目的探讨线状体肌病的临床特点、病理学特征。方法结合近3年国外文献复习,分析1例线状体肌病患者的临床特点和超微病理学特征。结果患者3岁发病,症状逐渐加重,病程逾20年,表现为典型的肌张力减低和肢体无力,症状以双下肢为主,近端和远端同时受累,伸肌为著,远端为甚,肌萎缩明显;病理检查发现线状小体、肌膜皱缩、肌纤维断裂,α和β颗粒以及一小块变性的神经纤维束。结论线状体肌病可累及全身骨骼肌,临床表现为肌张力减低和肌无力,病理学特征为线状小体。 Objective To investigate the clinical features and pathological features of myoeiomyopathy. Methods Combined with the review of foreign literature in the past three years, the clinical features and ultrastructural features of one patient with myogenic leiomyopathy were analyzed. Results The patient developed symptoms at 3 years of age and the symptoms gradually worsen over a 20-year period. The symptoms were typical hypotension and limb weakness. The symptoms were mainly both lower extremities, with proximal and distal involvement, extensor muscles, distal , Muscle atrophy obvious; pathological examination revealed linear bodies, sarcopenia, muscle fiber rupture, α and β particles and a small piece of degenerative nerve fiber bundle. Conclusions Linear myopathy may affect whole body skeletal muscle. Its clinical manifestations include hypotonia and muscle weakness, and its pathological features are linear bodies.