脊髓前角细胞以下二级神经元受侵害时,会引起神经原性肌萎缩,如Werdnig—Hoffmann 病、Kugelberg-Welander 病,肌萎缩性侧索硬化症,遗传性末梢神经障碍等。这些病一般不伴中枢神经症状,通过临床症状、肌电图、血液生化等即可确诊,不一定必须进行肌活检。在神经系统疾病中,肌活检可作为重要辅助诊断方法的是代谢疾病。最近报告增加的线粒体异常的疾病多有骨骼肌纤维形态异常,故肌活检可有重要参考价值。而肌纤维内有蓄积物的代谢性疾病,如糖原 Spinal cord anterior horn cells below secondary neurons affected, can cause neurogenic muscle atrophy, such as Werdnig-Hoffmann disease, Kugelberg-Welander disease, amyotrophic lateral sclerosis, hereditary peripheral nerve disorders. These diseases are generally not associated with CNS symptoms and may be confirmed by clinical symptoms, electromyography, blood biochemistry, etc., and may not necessarily require muscle biopsy. In neurological diseases, muscle biopsy can be used as an important auxiliary diagnosis of metabolic diseases. Recent reports of increased mitochondrial abnormalities have skeletal muscle fiber morphology abnormalities, so muscle biopsy may have important reference value. The muscle fiber accumulation of metabolic diseases, such as glycogen