Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study

Maryse Feron, Annabelle Couillandre, Eya Mseddi, Nicolas Termoz, Malek Abidi, Eric Bardinet, Daniel Delgadillo, Timothée Lenglet, Giorgia Querin, Marie Laure Welter, Nadine Le Forestier, François Salachas, Gaelle Bruneteau, Maria del Mar Amador, Rabab Debs, Lucette Lacomblez, Vincent Meininger, Mélanie Pélégrini-Issac, Peter Bede, Pierre François Pradat & 1 others Giovanni de Marco

    Research output: Contribution to journalArticle

    7 Citations (Scopus)

    Abstract

    Introduction: Extrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall risk and their quality-of-life implications. Given the concomitant pyramidal and cerebellar degeneration in ALS, the clinical assessment of extrapyramidal features is particularly challenging. Objective: The comprehensive characterisation of postural instability in ALS using standardised clinical assessments, gait analyses and computational neuroimaging tools in a prospective study design. Methods: Parameters of gait initiation in the anticipatory postural adjustment phase (APA) and execution phase (EP) were evaluated in ALS patients with and without postural instability and healthy controls. Clinical and gait analysis parameters were interpreted in the context of brain imaging findings. Results: ALS patients with postural instability exhibit impaired gait initiation with an altered APA phase, poor dynamic postural control and significantly decreased braking index. Consistent with their clinical profile, “unsteady” ALS patients have reduced caudate and brain stem volumes compared to “steady” ALS patients. Interpretation: Our findings highlight that the ALS functional rating scale (ALSFRS-r) does not account for extrapyramidal deficits, which are major contributors to gait impairment in a subset of ALS patients. Basal ganglia degeneration in ALS does not only contribute to cognitive and behavioural deficits, but also adds to the heterogeneity of motor disability.

    LanguageEnglish
    Pages2125-2136
    Number of pages12
    JournalJournal of Neurology
    Volume265
    Issue number9
    DOIs
    Publication statusPublished - 1 Sep 2018

    Fingerprint

    Amyotrophic Lateral Sclerosis
    Neuroimaging
    Gait
    Basal Ganglia
    Brain Stem
    Quality of Life
    Prospective Studies

    Keywords

    • Amyotrophic lateral sclerosis
    • Basal ganglia
    • Gait impairment
    • Magnetic resonance imaging
    • Postural instability

    Cite this

    Feron, M., Couillandre, A., Mseddi, E., Termoz, N., Abidi, M., Bardinet, E., ... de Marco, G. (2018). Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study. Journal of Neurology, 265(9), 2125-2136. https://doi.org/10.1007/s00415-018-8964-y
    Feron, Maryse ; Couillandre, Annabelle ; Mseddi, Eya ; Termoz, Nicolas ; Abidi, Malek ; Bardinet, Eric ; Delgadillo, Daniel ; Lenglet, Timothée ; Querin, Giorgia ; Welter, Marie Laure ; Le Forestier, Nadine ; Salachas, François ; Bruneteau, Gaelle ; del Mar Amador, Maria ; Debs, Rabab ; Lacomblez, Lucette ; Meininger, Vincent ; Pélégrini-Issac, Mélanie ; Bede, Peter ; Pradat, Pierre François ; de Marco, Giovanni. / Extrapyramidal deficits in ALS : a combined biomechanical and neuroimaging study. In: Journal of Neurology. 2018 ; Vol. 265, No. 9. pp. 2125-2136.
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    abstract = "Introduction: Extrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall risk and their quality-of-life implications. Given the concomitant pyramidal and cerebellar degeneration in ALS, the clinical assessment of extrapyramidal features is particularly challenging. Objective: The comprehensive characterisation of postural instability in ALS using standardised clinical assessments, gait analyses and computational neuroimaging tools in a prospective study design. Methods: Parameters of gait initiation in the anticipatory postural adjustment phase (APA) and execution phase (EP) were evaluated in ALS patients with and without postural instability and healthy controls. Clinical and gait analysis parameters were interpreted in the context of brain imaging findings. Results: ALS patients with postural instability exhibit impaired gait initiation with an altered APA phase, poor dynamic postural control and significantly decreased braking index. Consistent with their clinical profile, “unsteady” ALS patients have reduced caudate and brain stem volumes compared to “steady” ALS patients. Interpretation: Our findings highlight that the ALS functional rating scale (ALSFRS-r) does not account for extrapyramidal deficits, which are major contributors to gait impairment in a subset of ALS patients. Basal ganglia degeneration in ALS does not only contribute to cognitive and behavioural deficits, but also adds to the heterogeneity of motor disability.",
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    Feron, M, Couillandre, A, Mseddi, E, Termoz, N, Abidi, M, Bardinet, E, Delgadillo, D, Lenglet, T, Querin, G, Welter, ML, Le Forestier, N, Salachas, F, Bruneteau, G, del Mar Amador, M, Debs, R, Lacomblez, L, Meininger, V, Pélégrini-Issac, M, Bede, P, Pradat, PF & de Marco, G 2018, 'Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study', Journal of Neurology, vol. 265, no. 9, pp. 2125-2136. https://doi.org/10.1007/s00415-018-8964-y

    Extrapyramidal deficits in ALS : a combined biomechanical and neuroimaging study. / Feron, Maryse; Couillandre, Annabelle; Mseddi, Eya; Termoz, Nicolas; Abidi, Malek; Bardinet, Eric; Delgadillo, Daniel; Lenglet, Timothée; Querin, Giorgia; Welter, Marie Laure; Le Forestier, Nadine; Salachas, François; Bruneteau, Gaelle; del Mar Amador, Maria; Debs, Rabab; Lacomblez, Lucette; Meininger, Vincent; Pélégrini-Issac, Mélanie; Bede, Peter; Pradat, Pierre François; de Marco, Giovanni.

    In: Journal of Neurology, Vol. 265, No. 9, 01.09.2018, p. 2125-2136.

    Research output: Contribution to journalArticle

    TY - JOUR

    T1 - Extrapyramidal deficits in ALS

    T2 - Journal of Neurology

    AU - Feron, Maryse

    AU - Couillandre, Annabelle

    AU - Mseddi, Eya

    AU - Termoz, Nicolas

    AU - Abidi, Malek

    AU - Bardinet, Eric

    AU - Delgadillo, Daniel

    AU - Lenglet, Timothée

    AU - Querin, Giorgia

    AU - Welter, Marie Laure

    AU - Le Forestier, Nadine

    AU - Salachas, François

    AU - Bruneteau, Gaelle

    AU - del Mar Amador, Maria

    AU - Debs, Rabab

    AU - Lacomblez, Lucette

    AU - Meininger, Vincent

    AU - Pélégrini-Issac, Mélanie

    AU - Bede, Peter

    AU - Pradat, Pierre François

    AU - de Marco, Giovanni

    PY - 2018/9/1

    Y1 - 2018/9/1

    N2 - Introduction: Extrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall risk and their quality-of-life implications. Given the concomitant pyramidal and cerebellar degeneration in ALS, the clinical assessment of extrapyramidal features is particularly challenging. Objective: The comprehensive characterisation of postural instability in ALS using standardised clinical assessments, gait analyses and computational neuroimaging tools in a prospective study design. Methods: Parameters of gait initiation in the anticipatory postural adjustment phase (APA) and execution phase (EP) were evaluated in ALS patients with and without postural instability and healthy controls. Clinical and gait analysis parameters were interpreted in the context of brain imaging findings. Results: ALS patients with postural instability exhibit impaired gait initiation with an altered APA phase, poor dynamic postural control and significantly decreased braking index. Consistent with their clinical profile, “unsteady” ALS patients have reduced caudate and brain stem volumes compared to “steady” ALS patients. Interpretation: Our findings highlight that the ALS functional rating scale (ALSFRS-r) does not account for extrapyramidal deficits, which are major contributors to gait impairment in a subset of ALS patients. Basal ganglia degeneration in ALS does not only contribute to cognitive and behavioural deficits, but also adds to the heterogeneity of motor disability.

    AB - Introduction: Extrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall risk and their quality-of-life implications. Given the concomitant pyramidal and cerebellar degeneration in ALS, the clinical assessment of extrapyramidal features is particularly challenging. Objective: The comprehensive characterisation of postural instability in ALS using standardised clinical assessments, gait analyses and computational neuroimaging tools in a prospective study design. Methods: Parameters of gait initiation in the anticipatory postural adjustment phase (APA) and execution phase (EP) were evaluated in ALS patients with and without postural instability and healthy controls. Clinical and gait analysis parameters were interpreted in the context of brain imaging findings. Results: ALS patients with postural instability exhibit impaired gait initiation with an altered APA phase, poor dynamic postural control and significantly decreased braking index. Consistent with their clinical profile, “unsteady” ALS patients have reduced caudate and brain stem volumes compared to “steady” ALS patients. Interpretation: Our findings highlight that the ALS functional rating scale (ALSFRS-r) does not account for extrapyramidal deficits, which are major contributors to gait impairment in a subset of ALS patients. Basal ganglia degeneration in ALS does not only contribute to cognitive and behavioural deficits, but also adds to the heterogeneity of motor disability.

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    Feron M, Couillandre A, Mseddi E, Termoz N, Abidi M, Bardinet E et al. Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study. Journal of Neurology. 2018 Sep 1;265(9):2125-2136. https://doi.org/10.1007/s00415-018-8964-y