Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome.
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).
Dörr
Jan
J
NeuroCure Clinical Research Center Charité-Universitätsmedizin Berlin, Berlin, Germany.
Radbruch
Helena
H
Bock
Markus
M
Wuerfel
Jens
J
Brüggemann
Anne
A
Wandinger
Klaus P
KP
Zeise
Daniel
D
Pfueller
Caspar F
CF
Zipp
Frauke
F
Paul
Friedemann
F
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
D005343:Fibrinolytic Agents; D001241:Aspirin