Neuromuscular Diseases
Conditions
Keywords
Tongue strength, neuromuscular, swallowing
Brief summary
The main objective is prospectively study the upper airways function in an adult population of neuromuscular patients. The main measurement will be the evolution of tongue strength recorded each year during annual clinical evaluation on a 5 years period.
Detailed description
The main objective is to study the function of the superior airways (tongue and pharynx) in an non invasive way in neuromuscular patients followed in the home ventilation unit of the Raymond POINCARE hospital (myopathy, neuropathy, disease of the neuromuscular junction or anterior horn) or of Necker Hospital and to detect prematurely the bulbar dysfunction. The secondary objectives of this prospective monocentric observational physiopathological study are: 1. to study the correlation between the possible swallowing dysfunction and the anomalies of the respiratory parameters 2. to establish correlations between infraclinical or clinical and presence of morphological tongue anomalies detected by magnetic resonance imaging like in acid maltase deficiency. The maximal tongue strength protrusion will be measured according to a method already used in the laboratory to explore sleep apnea syndromes. Swallowing will be estimated by a non invasive method already used to highlight swallowing disorders in neurological and muscular pathologies This method will be associated with a method of recording of thoracic and abdominal movements. Data wil be compared to a control database obtained for a previous study
Interventions
PROCEDUREtongue strength measurement
tongue strength measurement
PROCEDURErespiratory function measurement
respiratory function measurement
PROCEDUREswallowing tests
swallowing tests
OTHERMagnetic resonance imaging
Magnetic resonance imaging
Sponsors
Centre d'Investigation Clinique et Technologique 805
Study design
Observational model
OTHER
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
6 Years to 70 Years
Healthy volunteers
No
Inclusion criteria
* neuromuscular disorder * age greater than or equal to 6 * Hospitalization in Home Ventilation Unit - Written informed consent
Exclusion criteria
* patient's refusal to participate * major swallowing disfunction * Acute respiratory failure * no affiliation to social security
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change of tongue strength measurement | 5 years | Measurement using mechanical force transducer |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Respiratory parameters | 5 years | Vital capacity and respiratory muscle strength measurement using spirometry and manometry |
| swallowing tests | 5 years | Non invasive measurement of swallowing function using surface electromyography, accelerometry and inductance plethysmography |
| Vital capacity | 5 years | Spirometry |
| Respiratory muscle strength | 5 years | manometry (maximal inspiratory pressure and expiratory pressure) |
Countries
France
Contacts
Primary ContactDavid Orlikowski, MD PhD
Backup ContactFrederic Barbot, MD
Outcome results
None listed