Said G, Ropert A, Faux N
Neurology. 1984 Aug;34(8):1025-32. doi: 10.1212/wnl.34.8.1025.
We investigated two patients with Portuguese amyloid polyneuropathy to learn more about the role played by amyloid in this condition. In sural nerves, axonal loss predominated in unmyelinated axons. Different abnormalities of single fibers near amyloid deposits included distortion of the myelin sheath, segmental demyelination, and wallerian degeneration. Electronmicroscopic studies showed degenerative changes of endoneurial cells in contact with fibrils of amyloid. Therefore, amyloid plays an important role in the length-dependent degeneration of fibers of this condition. Why unmyelinated fibers are so heavily affected remains unclear.
我们研究了两名患有葡萄牙型淀粉样多神经病的患者，以进一步了解淀粉样蛋白在这种疾病中所起的作用。在腓肠神经中，轴突损失主要发生在无髓鞘轴突。淀粉样沉积物附近单纤维的不同异常包括髓鞘扭曲、节段性脱髓鞘和华勒氏变性。电子显微镜研究显示，与淀粉样蛋白原纤维接触的神经内膜细胞出现退行性变化。因此，淀粉样蛋白在这种疾病纤维的长度依赖性变性中起重要作用。无髓鞘纤维为何受到如此严重的影响仍不清楚。
