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The Study of Language in the Amyotrophic Lateral Sclerosis - Frontotemporal Spectrum Disorder: a Systematic Review of Findings and New Perspectives

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Abstract

Amyotrophic Lateral Sclerosis is a neurodegenerative disorder characterized primarily by motor network disruption. Extra-motor manifestations including executive functions, social cognition, and behavioral changes are now well recognized as important features of ALS, and are associated with frontotemporal and frontostriatal network disruption. However, the presence and characterization of language changes has received less attention. This systematic review characterizes the profile of reported language dysfunction in ALS. PRISMA guidelines were implemented to carry out and report the review. Current evidence suggests that areas of neuroanatomical disruption in ALS spread to language centers such as posterior, inferior frontal and superior temporal areas leading to deficits in word retrieval, syntactic and grammatical processing, and spelling. However, the majority of studies of language in ALS have been limited by the recruitment of small clinic-based prevalent samples and important questions remain regarding the incidence and progression of language impairment in ALS. Further studies from population-based incident cohorts will help to determine the range of language deficits in ALS, and how these relate to previously defined executive and behavioral sub-phenotypes.

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Acknowledgements

This work is funded by The Motor Neuron Disease Association (MNDA).

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Authors and Affiliations

  1. Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College Dublin, 152-160 Pearse Street, Dublin 2, Ireland

    Marta Pinto-Grau, Orla Hardiman & Niall Pender

  2. Department of Psychology, Beaumont Hospital, Dublin, Ireland

    Marta Pinto-Grau & Niall Pender

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  1. Marta Pinto-Grau
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  2. Orla Hardiman
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Correspondence to Marta Pinto-Grau.

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Ms. Marta Pinto-Grau and Professor Niall Pender have nothing to disclose. Professor Orla Hardiman serves as Editor-in-Chief of the journal Amyotrophic Lateral Sclerosis and the Frontotemporal Degenerations.

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Pinto-Grau, M., Hardiman, O. & Pender, N. The Study of Language in the Amyotrophic Lateral Sclerosis - Frontotemporal Spectrum Disorder: a Systematic Review of Findings and New Perspectives. Neuropsychol Rev 28, 251–268 (2018). https://doi.org/10.1007/s11065-018-9375-7

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