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Nat Rev Neurol. 2009 Dec;5(12):683-8. doi: 10.1038/nrneurol.2009.176.

Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome.

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  • 1NeuroCure Clinical Research Center Charité-Universitätsmedizin Berlin, Berlin, Germany.

Abstract

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 (1)H 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).

[PubMed - indexed for MEDLINE]
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