Evidence map›Paper›PMID 39475283›Full record

ArticleEuropean journal of neurology2025

Amyotrophic lateral sclerosis established as a multistep process across phenotypes.

Laura Ziser, Ruben P A van Eijk, Matthew C Kiernan, Allan McRae, Robert D Henderson, David Schultz, Merrilee Needham, Susan Mathers, Pam McCombe, Paul Talman and 1 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in European journal of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed.

  1. Review
  2. Review of the Pathology of Muscle in Amyotrophic Lateral Sclerosis.International journal of molecular sciences · 2026
    Review
  3. Review
  4. Article
  5. Review
  6. Review
  7. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

11 authors.

Laura ZiserInstitute for Molecular Bioscience, University of Queensland, Brisbane, Queensland, Australia.
Ruben P A van EijkDepartment of Neurology, UMC Utrecht Brain Centre, University Medical Center Utrecht, Utrecht, The Netherlands.ORCID 0000-0002-7132-5967
Matthew C KiernanNeuroscience Research, Sydney, New South Wales, Australia.
Allan McRaeInstitute for Molecular Bioscience, University of Queensland, Brisbane, Queensland, Australia.
Robert D HendersonDepartment of Neurology, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia.
David SchultzDepartment of Neurology, Flinders University of South Australia, and Flinders Medical Centre, Bedford Park, South Australia, Australia.
Merrilee NeedhamDepartment of Neurology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
Susan MathersDepartment of Neurology, Calvary Health Care Bethlehem, Melbourne, Victoria, Australia.
Pam McCombeDepartment of Neurology, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia.
Paul TalmanDeakin University, University Hospital Geelong, Geelong, Victoria, Australia.
Steve VucicBrain and Nerve Research Centre, The University of Sydney, Concord Hospital, Sydney, New South Wales, Australia.ORCID 0000-0002-8323-873X

Funding

National Health and Medical Research Council
6 · The paper itself

Abstract

background and purposeGiven the accepted multistep process of disease causation in amyotrophic lateral sclerosis (ALS), the present study was undertaken to determine the number of steps required for disease onset across each of the ALS phenotypes.

methodsClinical and demographic data were prospectively accumulated using the Australian Motor Neurone Disease Registry (2005-2016), and age-specific incidence rates were calculated. Poisson regression was utilized to assess the relationship between log age-specific incidence and log age of onset, with McFadden's R

resultsIn total, 2647 ALS patients were included, with mean disease-onset age being 62.2 ± 12.1 years. A linear relationship between log incidence and log age was established across ALS phenotypes, with variable slope estimates: bulbar 5.1 (95% confidence interval [CI] 4.6-5.6); cervical 2.7 (95% CI 2.3-3.0); lumbar 3.5 (95% CI 3.2-3.9); flail arm 4.7 (95% CI 3.9-5.5); flail leg 3.6 (95% CI 2.6-4.5); primary lateral sclerosis 2.7 (95% CI 1.8-3.7). Slope estimates were significantly higher in the bulbar compared to the cervical, lumbar and primary lateral sclerosis phenotypes. McFadden's R DISCUSSION: A multistep process has been established across all ALS phenotypes with variable slope estimates, suggesting that the number of steps to develop disease is different across clinical presentations. Identification of mechanisms underlying slope estimate variability could exert pathophysiological significance.

Indexed as

Age of OnsetAmyotrophic Lateral SclerosisPhenotypeRegistriesAdultAgedAustraliaDisease ProgressionFemaleHumansIncidenceMaleMiddle Agedamyotrophic lateral sclerosisincidencemultistep process

Identifiers

PMID39475283
PMCPMC11622508

What Socratic holds

Textmetadata
LicenceCC BY
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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.