Bulbar Function in Neuromuscular Patients
- Conditions
- Neuromuscular Diseases
- Interventions
- Procedure: tongue strength measurementProcedure: respiratory function measurementProcedure: swallowing testsOther: Magnetic resonance imaging
- Registration Number
- NCT02097368
- Lead Sponsor
- Centre d'Investigation Clinique et Technologique 805
- 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
Recruitment & Eligibility
- Status
- UNKNOWN
- Sex
- All
- Target Recruitment
- 100
- neuromuscular disorder
- age greater than or equal to 6
- Hospitalization in Home Ventilation Unit - Written informed consent
- patient's refusal to participate
- major swallowing disfunction
- Acute respiratory failure
- no affiliation to social security
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description neuromuscular patient swallowing tests Patients affected by neuromuscular pathology will be explored by tongue strength measurement, respiratory function measurement, swallowing tests and Magnetic resonance imaging neuromuscular patient Magnetic resonance imaging Patients affected by neuromuscular pathology will be explored by tongue strength measurement, respiratory function measurement, swallowing tests and Magnetic resonance imaging neuromuscular patient respiratory function measurement Patients affected by neuromuscular pathology will be explored by tongue strength measurement, respiratory function measurement, swallowing tests and Magnetic resonance imaging neuromuscular patient tongue strength measurement Patients affected by neuromuscular pathology will be explored by tongue strength measurement, respiratory function measurement, swallowing tests and Magnetic resonance imaging
- Primary Outcome Measures
Name Time Method Change of tongue strength measurement 5 years Measurement using mechanical force transducer
- Secondary Outcome Measures
Name Time Method swallowing tests 5 years Non invasive measurement of swallowing function using surface electromyography, accelerometry and inductance plethysmography
Respiratory parameters 5 years Vital capacity and respiratory muscle strength measurement using spirometry and manometry
Vital capacity 5 years Spirometry
Respiratory muscle strength 5 years manometry (maximal inspiratory pressure and expiratory pressure)
Trial Locations
- Locations (2)
Non invasive ventilation Unit - Necker Hospital
🇫🇷Paris, France
Home Ventilation Unit, Raymond Poincare Hospital
🇫🇷Garches, France