Neuromuscular Diseases in Children, Neuromuscular Diseases in Infants
Conditions
Brief summary
Preliminary data underline the fact that muscular deoxygenation during training happens earlier in adult patients suffering from neuromuscular disorders in comparison to controls. Up to date, pediatric data are lacking. The study aims at demonstrating if a correlation exists between the motor deficiency and the muscular deoxygenation in children suffering from neuromuscular disorders.
Detailed description
Preliminary data underline the fact that muscular deoxygenation during training happens earlier in adult patients suffering from neuromuscular disorders in comparison to controls, as it was demonstrated in Becker muscular dystrophy patients and fascioscapulo humeral muscular dystrophy. Moreover, the deoxygenation kinetics was positively correlated to the functional status and the muscular deficiency severity. However, no pediatric study has been conducted on this topic up to date. The aim of the study is to determine if a correlation exists in children suffering from neuromuscular diseases: * between muscular oxygenation and the percentage of the 6 minutes walking test performed in the routine medical care * between muscular oxygenation and the total and D1 motor function measure value
Interventions
6 minutes walking test performed in the routine medical care with muscle oximetry recording
Sponsors
Study design
Eligibility
Inclusion criteria
* Children from 6 to 18 years-old suffering from a neuromuscular disorder * Able to walk, with or without help
Exclusion criteria
* Hypoxia secondary to heart or lung disease * Visual impairment (\<5/10) * Parents refusal concerning data storage
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Muscular oxygenation | 6 minutes | Muscular oximetry |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| 6 minutes walking distance | 6 minutes | 6 minutes walking distance |
| Motor function | 25 minutes | Motor function measure (MFM scale) |
Countries
France