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[先天性导水管狭窄及出血后脑积水的病理学]

[Pathology of congenital aqueductal stenosis and posthemorrhagic hydrocephalus].

作者信息

Takashima S, Fukumizu M

机构信息

Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, Kodaira, Tokyo.

出版信息

No To Hattatsu. 1994 May;26(3):216-21.

PMID:8185973
Abstract

The brain stem of children with congenital aqueductal stenosis or posthemorrhagic hydrocephalus were examined histologically and immunohistochemically. Congenital aqueductal stenosis occurs in various stages of CNS development, and is associated with microdysplasia and maturational abnormalities including astrogliosis, loss of neurons (tyrosine hydroxylase reactivity) and decreased myelination (myelin basic protein reactivity) in the periaqueductal gray matter. In Arnold-Chiari type II malformation there are few dysplasias, and poor development of glial and neural components in the periventricular brainstem. On the other hand, there are marked astroglial proliferation and defects of neural fibers in the periaqueductal area in children with posthemorrhagic hydrocephalus. Fibrous proliferation in aqueduct makes stenosis, and its pathogenesis seems to be caused by edema, ependymal cell desquamation, microglial proliferation and astrogliosis in parts of hemosiderin deposition. Thus, different CSF circulation disturbance and neural transmission disorder of the brainstem may develop with occurrence time, etiology or secondary complication of hydrocephalus.

摘要

对患有先天性导水管狭窄或出血后脑积水的儿童脑干进行了组织学和免疫组织化学检查。先天性导水管狭窄发生在中枢神经系统发育的各个阶段,并与微发育异常和成熟异常有关,包括导水管周围灰质中的星形胶质细胞增生、神经元丢失(酪氨酸羟化酶反应性)和髓鞘形成减少(髓鞘碱性蛋白反应性)。在Arnold-Chiari II型畸形中,发育异常较少,脑室周围脑干中的胶质和神经成分发育不良。另一方面,出血后脑积水儿童的导水管周围区域存在明显的星形胶质细胞增生和神经纤维缺陷。导水管中的纤维增生导致狭窄,其发病机制似乎是由部分含铁血黄素沉积处的水肿、室管膜细胞脱落、小胶质细胞增生和星形胶质细胞增生引起的。因此,脑干不同的脑脊液循环障碍和神经传递障碍可能随着脑积水的发生时间、病因或继发并发症而出现。

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