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Intermittent negative pressure ventilation in the treatment of respiratory failure in progressive neuromuscular disease

Department of Medicine, University of Missouri, Columbia, MO, and the Department of Neurology and the Pulmonary Rehabilitation/RESTOR Program, University of Wisconsin Center Health Sciences and William S. Middleton VA Hospital, Madison, WI.

Five men with degenerative neuromuscular diseases (three with amyotrophic lateral sclerosis [ALS] and two with Duchenne's muscular dystrophy [DMD]) who had respiratory failure were treated with intermittent negative pressure ventilation (NPV). One patient with ALS in severe acute respiratory failure was successfully treated with NPV alone. This patient and two other ALS patients in chronic respiratory failure with Paco₂ elevation stabilized or improved their vital capacity (VC) and lowered their Paco₂ after 5 to 11 weeks of therapy. Finally, intermittent NPV was used to replace 24-hour positive pressure ventilation in two patients with DMD. It is concluded that intermittent NPV may stabilize or temporarily improve the respiratory status in patients with progressive neuromuscular diseases.

Address correspondence and reprint requests to Dr. Braun, Department of Medicine, University of Missouri, One Hospital Drive, Columbia, MO 65212.

Received November 19, 1986. Accepted for publication in final form February 26, 1987.

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