Prognostic factors in ALS: A critical review

Adriano Chiò, Giancarlo Logroscino, Orla Hardiman, Robert Swingler, Douglas Mitchell, Ettore Beghi, Bryan G. Traynor

Research output: Contribution to journalArticle

Abstract

We have performed a systematic review to summarize current knowledge concerning factors related to survival in ALS and to evaluate the implications of these data for clinical trials design. The median survival time from onset to death ranges from 20 to 48 ;months, but 1020% of ALS patients have a survival longer than 10 ;years. Older age and bulbar onset are consistently reported to have a worse outcome. There are conflicting data on gender, diagnostic delay and El Escorial criteria. The rate of symptom progression was revealed to be an independent prognostic factor. Psychosocial factors, FTD, nutritional status, and respiratory function are also related to ALS outcome. The effect of enteral nutrition on survival is still unclear, while NIPPV has been found to improve survival. There are no well established biological markers of progression, although some are likely to emerge in the near future. These findings have relevant implications for the design of future trials. Randomization, besides the type of onset, should take into account age, respiratory status at entry, and a measure of disease progression pre-entry. Alternative trial designs can include the use of natural history controls, the so-called minimization method for treatment allocation, and the futility approach.

Original languageEnglish
Pages (from-to)310-323
Number of pages14
JournalAmyotrophic Lateral Sclerosis
Volume10
Issue number5-6
DOIs
Publication statusPublished - 2009

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Keywords

  • Amyotrophic lateral sclerosis
  • Prognostic factors
  • Survival
  • Trials

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Chiò, A., Logroscino, G., Hardiman, O., Swingler, R., Mitchell, D., Beghi, E., & Traynor, B. G. (2009). Prognostic factors in ALS: A critical review. Amyotrophic Lateral Sclerosis, 10(5-6), 310-323. https://doi.org/10.3109/17482960802566824