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27.09.2023 | Original Communication

Language deficits in primary lateral sclerosis: cortical atrophy, white matter degeneration and functional disconnection between cerebral regions

verfasst von: Ee Ling Tan, Marlene Tahedl, Jasmin Lope, Jennifer C. Hengeveld, Mark A. Doherty, Russell L. McLaughlin, Orla Hardiman, Kai Ming Chang, Eoin Finegan, Peter Bede

Erschienen in: Journal of Neurology | Ausgabe 1/2024

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Abstract

Background

Primary lateral sclerosis (PLS) is traditionally regarded as a pure upper motor neuron disorder, but recent cases series have highlighted cognitive deficits in executive and language domains.

Methods

A single-centre, prospective neuroimaging study was conducted with comprehensive clinical and genetic profiling. The structural and functional integrity of language-associated brain regions and networks were systematically evaluated in 40 patients with PLS in comparison to 111 healthy controls. The structural integrity of the arcuate fascicle, frontal aslant tract, inferior occipito-frontal fascicle, inferior longitudinal fascicle, superior longitudinal fascicle and uncinate fascicle was evaluated. Functional connectivity between the supplementary motor region and the inferior frontal gyrus and connectivity between Wernicke’s and Broca’s areas was also assessed.

Results

Cortical thickness reductions were observed in both Wernicke’s and Broca’s areas. Fractional anisotropy reduction was noted in the aslant tract and increased radical diffusivity (RD) identified in the aslant tract, arcuate fascicle and superior longitudinal fascicle in the left hemisphere. Functional connectivity was reduced along the aslant track, i.e. between the supplementary motor region and the inferior frontal gyrus, but unaffected between Wernicke’s and Broca’s areas. Cortical thickness alterations, structural and functional connectivity changes were also noted in the right hemisphere.

Conclusions

Disease-burden in PLS is not confined to motor regions, but there is also a marked involvement of language-associated tracts, networks and cortical regions. Given the considerably longer survival in PLS compared to ALS, the impact of language impairment on the management of PLS needs to be carefully considered.

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Titel: Language deficits in primary lateral sclerosis: cortical atrophy, white matter degeneration and functional disconnection between cerebral regions
verfasst von: Ee Ling Tan
Marlene Tahedl
Jasmin Lope
Jennifer C. Hengeveld
Mark A. Doherty
Russell L. McLaughlin
Orla Hardiman
Kai Ming Chang
Eoin Finegan
Peter Bede
Publikationsdatum: 27.09.2023
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 1/2024
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-023-11994-7

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