• Jan Dörr, Helena Radbruch, Markus Bock, Jens Wuerfel, Anne Brüggemann, Klaus P Wandinger, Daniel Zeise, Caspar F Pfueller, Frauke Zipp, and Friedemann Paul.
• NeuroCure Clinical Research Center Charité-Universitätsmedizin Berlin, Berlin, Germany.
• Nat Rev Neurol. 2009 Dec 1; 5 (12): 683-8.

BackgroundA 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.InvestigationsDetailed 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).DiagnosisSusac syndrome, characterized by a combination of encephalopathy, branch retinal artery occlusions, and hearing loss.ManagementLong-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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