TABLE 1

Problems related to bulbar dysfunction during NIV in ALS and proposed solutions from the literature

ProblemsProposed solutions
NIVCheck effectiveness (built-in software, polygraphy, polysomnography) [12, 17]
 Obstructive eventsIncrease EPAP [16, 24, 25]
Decrease ventilator support [12, 16, 24, 25]
Reduce inspiratory time [12, 16, 24, 25]
Switch to volume assist/control mode [12, 16, 24, 25]
 Orofacial muscle weaknessSwitch from nasal or MPV during day to a nasal mask with chin strap, leap-seal MPV or an oronasal mask [18]
SialorrhoeaAnticholinergic drugs [30]
Botulinum toxin [30]
Radiotherapy [30]
Respiratory secretionsAssisted coughing techniques [3, 11, 12, 26, 27, 29]
MI-EOptimal adjustments and individualised settings (laryngoscopy or waveforms analysis) [26, 27]
 Obstruction during insufflationDecrease insufflation pressure [26, 27]
 Obstruction during exsufflationDecrease exsufflation pressure [26, 27]

NIV: noninvasive ventilation; ALS: amyotrophic lateral sclerosis; EPAP: expiratory positive airway pressure; MI-E: mechanical in-exsufflation; MPV: mouthpiece ventilation.