Motor Parameters in Patients With Limb Girdle Muscular Dystrophy

Characterization and Identification of Motor Parameters Using Instrumental Assessment in Patients With Limb Girdle Muscular Dystrophy

The primary objective of the study is to perform 2-year follow up with motor parameters evolution using instrumental assessments in patients with limb girdle muscular dystrophie, and to identify which motor parameters are sensitive to change.

The secondary objectives of the study are:

• to describe the changes of the parameters obtained from instrumental evaluations in comparison with the changes obtained from clinical assessments.
• to characterize the muscular impairments, the biomechanical gait disorders, the standing postural control disorders, the biomechanical upper limb disorders in spatial exploration, the limitation of upper limb capacities, the fatigue, the endurance, the patients' participation to their activities and their quality of life, in comparison with a healthy paired population.
• to highlight the relationships between muscular assessment parameters, biomechanical gait parameters, standing posture control and upper limb spatial exploration.
• to highlight the relationships between data from instrumental assessments and data from clinical assessments.
• to highlight the relationships between instrumental assessments data and clinical assessments data on one side, and features of patients (age, sex, duration since diagnosis, type of LGMD, rehabilitation in follow-up, sportive practices...) on the other side.

This is a monocentric study in which 2 sub-groups of people will be enrolled: LGMD patients and healthy volunteers. Each enrolled LGMD patient will have 5 visits during 2 years, one baseline visit and four half-yearly visits. Each enrolled healthy volunteer will have one planned visit only. The duration of enrollments will last 20 months.

Inclusion Criteria:

• For limb girdle muscular dystrophie group:

  • Patients diagnosed limb girdle muscular dystrophie;
  • Aged between 18 and 70 years;
  • Covered by the French social security scheme;
  • Patient able to maintain upright position alone;
  • Able to walk at least 10 meters and 6 minutes consecutively without help.

• For Volunteer group:

  • Adult (18 to 70 years) without neuromuscular or squeletic disorder.

Exclusion Criteria:

§ For all:

• Without associated neuromuscular disorders such as respiratory or muscular-squeletic diseases, apart from the consequences of dystrophy;
• Contraindication to the tests;
• Noncontrol cardiac rhythm disorders;
• Lying down position intolerance due to severe respiratory troubles;
• Pregnant or breastfeeding woman.