnosis. Susac syndrome, characterized by a combination of encephalopathy, branch retinal artery occlusions, and hearing loss. Management. Long-term immunosuppressive treatment with azathioprine (150 mg/day) and prednisolone (10 mg/day), and inhibition of thrombocyte function with acetylsalicylic acid (100 mg/day).
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