Behse F, Buchthal F
Brain. 1977 Mar;100 Pt 1:67-85. doi: 10.1093/brain/100.1.67.
Biopsy of the sural nerves distinguished two groups of patients with peroneal muscular atrophy (Charcot-Marie-Tooth): a hypertrophic type and a neuronal type. In patients with the hypertrophic type (10 nerves), 30-100 per cent of teased fibres of the sural nerve had demyelinated segments, numerous onion-bulb formations and often an increase in endoneurial space. Large and small fibres, with a diameter of more than 7 micron and less than 5 micron were diminished in number. Regeneration was scarce. There were more fibres with 60-120 myelin lamellae than in normal nerve, suggesting an atrophy of the axon. Biopsy of 19 sural nerves of patients with the neuronal type of PMA showed loss of large fibres (more than 7 micron in diameter). The number of small fibres was normal, presumably due to regeneration, since there were many "clusters" of small myelinated fibres. Fibres with demyelinated segments and onion-bulb formations were absent or rare and the endoneurial space was normal or slightly increased. Neither in the hypertrophic nor in the neuronal type did fibre loss occur selectively among the very largest fibres. Nine nerves from patients with hereditary spastic paraplegia and from a family with tremor and spasticity in addition to PMA showed changes similar in type but often milder in degree than nerves of the neuronal type of PMA. The number of unmyelinated fibres was normal in 12 of 21 nerves from patients with PMA; it was increased in 5 and diminished in 3 nerves.
腓肠神经活检将腓骨肌萎缩症（夏科-马里-图斯病）患者分为两组：肥厚型和神经元型。在肥厚型患者（10条神经）中，腓肠神经30% - 100%的分离纤维有脱髓鞘节段、大量洋葱球样结构，且神经内膜间隙常增大。直径大于7微米和小于5微米的大、小纤维数量减少。再生现象少见。与正常神经相比，有60 - 120层髓鞘板的纤维更多，提示轴突萎缩。对神经元型腓骨肌萎缩症患者的19条腓肠神经进行活检，结果显示大纤维（直径大于7微米）缺失。小纤维数量正常，推测是由于再生，因为有许多小的有髓纤维“簇”。有脱髓鞘节段和洋葱球样结构的纤维不存在或少见，神经内膜间隙正常或略有增大。无论是肥厚型还是神经元型，最大的纤维中均未出现选择性纤维丢失。来自遗传性痉挛性截瘫患者以及一个除腓骨肌萎缩症外还有震颤和痉挛症状的家族的9条神经，其变化类型相似，但程度通常比神经元型腓骨肌萎缩症的神经轻。在21条腓骨肌萎缩症患者的神经中，12条神经的无髓纤维数量正常；5条神经的无髓纤维数量增加，3条神经的无髓纤维数量减少。
