title: Amyotrophic lateral sclerosis: impact of pulmonary follow-up and mechanical ventilation on survival. A study of 114 cases.
abstract: OBJECTIVE
  To study the impact of ventilatory management and treatment on the survival of patients with amyotrophic lateral sclerosis (ALS).
  
  
  METHODS
  Retrospective analysis of 114 consecutive patients admitted to a general hospital, evaluating demographic data, type of presentation, clinical management, treatment with mechanical ventilation and survival.
  
  
  METHODS
  descriptive and Kaplan-Meier estimator.
  
  
  RESULTS
  Sixty four patients presented initial bulbar involvement. Overall mean survival after diagnosis was 28.0 months (95%CI, 21.1-34.8). Seventy patients were referred to the pulmonary specialist (61.4%) and 43 received non-invasive ventilation (NIV) at 12.7 months (median) after diagnosis. Thirty seven patients continued to receive NIV with no subsequent invasive ventilation. The mean survival of these patients was 23.3 months (95%CI, 16.7-28.8), higher in those without bulbar involvement, although below the range of significance. Survival in the 26 patients receiving programmed NIV was higher than in the 11 patients in whom this was indicated without prior pulmonary assessment (considered following diagnosis, P<.012, and in accordance with the start of ventilation, P<.004). A total of 7 patients were treated invasively; mean survival in this group was 72 months (95%CI, 14.36-129.6), median 49.6±17.5 (95%CI, 15.3-83.8), and despite the difficulties involved in home care, acceptance and tolerance was acceptable.
  
  
  CONCLUSIONS
  Long-term mechanical ventilation prolongs survival in ALS. Programmed pulmonary assessment has a positive impact on survival of ALS patients and is key to the multidisciplinary management of this disease.
authors.affiliation: Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, CHUAC, A Coruña, España.
authors.forename: Pilar
authors.initials: P
authors.lastname: Sanjuán-López
authors.affiliation: Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, CHUAC, A Coruña, España.
authors.forename: Paz
authors.initials: P
authors.lastname: Valiño-López
authors.affiliation: Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, CHUAC, A Coruña, España.
authors.forename: Jorge
authors.initials: J
authors.lastname: Ricoy-Gabaldón
authors.affiliation: Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, CHUAC, A Coruña, España. Electronic address: hector.verea.hernando@sergas.es.
authors.forename: Héctor
authors.initials: H
authors.lastname: Verea-Hernando
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D000818:Animals; D018450:Disease Progression; D004285:Dogs; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation; D012121:Respiration, Artificial; D012125:Respiratory Center / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy; D012189:Retrospective Studies; D016019:Survival Analysis; D014139:Tracheostomy
keywords: Amyotrophic lateral sclerosis; Esclerosis lateral amiotrófica; Insuficiencia respiratoria; Manejo respiratorio; Respiratory management; Ventilatory failure