Bulbar Function in Neuromuscular Patients
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