Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech
Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech
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DOI：
10.1093/brain/awl078
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发表时间：
2006-06-01
期刊：
影响因子：
14.5
通讯作者：
Petersen, Ronald C.
中科院分区：
文献类型：
--
作者：
Josephs, Keith A.;Duffy, Joseph R.;Petersen, Ronald C.
言语失用症（Apraxia of speech，AOS）是一种运动性言语障碍，其特征是语速缓慢，韵律异常，语音替换、添加、重复和重复失真，有时伴有摸索和试错发音运动。虽然AOS经常被归入失语症的标题下，实际上最经常与失语症共同发生，但它可以是退行性神经系统疾病的主要表现，甚至是唯一表现。在这项研究中，我们确定失语症和AOS的临床分类是否与病理诊断和特定的生化和解剖结构异常相关。17例初步诊断为退行性失语症或AOS的病例被两名语言病理学家重新独立分类，他们对病理和生化结果不知情，将其分为五个操作定义的失语症和AOS类别之一。病理诊断为进行性核上性麻痹6例，皮质基底节变性5例，额颞叶变性伴仅泛素免疫反应性改变5例，皮克病1例。使用基于体素的形态测量法（VBM）和单光子发射断层扫描完成磁共振成像分析，对临床诊断设盲，然后寻找临床成像和临床病理学关联。所有评价的法官间临床分类可靠性为87%（kappa = 0.8）。11例病例有AOS证据，其中所有（100%）的病理诊断特征为基础的tau生物化学，而其他6例无AOS的病例中有5例没有tau生物化学（P = 0.001）。17例病例中的大多数进行了一年以上的评估，证明了言语和语言综合征以及运动体征的演变。VBM显示运动前区和辅助运动皮层是与AOS相关的主要皮层区域，而前外侧裂周围区与非流利性失语相关。对退行性失语症和AOS的分类进行细化，可能有助于提高我们对行为、病理和影像学相关性之间关系的理解。
Apraxia of speech (AOS) is a motor speech disorder characterized by slow speaking rate, abnormal prosody and distorted sound substitutions, additions, repetitions and prolongations, sometimes accompanied by groping, and trial and error articulatory movements. Although AOS is frequently subsumed under the heading of aphasia, and indeed most often co-occurs with aphasia, it can be the predominant or even the sole manifestation of a degenerative neurological disease. In this study we determine whether the clinical classifications of aphasia and AOS correlated with pathological diagnoses and specific biochemical and anatomical structural abnormalities. Seventeen cases with initial diagnoses of a degenerative aphasia or AOS were re-classified independently by two speech-language pathologists-blinded to pathological and biochemical findings-into one of five operationally defined categories of aphasia and AOS. Pathological diagnoses in the 17 cases were progressive supranuclear palsy in 6, corticobasal degeneration in 5, frontotemporal lobar degeneration with ubiquitin-only-immunoreactive changes in 5 and Pick's disease in 1. Magnetic resonance imaging analysis using voxel-based morphometry (VBM), and single photon emission tomography were completed, blinded to the clinical diagnoses, and clinicoimaging and clinicopathological associations were then sought. Interjudge clinical classification reliability was 87% (kappa = 0.8) for all evaluations. Eleven cases had evidence of AOS, of which all (100%) had a pathological diagnosis characterized by underlying tau biochemistry, while five of the other six cases without AOS did not have tau biochemistry (P = 0.001). A majority of the 17 cases had more than one yearly evaluation, demonstrating the evolution of the speech and language syndromes, as well as motor signs. VBM revealed the premotor and supplemental motor cortices to be the main cortical regions associated with AOS, while the anterior peri-sylvian region was associated with non-fluent aphasia. Refining the classification of the degenerative aphasias and AOS may be necessary to improve our understanding of the relationships among behavioural, pathological and imaging correlations.