Quantitative motor assessment in myotonic dystrophy.
OBJECTIVE
To establish baseline data, using a quantitative motor evaluation protocol, prior to a prospective longitudinal study of the natural history of muscular involvement in myotonic dystrophy (DM).


METHODS
We conducted a cross-sectional study using a protocol consisting of manual muscle testing (MMT), quantitative muscle testing (QMT), and timed functional testing (TFT) on 50 definite DM patients (27 men, 23 women), aged 16 to 67 years. The relationships between MMT, QMT and TFT scores and disease duration were examined using linear regression analysis.


RESULTS
The muscle weakness was symmetric and the neck flexors and the distal muscles of upper and lower extremities were weaker than proximal muscles. Using MMT scores, the average strength decline was 0.95% per year and was similar for men and women. The strength decline was significantly more rapid for distal muscles than for proximal muscles. Quantitative muscle testing scores documented a strength decline per year of disease duration of 1.2-1.6% for the hip flexors and of 2.0-3.0% for the hand grip flexors.


CONCLUSIONS
We observed significant linear relationships between the scores generated by this protocol and disease duration. These data illustrate the distal to proximal progression of muscular involvement in DM, a pattern of progression well-recognized by the clinicians. The follow-up assessment of a large DM cohort in a longitudinal study will establish whether this quantitative protocol provides sensitive measures of the disease progression.
Mathieu
J
J
Neuromuscular Clinic, Centre de réadaptation en déficience physique, Jonquière, QC, Canada.
Boivin
H
H
Richards
C L
CL
D000293:Adolescent; D000328:Adult; D000368:Aged; D000375:Aging / Q000502:physiology; D001132:Arm / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology; D007866:Leg / Q000503:physiopathology; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology*; D018482:Muscle, Skeletal / Q000503:physiopathology*; D009223:Myotonic Dystrophy / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology*; D009334:Neck Muscles / Q000503:physiopathology; D013997:Time Factors