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J Neurol. 2018 Sep;265(9):2125-2136. doi: 10.1007/s00415-018-8964-y. Epub 2018 Jul 11.

Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study.

Author information

1
Laboratoire CeRSM - EA 2931 Paris Ouest, Nanterre, France.
2
COMUE Université Paris Lumières, Paris, France.
3
Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), UMR-S975, 75013, Paris, France.
4
Inserm, U975, 75013, Paris, France.
5
CNRS, UMR 7225, 75013, Paris, France.
6
Institut du Cerveau et de la Moelle Epinière, Centre de Neuroimagerie de Recherche (CENIR), 75013, Paris, France.
7
Service de Neuro-Oncologie, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Pitié-Salpêtrière/Charles Foix, 75013, Paris, France.
8
Département de Neurologie, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Pitié-Salpêtrière/Charles Foix, 75013, Paris, France.
9
Sorbonne Université, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, LIB, 75006, Paris, France.
10
Institut du Cerveau et de la Moelle Epinière, Plateforme d'Analyse du Mouvement (PANAM), 75013, Paris, France.
11
Neurophysiology Department, CHU Charles Nicolle, Université de Rouen, Rouen, France.
12
Département de recherche en éthique, Université Paris-Sud-Saclay, Le Kremlin-Bicêtre, France.
13
Hôpital des Peupliers, Ramsay générale de santé, Paris, France.
14
Computational Neuroimaging Group, Academic Unit of Neurology, Trinity College, Dublin, Ireland.
15
COMUE Université Paris Lumières, Paris, France. pierre-francois.pradat@psl.aphp.fr.
16
Département de Neurologie, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Pitié-Salpêtrière/Charles Foix, 75013, Paris, France. pierre-francois.pradat@psl.aphp.fr.
17
Sorbonne Université, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, LIB, 75006, Paris, France. pierre-francois.pradat@psl.aphp.fr.
18
Northern Ireland Centre for Stratified Medicine, Biomedical Sciences Research Institute, Ulster University, C-TRIC, Altnagelvin Hospital, Londonderry, UK. pierre-francois.pradat@psl.aphp.fr.

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.

KEYWORDS:

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

PMID:
29995291
DOI:
10.1007/s00415-018-8964-y

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