Evidence map›Paper›PMID 24306004›Full record

ArticleNeurology2014

Familial clustering of ALS in a population-based resource.

Summer B Gibson, Karla P Figueroa, Mark B Bromberg, Stefan-M Pulst, Lisa Cannon-Albright

Open access · bronzeAbstract read
In one paragraph

Article in Neurology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
23citing papers in PubMed, 1 pooled it
2.9field-weighted citation impact, top 9% of its field
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

23 citing papers in PubMed, 1 synthesis or guideline pooled it, 43 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Review
  4. Review
  5. Article
  6. Review
  7. Article
  8. Evidence-based consensus guidelines for ALS genetic testing and counseling.Annals of clinical and translational neurology · 2023
    Article
  9. Article
  10. Histone Methylation Regulation in Neurodegenerative Disorders.International journal of molecular sciences · 2021
    Review
  11. The impact of histone post-translational modifications in neurodegenerative diseases.Biochimica et biophysica acta. Molecular basis of disease · 2019
    Review
  12. Review
  13. Article
  14. Article
  15. Article
  16. Article
  17. Article
  18. ALS and FTD: an epigenetic perspective.Acta neuropathologica · 2016
    Review
  19. Article
  20. Observational
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

5 authors at 1 institution in 1 country.

Summer B GibsonFrom the Department of Neurology (S.B.G., K.P.F., M.B.B., S.-M.P.) and Department of Medicine, Division of Genetic Epidemiology (L.C.-A.), University of Utah School of Medicine; and George E. Wahlen Department of Veterans Affairs Medical Center (L.C.-A.), Salt Lake City, UT.
Karla P Figueroa
Mark B Bromberg
Stefan-M Pulst
Lisa Cannon-Albright
George E. Wahlen Department of VA Medical Center · US

Funding

UTAH REGIONAL CANCER CENTERP30CA042014 · NCI · UTAH STATE HIGHER EDUCATION SYSTEM--UNIVERSITY OF UTAH · PI Jared P Rutter · 1986 to 2026
$72.6M
University of Utah Center for Clinical and Translational ScienceUL1TR001067 · NCATS · UNIVERSITY OF UTAH · PI DERE, WILLARD H., HESS, RACHEL · 2013 to 2017
$19.8M
SPINOCEREBELLAR ATAXIA TYPE 2--GENE AND GENE PRODUCTR01NS033123 · NINDS · UNIVERSITY OF UTAH · PI PULST, STEFAN M. · 1996 to 2012
$2.9M
Analysis of the Familial Component to Disease in a Biomedical Resource w/Link...R01LM009331 · NLM · UNIVERSITY OF UTAH · PI ALBRIGHT, LISA CANNON · 2007 to 2010
$1.6M
University of Utah Center for clinical and Translational ScienceTL1TR001066 · NCATS · UNIVERSITY OF UTAH · PI DERE, WILLARD H., HESS, RACHEL · 2013 to 2017
$558k
NCATS NIH HHS TL1 TR001066NCATS NIH HHS UL1 TR001067NCI NIH HHS P30 CA042014NCI NIH HHS P30 CA42014NINDS NIH HHS R01 NS033123NLM NIH HHS R01 LM009331
6 · The paper itself

Abstract

objectiveTo determine the extent of an inherited contribution to amyotrophic lateral sclerosis (ALS) mortality.

methodsDeath certificates (DCs) from 1904 to 2009 were analyzed from patients with at least 3 generations recorded in the Utah Population Database, a genealogic and medical database of more than 2 million Utah residents. Among probands whose DCs listed ALS, the relative risk (RR) of death with ALS was determined among spouses and first- through fifth-degree relatives, using birth year-, sex-, and birthplace-matched cohorts.

resultsEight hundred seventy-three patients with ALS met the inclusion criteria. Among 3,531 deceased first-degree relatives of probands, the RR of dying with ALS was increased compared with control cohorts (RR = 4.91, 95% confidence interval 3.36, 6.94). The RR of dying with ALS was also increased among 9,386 deceased second-degree relatives (RR = 2.85, 95% confidence interval 2.06, 3.84). The RR of dying with ALS was not increased among third- through fifth-degree relatives. More affected first-degree relatives were male (p = 0.014). No cases of conjugal ALS were observed.

conclusionsThis study is suggestive of familial clustering in excess of expected for ALS. Our results confirm the results of prior studies of familial ALS, suggesting applicability of our findings to other mixed European populations. Furthermore, this work expands on previous studies by quantifying the RR of ALS among more distant relatives. The use of mortality data obtained from DCs reduces the ascertainment and recall bias of many previous studies. Finally, the excess of ALS among second-degree relatives and lack of conjugal ALS are strongly supportive of a genetic contribution.

Indexed as

AgedAmyotrophic Lateral SclerosisCluster AnalysisCohort StudiesDatabases, FactualFemaleHumansMaleMiddle AgedPopulation SurveillanceUtah

Identifiers

PMID24306004
PMCPMC3873619
OpenAlexW2155418687

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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 OpenQuestion graph.