Responsiveness and Minimal Clinically Important Difference of the Motor Function Measure in Collagen VI-Related Dystrophies and Laminin Alpha2-Related Muscular Dystrophy.


Journal

Archives of physical medicine and rehabilitation
ISSN: 1532-821X
Titre abrégé: Arch Phys Med Rehabil
Pays: United States
ID NLM: 2985158R

Informations de publication

Date de publication:
04 2021
Historique:
received: 23 06 2020
revised: 16 09 2020
accepted: 01 10 2020
pubmed: 10 11 2020
medline: 20 4 2021
entrez: 9 11 2020
Statut: ppublish

Résumé

To investigate the responsiveness of the motor function measure (MFM) and determine the minimal clinically important difference (MCID) in individuals with 2 common types of congenital muscular dystrophy (CMD). Observational, prospective, single center, cohort study. National Institute of Neurological Disorders and Stroke (NINDS) of the National Institutes of Health (NIH). Individuals (N=44) with collagen VI-related dystrophies (COL6-RD, n=23) and 21 individuals laminin alpha2-related muscular dystrophy (LAMA2-RD, n=21) enrolled in a 4-year longitudinal natural history study. Not applicable. Responsiveness of the MFM-32 and the Rasch-scaled MFM-25 and the MCID of the MFM-32 determined from a patient-reported anchor with 2 different methods, within-patient and between-patient. The original MFM-32 and Rasch-scaled MFM-25 performed similarly overall in both the COL6-RD and LAMA2-RD populations, with all subscores (D1, standing and transfers; D2, axial and proximal; D3, distal) showing a significant decrease over time, except MFM D1 and D3 for LAMA2-RD. The MFM D1 subscore was the most sensitive to change for ambulant individuals, whereas the MFM D2 subscore was the most sensitive to change for nonambulant individuals. The MCID for the MFM-32 total score was calculated as 2.5 and 3.9 percentage points according to 2 different methods. The MFM showed strong responsiveness in individuals with LAMA2-RD and COL6-RD. Because a floor effect was identified more prominently with the Rasch-Scaled MFM-25, the use of the original MFM-32 as a quantitative variable with the assumption of scale linearity appears to be a good compromise. When designing clinical trials in congenital muscular dystrophies, the use of MCID for MFM should be considered to determine if a given intervention effects show not only a statistically significant change but also a clinically meaningful change.

Identifiants

pubmed: 33166523
pii: S0003-9993(20)31214-4
doi: 10.1016/j.apmr.2020.10.116
pmc: PMC10363856
mid: NIHMS1865249
pii:
doi:

Types de publication

Journal Article Observational Study Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

604-610

Subventions

Organisme : Intramural NIH HHS
ID : Z99 CL999999
Pays : United States

Informations de copyright

Copyright © 2020 American Congress of Rehabilitation Medicine. All rights reserved.

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Auteurs

Laure Le Goff (L)

Department of Pediatric Physical Medicine and Rehabilitation, Hospices Civils de Lyon, Bron, France. Electronic address: laure.le-goff@chu-lyon.fr.

Katherine G Meilleur (KG)

National Institute of Nursing Research, National Institutes of Health, Bethesda, MD; Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

Gina Norato (G)

Office of Biostatistics, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

Pascal Rippert (P)

Public Health Center, Research and Clinical Epidemiology Department, Hospices Civils de Lyon, Lyon, France.

Minal Jain (M)

Mark O. Hatfield Clinical Research Center, National Institutes of Health, Bethesda, MD.

Margaret Fink (M)

Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

A Reghan Foley (AR)

Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

Melissa Waite (M)

Mark O. Hatfield Clinical Research Center, National Institutes of Health, Bethesda, MD.

Sandra Donkervoort (S)

Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

Carsten G Bönnemann (CG)

Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

Carole Vuillerot (C)

Department of Pediatric Physical Medicine and Rehabilitation, Hospices Civils de Lyon, Bron, France; Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD; Neuromyogen Institute, CNRS UMR 5310-INSERM, University of Lyon, Lyon, France; University of Lyon 1, F-69100, Villeurbanne, France.

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