The phenotype spectrum of Japanese multiple system atrophy
The phenotype spectrum of Japanese multiple system atrophy
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DOI：
10.1136/jnnp.2009.182576
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发表时间：
2010-11-01
影响因子：
11
通讯作者：
Nishizawa, M.
中科院分区：
文献类型：
--
作者：
Ozawa, T.;Tada, M.;Nishizawa, M.
目的本研究旨在确定日本多系统萎缩（MSA）患者纹状体黑质（StrN）和橄榄桥脑小脑（OPC）系统的病理受累谱。本研究还旨在比较日本MSA患者的病理谱与以前报道的结果在英国MSA patient.Methods的50 MSA患者的大脑，被称为脑研究所，新泻大学，日本，进行了半定量病理分析。神经元细胞损失的严重程度，确定为先前所述的研究从皇后广场脑银行（QSBB），UK.Results的平均神经元细胞损失评分是显着高于在OPC领域比基底神经节网站检查，除了背外侧壳核。病理表型的相对患病率显示，40%的病例具有OPC为主的病理，18%具有StrN为主的病理，其余（42%）具有等同的StrN和OPC病理。所有MSA患者的迷走神经背侧运动核和黑质内均无Lewy小体共存。结论与以往报道的英国患者的QSBB结果相反，（OPC为主的病理17%，StrN为主的病理34%，等同的StrN和OPC病理49%），本研究的结果显示OPC系统比StrN系统有更多的病理学参与。Lewy小体的罕见可能是日本MSA表型表达的基础。目前的观察结果反映了两个群体之间的表型分布的不平衡。
Objective This study aimed to determine the spectrum of pathological involvement of the striatonigral (StrN) and olivopontocerebellar (OPC) systems in Japanese patients with multiple system atrophy (MSA). This study also aimed to compare the pathological spectrum of Japanese MSA patients with the previously reported results in British MSA patients.Methods A semiquantitative pathological analysis of 50 MSA patients' brains that were referred to the Brain Research Institute, Niigata University, Japan, was performed. The severity of neuronal cell loss was determined as previously described by the study from the Queen Square Brain Bank (QSBB), UK.Results The mean neuronal cell loss score was significantly higher in the OPC area than in the basal ganglia sites examined, except the dorsolateral putamen. The relative prevalence of pathological phenotypes showed that 40% of cases had OPC-predominant pathology, 18% had StrN-predominant pathology and the remaining (42%) had equivalent StrN and OPC pathology. None of the MSA cases had coexistent Lewy bodies in the dorsal motor nucleus of the vagus and the substantia nigra.Conclusions In contrast to the previously reported results involving British patients' brains from the QSBB (OPC-predominant pathology 17%, StrN-predominant pathology 34%, equivalent StrN and OPC pathology 49%), the results of the present study showed more pathological involvement of the OPC system than of the StrN system. The rarity of Lewy bodies may underlie the phenotypic expression of Japanese MSA. The present observations reflect the disequilibrium in the phenotype distribution between the two populations.