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Abstract

Using 42 strength and functional assessments recorded monthly, the natural history of amyotrophic lateral sclerosis (ALS) is described in 167 patients (98 men, 67 women) followed in five medical centers in the western United States. The mean age at onset was 57.4 years, and symptoms were present for 2.64 years before study entry. Although there was a highly variable rate of decline within the group of patients, there were no differences in rate of decline by age or gender. Older patients and women were weaker on entry. Forty-eight patients died during the study. The median survival was 4.0 years for the study cohort but 2.1 years for newly diagnosed cases. Decline in pulmonary function most closely correlated with death. Our results emphasize the importance of considering clinical variability in planning clinical trials. One possible strategy is to identify and stratify patients by rate of decline in pulmonary function since prospectively identifying homogeneous subgroups allows investigators to substantially reduce sample size in therapeutic trials.

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Published In

Neurology®
Volume 43Number 7July 1993
Pages: 1316
PubMed: 8327132

Publication History

Published online: July 1, 1993
Published in print: July 1993

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Authors

Affiliations & Disclosures

S. P. Ringel, MD
Western Amyotrophic Lateral Sclerosis Group.
J. R. Murphy, PhD
Western Amyotrophic Lateral Sclerosis Group.
M. K. Alderson, MD
Western Amyotrophic Lateral Sclerosis Group.
W. Bryan, MD
Western Amyotrophic Lateral Sclerosis Group.
J. D. England, MD
Western Amyotrophic Lateral Sclerosis Group.
R. G. Miller, MD
Western Amyotrophic Lateral Sclerosis Group.
J. H. Petajan, MD, PhD
Western Amyotrophic Lateral Sclerosis Group.
S. A. Smith, MD
Western Amyotrophic Lateral Sclerosis Group.
R. I. Roelofs, MD
Western Amyotrophic Lateral Sclerosis Group.
F. Ziter, MD
Western Amyotrophic Lateral Sclerosis Group.
M. Y. Lee, MA
Western Amyotrophic Lateral Sclerosis Group.
J. R. Brinkmann, MS
Western Amyotrophic Lateral Sclerosis Group.
A. Almada
Western Amyotrophic Lateral Sclerosis Group.
E. Gappmaier, MS
Western Amyotrophic Lateral Sclerosis Group.
J. Graves, MS
Western Amyotrophic Lateral Sclerosis Group.
L. Herbelin
Western Amyotrophic Lateral Sclerosis Group.
M. Mendoza, MS
Western Amyotrophic Lateral Sclerosis Group.
D. Mylar, MS
Western Amyotrophic Lateral Sclerosis Group.
P. Smith, MS
Western Amyotrophic Lateral Sclerosis Group.
P. Yu
Western Amyotrophic Lateral Sclerosis Group.

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Cited By
  1. Cannabinoid Neurobiology and Medical Cannabis Intervention for Amyotrophic Lateral Sclerosis (ALS), Medical Cannabis and the Effects of Cannabinoids on Fighting Cancer, Multiple Sclerosis, Epilepsy, Parkinson's, and Other Neurodegenerative Diseases, (147-169), (2023).https://doi.org/10.4018/978-1-6684-5652-1.ch006
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  2. Genetic Variability of Inflammation and Oxidative Stress Genes Affects Onset, Progression of the Disease and Survival of Patients with Amyotrophic Lateral Sclerosis, Genes, 13, 5, (757), (2022).https://doi.org/10.3390/genes13050757
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  3. A review: Management of motor neuron diseases, IP Indian Journal of Neurosciences, 7, 4, (292-294), (2022).https://doi.org/10.18231/j.ijn.2021.053
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  4. Predictive parameters of early respiratory decline in amyotrophic lateral sclerosis, European Journal of Neurology, 29, 11, (3170-3176), (2022).https://doi.org/10.1111/ene.15486
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  5. A Multi-Center Cohort Study on Characteristics of Pain, Its Impact and Pharmacotherapeutic Management in Patients with ALS, Journal of Clinical Medicine, 10, 19, (4552), (2021).https://doi.org/10.3390/jcm10194552
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  7. Single breath counting is an effective screening tool for forced vital capacity in ALS, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22, sup1, (5-8), (2021).https://doi.org/10.1080/21678421.2021.1915337
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  8. Cyclophosphamide: a Therapeutic Option for Amyotrophic Lateral Sclerosis, Journal of Neuroimmune Pharmacology, 17, 3-4, (423-424), (2021).https://doi.org/10.1007/s11481-021-10032-5
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  9. The Benefit of Non-invasive Ventilation in Motor Neuron Disease, The Open Respiratory Medicine Journal, 14, 1, (53-61), (2020).https://doi.org/10.2174/1874306402014010053
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  10. Interleukin 6 (IL6) level is a biomarker for functional disease progression within IL6R 358 Ala variant groups in amyotrophic lateral sclerosis patients , Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22, 3-4, (248-259), (2020).https://doi.org/10.1080/21678421.2020.1813310
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