摘要
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。
Nemaline myopathy is a rare congenital myopathy.Here we report the clinical features, laboratory examination and pathologic findings of an adult myopathy patient.Most myofibers containing large number of nemaline rod bodies emanated from the Z discs were observed by electron microscope,so that the patient was finally diagnosed late onset NM by muscle biopsy.In adult patients with proximal muscle weakness, especially in those with underlying immunologic disorders, a modified Gomori's trichrome stain on cryostat sections with possible confirmatory electron microscopy should be performed in order to prevent misdiagnosis. Functional training for these patients in the early stage will improve the prognosis.
出处
《中国临床康复》
CSCD
2003年第16期2330-2331,F003,共3页
Chinese Journal of Clinical Rehabilitation
