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

Item Type:Article
Title:Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome
Creators Name:Doerr, J., Radbruch, H., Bock, M., Wuerfel, J., Brueggemann, A., Wandinger, K.P., Zeise, D., Pfueller, C.F., Zipp, F. and Paul, F.
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).
Keywords:Aspirin, Auditory Perceptual Disorders, Brain, Cerebral Angiography, Differential Diagnosis, Disease Progression, Fibrinolytic Agents, Hearing Loss, Magnetic Resonance Imaging, Susac Syndrome, Vision Disorders
Source:Nature Reviews Neurology
Publisher:Nature Publishing Group
Page Range:683-688
Date:December 2009
Official Publication:https://doi.org/10.1038/nrneurol.2009.176
PubMed:View item in PubMed

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