A quantitative investigation of neuronal cytoplasmic and intranuclear inclusions in the pontine and inferior olivary nuclei in multiple system atrophy
A quantitative investigation of neuronal cytoplasmic and intranuclear inclusions in the pontine and inferior olivary nuclei in multiple system atrophy
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DOI：
10.1111/j.1365-2990.2004.00564.x
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发表时间：
2004-10-01
影响因子：
5
通讯作者：
Wakabayashi, K
中科院分区：
文献类型：
--
作者：
Nishie, M;Mori, F;Wakabayashi, K
多系统萎缩(MSA)是一种以神经元和少突胶质细胞α-突触核蛋白聚集体存在为特征的散发性神经退行性疾病。为了探讨神经元胞浆和核内包涵体(NCIS和NNIs)的发生与神经元退变的关系，我们对14例MSA患者的脑桥和下橄榄核进行了定量分析。α-突触核蛋白免疫组织化学显示NCIS和NNIs在所有病例的双侧脑核均有表达。脑桥核和下橄榄核NCIS的平均发生率分别为9.1%和25.8%，NNIS的平均发生率分别为9.2%和9.0%。NNI的数量与桥脑和下橄榄核内神经元的数量密切相关。尽管NCI的数量与两个核团的神经元数目无关，但中度MSA患者的NCI计数高于轻度MSA患者。4例患者脑桥核的NNI计数远高于NCI计数，3例患者的橄榄核NNI计数与NCI计数相同。此外，NNI占优势的病例中神经元数量显著高于NCI占优势的病例。这些发现表明，NCI的形成随着疾病进程的进展而加速，并且在MSA中，NNI的形成比NCI的形成更早。
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterized by the presence of neuronal and oligodendroglial alpha-synuclein aggregates. To investigate the relationship between the occurrence of neuronal cytoplasmic and intranuclear inclusions (NCIs and NNIs, respectively) and the progression of neuronal degeneration, we performed a quantitative analysis of the pontine and inferior olivary nuclei based on 14 cases of MSA. alpha-Synuclein immunohistochemistry revealed that NCIs and NNIs were present in both brain nuclei in all the cases. The average incidence of NCIs in the pontine and inferior olivary nuclei was 9.1% and 25.8%, respectively, and that of NNIs was 9.2% and 9.0%, respectively. The number of NNIs was strongly correlated with that of neurones in the pontine and inferior olivary nuclei. Although the number of NCIs was not correlated with the neuronal population in both nuclei, the NCI count in patients with moderate MSA was higher than in patients with mild MSA. The NNI count was much higher than the NCI count in the pontine nucleus in four patients, and was the same in the olivary nucleus in three of the four patients. Moreover, the neuronal population in the NNI-predominant cases was significantly higher than in the NCI-predominant cases. These findings suggest that NCI formation is accelerated by the progression of the disease process, and that in MSA, NNI formation is an earlier phenomenon than NCI formation.