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Language Disorder in Progressive Supranuclear Palsy and Corticobasal Syndrome: Neural Correlates and Detection by the MLSE Screening Tool.

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Peer-reviewed

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Article

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Authors

Peterson, Katie A 
Jones, P Simon 
Patel, Nikil 
Tsvetanov, Kamen A.  ORCID logo  https://orcid.org/0000-0002-3178-6363
Ingram, Ruth 

Abstract

Background: 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). Methods: Fifty-one patients (PSP N = 13, CBS N = 19, nfvPPA N = 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. Results: 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. Conclusions: 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.

Description

Keywords

aphasia, corticobasal syndrome, language, progressive supranuclear palsy, speech

Journal Title

Front Aging Neurosci

Conference Name

Journal ISSN

1663-4365
1663-4365

Volume Title

13

Publisher

Frontiers Media SA

Rights

All rights reserved
Sponsorship
MRC (via St George's, University of London) (13120-10)
Guarantors of Brain (Unknown)
MRC (unknown)
Cambridge University Hospitals NHS Foundation Trust (CUH) (146281)
Patrick Berthoud Charitable Trust (via Charities Aid Foundation) (Unknown)
Medical Research Council (MC_UU_00005/18)
National Institute for Health and Care Research (IS-BRC-1215-20014)
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