Horoupian D S
Acta Neuropathol. 1982;57(2-3):165-70. doi: 10.1007/BF00685385.
A 6 1/2 month old male infant presented a week after his birth with secretory diarrhea of unknown etiology. He was sustained by central hyperalimentation for the rest of his life, and treated for presumed sepsis with a wide variety of antibiotics. The brain showed vacuolation in the diencephalic nuclei and white matter of the brain stem. There were also many clusters of enlarged Purkinje cell dendrites in the molecular layer. In Golgi preparations the primary and secondary dendrites showed segmental swellings and absent tertiary branchlets. The swellings were due to remarkable accumulations of mitochondria. The pathogenesis of the dendritic changes is discussed, and 'dying back' phenomenon is proposed to explain the changes.
一名6个半月大的男婴在出生一周后出现病因不明的分泌性腹泻。他此后一直依靠中心静脉高营养维持生命，并因疑似败血症接受了多种抗生素治疗。大脑显示间脑核和脑干白质有空泡形成。分子层中也有许多成簇的浦肯野细胞树突增大。在高尔基染色标本中，一级和二级树突显示节段性肿胀，三级分支缺失。肿胀是由于线粒体的显著积聚。本文讨论了树突变化的发病机制，并提出“逆行性死亡”现象来解释这些变化。
