Prediction of pathology in primary progressive language and speech disorders
Prediction of pathology in primary progressive language and speech disorders
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DOI：
10.1212/wnl.0b013e3181c7198e
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发表时间：
2010-01-05
期刊：
影响因子：
9.9
通讯作者：
Pasquier, F.
中科院分区：
文献类型：
--
作者：
Deramecourt, V.;Lebert, F.;Pasquier, F.
目的：额颞叶变性（FTLD）包括多种临床病理实体。生前预测潜在的病理损害被认为是困难的。本研究旨在描述1）原发性进行性语言和言语障碍的不同临床变异与2）病理诊断之间的相关性。方法：后者适用于1993年至2008年期间在法国里尔记忆诊所（Lille Memory Clinic）进行前瞻性监测的18例患者。进行性构音障碍5例，无语法性进行性失语6例，逻辑缺乏性进行性失语1例，进行性行话性失语2例，典型语义性痴呆（n = 2）和非典型语义性痴呆（n = 2）。所有进行性构音障碍患者在尸检评估时均具有tau病理学：进行性核上性麻痹（n = 2）、皮克病（n = 2）和皮质基底节变性（n = 1）。所有无语法型原发性进行性失语患者均有TDP-43阳性FTLD（FTLD-TDP）。进行性失语和进行性行话失语患者均为Alzheimer病。这两例典型的语义性痴呆都有FTLD-TDP。非典型语义性痴呆患者有tau蛋白病理改变：嗜银颗粒病和皮质基底节变性。结论：病理改变的不同解剖分布可以解释这些结果：在伴有进行性构音障碍的tau病变中，鳃盖和皮质下区域，在TDP-43阳性额颞叶变性中，左侧额颞皮质在FTLD-TDP或嗜银颗粒病伴语义性痴呆患者中，双侧颞叶外侧和前颞叶皮质出现异常;在Alzheimer病伴语言缺乏性进行性失语或行话性失语患者中，左侧颞叶顶叶皮质出现异常。这些相关性必须在更大的系列中得到证实。神经病学（R）2010;74：42-49
Objective: Frontotemporal lobar degeneration (FTLD) encompasses a variety of clinicopathologic entities. The antemortem prediction of the underlying pathologic lesions is reputed to be difficult. This study sought to characterize correlations between 1) the different clinical variants of primary progressive language and speech disorders and 2) the pathologic diagnosis.Methods: The latter was available for 18 patients having been prospectively monitored in the Lille Memory Clinic (France) between 1993 and 2008.Results: The patients were diagnosed with progressive anarthria (n = 5), agrammatic progressive aphasia (n = 6), logopenic progressive aphasia (n = 1), progressive jargon aphasia (n = 2), typical semantic dementia (n = 2), and atypical semantic dementia (n = 2). All patients with progressive anarthria had a tau pathology at postmortem evaluation: progressive supranuclear palsy (n = 2), Pick disease (n = 2), and corticobasal degeneration (n = 1). All patients with agrammatic primary progressive aphasia had TDP-43-positive FTLD (FTLD-TDP). The patients with logopenic progressive aphasia and progressive jargon aphasia had Alzheimer disease. Both cases of typical semantic dementia had FTLD-TDP. The patients with atypical semantic dementia had tau pathologies: argyrophilic grain disease and corticobasal degeneration.Conclusions: The different anatomic distribution of the pathologic lesions could explain these results: opercular and subcortical regions in tau pathologies with progressive anarthria, the left frontotemporal cortex in TDP-43-positive frontotemporal lobar degeneration (FTLD-TDP) with agrammatic progressive aphasia, the bilateral lateral and anterior temporal cortex in FTLD-TDP or argyrophilic grain disease with semantic dementia, and the left parietotemporal cortex in Alzheimer disease with logopenic progressive aphasia or jargon aphasia. These correlations have to be confirmed in larger series. Neurology (R) 2010;74:42-49