Muscular Dystrophies
Conditions
Keywords
diaphragm, ultrasound, echocardiography
Brief summary
Muscular dystrophies are neurologic disease with higher morbidity and mortality. Cardiac function and respiratory muscles can be affected, in addition with skeletal muscle deficiency . Lung function is classically assessed with functionnal pulmonary tests (FPT) . Ultrasound recently emerged as a non-invasive tool to assess diaphragm function. The aims of the study are: * to provide the spectrum of diaphragm ultrasound in patients with muscular dystrophies * to assess the correlation between diaphragm ultrasound and FPT * to assess the correlation between diaphragm ultrasound and cardiac function
Interventions
None listed
Sponsors
Centre d'Investigation Clinique et Technologique 805
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Inclusion criteria
* Duchenne muscular dystrophy, * Becker muscular dystrophy, * LGMD, * myotonic dystrophy type 1 and type 2, * metabolic myopathy, * mitochondrial myopathy, * FSHD1 and 2, * myasthenia
Exclusion criteria
* history of cardiac surgery * history of thoracic surgery * brain traumatism
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| diaphragm ultrasound motion | 1 year | using echography |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| correlation between diaphragm motion (mm) and forced vital capacity(ml) | 1 year | using echography and FPT |
| correlation between diaphragm motion (mm) and inspiratory capacity (ml) | 1 year | using echography and FPT |
| diaphragm thickness (mm) | 1 year | using echography |
| correlation between diaphargm thickness and maximal inspiratory pressure (cmH20) | 1 year | using echography and FPT |
Countries
France
Outcome results
None listed