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Abstract

We analyzed data from the 245-patient placebo group of the ALS CNTF Treatment Study Group study, a large, prospective, multicenter study of recombinant human ciliary neurotrophic factor to determine prognostic factors for length of survival in ALS. Variables examined included baseline demographic characteristics, indices of disease severity, pulmonary function, and clinical laboratory tests. Shorter survival was associated with greater age, lower percent-predicted forced vital capacity (FVC%), and lower serum chloride at study entry. A shorter interval from symptom onset to diagnosis of ALS and greater weight loss in the 2 months before study entry also predicted shortened survival times. The rate of muscle strength loss before study entry did not predict risk of mortality. Serum chloride, reflecting the degree of respiratory acidosis, was identified for the first time as being correlated with prognosis in ALS. The relationship between a patient's FVC% and the probability of survival is described.

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References

1.
Haverkamp LJ, Appel V, Appel SH. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 1995;118:707-719.
2.
Ringel SP, Murphy JR, Alderson MK, et al. The natural history of amyotrophic lateral sclerosis. Neurology 1993;43:1316-1322.
3.
Gubbay SS, Kahana E, Zilber N, et al. Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol 1985;232:295-300.
4.
Kondo K, Hemmi I. Clinical statistics in 515 fatal cases of motor neuron disease. Neuroepidemiology 1984;3:129-148.
5.
Preux P-M, Couratier PH, Boutros-Toni F, et al. Survival prediction in sporadic amyotrophic lateral sclerosis. Neuroepidemiology 1996;15:153-160.
6.
Cedarbaum JM, Stambler N. Performance of the ALS Functional Rating Scale (ALSFRS) in multicenter clinical trials. J Neurol Sci 1997;152(Suppl 1):1-9.
7.
Bensimon G, Lacomblez L, Meininger, the ALS Riluzole Study Group. A controlled trial of riluzole in amyotrophic lateral sclerosis. N Engl J Med 1994;330:585-591.
8.
Fallat RJ, Jewitt B, Bass M, et al. Spirometry in amyotrophic lateral sclerosis. Arch Neurol 1979;36:74-80.
9.
ALS CNTF Treatment Study (A.C.T.S.) Group. A double-blind, placebo-controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis(ALS). Neurology 1996;46:1244-1249.
10.
Andres PL, Hedlund W, Finison L, et al. Quantitative motor assessment in amyotrophic lateral sclerosis. Neurology 1986;36:937-941.
11.
Brooks BR. Amyotrophic lateral sclerosis clinimetric scales-guidelines for administration and scoring. In: Herndon R, ed. Handbook of clinical neurologic scales. New York: Demos Vermande, 1997:27-79.
12.
Traver GA, Cline MG, Burrows B. Predictors of mortality in chronic obstructive pulmonary disease. A 15-year follow-up study. Am Rev Respir Dis 1979;119:895-902.
13.
Oswald-Mammosser M, Weitzenblum E, Quoix E, et al. Prognostic factors in COPD patients receiving long-term oxygen therapy. Importance of pulmonary artery pressure. Chest 1995;107:1193-1198.
14.
Gold WM. Pulmonary function testing. In: Murray JF, Nadel JA, eds. Respiratory medicine, 2nd ed. Philadelphia: WB Saunders, 1994:871.

Information & Authors

Information

Published In

Neurology®
Volume 50Number 1January 1998
Pages: 66-72
PubMed: 9443459

Publication History

Published online: January 1, 1998
Published in print: January 1998

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Authors

Affiliations & Disclosures

Nancy Stambler, MS
From Regeneron Pharmaceuticals, Inc., Tarrytown, NY.
Matthew Charatan, RPh
From Regeneron Pharmaceuticals, Inc., Tarrytown, NY.
Jesse M. Cedarbaum, MD
From Regeneron Pharmaceuticals, Inc., Tarrytown, NY.
ALS CNTF Treatment Study Group*
From Regeneron Pharmaceuticals, Inc., Tarrytown, NY.

Notes

Address correspondence and reprint requests to Dr. Jesse M. Cedarbaum, Regeneron Pharmaceuticals, Inc., 777 Old Saw Mill River Rd., Tarrytown, NY 10591.

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