title: Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease.
abstract: BACKGROUND
  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.
authors.affiliation: Department of Physical Medicine and Rehabilitation, University of Medicine and Dentistry of New Jersey-New Jersey Medical School, University Hospital B-403, 150 Bergen Street, Newark, New Jersey 07103, USA.
authors.forename: Kedar R
authors.initials: KR
authors.lastname: Mahajan
authors.forename: John Robert
authors.initials: JR
authors.lastname: Bach
authors.forename: Lou
authors.initials: L
authors.lastname: Saporito
authors.forename: Nick
authors.initials: N
authors.lastname: Perez
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D000704:Analysis of Variance; D003964:Diaphragm / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation / Q000379:methods*; D012133:Respiratory Paralysis / Q000209:etiology* / Q000534:rehabilitation*; D012189:Retrospective Studies; D015996:Survival Rate; D014797:Vital Capacity / Q000502:physiology