MORPHOMETRIC AND BIOCHEMICAL-STUDIES OF PERIPHERAL-NERVES IN AMYOTROPHIC LATERAL SCLEROSIS
MORPHOMETRIC AND BIOCHEMICAL-STUDIES OF PERIPHERAL-NERVES IN AMYOTROPHIC LATERAL SCLEROSIS
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DOI：
10.1002/ana.410140304
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发表时间：
1983-01-01
影响因子：
11.2
通讯作者：
ADELMAN, LS
中科院分区：
文献类型：
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作者：
BRADLEY, WG;GOOD, P;ADELMAN, LS
11例肌萎缩性侧索硬化症（ALS）患者死后的膈神经仅含有正常数量33%的大髓鞘纤维（对照9例，P < 0.001）。在这些患者的膈神经中，远段大髓鞘纤维比近段少18% （P < 0.025）。大髓鞘纤维远段轴突周长与髓鞘片的比值比对照纤维大34% （P < 0.002）。急性轴突变性在各水平的比例相同(48.0。13.7%)。与对照组相比，21例ALS患者腓肠神经急性轴突变性增多，髓鞘纤维减少30% (P < 0.05)；变性的证据也扩展到无髓鞘纤维。体外测定的9条腓肠神经乙酰胆碱酯酶轴突转运量比对照组降低24% (P < 0.05)，表观转运率比对照组降低44% （P < 0.01）。在肌萎缩侧索硬化症中，运动神经元细胞体的退化叠加了小程度的死变和远端轴突萎缩。这种疾病的影响扩散到运动神经元之外。
Phrenic nerves of 11 patients with amyotrophic lateral sclerosis [ALS] studied postmortem contained only 33% of the normal number of large myelinated fibers (9 controls; P < 0.001). In the phrenic nerves of these patients, there were 18% fewer large myelinated fibers in the distal segment than in the proximal segment (P < 0.025). The ratio of axonal circumference to myelin lamellae in large myelinated fibers in the distal segment was 34% greater than that in control fibers (P < 0.002). The proportion of acute axonal degeneration was the same at all levels (48.0 .+-. 13.7%). Sural nerves of 21 patients with ALS had more acute axonal degeneration and 30% fewer myelinated fibers (P < 0.05) than controls; evidence of degeneration also extended to unmyelinated fibers. The amount of axonal transport of acetylcholinesterase in 9 sural nerves determined in vitro was reduced by 24% (P < 0.05) and the apparent transport rate was reduced by 44% (P < 0.01) compared with 4 controls. In ALS a small degree of dying-back change and of distal axonal atrophy is superimposed on the degeneration of motor neuron cell bodies. The disease effects spread beyond the motor neurons.