NeuroCure Clinical Research Center Charité-Universitätsmedizin Berlin, Berlin, Germany.
Nat Rev Neurol. 2009 Dec;5(12):683-8. doi: 10.1038/nrneurol.2009.176.
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.
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).
Susac syndrome, characterized by a combination of encephalopathy, branch retinal artery occlusions, and hearing loss.
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).
一位 23 岁女性因反复发作的听力损失、脑病、局灶性神经功能缺损和视野缺损而到神经内科就诊。在就诊前 3 年，该患者曾因疑似急性脱髓鞘性脑脊髓炎和外周耳源性功能障碍接受甲基强的松龙治疗，后者完全恢复；也曾因双侧视网膜分支动脉阻塞导致双眼视力缺陷接受治疗，部分恢复后开始长期服用乙酰水杨酸。
详细的病史、临床检查、广泛的实验室检查、脑脊液分析、脑和脊髓 MRI、室周单体素（1）H 磁共振波谱、视网膜荧光血管造影、光学相干断层扫描、测听、神经生理学检查（脑电图、诱发电位）。
Susac 综合征，其特征为脑病、视网膜分支动脉阻塞和听力损失的组合。
长期免疫抑制治疗用硫唑嘌呤（150mg/天）和泼尼松龙（10mg/天），以及用乙酰水杨酸抑制血小板功能（100mg/天）。
