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.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Petitclerc É,Hébert LJ,Mathieu J,Desrosiers J,Gagnon Cdoi
10.1002/mus.25451subject
Has Abstractpub_date
2017-07-01 00:00:00pages
57-63issue
1eissn
0148-639Xissn
1097-4598journal_volume
56pub_type
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