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

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.