Neuropathological analysis on pathogenesis in infantile spasms

婴儿痉挛症发病机制的神经病理学分析

• 批准号: 11670809
• 负责人: HAYASHI Masaharu
• 金额: $ 1.09万
• 依托单位: Tokyo Metropolitan Institute for Neuroscience
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 1999
• 资助国家: 日本
• 起止时间: 1999 至 2001
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-11670809/
• 关键词: infantile spasms; Lennox syndrome; brainstem; neurotransmitter; limbic system; progressive myoclonic epilepsy; dentatorubral-pallidoluysian atrophy; severely handicapped; ウエスト症候群; 大脳皮質形成異常; ウェスト症候群; 重障児・者; 扁桃体; 滑脳症; 新生児仮死後遺症; 側頭葉; グルタミン酸トラ

Previously, we reported the common functional brainstem lesions in the autopsy cases of lissencephaly (LIS) and perinatal hypoxic ischemic encephalopathy (HIE) having both infantile spasms (IS) and Lennox-Gastaut Isyndrome (LGS). To investigate the pathogenesis of IS and LGS, we immunohistochemically examined the expression of neiuotransmitters, neuropeptides, calcium-binding proteins and/or glutamate transporters in the brainstem and limbic system in child autopsy cases. In cases of LIS and HIE suffering from both epileptic syndromes, there were no IS/LGS-specific changes in the expressions of either calcium-binding proteins or glutamate transporters in the brainstem and limbic system. The brainstem showed reduced expressions of neurotransmitters in both the cases of hereditary dentatorubral-pallidoluysian atrophy suffering from another refractory progressive myoclonic epilepsy and the cases of subtle cerebral dysgenesis having IS but not LGS, although the changes were not related to the severity of epileptic seizures. In addition, the amygdaloid lesions in the severely handicapped were clarified to have relationships with the etiology but not either the severity of epileptic seizures or the hippocampal lesions. It is suggested that the brainstem lesions in IS and/or LGS can be involved in the disease progression more than the limbic lesions.

在此之前，我们报告了常见的功能性脑干病变，包括无脑(Lis)和围产期缺氧缺血性脑病(HIE)合并婴儿痉挛(IS)和Lennox-Gastaut I综合征(LGS)。为了探讨IS和LGS的发病机制，我们用免疫组织化学方法检测了脑干和边缘系统中神经递质、神经肽、钙结合蛋白和/或谷氨酸转运体的表达。在同时患有癫痫综合征的LIS和HIE病例中，脑干和边缘系统的钙结合蛋白或谷氨酸转运体的表达没有发生IS/LGS特异性的变化。遗传性齿状核-苍白球萎缩伴难治性进行性肌阵挛癫痫和轻度脑发育不良伴IS而不伴LGS的脑干神经递质表达均降低，但与癫痫发作的严重程度无关。此外，严重残障患者的杏仁核损害与病因有关，但与癫痫发作的严重程度或海马区损害无关。提示IS和/或LGS的脑干病变可能比边缘病变更多地参与疾病的进展。

项目成果

Kumada S, Hayashi M, Araki S, et al.: "Cerebellar degeneration in hereditary dentatombral-pallidoluysian atrophy and Machado-Joseph disease"Acta Neuropathol. 99(1). 48-54 (2000)

Kumada S、Hayashi M、Araki S 等人：“遗传性齿瘤-苍白球路易体萎缩和马查多-约瑟夫病中的小脑变性”《神经病理学报》。

林雅晴, 伊藤昌弘, 森松義雄 他: "重症心身障害児(者)剖検脳における扁桃体病変の検討"日本重症心身障害学会誌. 25・2. 1-4 (2000)

Masaharu Hayashi、Masahiro Ito、Yoshio Morimatsu 等：“患有严重精神和身体残疾的儿童尸检大脑中杏仁核病变的检查”，日本严重精神和身体疾病学会杂志 25, 2. 1-4 ( 2000)

Hayashi M, Araki S, Kumada S et al.: "Neurodegenerotive features in dcvdopnentul brain discovers."Neuropathology. 21・2. 32-39 (2001)

Hayashi M、Araki S、Kumada S 等人：“dcvdopnentul 大脑中的神经退行性疾病特征的发现。”神经病理学 21・2（2001）。

Hayashi M, Itoh M, Araki S, et al.: "Inmmunohistochemical analysis of biainstem lesions in infantile spasms"Neuropathology. 20(4). 297-303 (2000)

Hayashi M、Itoh M、Araki S 等人：“婴儿痉挛症中双干损伤的免疫组织化学分析”神经病理学。

Hayashi M, Araki S, Kumada S, et al.: "Neurodegenrative features in developmental brain disorders"Neuropathology. 21(1). 32-39 (2001)

Hayashi M、Araki S、Kumada S 等人：“发育性脑疾病的神经退行性特征”神经病理学。