Article

OBJECTIVE: To study the applicability and responsiveness of the motor function
measure (total score and sub-scores D1, D2 and D3) in patients with
Charcot-Marie-Tooth disease. METHODS: Two hundred and thirty-three
patients aged 4-86 years were included in the descriptive study. Scores and
sub-scores were analyzed by age and by disease subtypes. Sensitivity to change
(responsiveness) was estimated in patients having had at least two evaluations
with at least six months between the first and the second. RESULTS: Motor
function measure scores decrease with age, especially sub-scores D1 and D3. There
were no significant differences between the scores according to type of
Charcot-Marie-Tooth disease. The scores were significantly higher for ambulatory
than for non-ambulatory patients. Significant responsiveness was demonstrated
only in type 2 Charcot-Marie-Tooth disease. DISCUSSION/CONCLUSIONS: Our results
suggest that, especially for D1 and D3 sub-scores, the motor function measure is
a reliable and valid outcome measure that can be usefully applied in longitudinal
follow-up. Studies of longer duration could demonstrate its responsiveness in
other Charcot-Marie-Tooth disease subtypes.
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Langue : ANGLAIS ; FRANCAIS