Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome.
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Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. / Dörr, Jan; Radbruch, Helena; Bock, Markus; Wuerfel, Jens; Brüggemann, Anne; Wandinger, Klaus-Peter; Zeise, Daniel; Pfueller, Caspar F; Zipp, Frauke; Paul, Friedemann.
In: NAT REV NEUROL, Vol. 5, No. 12, 12, 2009, p. 683-688.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome.
AU - Dörr, Jan
AU - Radbruch, Helena
AU - Bock, Markus
AU - Wuerfel, Jens
AU - Brüggemann, Anne
AU - Wandinger, Klaus-Peter
AU - Zeise, Daniel
AU - Pfueller, Caspar F
AU - Zipp, Frauke
AU - Paul, Friedemann
PY - 2009
Y1 - 2009
N2 - 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).
AB - 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).
M3 - SCORING: Zeitschriftenaufsatz
VL - 5
SP - 683
EP - 688
JO - NAT REV NEUROL
JF - NAT REV NEUROL
SN - 1759-4758
IS - 12
M1 - 12
ER -
