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Lower limb muscle strength impairment in late‐onset and adult myotonic dystrophy type 1 phenotypes

ABSTRACT Introduction Lower limb strength has never been characterized separately for late‐onset and adult myotonic dystrophy type 1 (DM1) phenotypes. Methods The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment p...

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Published in:Muscle & nerve 2017-07, Vol.56 (1), p.57-63
Main Authors: Petitclerc, Émilie, Hébert, Luc J., Mathieu, Jean, Desrosiers, Johanne, Gagnon, Cynthia
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creator Petitclerc, Émilie
Hébert, Luc J.
Mathieu, Jean
Desrosiers, Johanne
Gagnon, Cynthia
description ABSTRACT Introduction Lower limb strength has never been characterized separately for late‐onset and adult myotonic dystrophy type 1 (DM1) phenotypes. Methods The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 assessment methods [manual (MMT) and quantitative (QMT) muscle testing] among 107 patients. Results Both MMT and QMT showed more pronounced weakness in the adult phenotype. In the late‐onset phenotype, although MMT showed normal strength, QMT revealed a loss of 11.7%–20.4%. Participants with grade 1 or 2 on the Muscle Impairment Rating Scale had weakness detected using QMT, which suggests earlier muscle impairment than MMT alone would suggest. Conclusions To avoid muscle wasting, physical activity recommendations should be made for the late‐onset phenotype and in the early stages of the disease for the adult phenotype. MMT is not recommended for use in clinical trials. Muscle Nerve 56: 57–63, 2017.
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Methods The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 assessment methods [manual (MMT) and quantitative (QMT) muscle testing] among 107 patients. Results Both MMT and QMT showed more pronounced weakness in the adult phenotype. In the late‐onset phenotype, although MMT showed normal strength, QMT revealed a loss of 11.7%–20.4%. Participants with grade 1 or 2 on the Muscle Impairment Rating Scale had weakness detected using QMT, which suggests earlier muscle impairment than MMT alone would suggest. Conclusions To avoid muscle wasting, physical activity recommendations should be made for the late‐onset phenotype and in the early stages of the disease for the adult phenotype. MMT is not recommended for use in clinical trials. 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Methods The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 assessment methods [manual (MMT) and quantitative (QMT) muscle testing] among 107 patients. Results Both MMT and QMT showed more pronounced weakness in the adult phenotype. In the late‐onset phenotype, although MMT showed normal strength, QMT revealed a loss of 11.7%–20.4%. Participants with grade 1 or 2 on the Muscle Impairment Rating Scale had weakness detected using QMT, which suggests earlier muscle impairment than MMT alone would suggest. Conclusions To avoid muscle wasting, physical activity recommendations should be made for the late‐onset phenotype and in the early stages of the disease for the adult phenotype. MMT is not recommended for use in clinical trials. 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Hébert, Luc J. ; Mathieu, Jean ; Desrosiers, Johanne ; Gagnon, Cynthia</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3531-86d32fa9274d90b713dc0d3c8b400006013651061b0f495f95fb65fb9d6d841d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Clinical trials</topic><topic>Dystrophy</topic><topic>Electromyography</topic><topic>Female</topic><topic>Genotype &amp; phenotype</topic><topic>Humans</topic><topic>Impairment</topic><topic>Isometric Contraction - physiology</topic><topic>Lower Extremity - innervation</topic><topic>lower limb</topic><topic>Male</topic><topic>Medical research</topic><topic>Middle Aged</topic><topic>Muscle strength</topic><topic>Muscle Strength - physiology</topic><topic>Muscle Weakness - etiology</topic><topic>Muscle, Skeletal - physiopathology</topic><topic>Myotonic dystrophy</topic><topic>Myotonic Dystrophy - complications</topic><topic>myotonic dystrophy type 1 (DM1)</topic><topic>outcome measures</topic><topic>phenotype comparison</topic><topic>Physical activity</topic><topic>quantitative muscle testing</topic><topic>Severity of Illness Index</topic><topic>strength</topic><topic>Yield strength</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Petitclerc, Émilie</creatorcontrib><creatorcontrib>Hébert, Luc J.</creatorcontrib><creatorcontrib>Mathieu, Jean</creatorcontrib><creatorcontrib>Desrosiers, Johanne</creatorcontrib><creatorcontrib>Gagnon, Cynthia</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Physical Education Index</collection><collection>Toxicology Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Environmental Sciences and Pollution Management</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>Nursing &amp; Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Muscle &amp; nerve</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Petitclerc, Émilie</au><au>Hébert, Luc J.</au><au>Mathieu, Jean</au><au>Desrosiers, Johanne</au><au>Gagnon, Cynthia</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Lower limb muscle strength impairment in late‐onset and adult myotonic dystrophy type 1 phenotypes</atitle><jtitle>Muscle &amp; nerve</jtitle><addtitle>Muscle Nerve</addtitle><date>2017-07</date><risdate>2017</risdate><volume>56</volume><issue>1</issue><spage>57</spage><epage>63</epage><pages>57-63</pages><issn>0148-639X</issn><eissn>1097-4598</eissn><abstract>ABSTRACT Introduction Lower limb strength has never been characterized separately for late‐onset and adult myotonic dystrophy type 1 (DM1) phenotypes. Methods The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 assessment methods [manual (MMT) and quantitative (QMT) muscle testing] among 107 patients. Results Both MMT and QMT showed more pronounced weakness in the adult phenotype. In the late‐onset phenotype, although MMT showed normal strength, QMT revealed a loss of 11.7%–20.4%. Participants with grade 1 or 2 on the Muscle Impairment Rating Scale had weakness detected using QMT, which suggests earlier muscle impairment than MMT alone would suggest. Conclusions To avoid muscle wasting, physical activity recommendations should be made for the late‐onset phenotype and in the early stages of the disease for the adult phenotype. MMT is not recommended for use in clinical trials. 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subjects Adult
Aged
Clinical trials
Dystrophy
Electromyography
Female
Genotype & phenotype
Humans
Impairment
Isometric Contraction - physiology
Lower Extremity - innervation
lower limb
Male
Medical research
Middle Aged
Muscle strength
Muscle Strength - physiology
Muscle Weakness - etiology
Muscle, Skeletal - physiopathology
Myotonic dystrophy
Myotonic Dystrophy - complications
myotonic dystrophy type 1 (DM1)
outcome measures
phenotype comparison
Physical activity
quantitative muscle testing
Severity of Illness Index
strength
Yield strength
Young Adult
title Lower limb muscle strength impairment in late‐onset and adult myotonic dystrophy type 1 phenotypes
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