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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

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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References

1.

Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet. 1996; 347: 1425–1431.

2.

A controlled trial of recombinant methionyl human BDNF in ALS: The BDNF Study Group (Phase III). Neurology. 1999; 52: 1427–1433.

3.

Groeneveld GJ, Veldink JH, van der Tweel I, et al. A randomized sequential trial of creatine in amyotrophic lateral sclerosis. Ann Neurol. 2003; 53: 437–445.

4.

Bensimon G, Lacomblez L, Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med. 1994; 330: 585–591.

5.

A double-blind placebo-controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis. ALS CNTF Treatment Study Group. Neurology. 1996; 46: 1244–1249.

6.

Miller RG, Moore D, Young LA, et al. Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis. WALS Study Group. Western Amyotrophic Lateral Sclerosis Study Group. Neurology. 1996; 47: 1383–1388.

7.

Miller RG, Moore DH, 2nd, Gelinas DF, et al. Phase III randomized trial of gabapentin in patients with amyotrophic lateral sclerosis. Neurology. 2001; 56: 843–848.

8.

Cudkowicz ME, Shefner JM, Schoenfeld DA, et al. A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis. Neurology. 2003; 61: 456–464.

9.

Lai EC, Felice KJ, Festoff BW, et al. Effect of recombinant human insulin-like growth factor-I on progression of ALS. A placebo-controlled study. The North America ALS/IGF-I Study Group. Neurology. 1997; 49: 1621–1630.

10.

Borasio GD, Robberecht W, Leigh PN, et al. A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. European ALS/IGF-I Study Group. Neurology. 1998; 51: 583–586.

11.

Shefner JM, Cudkowicz ME, Schoenfeld D, et al, and the NEALS Consortium. Clinical trial of creatine in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4 (suppl): 28, C31.

12.

Brooks BR. Functional scales: summary. Amyotroph Lateral Scler Other Motor Neuron Disord. 2002; 3 (suppl 1): S13–18.

13.

The Amyotrophic Lateral Sclerosis Functional Rating Scale. Assessment of activities of daily living in patients with amyotrophic lateral sclerosis. The ALS CNTF treatment study (ACTS) phase I-II Study Group. Arch Neurol. 1996; 53: 141–147.

14.

Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci. 1999; 169: 13–21.

15.

Cedarbaum JM, Stambler N. Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter clinical trials. J Neurol Sci. 1997; 152 suppl 1: S1–9.

16.

Moore DH, Miller RG, WALS Study Group, ALS CARE Study Group. ALSFRS as a measure of disease progression and survival. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4 (suppl 1): 42, C50.

17.

Traynor BJ, Zhang H, Shefner JM, Schoenfeld D, Cudkowicz CE. Functional outcome measures as a clinical trial endpoint in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4 (suppl 1): 42, C51.

18.

Magnus T, Beck M, Giess R, Puls I, Naumann M, Toyka KV. Disease progression in amyotrophic lateral sclerosis: predictors of survival. Muscle Nerve. 2002; 25: 709–714.

19.

Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors. J Neurol Sci. 1994; 124 suppl: 96–107.

20.

Cox DR. Regression models and life tables. J R Statist Soc B. 1972; 34: 187–220.

21.

Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc. 1958; 53: 547–481.

22.

Turner M, Al-Chalabi A. Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology. 2002; 59: 2012–2013;author reply 2013.

23.

Chio A, Mora G, Leone M, et al. Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology. 2002; 59: 99–103.

24.

Turner MR, Bakker M, Sham P, Shaw CE, Leigh PN, Al-Chalabi A. Prognostic modelling of therapeutic interventions in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2002; 3: 15–21.

25.

Cedarbaum JM, Stambler N. Disease status and use of ventilatory support by ALS patients. BDNF Study Group. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001; 2: 19–22.

26.

Kasarskis EJ, Scarlata D, Hill R, Fuller C, Stambler N, Cedarbaum JM. A retrospective study of percutaneous endoscopic gastrostomy in ALS patients during the BDNF and CNTF trials. J Neurol Sci. 1999; 169: 118–125.

27.

Mitsumoto H, Davidson M, Moore D, et al. Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4: 177–185.

28.

Traynor BJ, Alexander M, Corr B, Frost E, Hardiman O. An outcome study of riluzole in amyotrophic lateral sclerosis–a population-based study in Ireland, 1996–2000. J Neurol. 2003; 250: 473–479.

29.

Bruijn LI, Trotti D, Kristal BS, et al. Pre-clinical trials of three FDA-approved compounds in a mouse model of ALS. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4 (suppl 1): 76, C96.

30.

Fleiss JL, Levin B, Paik MC. Statistical methods for rates and proportions. Hoboken, NJ: Wiley Interscience, 2003.

Information & Authors

Information

Published In

Neurology^®

Volume 64 • Number 1 • January 11, 2005

Pages: 38-43

PubMed: 15642901

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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