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Patients who survive 5 years or more with ALS in Olmsted County, 1925–2004
  1. Farrah J Mateen1,2,
  2. Marco Carone3,
  3. Eric J Sorenson1
  1. 1Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  2. 2Department of International Health, The Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA
  3. 3Department of Biostatistics, The Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA
  1. Correspondence to Dr Farrah J Mateen, Johns Hopkins University, 615 North Wolfe Street, E8518, Baltimore, MD 21205-2179, USA; fmateen{at}


Background A small proportion of patients with amyotrophic lateral sclerosis (ALS) survive more than 5 years. The frequency of 5-year or longer survival with ALS in a USA population is unknown but may provide a baseline for studies that employ survival as a primary endpoint of analysis.

Methods All persons diagnosed as having ALS in Olmsted County between 1925 and 2004 were studied for demographic and clinical features. Longer-term survivors were defined as patients who lived 5 years or longer, tracheostomy-free, following symptomatic onset.

Results 94 patients (mean survival from symptomatic onset 2.95 years (95% CI 2.54 to 3.35), mean survival from diagnosis 1.89 years (95% CI 1.54 to 2.24)) were diagnosed as having ALS. Five-year or longer survivors accounted for 14% of the population of patients (95% CI 7.9% to 22.8%). The frequency of 5 year or longer survivors did not change over time. The mean survival of these individuals was 7.04 years (95% CI 6.14 to 7.94 years; range 5.11–9.35 years). They had a significantly longer mean time to diagnosis (1.77 years, 95% CI 0.95 to 2.58 years) as compared with survivors of less than 5 years (0.94 years, 95% CI 0.75 to 1.13 years) (p=0.02) but could not be reliably identified at the time of diagnosis by age, sex, clinical presentation or El Escorial category.

Conclusion Patients surviving more than 5 years following the symptomatic onset of ALS account for 14% of the total ALS population. This frequency has not changed over time. Patients with a survival of 5 years or longer are clinically similar to the total population ALS population in terms of age, gender, presentation and site of onset but have a longer time from symptomatic onset to diagnosis.

  • Amyotrophic lateral sclerosis
  • natural history
  • prognosis
  • survival
  • clinical neurology
  • epidemiology
  • motor neuron disease
  • statistics

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  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by the Mayo Clinic Institutional Review Board.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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