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Forschungsdatenbank PMU-SQQUID

[Survival with artificial respiration at home. An open, prospective study on home ventilation for neuromuscular diseases, in particular, the situation of ALS patients].
Winterholler, MG; Erbguth, FJ; Hecht, MJ; Heuss, D; Neundörfer, B;
Nervenarzt. 2001; 72(4):293-301
Originalarbeiten (Zeitschrift)


Erbguth Frank


A growing number of patients with neuromuscular disease have been treated with home mechanical ventilation during the past 15 years. We prospectively examined the long-term effects and complications of this method, particularly with regard to noninvasive positive pressure ventilation (NPPV). Thirty-one patients with amyotrophic lateral sclerosis (ALS, n = 20) or other slowly progressive neuromuscular diseases (NMD, n = 11) were observed for 17,517 home ventilation days (almost 48 ventilation years). The mean observed ventilation time was 565 days (min/max: 30/2930). Twenty-five patients were ventilated noninvasively with different masks. The calculated mean survival with NPPV ventilation (criteria: death, tracheostomy, or patient deciding to break off) was 2052 (SE: +/- 317.8) days in the NMD group, 248 days (+/- 35.7) for ALS patients without bulbar symptoms, and 82 days (+/- 27.4) with bulbar paralysis. Complications with the need for intervention were observed six times more frequently with ALS than with NMD. NPPV is effective for years in patients with slowly progressing NMD. Those ALS patients without bulbar symptoms can profit for up to a year from NPPV, while those with bulbar paralysis can have some symptom relief. Complications of every kind are much more frequent in ALS patients.

Useful keywords (using NLM MeSH Indexing)



Amyotrophic Lateral Sclerosis/nursing*

Amyotrophic Lateral Sclerosis/psychology




Home Nursing*


Intermittent Positive-Pressure Ventilation/adverse effects

Intermittent Positive-Pressure Ventilation/nursing*


Middle Aged

Neuromuscular Diseases/nursing*

Neuromuscular Diseases/psychology

Patient Satisfaction

Prospective Studies

Respiratory Insufficiency/nursing

Survival Analysis

Treatment Outcome

Treatment Refusal

Ventilator Weaning

Find related publications in this database (Keywords)

home ventilation
neuromuscular disease
survival analysis
amyotrophic lateral sclerosis