Selective neurofibrillary degeneration of the hippocampal CA2 sector is associated with four-repeat tauopathies
Selective neurofibrillary degeneration of the hippocampal CA2 sector is associated with four-repeat tauopathies
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DOI：
10.1093/jnen/61.12.1040
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发表时间：
2002-12-01
影响因子：
3.2
通讯作者：
Dickson, DW
中科院分区：
文献类型：
--
作者：
Ishizawa, T;Ko, LW;Dickson, DW
海马体的 CA2 部分在衰老和阿尔茨海默病中相对抵抗大量的欧洲原纤维缠结；然而，有些病例的 CA2 区出现选择性神经原纤维变性，而更脆弱的 CA1 区则不受影响。此类病例不符合 Braak 和 Braak 分期方案，可被视为具有空气非典型神经原纤维变性模式。我们鉴定了 24 例非典型病例，平均死亡年龄为 78.6 +/- 1.4 岁，平均 Braak 分期为 3.2 +/- 0.4，并通过 Gallyas 银染色、tau 和 alphaB-晶状体蛋白免疫组织化学描述了其病理和遗传特征；以及载脂蛋白-E (ApoE) 和 tau 基因分型。由于存在海马硬化，三例病例被排除在进一步分析之外。其余 21 例非典型病例中，除 1 例外，其余均为四次重复 (4R) tau蛋白病，包括进行性核上性麻痹；皮质基底节变性和嗜银颗粒病（AGD）。值得注意的是； 21例非典型病例中有19例为单纯或混合型AGD病例。 ApoE epsilon4 等位基因频率与正常对照相似；而在非典型病例中，延伸tau H1单倍型的频率有增加的趋势：海马CA2区的选择性神经原纤维变性尚未得到广泛认可；但当检测到时应提示 4R-tau 蛋白病的可能性，特别是 AGD。
The CA2 sector of the hippocampus is relatively resistant ton eurofibrillary tangles in aging and Alzheimer disease; however, some cases have selective neurofibrillary degeneration in CA2 with sparing of the, more vulnerable CA1 sector. Cases such as this do not fit in the Braak and Braak staging scheme and can be considered to have air atypical pattern of neurofibrillary degeneration. We identified 24, atypical cases with an average, age at death of 78.6 +/- 1.4 yr and average Braak stage of 3.2 +/- 0.4 and describe their pathologic and genetic characteristics with Gallyas silver staining, immunohistochemistry for tau and alphaB-crystallin; as well as apolipoprotein-E (ApoE) and tau genotyping. Three cases were excluded from further analysis due to presence of hippocampal sclerosis. All but 1 of the remaining 21 atypical cases were four-repeat (4R) tauopathies, including progressive supranuclear palsy; corticobasal degeneration, and argyrophilic grain disease (AGD). Remarkably; 19 of the 21 atypical cases were pure or mixed cases of AGD. The ApoE epsilon4 allele frequency was similar to normal controls; while there was a trend for an increased frequency of the extended tau H1 haplotype in atypical cases: Selective neurofibrillary degeneration in CA2 sector of the hippocampus is not widely recognized; but when detected should suggest the possibility of a 4R-tauopathy, particularly AGD.