重症肌无力全身表现及发病机制探讨  

SYSTEMIC MANIFESTATIONS OF MYASTHENIA GRAVIS AND ITS PUTATIVE PATHOGENESIS

作　　者：张华[1,2] 许贤豪[1,2] 国红[1,2] 苏闻[1,2] 王秀云[1,2] 孙宏[1,2] 张振馨[1,2] 谭铭勋[1,2] 

机构地区：[1]北京医院神经内科 [2]北京协和医院神经内科

出　　处：《中华内科杂志》1997年第6期368-371,共4页Chinese Journal of Internal Medicine

基　　金：卫生部基金

摘　　要：６４４例重症肌无力（ＭＧ）患者合并多种免疫有关性疾病，并且有多种自身抗体阳性，同时发现了一些骨骼肌以外受损害的表现，伴锥体束征者（ＰＳ）１１例，伴发癫痫４例，原因未明的周围神经病变３例，部分重症肌无力病人伴有血清ＡＬＴ增高，１４例ＭＧ患者的超声心动图，射血指数，２例心脏核素扫描，均未见明显异常。ＭＧ病人的骨骼肌以外损伤的表现，一般不需要特殊处理，随着重症肌无力的好转，绝大部分能够好转，其发病机制可能与乙酰胆碱受体抗体（ＡＣｈＲＡｂ）有关，或者是免疫泛化产生的其他免疫因素的作用，由此可见ＭＧ可能是一种主要累及神经肌肉接头处突触后膜上ＡＣｈＲ的全身性自身免疫性疾病。We studied the systemic manifestations of myasthenia gravis and its putative pathogenesis. 644 clinically, pharmacologically and electrophysiologically diagnosed patients with myasthenia gravis (MG) were studied. 33 patients had Graves disease, 5 periodic paralysis, 4 Hashimoto disease, and 4 epilepsy, polymyositis, rheumatoid arthritis and Guillain Barre syndrome each. 45 patients had positive anti skeletal muscle antibodies, 9 positive anti thyroid antibody, and 3 positive SSA and SSB. 11 MG patients with positive pyramidal signs (MG+PS) had no evidence of multiple sclerosis (MS) or other neurologic diseases. Compared with 12 MG patients without pyramidal signs (MG PS) and 23 normal controls (NCs), they had not only high levels of IgGcsf and IgG syn, but also increased ratio of AChRAbcsf/AChRAbs ( P <0.05). Four MG patients had epilepsy. Four MG patients had severe memory disorders. Three MG patients had diseases of the peripheral nerves without diabetes mellitus and toxin. SGPT was significantly increased in 22 MG patients than in 9 normal controls ( P <0.05). These systemic manifestations were improved while MG was improved by immunological therapy. MG is a systemic autoimmune disease predominantly involving AChR on the postsynaptic membrane of N M conjunction.

关 键 词：重症肌无力 免疫学试验 发病机制 

分 类 号：R746.102[医药卫生—神经病学与精神病学]