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Arch Bronconeumol. 2014 Dec;50(12):509-13. doi: 10.1016/j.arbres.2014.04.010. Epub 2014 Jun 12.

Amyotrophic lateral sclerosis: impact of pulmonary follow-up and mechanical ventilation on survival. A study of 114 cases.

[Article in English, Spanish]

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Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, CHUAC, A Coruña, España.
Servicio de Neumología, Complexo Hospitalario Universitario A Coruña, CHUAC, A Coruña, España. Electronic address:



To study the impact of ventilatory management and treatment on the survival of patients with amyotrophic lateral sclerosis (ALS).


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.


descriptive and Kaplan-Meier estimator.


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.


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.


Amyotrophic lateral sclerosis; Esclerosis lateral amiotrófica; Insuficiencia respiratoria; Manejo respiratorio; Respiratory management; Ventilatory failure

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