Stiff man syndrome and variants

被引:0
作者
Meinck, H-M [1 ]
机构
[1] Neurol Univ Klin Heidelberg, D-69120 Heidelberg, Germany
来源
NERVENARZT | 2013年 / 84卷 / 04期
关键词
Stiff man syndrome; Stiff person syndrome; GAD autoantibodies; GlyR autoantibodies; Paraneoplastic disorder; PERSON SYNDROME; INTRATHECAL BACLOFEN; RIGIDITY; AUTOIMMUNITY; ANTIBODIES; MYOCLONUS;
D O I
10.1007/s00115-012-3604-8
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:450 / 454
页数:5
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