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Article Dans Une Revue Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders Année : 2009

Prognostic factors in ALS: A critical review.

Adriano Chiò
Giancarlo Logroscino
  • Fonction : Auteur
Orla Hardiman
Robert Swingler
  • Fonction : Auteur
Douglas Mitchell
  • Fonction : Auteur
Bryan G Traynor
  • Fonction : Auteur


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 10-20% 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.

Dates et versions

hal-00603350 , version 1 (24-06-2011)



Adriano Chiò, Giancarlo Logroscino, Orla Hardiman, Robert Swingler, Douglas Mitchell, et al.. Prognostic factors in ALS: A critical review.. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders, 2009, 10 (5-6), pp.310-23. ⟨10.3109/17482960802566824⟩. ⟨hal-00603350⟩
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