Bulbar Function in Neuromuscular Patients (ORDEG)

• Change of tongue strength measurement [ Time Frame: 5 years ]
  Measurement using mechanical force transducer
  
  

• swallowing tests [ Time Frame: 5 years ]
  Non invasive measurement of swallowing function using surface electromyography, accelerometry and inductance plethysmography
  
  
• Respiratory parameters [ Time Frame: 5 years ]
  Vital capacity and respiratory muscle strength measurement using spirometry and manometry
  
  
• Vital capacity [ Time Frame: 5 years ]
  Spirometry
  
  
• Respiratory muscle strength [ Time Frame: 5 years ]
  manometry (maximal inspiratory pressure and expiratory pressure)
  
  

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