title: The motor function measure to study limitation of activity in children and adults with Charcot-Marie-Tooth disease.
abstract: 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.
  
  
  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.
authors.affiliation: Service de médecine physique et réadaptation, hôpital Henry-Gabrielle, hospices civils de Lyon, 20, route de Vourles, 69230 Saint-Genis-Laval, France. Electronic address: laurenceallard@free.fr.
authors.forename: L
authors.initials: L
authors.lastname: Allard
authors.affiliation: Service de médecine physique et réadaptation, hôpital Henry-Gabrielle, hospices civils de Lyon, 20, route de Vourles, 69230 Saint-Genis-Laval, France.
authors.forename: G
authors.initials: G
authors.lastname: Rode
authors.affiliation: Service de médecine physique et réadaptation, hôpital Henry-Gabrielle, hospices civils de Lyon, 20, route de Vourles, 69230 Saint-Genis-Laval, France.
authors.forename: S
authors.initials: S
authors.lastname: Jacquin-Courtois
authors.affiliation: Service de médecine physique et réadaptation, hôpital Henry-Gabrielle, hospices civils de Lyon, 20, route de Vourles, 69230 Saint-Genis-Laval, France.
authors.forename: M C
authors.initials: MC
authors.lastname: Pouget
authors.affiliation: L'Escale, service central de rééducation pédiatrique, hôpital femme Mère-Enfant, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron cedex, France; Pôle information médicale évaluation recherche, hospices civils de Lyon, 69003 Lyon, France.
authors.forename: P
authors.initials: P
authors.lastname: Rippert
authors.affiliation: Direction de la recherche et de l'innovation, centre hospitalo-universitaire de Montpellier, 34000 Montpellier, France.
authors.forename: D
authors.initials: D
authors.lastname: Hamroun
authors.affiliation: L'Escale, service central de rééducation pédiatrique, hôpital femme Mère-Enfant, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: I
authors.initials: I
authors.lastname: Poirot
authors.affiliation: L'Escale, service central de rééducation pédiatrique, hôpital femme Mère-Enfant, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Bérard
authors.affiliation: L'Escale, service central de rééducation pédiatrique, hôpital femme Mère-Enfant, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron cedex, France; Université de Lyon, 69000 Lyon, France; Université Lyon I, 69100 Villeurbanne, France; CNRS, UMR 5558, laboratoire de biométrie et biologie évolutive, équipe biostatistique santé, 69310 Pierre-Bénite, France.
authors.forename: C
authors.initials: C
authors.lastname: Vuillerot
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002607:Charcot-Marie-Tooth Disease / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology*; D009460:Neurologic Examination; D013647:Task Performance and Analysis; D055815:Young Adult
keywords: Activity limitation; Charcot-Marie-Tooth disease; Functional scale; Limitation d’activités; Maladie de Charcot-Marie-Tooth; Maladie neuromusculaire; Mesure de Fonction Motrice; Motor Function Measure; Neuromuscular disease; Échelle fonctionnelle