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Keywords:

  • amyotrophic lateral sclerosis;
  • diaphragm pacing;
  • motor neuron disease;
  • neuromuscular disease;
  • noninvasive mechanical ventilation;
  • survival;
  • vital capacity

Abstract

Introduction: Although it is known that continuous noninvasive ventilation (CNIV) can prolong life in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND), in this study we explore similar claims for diaphragm pacing (DP). Methods: NIV and DP users' vital capacities (VCs) over time and duration of NIV and CNIV dependence were analyzed for 354 non-DP and 8 DP ALS/MND patients. Results: Patients had a higher rate of monthly VC decline before NIV use (5.1 ± 7.6%) than during NIV use (2.5 ± 3.6%) (P < 0.01, 95% CI 0.84–4.5); the decline for 4 DP users was 3.7–20%. Fifty-five ALS/MND patients used part-time NIV for 19.9 ± 27.6 months until tracheostomy/death, whereas 113 others used it for 10.9 ± 10.5 months until CNIV dependence for another 12.8 ± 16.2 months. After placement, 7 DP users were CNIV dependent in 8.0 ± 7.0 months, whereas 6 underwent tracheostomy/died in 18.2 ± 13.7 months. Conclusions: CNIV prolonged the survival of 113 of the 354 non-DP and 6 DP ALS/MND patients by 12.8 and 10.2 months, respectively. DP provided no benefit on VC or mechanical ventilation–free survival. Muscle Nerve, 2012