Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations.

Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations.
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DOI:
10.1007/s00401-008-0460-5
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发表时间:
2009-01
影响因子:
12.7
通讯作者:
Mann DM
Mann DM
中科院分区:
医学1区
文献类型:
--
作者:
Mackenzie IR;Neumann M;Bigio EH;Cairns NJ;Alafuzoff I;Kril J;Kovacs GG;Ghetti B;Halliday G;Holm IE;Ince PG;Kamphorst W;Revesz T;Rozemuller AJ;Kumar-Singh S;Akiyama H;Baborie A;Spina S;Dickson DW;Trojanowski JQ;Mann DM

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与临床症状额颞叶痴呆(FTD,行为变异性FTD)、进行性非语意性失语症(PNFA)和语义性痴呆(SD)相关的神经病理学是异质性的,其共同特征是相对选择性的额叶和颞叶变性(frontotemporal lobar degeneration, FTLD)。与其他神经退行性疾病一样,大多数FTLD病理亚型以特定种类的细胞内蛋白包涵体为特征。在过去的几十年里,许多包涵体的生化组成已经被确定。基于假定的分子缺陷对FTLD进行分类的趋势越来越多,人们相信这最能反映潜在的致病过程,而且因为过去许多同名和描述性命名的综合征现在已知具有不完善的临床病理相关性。
The neuropathology associated with the clinical entities frontotemporal dementia (FTD, behavioral variant FTD), progressive non-Xuent aphasia (PNFA) and semantic dementia (SD), is heterogeneous with the common feature being a relatively selective degeneration of the frontal and temporal lobes (frontotemporal lobar degeneration, FTLD). As in other neurodegenerative conditions, most pathological subtypes of FTLD are characterized by specific kinds of intracellular protein inclusions. In the past few decades, the biochemical composition of many of these inclusion bodies has been determined. There is a growing trend to classify FTLD based on the presumed molecular defect, in the belief that this most closely reXects the underlying pathogenic process and because many of the eponymous and descriptively named syndromes of the past are now known to have imperfect clinicopathological correlation.
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