Abstract
Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder presenting with major muscle impairments. However, few studies have addressed muscle strength progression using quantitative methods. The aims of this study were to document muscle strength changes in eight muscle groups among adults with DM1 over a 9-year period, and to compare this progression between phenotypes (adult and late-onset) and sex. Patients with a genetic diagnosis of DM1 with the late-onset or the adult phenotype were recruited at baseline through the clinical registry of the Saguenay Neuromuscular Clinic. The maximum isometric muscle strength was measured at baseline and 9 years later using a standardized protocol of quantitative muscle testing. Muscle groups included were shoulder abductors, elbow flexors/extensors, wrist extensors, hip flexors, knee flexors/extensors, and ankle dorsiflexors. For the whole group, a mean loss of 24.5–52.8% was observed over the 9-year period for all muscle groups, except for hip flexors which remained stable. Generally, men were stronger and showed a significant greater rate of decline of muscle strength than women. The adult and late-onset phenotypes taken separately also showed a significant and similar decline over the 9-year period, except for the wrist and knee extensors where muscle strength of participants with the adult phenotype decreased faster than in the late-onset phenotype. The similar rate of decline of muscle strength loss observed between phenotypes highlights the need to develop interventions to prevent this decline, even for patients with the late-onset phenotype who are often considered as mildly impaired, and therefore neglected by the rehabilitation services.
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Acknowledgements
We would like to thank all the participants who took the time to take part in this study. Cynthia Gagnon (grant no. 31011) was funded by the Fonds de recherche du Québec – Santé (FRQS) as junior 2 researcher. The study was funded (grant no. JNM-108412) by the Canadian Institutes of Health Research (CIHR) and Muscular Dystrophy Canada, the Réseau Provincial de recherche en adaptation-réadaptation (REPAR-FRQS), Canada, and the Einar Belvén Foundation, Sweden.
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This study has been approved by the appropriate ethics committee (Ethics Review Board of the CIUSSS du Saguenay–Lac-St-Jean (Quebec, Canada)) and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
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Mean muscle strength decline in Newton-meters (Nm) between baseline (T1) and follow-up (T2) for a shoulder abductors, b wrist extensors, c elbow flexors, and d elbow extensors comparing between adult and late onset phenotypes, and between male and female (PNG 1893 KB)
415_2018_8898_MOESM2_ESM.png
Mean muscle strength decline in Newton-meters (Nm) between baseline (T1) and follow-up (T2) for a knee flexors, b knee extensors, c hip flexors, and d ankle dorsiflexors comparing between adult and late onset phenotypes, and between male and female (PNG 1943 KB)
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Gagnon, C., Petitclerc, É., Kierkegaard, M. et al. A 9-year follow-up study of quantitative muscle strength changes in myotonic dystrophy type 1. J Neurol 265, 1698–1705 (2018). https://doi.org/10.1007/s00415-018-8898-4
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DOI: https://doi.org/10.1007/s00415-018-8898-4