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January 10, 2005

The ALSFRSr predicts survival time in an ALS clinic population

P. Kaufmann, MD, MSc, G. Levy, MD, J. L.P. Thompson, PhD, M. L. DelBene, NP-P, RN, MS, V. Battista, BA, P. H. Gordon, MD, L. P. Rowland, MD, B. Levin, PhD, and H. Mitsumoto, MDAuthors Info & Affiliations
January 11, 2005 issue
64 (1) 38-43
https://doi.org/10.1212/01.WNL.0000148648.38313.64
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Abstract

Objective: To determine whether the Amyotrophic Lateral Sclerosis Functional Rating Scale–revised (ALSFRSr), a predictor of survival time in ALS clinical trials, predicts survival time in an ALS clinic population.
Methods: The authors prospectively evaluated 267 consecutive patients with ALS at first visit to an ALS clinic using the ALSFRSr and pulmonary function testing. The association of ALSFRSr score at baseline with death or tracheostomy in ALS was examined using Cox proportional hazards models, adjusting for age at baseline, sex, and symptom duration.
Results: Of 267 patients with ALS, 103 (39%) reached the endpoint, defined as either death (79 patients) or tracheostomy (24 patients), during a mean follow-up of 1.0 ± 0.7 years. Among the 103 patients who reached the endpoint during follow-up, 77 (75%) had a baseline ALSFRSr score of less than 38 (the median baseline score of all patients), compared to 53 of 164 (32%) who remained alive without tracheostomy. Patients with a total ALSFRSr score below the median had a 4.4-fold increased risk of death or tracheostomy compared to those who scored above the median (HR: 4.38, 95% CI: 2.79 to 6.86, p < 0.001). Both the total ALSFRSr score at baseline (HR: 0.94, 95% CI: 0.91 to 0.98, p < 0.001) and forced vital capacity at baseline (HR: 0.99, 95% CI: 0.98 to 1.00, p = 0.02) were associated with death or tracheostomy when included in the same Cox model.
Conclusions: In an ALS clinic population, the total Amyotrophic Lateral Sclerosis Functional Rating Scale–revised score at baseline is a strong predictor of death or tracheostomy independently of forced vital capacity and after adjustment for age at baseline, sex, and symptom duration.

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Information & Authors

Information

Published In

Neurology®
Volume 64 • Number 1 • January 11, 2005
Pages: 38-43

Copyright

© 2005.

Publication History

Received: February 10, 2004
Accepted: September 15, 2004
Published online: January 10, 2005
Published in print: January 11, 2005

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Authors

Affiliations & Disclosures

P. Kaufmann, MD, MSc
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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G. Levy, MD
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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J. L.P. Thompson, PhD
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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M. L. DelBene, NP-P, RN, MS
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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V. Battista, BA
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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P. H. Gordon, MD
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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L. P. Rowland, MD
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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B. Levin, PhD
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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H. Mitsumoto, MD
From the Department of Neurology, College of Physicians and Surgeons (Drs. Kaufmann, Gordon, Rowland, and Mitsumoto, and M.L. DelBene and V. Battista), and the Department of Biostatistics, Mailman School of Public Health (Drs. Levy, Thompson, and Levin), Columbia University, New York, NY.
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Notes

Address correspondence and reprint requests to Dr. Petra Kaufmann, The Neurologic Institute, Columbia University, 710 W 168th Street, New York, NY 10032; e-mail: [email protected]

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