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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 |
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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. |
doi_str_mv | 10.1002/mus.25451 |
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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.</description><identifier>ISSN: 0148-639X</identifier><identifier>EISSN: 1097-4598</identifier><identifier>DOI: 10.1002/mus.25451</identifier><identifier>PMID: 27784130</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>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</subject><ispartof>Muscle & nerve, 2017-07, Vol.56 (1), p.57-63</ispartof><rights>2016 Wiley Periodicals, Inc.</rights><rights>2017 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3531-86d32fa9274d90b713dc0d3c8b400006013651061b0f495f95fb65fb9d6d841d3</citedby><cites>FETCH-LOGICAL-c3531-86d32fa9274d90b713dc0d3c8b400006013651061b0f495f95fb65fb9d6d841d3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,777,781,27905,27906</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27784130$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Petitclerc, Émilie</creatorcontrib><creatorcontrib>Hébert, Luc J.</creatorcontrib><creatorcontrib>Mathieu, Jean</creatorcontrib><creatorcontrib>Desrosiers, Johanne</creatorcontrib><creatorcontrib>Gagnon, Cynthia</creatorcontrib><title>Lower limb muscle strength impairment in late‐onset and adult myotonic dystrophy type 1 phenotypes</title><title>Muscle & nerve</title><addtitle>Muscle Nerve</addtitle><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.</description><subject>Adult</subject><subject>Aged</subject><subject>Clinical trials</subject><subject>Dystrophy</subject><subject>Electromyography</subject><subject>Female</subject><subject>Genotype & phenotype</subject><subject>Humans</subject><subject>Impairment</subject><subject>Isometric Contraction - physiology</subject><subject>Lower Extremity - innervation</subject><subject>lower limb</subject><subject>Male</subject><subject>Medical research</subject><subject>Middle Aged</subject><subject>Muscle strength</subject><subject>Muscle Strength - physiology</subject><subject>Muscle Weakness - etiology</subject><subject>Muscle, Skeletal - physiopathology</subject><subject>Myotonic dystrophy</subject><subject>Myotonic Dystrophy - complications</subject><subject>myotonic dystrophy type 1 (DM1)</subject><subject>outcome measures</subject><subject>phenotype comparison</subject><subject>Physical activity</subject><subject>quantitative muscle testing</subject><subject>Severity of Illness Index</subject><subject>strength</subject><subject>Yield strength</subject><subject>Young Adult</subject><issn>0148-639X</issn><issn>1097-4598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><recordid>eNp1kM1O3DAURq2qqDMMXfACyBKbsgj4xrEdL9Gof9IgFoDUnZXEDhOU2MF2NMquj9Bn5EkwHcoCqdK1rhfHR58_hI6BnAMh-cUwhfOcFQw-oCUQKbKCyfIjWhIoyoxT-WuBDkN4IIRAycUntMiFKAugZIn0xu2Mx3031Dhpmt7gEL2x93GLu2GsOj8YG3FncV9F8_T7j7PBRFxZjSs99REPs4vOdg3Wc3roxu2M4zwaDHjcGute7uEIHbRVH8zn171Cd9--3q5_ZJvr7z_Xl5usoYxCVnJN87aSuSi0JLUAqhuiaVPWRYpOOAHKGRAONWkLydo0NU9Haq7TdzRdoS977-jd42RCVEMXGtP3lTVuCgpKyngpBCsSevoOfXCTtymdAgk5MCaFTNTZnmq8C8GbVo2-Gyo_KyDqpXqVOlN_q0_syatxqgej38h_XSfgYg_sut7M_zepq7ubvfIZWqSOdw</recordid><startdate>201707</startdate><enddate>201707</enddate><creator>Petitclerc, Émilie</creator><creator>Hébert, Luc J.</creator><creator>Mathieu, Jean</creator><creator>Desrosiers, Johanne</creator><creator>Gagnon, Cynthia</creator><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7TM</scope><scope>7TS</scope><scope>7U7</scope><scope>7U9</scope><scope>C1K</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope></search><sort><creationdate>201707</creationdate><title>Lower limb muscle strength impairment in late‐onset and adult myotonic dystrophy type 1 phenotypes</title><author>Petitclerc, Émilie ; 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 & 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 & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Muscle & 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 & 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. Muscle Nerve 56: 57–63, 2017.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>27784130</pmid><doi>10.1002/mus.25451</doi><tpages>7</tpages></addata></record> |
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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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