Our objective was to describe patient characteristics, survival and long-term outcome in amyotrophic lateral sclerosis (ALS) patients treated with non-invasive and invasive home mechanical ventilation (HMV). A single-centre cohort study over a 15-years period (1998-2012) was performed. All ALS patients admitted to the centre were enrolled in the study. All patients were offered treatment with non-invasive and/or invasive HMV. The patients were divided into four groups: 1) no treatment; 2) treatment with non-invasive HMV; 3) treatment with non-invasive HMV followed by invasive HMV by tracheostomy; and 4) treatment with invasive HMV by tracheostomy. Patient characteristics and effects on survival were studied. Four hundred and thirty-one patients with ALS were admitted to a referral respiratory care unit (RCU) in the period January 1998 to June 2012. The average treatment time in the groups was: 1) 22.9 months (range 1-164); 2) 25.8 months (range 1-145); 3) 56.8 months (range 14-207); and 4) 33.8 months (range 6-88). Non-invasive HMV followed by invasive HMV is a possible treatment of respiratory symptoms in ALS and has a significant effect on survival.

Patients' requests for the withdrawal of IHMV in advanced stage ALS was related to a perceived "loss of meaning in life." Termination of treatment was performed under deep sedation as a medically, legally, and ethically justified procedure.