Peterson Katie A, Jones P Simon, Patel Nikil, Tsvetanov Kamen A, Ingram Ruth, Cappa Stefano F, Lambon Ralph Matthew A, Patterson Karalyn, Garrard Peter, Rowe James B
Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust, University of Cambridge, Cambridge, United Kingdom.
Department of Neurosciences, St. George's, University of London, London, United Kingdom.
Front Aging Neurosci. 2021 Jul 19;13:675739. doi: 10.3389/fnagi.2021.675739. eCollection 2021.
: Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) affect speech and language as well as motor functions. Clinical and neuropathological data indicate a close relationship between these two disorders and the non-fluent variant of primary progressive aphasia (nfvPPA). We use the recently developed Mini Linguistic State Examination tool (MLSE) to study speech and language disorders in patients with PSP, CBS, and nfvPPA, in combination with structural magnetic resonance imaging (MRI). : Fifty-one patients (PSP = 13, CBS = 19, nfvPPA = 19) and 30 age-matched controls completed the MLSE, the short form of the Boston Diagnostic Aphasia Examination (BDAE), and the Addenbrooke's Cognitive Examination III. Thirty-eight patients and all controls underwent structural MRI at 3 Tesla, with T1 and T2-weighted images processed by surface-based and subcortical segmentation within FreeSurfer 6.0.0 to extract cortical thickness and subcortical volumes. Morphometric differences were compared between groups and correlated with the severity of speech and language impairment. : CBS and PSP patients showed impaired MLSE performance, compared to controls, with a similar language profile to nfvPPA, albeit less severe. All patient groups showed reduced cortical thickness in bilateral frontal regions and striatal volume. PSP and nfvPPA patients also showed reduced superior temporal cortical thickness, with additional thalamic and amygdalo-hippocampal volume reductions in nfvPPA. Multivariate analysis of brain-wide cortical thickness and subcortical volumes with MLSE domain scores revealed associations between performance on multiple speech and language domains with atrophy of left-lateralised fronto-temporal cortex, amygdala, hippocampus, putamen, and caudate. : The effect of PSP and CBS on speech and language overlaps with nfvPPA. These three disorders cause a common anatomical pattern of atrophy in the left frontotemporal language network and striatum. The MLSE is a short clinical screening tool that can identify the language disorder of PSP and CBS, facilitating clinical management and patient access to future clinical trials.
进行性核上性麻痹(PSP)和皮质基底节综合征(CBS)会影响言语和语言以及运动功能。临床和神经病理学数据表明,这两种疾病与原发性进行性失语的非流利型(nfvPPA)之间存在密切关系。我们使用最近开发的迷你语言状态检查工具(MLSE),结合结构磁共振成像(MRI),来研究PSP、CBS和nfvPPA患者的言语和语言障碍。
51名患者(PSP = 13例,CBS = 19例,nfvPPA = 19例)和30名年龄匹配的对照者完成了MLSE、波士顿诊断性失语检查简表(BDAE)和Addenbrooke认知检查III。38名患者和所有对照者在3特斯拉下接受了结构MRI检查,T1和T2加权图像在FreeSurfer 6.0.0中通过基于表面和皮质下分割进行处理,以提取皮质厚度和皮质下体积。比较了各组之间的形态学差异,并将其与言语和语言障碍的严重程度相关联。
与对照者相比,CBS和PSP患者的MLSE表现受损,其语言特征与nfvPPA相似,尽管程度较轻。所有患者组双侧额叶区域的皮质厚度和纹状体体积均减小。PSP和nfvPPA患者还表现出颞上皮质厚度减小,nfvPPA患者的丘脑和杏仁核 - 海马体积进一步减小。对全脑皮质厚度和皮质下体积与MLSE领域得分进行多变量分析发现,多个言语和语言领域的表现与左侧额颞叶皮质、杏仁核、海马、壳核和尾状核萎缩之间存在关联。
PSP和CBS对言语和语言的影响与nfvPPA重叠。这三种疾病在左侧额颞叶语言网络和纹状体中导致了共同的萎缩解剖模式。MLSE是一种简短的临床筛查工具,可以识别PSP和CBS的语言障碍,有助于临床管理并使患者能够参与未来的临床试验。