PMID- 11171898
OWN - NLM
STAT- MEDLINE
DCOM- 20010405
LR  - 20220310
IS  - 0028-3878 (Print)
IS  - 0028-3878 (Linking)
VI  - 56
IP  - 3
DP  - 2001 Feb 13
TI  - Assessment of a disease-specific muscular impairment rating scale in myotonic 
      dystrophy.
PG  - 336-40
AB  - OBJECTIVE: To document the intra/interrater reliability and the construct 
      validity of the Muscular Impairment Rating Scale (MIRS) in assessing patients 
      with myotonic dystrophy type 1 (DM1). The MIRS is a ordinal five-point rating 
      scale, established in accordance with the clinically recognized distal to 
      proximal progression of the muscular involvement in DM1, based partly on a manual 
      muscle testing (MMT) of 11 muscle groups. METHODS: To assess the reliability of 
      the MIRS, 55 patients with DM1 were examined by three different observers, one of 
      them evaluating each patient twice. Intra- and interobserver reliability of the 
      MIRS was measured using Cohen's weighted kappa. To assess the construct validity 
      of the MIRS, correlations were made with the Functional Status Index (FSI) and 
      eight timed functional tasks. RESULTS: The intraobserver reliability of the MIRS 
      was excellent (weighted kappa = 0.84), and the interobserver reliability was 
      interpreted as a substantial agreement (weighted kappa = 0.77 to 0.79). The 
      correlation coefficients between MMT scores and MIRS grades were all highly 
      significant (r(s) = -0.81 to -0.88, p < 0.001). The FSI showed a significant 
      progressive increase of the total median dependence score in activities of daily 
      living from 0 in MIRS grade 1 to 39 in MIRS grade 5 (p < 0.001). The time needed 
      to perform the eight functional tasks was also found to significantly increase in 
      relation with the progression of the MIRS grades. CONCLUSION: The MIRS is a 
      quick, simple, and reliable measurement of muscular impairment in DM1. The FSI 
      questionnaire and the timed motor activities supported its construct validity. 
      The MIRS is useful to monitor major stages of DM1 progression, to study the 
      natural history of the disease, and to identify homogeneous groups of patients 
      for clinical trials.
FAU - Mathieu, J
AU  - Mathieu J
AD  - Neuromuscular Clinic, Centre Regional de Readaptation en Deficience Physique, 
      Jonquiere, Quebec, Canada. jmathieu@saglac.qc.ca
FAU - Boivin, H
AU  - Boivin H
FAU - Meunier, D
AU  - Meunier D
FAU - Gaudreault, M
AU  - Gaudreault M
FAU - Begin, P
AU  - Begin P
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Muscles/*physiopathology
MH  - Myotonic Dystrophy/*physiopathology
MH  - Outcome Assessment, Health Care
MH  - Reproducibility of Results
MH  - Surveys and Questionnaires
EDAT- 2001/02/15 11:00
MHDA- 2001/04/06 10:01
CRDT- 2001/02/15 11:00
PHST- 2001/02/15 11:00 [pubmed]
PHST- 2001/04/06 10:01 [medline]
PHST- 2001/02/15 11:00 [entrez]
AID - 10.1212/wnl.56.3.336 [doi]
PST - ppublish
SO  - Neurology. 2001 Feb 13;56(3):336-40. doi: 10.1212/wnl.56.3.336.