Cited by 1,172Amyotrophic Lateral Sclerosis ResearchNeurogenetic and Muscular Disorders ResearchAmyloidosis: Diagnosis, Treatment, Outcomes

Prognostic factors in ALS: A critical review

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Chiò, Adriano, Logroscino, Giancarlo, Hardiman, Orla, Swingler, Robert, Mitchell, Douglas, Beghi, Ettore, Traynor, Bryan G., On Behalf of the Eurals Consortium·2009·Amyotrophic Lateral Sclerosis

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 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.

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  • Bulbar onset ALS has been associated with faster disease progression.

    “Although bulbar onset ALS has been associated with faster disease progression (44), DFS rates were not significantly different between participants with ALSþCTE and ALS only although this analysis is likely underpowered due to the small number of participants with CTE.”

    From Chronic Traumatic Encephalopathy Within an Amyotrophic Lateral Sclerosis Brain Bank Cohort (2018) · cites this paper as [44]

    The Site of onset section says bulbar-onset ALS is associated with a worse prognosis than spinal-onset ALS. The review discusses progression rates separately, but does not establish that bulbar onset itself is associated with faster disease progression. (LLM, conf=0.85)

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Authors
Chiò, A., Logroscino, G., Hardiman, O., Swingler, R., Mitchell, D., Beghi, E., Traynor, B. G., & On Behalf of the Eurals Consortium
Affiliations
  • Azienda Ospedaliero Universitaria San Giovanni Battista (IT)
  • Harvard University (US)
  • Trinity College Dublin (IE)
  • Ninewells Hospital (GB)
  • Royal Preston Hospital (GB)
  • Mario Negri Institute for Pharmacological Research (IT)
Corresponding author
Chiò, Adriano
Venue
Amyotrophic Lateral Sclerosis
Published
2009-01
Volume
10
Issue
5-6
Pages
310-323
ISSN
  • 1471-180X (ISSN-L)
  • 1748-2968
OpenAlex
W2155221045
Cited by
1,172
References
3
Citations by year
2026: 76 · 2025: 88 · 2024: 99 · 2023: 111 · 2022: 98 · 2021: 90 · 2020: 96 · 2019: 76 · 2018: 79 · 2017: 71 · 2016: 67 · 2015: 52 · 2014: 48 · 2013: 23 · 2012: 35
FWCI
3.53
Type
journal article
Language
en
Open access
Locations
  • Amyotrophic Lateral Sclerosis (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
  • HAL (submittedVersion)
Funders
  • National Institutes of Health
  • National Institute on Aging (ZIAAG000933)
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords
prognostic factors, survival, bulbar onset, age, symptom progression, frontotemporal dementia, nutritional status, respiratory function, enteral nutrition, noninvasive positive pressure ventilation, biomarkers, clinical trial design, randomization, minimization method, futility
MeSH terms
Amyotrophic Lateral Sclerosis, Clinical Trials as Topic, Humans, Nutritional Status, Prognosis, Prospective Studies, Respiration, Retrospective Studies, Severity of Illness Index, Biomarkers, Survival Rate, Age of Onset, Disease Progression

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