Pulmonary predictors of survival in amyotrophic lateral sclerosis: Use in clinical trial design

Eric P. Schmidt, Daniel B. Drachman, Charles M. Wiener, Lora Clawson, Richard Kimball, Noah Lechtzin

Research output: Contribution to journalArticlepeer-review

56 Scopus citations


The rapidity of progression of amyotrophic lateral sclerosis (ALS) to death or respiratory failure impacts patients, clinicians, and clinical investigators. This study compared the abilities of various pulmonary function tests to predict tracheostomy-free survival. We evaluated 95 ALS patients by determining upright and supine forced vital capacity (FVC), maximal inspiratory (MIP) and expiratory (MEP) pressures, arterial partial pressure of carbon dioxide (PaCO2), and transdiaphragmatic sniff pressures (Pdi-sniff). Tracheostomy-free survival time was measured from the date of spirometry. Supine FVC, upright FVC, MIP, MEP, and Pdi-sniff were significantly associated with tracheostomy-free survival after controlling for non-pulmonary factors, whereas PaCO2 was not. A normal supine FVC, MIP, or MEP was highly predictive for one-year survival. These tests are well suited to predict survival for trial enrollment and patient counseling. Supine FVC's simplicity of use and availability to ALS investigators makes it a particularly attractive predictor of one-year survival in ALS.

Original languageEnglish (US)
Pages (from-to)127-132
Number of pages6
JournalMuscle and Nerve
Issue number1
StatePublished - Jan 2006


  • Amyotrophic lateral sclerosis
  • Prognosis
  • Pulmonary function
  • Spirometry
  • Therapeutic trial design

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)


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