Marie-Christine Commare

Publications (2)5.34 Total impact

• Article: Motor and respiratory heterogeneity in Duchenne patients: implication for clinical trials.

  Véronique Humbertclaude, Dalil Hamroun, Kamel Bezzou, Carole Bérard, Odile Boespflug-Tanguy, Christine Bommelaer, Emmanuelle Campana-Salort, Claude Cances, Brigitte Chabrol, Marie-Christine Commare, [......], Elisabeth Ollagnon-Roman, Christian Richelme, François Rivier, Sabrina Sacconi, Vincent Tiffreau, Carole Vuillerot, Marie-Christine Picot, Mireille Claustres, Christophe Béroud, Sylvie Tuffery-Giraud
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  ABSTRACT: Our objective was to clarify the clinical heterogeneity in Duchenne muscular dystrophy (DMD). The French dystrophinopathy database provided clinical, histochemical and molecular data of 278 DMD patients (mean longitudinal follow-up: 14.2 years). Diagnosis was based on mutation identification in the DMD gene. Three groups were defined according to the age at ambulation loss: before 8 years (group A); between 8 and 11 years (group B); between 11 and 16 years (group C). Motor and respiratory declines were statistically different between the three groups, as opposed to heart involvement. When acquired, running ability was lost at the mean age of 5.41 (group A), 7.11 (group B), 9.19 (group C) years; climbing stairs ability at 6.24 (group A), 7.99 (group B), 10,42 (group C) years, and ambulation at 7.10 (group A), 9.25 (group B), 12.01 (group C) years. Pulmonary growth stopped at 10.26 (group A), 12.45 (group B), 14.58 (group C) years. Then, forced vital capacity decreased at the rate of 8.83 (group A), 7.52 (group B), 6.03 (group C) percent per year. Phenotypic variability did not rely on specific mutational spectrum. Beside the most common form of DMD (group B), we provide detailed description on two extreme clinical subgroups: a severe one (group A) characterized by early severe motor and respiratory decline and a milder subgroup (group C). Compared to group B or C, four to six times fewer patients from group A are needed to detect the same decrease in disease progression in a clinical trial.
  European journal of paediatric neurology: EJPN: official journal of the European Paediatric Neurology Society 09/2011; 16(2):149-60. · 2.01 Impact Factor
• Article: Self-perception of quality of life by adolescents with neuromuscular diseases.

  Carole Vuillerot, Isabelle Hodgkinson, Alvine Bissery, Anne-Marie Schott-Pethelaz, Jean Iwaz, René Ecochard, Marie-Charlotte D'Anjou, Marie-Christine Commare, Carole Berard
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  ABSTRACT: Little is known about quality of life of adolescents with neuromuscular diseases or the factors that influence it. We searched whether physical impairment, physical disability, and medical complications were predictors of low quality of life. Motor function, health, orthopedic status, and rehabilitation were assessed in 43 adolescents with neuromuscular diseases (mean age, 13.8 years, standard deviation 1.7 year; sex ratio 2.9/1). Quality of life was measured with the VSP-A ("Vécu Santé Perçu par l'Adolescent"; self-perceived health state in adolescents), a validated health-related quality of life self-perception test. A mixed linear regression related quality of life to impairment, disability, and respiratory status. Comparisons were made with results from an age/sex-matched nondisabled group. On the average, the VSP-A scores in physically disabled adolescents were: (1) similar to those of the nondisabled group with regard to vitality, body image, relationships with parents and friends, and physical and psychological well-being; (2) higher with regard to school performance (score 68 vs. 52.8) and relationships with teachers (67.4 vs. 43.2); and (3) lower with regard to leisure activities (43.9 vs. 60.9). Physical disability and physical impairment were not negatively associated with seven of the nine VSP-A dimensions, but physical impairment was negatively associated with leisure activities and vitality (p < .001 and p < .01, respectively). Adolescents with ventilatory support did not express lower scores than adolescents not requiring ventilatory support (67.7+/-11 vs. 62.9+/-15, p=.39). These surprising results should lead us question our medical, educational, and rehabilitation practices. Already well-managed disabled adolescents should benefit from less compassionate but more daring and dynamic interpersonal contacts.
  Journal of Adolescent Health 01/2010; 46(1):70-6. · 3.33 Impact Factor