Résumé :
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Background : The rate of sleep-disordered breathing is high in patients with neuromuscular diseases. Diaphragm pacing stimulation (DPS) can successfully treat central apnea in complete tetraplegics, replacing mechanical ventilation. This reports a single site experience of using DPS to treat acquired central sleep apnea in incomplete tetraplegia and in patients with amyotrophic lateral sclerosis or motor neuron disease (ALS/MND). Design : Prospective trial database of diaphragm pacing patients at a single institution. Participants/methods : Patients with tetraplegia with documented central sleep apnea but volitional diaphragm movement underwent laparoscopic diaphragm motor point mapping with electrode implantation. After conditioning the diaphragm during daytime, patients utilized DPS for sleep instead of positive pressure ventilation. ALS patients who were involved in trials of DPS and developed sleep-disordered breathing began using DPS at night. Results : Four tetraplegic patients with central sleep apnea were implanted from 2006-2008 (three males, one female). Average age was 38 years (range 18-59) with implantation an average of 10 years post injury (range 3-24). All patients were able to utilize DPS for nighttime ventilation alone with discontinuation of positive pressure mechanical ventilation. One patient who was completely dependent on ventilation was successfully weaned. One patient was able to undergo tracheostomy tube decannulation after 24 years. Out of a population of 51 ALS patients implanted with DPS, 50% (25) utilize DPS with or without non-invasive positive pressure (NIPPV) ventilation at night to improve their sleep disordered breathing or acquired central sleep apnea. Continuous diaphragm electro-myographic recordings in ALS/MND patients show suppression of diaphragm activity with NIPPV. The combination of NIPPV and DPS can optimize ventilation while maintaining diaphragm strength and reduce the deconditioning effects of NIPPV and resulting rapid dependence. Conclusion : This experience shows that diaphragm pacing can be used to treat acquired central sleep apnea. Recent reports describe the rapid deconditioning of the diaphragm when utilizing positive pressure ventilation without diaphragm activity. DPS maintains Type 1 muscle fibers and improves respiratory compliance by ventilating posterior lobes. These factors improve daytime ventilation and can decrease the work of breathing in patients with compromised strength. Aggressive use of polysomnography can identify patients that have developed acquired central sleep apnea allowing this new option in ventilation.
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