Bulbar Function in Neuromuscular Patients

Study Overview
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Brief Summary
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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
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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: to study the correlation between the possible swallowing dysfunction and the anomalies of the respiratory parameters 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

Official Title
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Prospective Evaluation of Bulbar Function in Neuromuscular Patients

Conditions
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Neuromuscular Diseases

Intervention / Treatment
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* Procedure: tongue strength measurement
* Procedure: respiratory function measurement
* Procedure: swallowing tests
* Other: Magnetic resonance imaging


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 70 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| neuromuscular patient<br>Patients affected by neuromuscular pathology will be explored by tongue strength measurement, respiratory function measurement, swallowing tests and Magnetic resonance imaging | Procedure: tongue strength measurement<br>* tongue strength measurement<br>Procedure: respiratory function measurement<br>* respiratory function measurement<br>Procedure: swallowing tests<br>* swallowing tests<br>Other: Magnetic resonance imaging<br>* Magnetic resonance imaging<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change of tongue strength measurement | Measurement using mechanical force transducer | 5 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| swallowing tests | Non invasive measurement of swallowing function using surface electromyography, accelerometry and inductance plethysmography | 5 years | 
| Respiratory parameters | Vital capacity and respiratory muscle strength measurement using spirometry and manometry | 5 years | 
| Vital capacity | Spirometry | 5 years | 
| Respiratory muscle strength | manometry (maximal inspiratory pressure and expiratory pressure) | 5 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Tongue strength, neuromuscular, swallowing