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先天性中脑导水管狭窄患儿的导水管周围微发育异常

Periaqueductal microdysplasia in children with congenital aqueductal stenosis.

作者信息

Fukumizu M, Becker L E, Takashima S

机构信息

Division of Mental Retardation and Birth Defect Research, National Center of Neurology and Psychiatry, Tokyo, Japan.

出版信息

Clin Neuropathol. 1994 Jan-Feb;13(1):46-52.

PMID:7913428
Abstract

The brainstems of children with aqueductal stenosis (AS) and controls were examined histologically and immunohistochemically. In the periaqueductal area of children with AS, there was astrogliosis, loss of neurons (tyrosine-hydroxylase (TH) reactivity) and decreased myelination (myelin basic protein reactivity). In locus coeruleus, there was a decreased neuronal cell density (TH-positive neurons) in those with AS. In other areas of the brainstem (medial reticular formation), there appeared to be more TH and substance P reactive neurons. These alterations (dysplasia of slight degree) in neurons were more remarkable in children with AS with other identified brain anomalies than in those with only AS, judged by specific immunocytochemical identification of neurons. The periaqueductal astrogliosis and delayed/insufficient myelination suggest secondary damage to this region.

摘要

对患有导水管狭窄(AS)的儿童和对照组儿童的脑干进行了组织学和免疫组织化学检查。在患有AS的儿童的导水管周围区域,存在星形胶质细胞增生、神经元丢失(酪氨酸羟化酶(TH)反应性)以及髓鞘形成减少(髓鞘碱性蛋白反应性)。在蓝斑核,AS患儿的神经元细胞密度(TH阳性神经元)降低。在脑干的其他区域(内侧网状结构),似乎有更多的TH和P物质反应性神经元。通过对神经元的特异性免疫细胞化学鉴定判断,这些神经元的改变(轻度发育异常)在患有其他已确定脑异常的AS患儿中比仅患有AS的患儿更为显著。导水管周围星形胶质细胞增生以及髓鞘形成延迟/不足提示该区域存在继发性损伤。

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