获得性神经性肌强直的临床、电生理及其免疫学异常(附并发于肺癌且有肌无力综合征肌电图特征的神经性肌强直1例报告)

Abnormality of Clinical Manifestations, Electrophysiology and Immunology in Acquired Neuromyotonia

本文报告1例38岁女性肺癌患者伴进行性全身肌肉连续性抽搐、痉挛、僵硬和无力，胸片和胸部CT提示右上肺中心型肺癌、肺不张。脑脊液IgG增高，寡克隆区带阳性。肌电图呈连续性高频肌强直放电，发作间期可见束颤电位。周围神经封闭可缓解全部肌强直症状，并消除肌电图异常连续性放电。低频重复电刺激出现衰减，高频重复电刺激动作电位波幅增高300％。患者同时有神经性肌强直和肌无力综合征肌电图特征，并与中心型肺癌和神经系统免疫学异常并存。上述改变可能与肺癌有关，提示患者神经系统IgG介导的自身免疫异常在神经性肌强直的发病机理中起重要作用。

Objective We report a 38-year-old woman with increased cramping, stiffening and weakness on her muscles. Results The X-ray and CT manifested right central lung cancer.The abnormal activity is characterized electromyographically by high intraburst frequency and neuromyotonic discharges, with fibrillation potentials. The spontaneous muscle discharges and muscular cramp were abolished by peripheral nerve block. Clinical electromyography also revealed that the amplitude of the resting compound muscle action potential reduced progressively during low frequency repetitive nerve stimulation,and increased by 300% during high frequency reptitive nerve stimulation. Conclusion The presence of oligoclonal bands and increased concentration of IgG in her spinal fluid suggests that a IgG antibodies mediated autoimmunity may play an important role in this patient's pathogenesis of acquired neuromyotonia and Lambert-Eaton myasthenic syndrome associated with lung cancer.