A 9-year follow-up study of the natural progression of upper limb performance in myotonic dystrophy type 1: A similar decline for phenotypes but not for gender

Neuromuscular Disorders : NMD
Kateri RaymondC Gagnon

Abstract

This study aimed to document and compare the decline of upper limb performance among adults with myotonic dystrophy type 1 according to phenotype and gender. A longitudinal descriptive design compared upper limb performance at baseline and follow-up of 70 women and 38 men with the late-onset or adult phenotypes. Grip strength and pinch strength as well as gross dexterity and fine dexterity were assessed. All four performance measures decreased significantly (p <0.001). The decline over time was similar for individuals with the late-onset and adult-onset phenotypes, but differed according to gender. For late-onset and adult-onset phenotypes respectively, women lost less grip strength than men: 0.4 and minus 0.8 kg (2.0% and -9.4%) in women vs. minus 7.4 and minus 3.1 kg (-19.2% and -30.7%) in men. A similar situation was found for gross dexterity: minus 3.0 and minus 3.2 blocks (-4.6% and -5.9%) in women vs. minus 12.4 and minus 8.7 blocks (-19.4% and -16.6%) in men. Pinch gauge had the smallest standard deviations and was one of the only measurement tools with significant detectable changes in relation to the standard error of measurement. Given these results, health professionals and researchers should consider phenotype and g...Continue Reading

Citations

Jul 25, 2019·Neurology. Genetics·Cécilia LégaréLuigi Bouchard
Apr 22, 2020·Muscle & Nerve·Elise DuchesneCynthia Gagnon
Aug 14, 2019·Annals of Clinical and Translational Neurology·Aura Cecilia Jimenez-MorenoHanns Lochmüller

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