Hirano A, Nakano I, Kurland L T, Mulder D W, Holley P W, Saccomanno G
J Neuropathol Exp Neurol. 1984 Sep;43(5):471-80. doi: 10.1097/00005072-198409000-00002.
Lewy body-like hyaline inclusions in the soma and swollen, cord-like cell processes are characteristic alterations of the anterior horn cells in familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement. A fine structural analysis of these two structures has been performed in two brothers from a family ("C" family) previously described by Kurland and Mulder in 1955. The perikaryal hyaline inclusions consisted of accumulations of randomly oriented neurofilaments interspersed with thick linear densities associated with granular material. Some of the accumulations showed a central condensation. Cord-like, swollen neuronal processes were composed, for the most part, of numerous neurofilaments arranged parallel to the long axes. Dense structures were sometimes observed within the large bundles of filaments. They were composed of ill-defined dense, granular and fibrillar material associated with scattered vesicles and mitochondria. These dense areas were sometimes surrounded by various amounts of fine filaments, approximately 5 nm in diameter.
在伴有后索和脊髓小脑束受累的家族性肌萎缩侧索硬化症（ALS）中，前角细胞的特征性改变为胞体出现路易小体样透明包涵体以及细胞突起肿胀、呈索状。对1955年Kurland和Mulder先前描述的一个家族（“C”家族）中的两兄弟的这两种结构进行了精细结构分析。核周透明包涵体由随机排列的神经丝聚集物组成，其间散布着与颗粒物质相关的粗线状致密物。一些聚集物呈现中央凝聚。索状、肿胀的神经元突起大部分由许多与长轴平行排列的神经丝组成。在大的细丝束内有时可观察到致密结构。它们由不明确的致密、颗粒状和纤维状物质组成，并伴有散在的小泡和线粒体。这些致密区域有时被不同数量的直径约5nm的细丝所包围。
