肌萎缩侧索硬化症的周围神经损害

目的 阐明肌萎缩侧索硬化症患者腓肠神经的病理改变特点。方法 选择已确诊的肌萎缩侧索硬化症患者行周围神经活检。取其腓肠神经进行光镜及电镜检查，观察其病理改变。结果 周围神经的改变以轴索损害为主，伴有髓鞘的脱失。根据病理改变可分为4组：正常；轻度轴索变性及髓鞘脱失；有髓神经纤维轻度脱失，轴索变性及脱髓鞘；有髓神经纤维严重脱失，轴索变性及脱髓鞘。结论 肌萎缩侧索硬化症患者常常早期即出现周围神经的损害，其中以轴索性的改变为主，同时可伴有不同程度的髓鞘脱失。

Lesion of the peripheral nerve in amyotrophic lateral sclerosis

ObjectiveTo investigate the pathologic characteristics of the sural nerve in amyotrophic lateral sclerosis. MethodsClinical, electrophysiologic, laboratory data and sural nerve biopsy of 11 patients were reviewed. The clinical and laboratory data were compatible with the diagnosis of ALS. The sural nerve was removed and immediately fixed in 10% formalin and phosphate-buffered 2.5% glutaraldehyde and processed according to the procedure used in our laboratory for light and ultrastructural examination.Results4 groups were distinguished based on pathologic changes: normal; with mild axon degeneration and demyelination; with mild loss of the myelinated nerve, axon degeneration and demyelination; with severe loss of the myelinated nerve, axon degeneration and demyelination.ConclusionPredominantly axonal neuropathies are common and occur early in ALS. Axon degeneration of the nerve fibers is predominant, and demyelination also can be performed in patients with ALS.