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NEUROLOGY 1998;50:66-72
© 1998 American Academy of Neurology

Prognostic indicators of survival in ALS

Nancy Stambler, MS, Matthew Charatan, RPh, Jesse M. Cedarbaum, MD and ALS CNTF Treatment Study Group*

From Regeneron Pharmaceuticals, Inc., Tarrytown, NY.

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

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.


*See the Appendix on page 71 for a list of ALS CNTF Treatment Study Group members.

Supported entirely by a grant from Regeneron Pharmaceuticals.

Presented in part at the 48th Annual Meeting of the American Academy of Neurology, San Francisco, CA, March 1996.

Received February 15, 1997. Accepted in final form August 8, 1997.




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