Meinck H-M
Neurologische Universitätsklinik Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Deutschland.
Nervenarzt. 2013 Apr;84(4):450-4. doi: 10.1007/s00115-012-3604-8.
Stiff man syndrome (SMS) and its variants are rare neurological disorders with unusual, often awkward motor and psychological symptoms. Misdiagnoses are frequent and differentiation from psychogenic movement disorder may be difficult. Clinical suspicion can be substantiated by neurophysiological and immunological testing. Autoimmunity against certain proteins of inhibitory synapses appears to be a key feature that links SMS to other autoimmune encephalopathies and endocrinopathies. According to retrospective analyses a front-loaded long-term methylprednisolone treatment appears to be most effective.
僵人综合征(SMS)及其变异型是罕见的神经系统疾病,具有异常且常显笨拙的运动和心理症状。误诊很常见,与精神性运动障碍进行鉴别可能很困难。神经生理学和免疫学检测可证实临床怀疑。针对抑制性突触某些蛋白的自身免疫似乎是将SMS与其他自身免疫性脑病和内分泌病联系起来的关键特征。根据回顾性分析,早期长期使用甲基泼尼松龙治疗似乎最为有效。