Nemaline myopathy rod myopathy
Conditions
Interventions
Active IMT consists of 15 minutes of IMT, twice a day, 5 days per week for 8
weeks, at a training workload of 30% of MIP using a resistive inspiratory
muscle training device. Sham IMT consists of a
Sponsors
Radboud Universitair Medisch Centrum
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: * genetically-confirmed nemaline myopathy * informed consent from participant or legal representative
Exclusion criteria
Exclusion criteria: * history of another condition that affects respiratory muscle strength or function (e.g. COPD)*
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The primary outcome parameter is the change in maximal inspiratory pressure (MIP) after active inspiratory muscle training. | — |
Secondary
| Measure | Time frame |
|---|---|
| Several secondary outcome parameters: * Diaphragm ultrasound (thickness, thickening, excursion). * Pulmonary function tests (peak cough flow, forced vital capacity, forced expiratory volume the first second, peak expiratory flow, vital capacity) * Respiratory muscle function tests (sniff nasal inspiratory pressure, maximal expiratory pressure, twitch mouth pressure) * Neurological examination * Maximal voluntary contraction * Rate of muscle relaxation * Mobility: Rivermead mobility index, and Community Balance and Mobility Scale * Falls: Falls efficacy scale and Falls History Questionnaire | — |
Countries
Netherlands
Outcome results
None listed