Document Detail

Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome.
MedLine Citation:
PMID:  19953118     Owner:  NLM     Status:  MEDLINE    
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).
Jan D?rr; Helena Radbruch; Markus Bock; Jens Wuerfel; Anne Br?ggemann; Klaus P Wandinger; Daniel Zeise; Caspar F Pfueller; Frauke Zipp; Friedemann Paul
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Nature reviews. Neurology     Volume:  5     ISSN:  1759-4766     ISO Abbreviation:  Nat Rev Neurol     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-12-02     Completed Date:  2010-02-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101500072     Medline TA:  Nat Rev Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  683-8     Citation Subset:  IM    
NeuroCure Clinical Research Center Charit?-Universit?tsmedizin Berlin, Berlin, Germany.
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MeSH Terms
Aspirin / therapeutic use
Auditory Perceptual Disorders / etiology*
Brain / pathology*
Cerebral Angiography / methods
Diagnosis, Differential
Disease Progression
Fibrinolytic Agents / therapeutic use
Hearing Loss / etiology*
Magnetic Resonance Imaging / methods
Susac Syndrome / complications*,  diagnosis,  drug therapy
Vision Disorders / etiology*
Young Adult
Reg. No./Substance:
0/Fibrinolytic Agents; 50-78-2/Aspirin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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