Classification and pathology of primary progressive aphasia
Classification and pathology of primary progressive aphasia
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DOI：
10.1212/01.wnl.0000436070.28137.7b
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发表时间：
2013-11-19
期刊：
影响因子：
9.9
通讯作者：
Jones, Matthew
中科院分区：
文献类型：
--
作者：
Harris, Jennifer M.;Gall, Claire;Jones, Matthew
目的：我们的目的是确定进行性语言障碍患者符合2011年原发性进行性失语症（PPA）分类的程度，并检查PPA variants.Methods内的临床病理相关性：62例连续病理证实的痴呆患者临床上出现失语症。排除临床信息不足的患者。PPA分类适用于匿名的临床数据，从患者的初步评估，由评分者谁是盲目的临床和病理diagnosis.Results：最后的队列包括52例患者，其中30人符合基本PPA标准。25例患者符合3种PPA分类之一（13例逻辑缺失，8例非流利/语法缺失，4例语义）。5名患者不符合任何PPA变体的标准。所有符合语义变体PPA的患者和75%符合非流利/语法变体PPA分类的患者均具有额颞叶变性谱病理学。病理学是异质性的患者谁满足logopenic变量PPA标准（46%阿尔茨海默病[AD]，8% AD混合痴呆与路易体，23%额颞叶变性，和23%其他hetero.Conclusion：2011年PPA的建议分类的大部分患者谁满足基本PPA标准。然而，一些患者的失语综合征无法分类，这表明2011年的建议没有涵盖PPA变体的全部范围。语义变体PPA的分类提供了对潜在病理的良好预测。逻辑缺失变异的分类不能成功区分AD引起的PPA与其他病理引起的PPA。
Objective: We aimed to determine the extent to which patients with progressive language impairment conform to 2011 primary progressive aphasia (PPA) classification and to examine clinicopathologic correlations within PPA variants.Methods: Sixty-two consecutive patients with pathologically confirmed dementia who presented clinically with aphasia were identified. Patients with insufficient clinical information were excluded. PPA classifications were applied to anonymized clinical data taken from patients' initial assessment by raters who were blinded to clinical and pathologic diagnosis.Results: The final cohort comprised 52 patients, 30 of whom met basic PPA criteria. Twenty-five patients met one of the 3 PPA classifications (13 logopenic, 8 nonfluent/agrammatic, and 4 semantic). Five patients did not meet the criteria for any of the PPA variants. All patients who met semantic variant PPA and 75% of patients who met nonfluent/agrammatic variant PPA classifications had frontotemporal lobar degeneration spectrum pathology. Pathologies were heterogeneous in patients who met logopenic variant PPA criteria (46% Alzheimer disease [AD], 8% AD mixed with dementia with Lewy bodies, 23% frontotemporal lobar degeneration, and 23% other).Conclusion: The 2011 PPA recommendations classify a large proportion of patients who meet basic PPA criteria. However, some patients had aphasic syndromes that could not be classified, suggesting that the 2011 recommendations do not cover the full range of PPA variants. Classification of semantic variant PPA provides a good prediction of underlying pathology. Classification of logopenic variant does not successfully differentiate PPA due to AD from PPA due to other pathologies.