Nouvelles méthodes d'exploration de la fonction respiratoire des patients neuromusculaires

Abstract : Neuromuscular disorders are liable to induce a restrictive syndrome. The weakness of respiratory muscles andthe associated spinal deformities lead the patients into a vicious circle, which progressively worsens the restrictivesyndrome and evolves into respiratory failure. This respiratory failure may be associated to additional factors such aschronic reduction of thoracic cage motion, sleep disordered breathing, bulbar dysfunction and ineffective cough. Therefore,dysfunction of respiratory system may have multiples origins and requires precise evaluations targeted on thecomprehension of the physiopathologic mechanisms for each disease, as they may not only allow to adapt specificallytreatment, but also to assess the new therapeutics targeted for muscle restoration that are emerging for some neuromusculardiseases. Accordingly, it is essential to acquire knowledge about the specific evolution of the different disorder, with andwithout classic treatments, and also to improve the understanding of the physiopathologic mechanisms of respiratoryfailure, combining the classic evaluation tools of respiratory function with new evaluation techniques which could provideadditional information.This thesis aims to increase the knowledge of respiratory dysfunction of some neuromuscular disorders using classicevaluation tools and also to propose new exploration methods of respiratory function for neuromuscular patients.Thus, we explored data of two specific neuromuscular disorders patients, regularly followed: FacioscapulohumeralMuscular Dystrophy (FSHD) and Duchenne Muscular Dystrophy (DMD). The analysis of FSHD seemed important asrespiratory failure is not well known and described for this disease. On the other hand, while the evolution of DMD is wellknown, the impact of noninvasive ventilation (NIV) on the evolution of respiratory function has been poorly described,whereas new therapeutic trials will be soon proposed to these patients, some of which are already under NIV.Next, to better predict the improvement of the restrictive syndrome that could be potentially obtained with muscle repairtherapies, we developed a measure of pulmonary volumes, using assistance for inspiratory and/or expiratory muscles.We also developed and validated a painless, noninvasive, non-volitional and indirect method of measurement of thediaphragmatic force, in order to obtain results independent of patients’ motivation.Hence, this thesis was able to advance knowledge of the evolution of respiratory function of some neuromuscular diseases,using traditional evaluation tools of respiratory function. Moreover, it validated new pulmonary function measuresindependent of patient’s voluntary efforts.
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Dante Brasil Santos. Nouvelles méthodes d'exploration de la fonction respiratoire des patients neuromusculaires. Neurosciences [q-bio.NC]. Université Paris-Saclay, 2016. Français. ⟨NNT : 2016SACLV025⟩. ⟨tel-01372536⟩

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