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Noninvasive positive-pressure ventilation in ALS

Predictors of tolerance and survival

From the ALS Research Center (D.L.C., V.L.B., F.P.), Department of Neurology, Ophthalmology, Otorhinolaryngology, and Psychiatry, Università di Palermo, Palermo, Italy; and the Pulmonary and Respiratory Intensive Care Unit (S.M., M.C.P., A.L.C.), Ospedale Civico, Palermo, Italy.

Address correspondence and reprint requests to Dr. Daniele Lo Coco, ALS Research Center, Department of Neurology, Ophthalmology, Otorhinolaryngology, and Psychiatry, Università di Palermo, Via G La Loggia, 1, 90129 Palermo, Italy; e-mail: danielelococo{at}yahoo.com

Objective: To identify factors associated with tolerance and survival after noninvasive positive-pressure ventilation (NIPPV) and to investigate the influence of NIPPV on lung function in patients with ALS.

Methods: NIPPV was offered to 71 patients with ALS in accordance with currently published guidelines. Effects of NIPPV on lung function and factors influencing tolerance and survival after NIPPV were studied.

Results: Forty-four patients (61.9%; 95% CI: 50.6 to 73.2) tolerated NIPPV (NIPPV use 4 h/day) and 27 (38.1%; 95% CI: 26.8 to 49.4) were intolerant (NIPPV use <4 h/day). Patients with mild or moderate bulbar symptoms were more likely to tolerate NIPPV than those with severe impairment (odds ratio = 6.09, 95% CI: 1.18 to 31.52, p = 0.031). After NIPPV introduction, a slower decline in forced vital capacity (FVC) was observed in tolerant vs intolerant patients (p = 0.002). The slope of FVC decline after NIPPV initiation (risk ratio [RR]: 0.78, 95% CI: 0.65 to 0.94, p = 0.01) together with NIPPV tolerance (RR: 0.32, 95% CI: 0.13 to 0.78, p = 0.013) were the only independent predictors of survival in the overall group of patients. In multivariate analysis, body mass index was the most powerful predictor of longer survival after NIPPV in tolerant patients (RR: 0.77, 95% CI: 0.61 to 0.96, p = 0.022).

Conclusion: Survival after noninvasive ventilation was independently related to ventilatory use (4 h/day) and to the modifications of forced vital capacity decline after treatment initiation. The severity of bulbar impairment and the nutritional status of the ALS patients at the introduction of ventilation may predict tolerance and survival.

Editorial, see page 736

This article was previously published in electronic format as an Expedited E-Pub on August 9, 2006, at www.neurology.org.

Disclosure: The authors report no conflicts of interest

Received November 9, 2005. Accepted in final form May 8, 2006.

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