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Effect of Noninvasive Positive-Pressure Ventilation on Survival in Amyotrophic Lateral Sclerosis

Loutfi Sami Aboussouan, MD; Saeed U. Khan, MD; David P. Meeker, MD; Kay Stelmach, RRT; and Hiroshi Mitsumoto, MD
[+] Article and Author Information

From Wayne State University School of Medicine, Detroit, Michigan; and the Cleveland Clinic Foundation, Cleveland, Ohio. Acknowledgment: The authors thank Dr. Gary T. Ferguson for his comments and extensive review of the manuscript. Requests for Reprints: Loutfi Sami Aboussouan, MD, Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, 3-Hudson, 3990 John R, Detroit, MI 48201. Current Author Addresses: Dr. Aboussouan: Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, 3-Hudson, 3990 John R, Detroit, MI 48201.


Copyright ©2004 by the American College of Physicians


Ann Intern Med. 1997;127(6):450-453. doi:10.7326/0003-4819-127-6-199709150-00006
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Background: Noninvasive positive-pressure ventilation may benefit patients with amyotrophic lateral sclerosis and respiratory insufficiency.

Objective: To determine 1) whether patients tolerant of noninvasive positive-pressure ventilation have better survival than intolerant patients and 2) whether bulbar symptoms account for intolerance of noninvasive positive-pressure ventilation.

Design: Observational cohort study.

Setting: Tertiary care referral center.

Patients: 39 patients with amyotrophic lateral sclerosis who were treated with noninvasive positive-pressure ventilation.

Intervention: Noninvasive positive-pressure ventilation was started for patients with new orthopnea, new hypercapnia, or both. Patients were divided into two groups: those tolerant of and those intolerant of noninvasive positive-pressure ventilation.

Results: The risk for death from onset of respiratory insufficiency was higher for intolerant patients than for tolerant patients (relative risk, 3.1 [95% CI, 1.8 to 9.6]). Moderate or severe bulbar symptoms were more prevalent among intolerant patients than among tolerant patients (67% compared with 33%; P = 0.04).

Conclusions: Among patients with amyotrophic lateral sclerosis, those who are tolerant of noninvasive positive-pressure ventilation have better survival than do those who are intolerant. Bulbar symptoms partially account for intolerance of noninvasive positive-pressure ventilation.

Figures

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Figure 1.
Kaplan-Meier survival plots from initiation of noninvasive positive-pressure ventilation. Top.PMiddle.PBottom.P

In patients intolerant of noninvasive positive-pressure ventilation, the relative risk for death was 3.1-fold greater (95% CI, 1.8-fold to 9.6-fold) than that in tolerant patients ( < 0.001). Median duration of survival in intolerant and tolerant patients was 2 and 15 months, respectively. In patients with mild or no bulbar symptoms, the relative risk for death of intolerant compared with tolerant patients was 3.5 (CI, 1.5 to 2.4; = 0.01). In patients with moderate or severe bulbar symptoms, the relative risk for death for intolerant compared with tolerant patients was 2.7 (CI, 1.1 to 10.6; = 0.03).

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