Moss T H
Acta Neuropathol. 1981;53(1):51-6. doi: 10.1007/BF00697184.
A teased fibre and electron-microscopical study was carried out on the sciatic nerves of mice affected with the peripheral neuropathy in dystonia musculorum. Widespread segmental demyelination was present in all the nerves. Focal axon swellings were also seen, but were relatively scarce and similar in appearance to post-traumatic reactive swellings. The variability of dystonic internodal lengths was indicative of segmental demyelination rather than axonal degeneration. The largely motor fibres of the phrenic nerve were seen to undergo a similar degenerative process, but with a later onset and more gradual progression. Segmental demyelination was found to be present before axon swellings and other degenerative changes became visible in developing phrenic nerve. Demyelination is thus an important pathological process in dystonia musculorum, and the present observations are consistent with a primary segmental demyelinating disorder in dystonic peripheral nerve.
对患有肌张力障碍性周围神经病的小鼠坐骨神经进行了 teased 纤维和电子显微镜研究。所有神经均存在广泛的节段性脱髓鞘。还可见局灶性轴突肿胀，但相对较少，且外观与创伤后反应性肿胀相似。肌张力障碍性结间长度的变异性表明是节段性脱髓鞘而非轴突变性。膈神经的大部分运动纤维也经历了类似的退行性过程，但发病较晚且进展较为缓慢。在发育中的膈神经中，发现节段性脱髓鞘在轴突肿胀和其他退行性变化可见之前就已存在。因此，脱髓鞘是肌张力障碍性周围神经病的一个重要病理过程，目前的观察结果与肌张力障碍性周围神经的原发性节段性脱髓鞘疾病一致。
