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婴儿猝死综合征中的中枢神经系统髓鞘形成延迟

Delayed central nervous system myelination in the sudden infant death syndrome.

作者信息

Kinney H C, Brody B A, Finkelstein D M, Vawter G F, Mandell F, Gilles F H

机构信息

Department of Pathology, Children's Hospital, Boston, Massachusetts 02115.

出版信息

J Neuropathol Exp Neurol. 1991 Jan;50(1):29-48. doi: 10.1097/00005072-199101000-00003.

DOI:10.1097/00005072-199101000-00003
PMID:1985152
Abstract

This study was designed to assess whether development of the central nervous system (CNS) is delayed in victims of the sudden infant death syndrome (SIDS). We selected the parameter of myelination because it is a continuously changing and readily accessible marker of CNS development in the SIDS age-range. We assessed myelination blindly in 61 SIDS and 89 autopsy controls. In 62 sites the degree of myelination was visually graded in myelin-stained histological sections on an ordinal scale of 0-4 using the inferior cerebellar peduncle as an internal standard of degree 3. Cases were stratified by postconceptional age at death and SIDS and controls were compared with respect to myelin degree at each site. Significantly delayed myelination (p less than 0.05) occurred in the SIDS group in 25 of the 62 sites examined. Hypomyelination affected fiber systems in which myelination is initiated before or after birth and which myelinate with different tempos and preferentially affect pyramidal and cerebellar (somatomotor) and prefrontal-temporal-limbic (visceromotor) systems. Hypomyelination was not associated with individual clinicopathologic variables in the SIDS group. Somatic growth and brain weight were significantly greater in SIDS than controls. Therefore, we suggest that SIDS is associated with a developmental CNS disorder. Although delayed CNS myelination most likely shares a common antecedent with sudden death and is not its cause, the role of somato- and viscero-motor systems in central cardiorespiratory control and arousal warrants further analysis in SIDS.

摘要

本研究旨在评估婴儿猝死综合征(SIDS)受害者的中枢神经系统(CNS)发育是否延迟。我们选择髓鞘形成参数,因为它是SIDS年龄范围内CNS发育中一个不断变化且易于获取的标志物。我们对61例SIDS病例和89例尸检对照进行了盲法髓鞘形成评估。在62个部位,使用小脑下脚作为3级的内部标准,在髓鞘染色的组织学切片上对髓鞘形成程度进行视觉分级,范围为0 - 4级。根据死亡时的孕龄对病例进行分层,并比较SIDS组和对照组在每个部位的髓鞘程度。在检查的62个部位中的25个部位,SIDS组出现了明显延迟的髓鞘形成(p小于0.05)。髓鞘形成不足影响了在出生前或出生后开始髓鞘形成、以不同速度髓鞘化且优先影响锥体和小脑(躯体运动)以及前额叶 - 颞叶 - 边缘(内脏运动)系统的纤维系统。SIDS组的髓鞘形成不足与个体临床病理变量无关。SIDS组的躯体生长和脑重量显著高于对照组。因此,我们认为SIDS与中枢神经系统发育障碍有关。虽然中枢神经系统髓鞘形成延迟很可能与猝死有共同的前期因素,而非其原因,但躯体和内脏运动系统在中枢心肺控制和唤醒中的作用值得在SIDS中进一步分析。

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