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Major involvement of trunk muscles in myotonic dystrophy type 1
Author(s) -
Solbakken G.,
Ørstavik K.,
Hagen T.,
Dietrichs E.,
Nærland T.
Publication year - 2016
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/ane.12565
Subject(s) - myotonic dystrophy , trunk , physical medicine and rehabilitation , medicine , myotonia , balance (ability) , muscular dystrophy , physical therapy , biology , ecology
Objectives The motor impairments in Myotonic Dystrophy 1 ( DM 1) are assumed to progress from distal toward proximal parts of the extremities in the Juvenile and Adult forms of DM 1. On occasion and late in progress spine deformity is observed. In this study we have examined whether and to what extent trunk muscles are impaired in DM 1, and if this impairment is correlated with the duration of the disorder, walking capacity, mobility, balance, and CTG ‐repeats. Materials & methods Manual muscle testing ( MMT ) of skeletal muscle strength in trunk and extremities, reassessment of the mutation size, time since first symptom, the 6 min walk test (6 MWT ), Rivermead mobility index ( RIM ) and Timed up & go ( TUG ) were sampled in 38 adult DM 1 outpatients. Results We found significant impairment in trunk muscles. Trunk muscle strength decreased significantly with increasing mutation size ( r = −0.64, P < 0.001). Reduced walking capacity, mobility and balance were significantly related to decreased trunk muscle strength. Conclusion DM 1 affects trunk muscle groups. The trunk impairments seem to occur relatively early in disease progression. Awareness of trunk impairments may be of importance for everyday functioning and for understanding the risk of injuries due to falls reported among DM 1 patients. It may also help in identification of DM 1 patients and considered outcome measure in future clinical trials.