Bulbar Function in Neuromuscular Patients

Prospective Evaluation of Bulbar Function in Neuromuscular Patients

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.

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

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