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

Katie A Peterson; P Simon Jones; Nikil Patel; Kamen A Tsvetanov; Ruth Ingram; Stefano F Cappa; Matthew A Lambon Ralph; Karalyn Patterson; Peter Garrard; James B Rowe

doi:10.3389/fnagi.2021.675739

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

Front Aging Neurosci. 2021 Jul 19:13:675739. doi: 10.3389/fnagi.2021.675739. eCollection 2021.

Authors

Katie A Peterson¹, P Simon Jones¹, Nikil Patel², Kamen A Tsvetanov³, Ruth Ingram⁴, Stefano F Cappa^{5

6}, Matthew A Lambon Ralph⁷, Karalyn Patterson^{1

7}, Peter Garrard², James B Rowe^{1

7}

Affiliations

¹ Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust, University of Cambridge, Cambridge, United Kingdom.
² Department of Neurosciences, St. George's, University of London, London, United Kingdom.
³ Department of Psychology, University of Cambridge, Cambridge, United Kingdom.
⁴ Division of Neuroscience and Experimental Psychology, University of Manchester, Manchester, United Kingdom.
⁵ IUSS Cognitive Neuroscience Center (ICoN), University Institute for Advanced Studies IUSS, Pavia, Italy.
⁶ Dementia Research Center, IRCCS Mondino Foundation, Pavia, Italy.
⁷ MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, United Kingdom.

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.

Keywords: aphasia; corticobasal syndrome; language; progressive supranuclear palsy; speech.

Abstract

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