杆状体肌病(NM)为一种少见的先天性肌病,报告1例成人晚发肌病患者的临床表现、实验室及肌肉病理检查,电镜下观察到多数肌纤维中含有大量源于Z线的杆状体,确诊为NM。结合国内外文献探讨NM的临床与病理特点,对于有近端肢体无力特别是合并有免疫系统疾病的患者行MGT染色或电镜检查将避免NM的误诊,早期功能锻炼可改善此类患者的预后。 (NM), a rare form of congenital myopathy, reports the clinical manifestations, laboratory and muscle pathology of one adult late-onset myopathy. Electron microscopy showed that most of the muscle fibers contained significant amounts of Line rod body, diagnosed as NM. To explore the clinical and pathological features of NM with literature both at home and abroad, MGT staining or electron microscopy in patients with proximal limb weakness, especially those with immune system diseases, will avoid the misdiagnosis of NM. Early functional exercise may improve the prognosis of these patients.