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夏伊-德雷格综合征与肌萎缩侧索硬化症。骶部自主神经元的细胞构筑学和形态测量学研究。

Shy-Drager syndrome and amyotrophic lateral sclerosis. Cytoarchitectonic and morphometric studies of sacral autonomic neurons.

作者信息

Konno H, Yamamoto T, Iwasaki Y, Iizuka H

出版信息

J Neurol Sci. 1986 Apr;73(2):193-204. doi: 10.1016/0022-510x(86)90130-9.

DOI:10.1016/0022-510x(86)90130-9
PMID:3701375
Abstract

In order to elucidate the morphological correlates of bladder-rectal dysfunctions in Shy-Drager syndrome, the sacral spinal cord was cytoarchitectonically studied and 3 groups of sacral motor neurons, the posterolateral motor neuron column (PL), inferior intermediolateral nucleus (IML) and cell group X of Onuf (Onuf), were morphometrically quantitated at the S3 level (5 cases), after which the results were compared with those from amyotrophic lateral sclerosis (5 cases) and an age-matched control group (4 cases). The sacral autonomic preganglionic nucleus of IML was localized chiefly in the S3-4 segments and was maximally developed in the caudal one-third of S3. The cell group X of Onuf was localized between the middle of S2 and the rostral one-third of S3 as a longitudinal slender column in the ventral horn. Between these two nuclei at the rostral S3 level, a connecting cellular bridge of neurons of intermediolateral cell type was identified. Morphometry disclosed a marked deprivation of IML, Onuf and somatic motor neurons in Shy-Drager syndrome and a severe loss of somatic motor neurons and a modest deprivation of IML neurons in ALS. These results imply that these two disorders distinguished by different clinical manifestations share a common loss of somatic motor and parasympathetic motor neurons at least in the sacral cord. There are, however, certain gradients in the severity of involvement in these heterogeneous cell groups.

摘要

为了阐明夏伊-德雷格综合征中膀胱直肠功能障碍的形态学关联,对骶脊髓进行了细胞构筑学研究,并在S3水平对3组骶运动神经元,即后外侧运动神经元柱(PL)、中间外侧核下部(IML)和奥努夫细胞群(Onuf)进行了形态计量学定量分析(5例),之后将结果与肌萎缩侧索硬化症(5例)和年龄匹配的对照组(4例)的结果进行比较。IML的骶自主神经节前核主要位于S3-4节段,在S3尾侧三分之一处发育最为充分。奥努夫细胞群位于S2中部和S3头侧三分之一之间,呈腹角内的纵向细长柱状。在S3头侧水平的这两个核之间,发现了一个中间外侧细胞类型神经元的连接细胞桥。形态计量学显示,夏伊-德雷格综合征中IML、奥努夫和躯体运动神经元明显减少,肌萎缩侧索硬化症中躯体运动神经元严重丢失,IML神经元轻度减少。这些结果表明,这两种以不同临床表现为特征的疾病至少在骶脊髓中存在躯体运动和副交感运动神经元的共同丢失。然而,在这些异质性细胞群的受累严重程度上存在一定的梯度。

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