title: Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome.
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.
  
  
  METHODS
  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).
  
  
  METHODS
  Susac syndrome, characterized by a combination of encephalopathy, branch retinal artery occlusions, and hearing loss.
  
  
  RESULTS
  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).
authors: NeuroCure Clinical Research Center Charité-Universitätsmedizin Berlin, Berlin, Germany.
authors: Jan
authors: J
authors: Dörr
authors: Helena
authors: H
authors: Radbruch
authors: Markus
authors: M
authors: Bock
authors: Jens
authors: J
authors: Wuerfel
authors: Anne
authors: A
authors: Brüggemann
authors: Klaus P
authors: KP
authors: Wandinger
authors: Daniel
authors: D
authors: Zeise
authors: Caspar F
authors: CF
authors: Pfueller
authors: Frauke
authors: F
authors: Zipp
authors: Friedemann
authors: F
authors: Paul
mesh_terms: D001241:Aspirin / Q000627:therapeutic use; D001308:Auditory Perceptual Disorders / Q000209:etiology*; D001921:Brain / Q000473:pathology*; D002533:Cerebral Angiography / Q000379:methods; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D055955:Susac Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D014786:Vision Disorders / Q000209:etiology*; D055815:Young Adult
chemical_list: D005343:Fibrinolytic Agents; D001241:Aspirin