PMID- 29865087
OWN - NLM
STAT- MEDLINE
DCOM- 20181101
LR  - 20181101
IS  - 2214-3599 (Print)
VI  - 5
IP  - 2
DP  - 2018
TI  - Relationships between Lower Limb Muscle Strength Impairments and Physical 
      Limitations in DM1.
PG  - 215-224
LID - 10.3233/JND-170291 [doi]
AB  - BACKGROUND: Although adult and late-onset DM1 phenotypes DM1 present distinct 
      lower limb weaknesses portraits, resulting physical limitations have never been 
      described separately for each phenotype. OBJECTIVE: To characterize the lower 
      limb weaknesses and physical limitations among the DM1 adult and late-onset 
      phenotypes separately and to document the contribution of weaknesses on mobility 
      to optimize the management of this population. METHODS: The strength of four 
      muscle groups among 198 participants was quantified. Participants were 
      categorized according to the severity of their muscular involvement using the 
      Muscular Impairment Rating Scale (MIRS). Physical limitations were assessed using 
      the Timed up-and-go (TUG), Berg Balance Scale (BBS) and 10 meters comfortable 
      walking speed (10MWT). Multiple linear regressions were performed to identify the 
      contribution of each muscle group to the mobility tests scores. RESULTS: 
      Late-onset demonstrated less weakness and physical limitations (p < 0.001 - 
      0.002) than the adult phenotype, but 21.9-47.5% of participants with this 
      phenotype showed mobility scores below reference values. Physical limitations 
      were observed in the first two MIRS grades (37.5-42.1% of the participants) for 
      the TUG and 10MWT. Ankle dorsiflexors and knee extensors were the two muscle 
      groups that showed the strongest relationships with mobility scores. CONCLUSION: 
      Although less impaired, the late-onset phenotype shows significant lower limb 
      muscle weakness associated with physical limitations. The surprising presence of 
      quantitative lower limb muscle weakness in the first two MIRS grades needs to be 
      considered when using this scale. Both ankle dorsiflexors and knee extensors 
      appear to be good indicators of physical limitations in DM1.
FAU - Petitclerc, Emilie
AU  - Petitclerc E
AD  - Faculty of Medicine and Health Sciences, Universite de Sherbrooke, Sherbrooke 
      (Quebec), Canada.
AD  - Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, 
      NeuromuscularClinic, Centre integre Universitaire de Sante et de Services Sociaux 
      du Saguenay-Lac-St-Jean, site Jonquiere, rue de l'Hopital, Jonquiere (Quebec), 
      Canada.
FAU - Hebert, Luc J
AU  - Hebert LJ
AD  - Faculty of Medicine, Rehabilitation (Physiotherapy) and Department of Radiology, 
      Universite Laval, avenue de la Medecine, Pavillon Ferdinand-Vandry, Quebec, QC, 
      Canada.
FAU - Mathieu, Jean
AU  - Mathieu J
AD  - Faculty of Medicine and Health Sciences, Universite de Sherbrooke, Sherbrooke 
      (Quebec), Canada.
AD  - Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, 
      NeuromuscularClinic, Centre integre Universitaire de Sante et de Services Sociaux 
      du Saguenay-Lac-St-Jean, site Jonquiere, rue de l'Hopital, Jonquiere (Quebec), 
      Canada.
FAU - Desrosiers, Johanne
AU  - Desrosiers J
AD  - Faculty of Medicine and Health Sciences, Universite de Sherbrooke, Sherbrooke 
      (Quebec), Canada.
FAU - Gagnon, Cynthia
AU  - Gagnon C
AD  - Faculty of Medicine and Health Sciences, Universite de Sherbrooke, Sherbrooke 
      (Quebec), Canada.
AD  - Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, 
      NeuromuscularClinic, Centre integre Universitaire de Sante et de Services Sociaux 
      du Saguenay-Lac-St-Jean, site Jonquiere, rue de l'Hopital, Jonquiere (Quebec), 
      Canada.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Netherlands
TA  - J Neuromuscul Dis
JT  - Journal of neuromuscular diseases
JID - 101649948
SB  - IM
MH  - *Activities of Daily Living
MH  - Adult
MH  - Age of Onset
MH  - Female
MH  - Humans
MH  - Linear Models
MH  - Lower Extremity/*physiopathology
MH  - Male
MH  - *Mobility Limitation
MH  - Muscle Strength/*physiology
MH  - Muscle Weakness/*physiopathology
MH  - Myotonic Dystrophy/*physiopathology
MH  - Phenotype
OTO - NOTNLM
OT  - Myotonic dystrophy
OT  - mobility limitation
OT  - muscle strength
OT  - phenotype
EDAT- 2018/06/06 06:00
MHDA- 2018/11/02 06:00
CRDT- 2018/06/06 06:00
PHST- 2018/06/06 06:00 [entrez]
PHST- 2018/06/06 06:00 [pubmed]
PHST- 2018/11/02 06:00 [medline]
AID - JND170291 [pii]
AID - 10.3233/JND-170291 [doi]
PST - ppublish
SO  - J Neuromuscul Dis. 2018;5(2):215-224. doi: 10.3233/JND-170291.