Neuromuscular Diseases Clinical Trial
Official title:
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
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