Overview

The objective of the ActiLiège Next study is to collect longitudinal data from patients and control subjects using a wearable magneto-inertial device. By collecting natural history data in various neuromuscular disorders (Duchenne Muscular Dystrophy, Fascioscapulohumeral Muscular Dystrophy, Myotonic Dystrophy 1, Charcot-Marie-Tooth, Centronuclear Myopathy, Congenital Muscular Dystrophy), we aim to validate digital outcome measures to continuously assess motor function in real-life.

Eligibility

Inclusion criteria

• For the patients:
  • Genetically confirmed diagnosis of DMD, FSHD, DM1, CMT or FKRP mutations or confirmed CNM based on muscle biopsy.
  • FSHD, DM1, CMT and CNM patients should be ambulant or in transition.
  • DM1 and CMT patients should present sensori-motor signs on physical examination.
  • Under the age of 20 years for patients with DMD, CNM or between the ages of 5 and 80 years for patients with FSHD, CMT and DM1.
  • More than 2 years old for patients with FKRP mutations
  • Non-ambulant DMD patients must be able to remain seated in an arm- or a wheelchair for at least one hour.
  • Patients with DMD treated with corticosteroids for at least 6 months or initiated corticosteroid at V0 (except for patients under 4).
  • Signed informed consent form by patient himself or, in case of minor patients, signed informed consent form by patient's parents or legal guardians.
• For the control subjects:
  • Ambulant boys and girls under 20 years old
  • Signed informed consent form by patient him/herself or, in case of minor patients, signed informed consent form by patient's parents or legal guardians.

Exclusion Criteria:

• For the patients:
  • Patients with extreme cognitive disorders that limit their understanding of the exercises to be performed.
  • Patients who have undergone a surgical procedure or who have experienced recent trauma (within fewer than 6 months) affecting the upper or lower limbs (for ambulant patients).
  • A concomitant chronic or acute neurological, endocrine, infectious, allergic, or inflammatory pathology within the 3-week period immediately prior to inclusion.
  • Patients who are participating in an interventional clinical trial.
  • DMD patients in transition who are not on corticosteroids.
• For the control subjects:
  • Patients who have undergone a surgical procedure or who have experienced recent trauma (within fewer than 6 months) affecting the upper or lower limbs.
  • Elite athletes (at the national level).
  • A chronic or acute muscular, neurological, infectious, or inflammatory pathology within the 3-week period immediately prior to inclusion.
  • An orthopedic, neuromuscular, or neurological pathology that affects the quality of the subject's walking gait.