重症肌无力伴骨骼肌以外受损的临床特征分析

目的 对伴心脏、肝脏损害,瞳孔不等,听力障碍,手足血管舒缩功能及皮肤营养障碍和阳痿等少见症状的重症肌无力(MG)患者的特点进行分析,提出诊断和鉴别诊断的方法。方法 (1)给予MG患者胆碱酯酶抑制剂(ChEI)及免疫治疗,再复查病情和随访;(2)伴瞳孔不等和听力障碍患者肌注新斯的明1mg,30～120min后观察症状变化,后者同时检查脑干听觉诱发电位2次。结果 55例MG患者临床症状随典型的骨骼肌病态疲劳的症状改善而好转,一般不需要特殊治疗。结论 ChEI治疗MG患者合并上述少见症状均有效,是与其他疾病鉴别的重要依据,也证实了MG是一种广泛的自身免疫性疾病。

The clinical features of myasthenia gravis affecting nonskeleton muscles

Objective To propose a method for diagnosis and differential diagnosis of myasthenia gravis (MG)with heart and liver injury, anisocoria , dysaudia, impatience etc. Methods (1) Before and after administering cholinesterase inhibitor and immune therapy , 55 patients with MG were re-examined for heart and liver and followed up . (2) 1 mg neostigmine was injected to the MG patients with anisocoria and dysaudia, the symptoms were observed and brainstem auditory evoked potential was performed before and after 30 minutes and 2 hours. Results With the relaxation of muscular weakless and fatigability distinctive of MG , other special symptoms will improve and no further special therapy is needed. Conclusions MG is an autoimmune disease mediated by auto-antibodies that attack the acetylcholine receptors not only at the skeletal muscle, but also at many other organs such as heart, liver, hearing apparatus pupillary sphincters and genitals. With the relaxation of muscular weakless , these symptoms will improve. The effect of administering cholinesterase inhibitors is the key point of differential diagnosis.