Neurology 2003;60:813-819
© 2003 American Academy of Neurology
Prognosis in amyotrophic lateral sclerosis
A population-based study
M.A. del Aguila, PhD,
W.T. Longstreth, Jr, MD MPH,
V. McGuire, PhD,
T.D. Koepsell, MD MPH and
G. van Belle, PhD
From the Departments of Dental Public Health Sciences (Dr. del Aguila), Neurology (Dr. Longstreth), Epidemiology (Drs. del Aguila, Longstreth, McGuire, and Koepsell), Health Services (Dr. Koepsell), Environmental Health (Dr. van Belle), and Biostatistics (Dr. van Belle), University of Washington, Seattle.
Address correspondence and reprint requests to Dr. Longstreth, Department of Neurology, Box 359775, Harborview Medical Center, 325 Ninth Avenue, Seattle, WA 98104-2420; e-mail: wl{at}u.washington.edu
Background: Accurate information on prognosis of ALS is useful to patients, families, and clinicians.
Methods: In a population-based study of ALS in western Washington, the authors assembled a cohort of 180 patients with incident ALS between 1990 and 1994. Information on potential prognostic factors was collected during an in-person interview. Patients also completed the Medical Outcomes Study Short Form 36 (SF-36). Vital status through December 1999 was known for all patients.
Results: Median survival was 32 months from onset of symptoms and 19 months from diagnosis. The 5-year survival after diagnosis was 7%. Older age and female sex were strongly associated with poor survival. In multivariable Cox proportional hazards regression models, factors significantly and independently associated with a worse prognosis included older age, any bulbar features at onset, shorter time from symptom onset to diagnosis, lack of a marital partner, and residence in King County. Recursive partitioning identified age, time from symptom onset to diagnosis, and marital status as the strongest predictors of survival. Good summary scores for physical health on the SF-36, but not for mental health, were significantly associated with longer survival than poor scores.
Conclusion: These findings are consistent with other population-based studies of ALS and confirm its pernicious nature. Older age, female sex, any bulbar features at onset, short time from symptom onset to diagnosis, lack of a marital partner, and disease severity are key prognostic factors. Serial measurement of severity would likely improve predictions.
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Correspondence:
Read all Correspondence
- Reply to Letter to the Editor
- WT Longstreth, et al.
- Neurology Online, 16 Jul 2003
[Full text]
- Prognosis in amyotrophic lateral sclerosis: A population-based study
- Adriano Chio, et al.
- Neurology Online, 16 Jul 2003
[Full text]
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