facioscapulohumeral dystrophy / Landouzy-Dejerine syndrome
Conditions
Interventions
None listed
Sponsors
Universitair Medisch Centrum Sint Radboud
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: - Diagnosis FSHD based on a positive (family) history, examination, genetically confirmed. - Age between 18-80 years. - Being able to stand independently and walk for ten meters without aids.
Exclusion criteria
Exclusion criteria: - Other neurological or musculoskeletal disorders. - Use of medication affecting balance (e.g. neuroleptics, antidepressants, anticonvulsants).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Maximum EMG amplitude, expressed as a percentage of the MVC during isolated contraction, is the primary outcome measure of the posture, balance and gait tasks. For the MRIS measurements, the primary outcome will be the cross-sectional area of the various leg and trunk muscles. | — |
Secondary
| Measure | Time frame |
|---|---|
| Posture: the joint angles of the lower back, hips, knees, and ankles in two positions. Gait: gait velocity; spatiotemporal variables (e.g. step lengths and cadence); joint angles and joint torques of the lower back, hips, knees and ankles during the gait cycle. Balance: spatiotemporal variables (e.g. displacement of the centre of mass) joint angles and joint torques. MRI: fatty degeneration on a four point scale proposed by Lamminen (1990). | — |
Countries
Netherlands
Outcome results
None listed