Perrie W T, Lee G T, Curtis E M, Sparke J, Buller J R, Rossi M L
Department of Neuropathology, Midland Centre for Neurosurgery and Neurology, Warley, U.K.
J Neural Transm Suppl. 1993;39:223-33.
Myelinated fibres in femoral nerves removed from amyotrophic lateral sclerosis (ALS) cases at post mortem were compared with age matched controls. A technique for processing whole transverse sections of the nerves for osmication and subsequent morphometric analysis is described. Although areas depleted in myelinated fibres were seen in the nerves from the ALS group, no statistically significant difference was shown due to wide variations in the controls. However, the ALS nerves showed a degree of disruption in the myelin which was not apparent in the controls. The most obvious effect was widespread "wrinkling" of the myelin in both large and small fibres from the ALS nerves. This phenomenon is the initial stage of a process which eventually results in uneven myelin thickness and nodal swellings and finally myelin ovoids and balls. We illustrate the steps in the progression of this degeneration with teased nerve studies and electron microscopy and propose that there are qualitative changes in the myelin of peripheral nerve in ALS. It seems likely that these are secondary effects resulting from axonal degeneration caused by deterioration and loss of anterior horn cells in the spinal cord.
对死后从肌萎缩侧索硬化症（ALS）病例中取出的股神经中的有髓纤维与年龄匹配的对照组进行了比较。描述了一种处理神经全横切片以进行锇酸固定及后续形态计量分析的技术。尽管在ALS组的神经中可见有髓纤维缺失区域，但由于对照组差异较大，未显示出统计学上的显著差异。然而，ALS神经的髓鞘出现了一定程度的破坏，而对照组中并不明显。最明显的影响是ALS神经的大小纤维中髓鞘普遍出现“起皱”。这种现象是一个过程的初始阶段，最终会导致髓鞘厚度不均和结肿胀，最终形成髓鞘卵圆体和小球。我们通过神经纤维分离研究和电子显微镜说明了这种变性的进展步骤，并提出ALS患者周围神经的髓鞘存在质的变化。这些变化似乎很可能是脊髓前角细胞退化和丧失导致轴突变性的继发效应。
