α-synuclein-positive structures in association with diffuse neurofibrillary tangles with calcification
α-synuclein-positive structures in association with diffuse neurofibrillary tangles with calcification
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DOI：
10.1046/j.1365-2990.2003.00470.x
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发表时间：
2003-06-01
影响因子：
5
通讯作者：
Sobue, G
中科院分区：
文献类型：
--
作者：
Hishikawa, N;Hashizume, Y;Sobue, G
已知α-突触核蛋白是帕金森病(PD)路易体(Lbs)和多系统萎缩的神经元和神经胶质细胞胞浆内含物(NCIS、GCIS)的主要成分。α-突触核蛋白阳性包涵体，如LBS、NCIS和GCIS，有时与tau阳性神经丝共存。使用α-突触核蛋白免疫组织化学的研究经常在家族性或散发性阿尔茨海默病(AD)患者的杏仁核中发现LBS，以及在唐氏综合症和AD患者中发现LBS。然而，还没有研究报道弥漫性神经纤维缠结伴钙化(DNTC)病例中的α-突触核蛋白阳性结构，其特征是大脑皮质中大量的神经纤维缠结(NFT)，但很少有老年斑。我们研究了两例DNTC中α-突触核蛋白阳性结构的分布：一名65岁的女性(脑重量，850克)和一名75岁的女性(脑重，800克)。在这两个病例中，都注意到以颞叶为主的严重脑萎缩。在显微镜下，在受NFTs影响最严重的区域--颞叶上叶(在颞叶内)、杏仁核、副海马体、内嗅皮层和岛叶可见α-突触蛋白阳性的胞浆内包涵体和轴突。在大脑皮层和脑干的其他区域，α-突触核蛋白阳性的胞浆内包涵体很少见或不存在。这种分布模式不同于帕金森病或痴呆伴LBS的分布模式。我们的发现表明，α-突触核蛋白的积聚模式是DNTC的一个病理特征，并且DNTC与tau和α-突触核蛋白的积聚有关。
alpha-Synuclein is known to be a major constituent of the Lewy bodies (LBs) in Parkinson's disease (PD) and the neuronal and glial cytoplasmic inclusions (NCIs, GCIs) in multiple system atrophy. alpha-Synuclein-positive inclusions such as LBs, NCIs and GCIs sometimes show colocalization with tau-positive neurofilaments. Studies using alpha-synuclein immunohistochemistry have often found LBs in the amygdala of patients with familial or sporadic Alzheimer's disease (AD), as well as in patients with Down's syndrome and AD. However, no studies have reported alpha-synuclein-positive structures in cases of diffuse neurofibrillary tangles with calcification (DNTC), which is characterized by numerous neurofibrillary tangles (NFTs) throughout the cerebral cortex but few, if any, senile plaques. We investigated the distribution of alpha-synuclein-positive structures in two cases of DNTC: a 65-year-old woman (brain weight, 850 g) and a 75-year-old woman (brain weight, 800 g). In both cases, severe cerebral atrophy predominant in the temporal lobe was noted. Microscopically, alpha-synuclein-positive intracytoplasmic inclusions and neurites were found in the superior temporal lobe (within the temporal pole), amygdala, parahippocampus, entorhinal cortex and insula, the regions most affected by the NFTs. alpha-Synuclein-positive intracytoplasmic inclusions were rare or absent in other regions of the cerebral cortex and brainstem. This distribution pattern differs from that of PD or dementia with LBs. Our findings suggest that the accumulation pattern of alpha-synuclein is a pathological feature of DNTC, and that DNTC is associated with accumulation of both tau and alpha-synuclein.