FAMILIAL AMYLOID POLYNEUROPATHY - ELECTRON MICROSCOPE STUDY OF PERIPHERAL NERVE IN 5 CASES .2. NERVE FIBRE CHANGES
FAMILIAL AMYLOID POLYNEUROPATHY - ELECTRON MICROSCOPE STUDY OF PERIPHERAL NERVE IN 5 CASES .2. NERVE FIBRE CHANGES
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DOI：
10.1093/brain/94.2.207
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发表时间：
1971-01-01
期刊：
影响因子：
14.5
通讯作者：
ANDRADE, C
中科院分区：
文献类型：
--
作者：
COIMBRA, A;ANDRADE, C
结果病例1和病例2的腓肠神经半薄切片显示所有神经束均出现神经纤维改变，而淀粉样蛋白沉积很少且大多数神经束不存在。横截面显示有核和无核细胞突起的胶原包围的胰岛中的有髓纤维数量减少（图 1，图板 XVII）。一些纤维看起来正常，而其他纤维则塌陷、扭曲或聚集成碎片（图 1）。纵向切片显示髓磷脂轮廓非常不规则，有时似乎沿其走向中断（图 2、3），并排列有细长的细胞突起束；类似束的索代替了完全缺失的髓鞘（图2）。很明显，这些以及横截面的圆形小岛都是邦纳带。纺锤形细胞束占据了没有髓鞘纤维的区域，其中一些仍含有髓鞘残余物（图 4）。病例3的变化与病例1和2的类型相同，但脱髓鞘更广泛，梭形细胞区域更广泛，淀粉样蛋白区域更大。病例 4 的神经中几乎不存在髓磷脂，而病例 5 的神经则完全缺乏髓磷脂，这两种神经主要由大量淀粉样蛋白沉积物中的纺锤形细胞区域组成。
RESULTSSemi-thin sections of sural nerve of Cases 1 and 2 showed nerve fibre changes in all nerve fascicles while amyloid deposits were rare and absent from most of them. Cross sections showed a decreased number of myelinated fibres among collagen-surrounded islets of nucleated and non-nucleated cell processes (fig. 1, Plate XVII). A few fibres looked normal, while others were collapsed, distorted or clumped into masses of debris (fig. 1). Longitudinal sections showed great irregularity of the myelin profiles which sometimes appeared to be interrupted along their course (figs. 2, 3), and were lined by bundles of elongated cell processes; cords of similar bundles were present in lieu of entirely absent myelin sheaths (fig. 2). It was evident that those, as well as the round islets of cross sections, were Bungner bands. Bundles of spindle-shaped cells, some still containing myelin remnants, occupied areas devoid of myelinated fibres (fig. 4). In Case 3, the changes were of the same type as in Cases 1 and 2 but demyelination was more widespread, the zones of spindle-shaped cells more extensive, and the amyloid areas larger. Myelin was practically absent from the nerve of Case 4 and completely lacking in Case 5, both nerves being mostly made up of areas of spindle-shaped cells among vast amyloid deposits.