title: Management of dyspnea in advanced motor neuron diseases.
abstract: OBJECTIVE
  Patients with amyotrophic lateral sclerosis or motor neuron disease (ALS/MND) invariably develop respiratory muscle weakness and most die from pulmonary complications. Little evidence is available that identifies optimal management approaches for caring for the dying patient. This review discusses the state of the art on dyspnea in advanced ALS/MND and its treatment.
  
  
  RESULTS
  Multiple observational studies have demonstrated that noninvasive positive pressure ventilation is beneficial in ALS/MND. It is a relatively safe intervention in the late stages of disease with additional survival benefits when it is started relatively early and it can improve survival. Despite guidelines related to pulmonary function testing about the use of noninvasive positive pressure ventilation, the factors, which are most closely associated with noninvasive positive pressure ventilation utilization, are dyspnea and orthopnea.
  
  
  CONCLUSIONS
  In ALS/MND, loss of function relentlessly progresses, and subsequent death occurs mostly in a predictable manner. Therefore, the end of life care is heavily influenced by the type and quality of care provided from the earliest stages. Most patients with ALS/MND develop dyspnea, agitation, anxiety and air hunger in the final phase. Noninvasive positive pressure ventilation has become the standard of care for patients with ALS/MND and advanced respiratory insufficiency. A multidisciplinary approach is strongly recommended.
authors.affiliation: Section of Palliative Care, Institute of Medical Research Alfredo Lanari, University of Buenos Aires, Argentina. vilma.tripodoro@gmail.com
authors.forename: Vilma Adriana
authors.initials: VA
authors.lastname: Tripodoro
authors.forename: Eduardo Luis
authors.initials: EL
authors.lastname: De Vito
mesh_terms: D016223:Advance Directives; D004417:Dyspnea / Q000209:etiology / Q000628:therapy*; D006801:Humans; D016472:Motor Neuron Disease / Q000150:complications*; D011175:Positive-Pressure Respiration / Q000379:methods*; D013727:Terminal Care / Q000458:organization & administration