Evidence map›Paper›PMID 27261500›Full record

ArticleJournal of neurology, neurosurgery, and psychiatry2017

Defining SOD1 ALS natural history to guide therapeutic clinical trial design.

Taha Bali, Wade Self, Jingxia Liu, Teepu Siddique, Leo H Wang, Thomas D Bird, Elena Ratti, Nazem Atassi, Kevin B Boylan, Jonathan D Glass and 17 more

Registry-linked trialAbstract read
In one paragraph

Article in Journal of neurology, neurosurgery, and psychiatry, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00821132 (Identification of Genes Causing Familial ALS or Increasing Risk for Sporadic ALS and ALS With Frontotemporal Dementia and Understanding Disease Mechanism.), which is not on this map. Cited by 56 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
56citing papers in PubMed, 3 pooled it
–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.

NCT00821132 completednot on this map

Identification of Genes Causing Familial ALS or Increasing Risk for Sporadic ALS and ALS With Frontotemporal Dementia and Understanding Disease Mechanism.

TypeobservationalSponsorNorthwestern UniversityRan1991 to 2023Enrolled13,521ConditionsAmyotrophic Lateral Sclerosis (ALS), Familial Amyotrophic Lateral Sclerosis, ALS With Frontotemporal Dementia (ALS/FTD), Lou Gehrig's DiseaseArmsGenetic study of ALS families
3 · Its place in the literature

Who cites it

56 citing papers in PubMed, 3 syntheses or guidelines pooled it.

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  18. Body mass index is lower in asymptomaticAmyotrophic lateral sclerosis & frontotemporal degeneration · 2024
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

27 authors.

Taha BaliDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.
Wade SelfDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.
Jingxia LiuDivision of Public Health Sciences, Washington University School of Medicine, St Louis, Missouri, USA.
Teepu SiddiqueDavee Department of Neurology and Clinical Neurosciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Leo H WangDepartment of Neurology at University of Washington Medical Center, University of Washington, Seattle, Washington, USA.
Thomas D BirdDepartment of Neurology at University of Washington Medical Center, University of Washington, Seattle, Washington, USA.
Elena RattiMassachusetts General Hospital, Neurology Clinical Research Institute, Boston, Massachusetts, USA.ORCID http://orcid.org/0000-0002-9321-7908
Nazem AtassiMassachusetts General Hospital, Neurology Clinical Research Institute, Boston, Massachusetts, USA.
Kevin B BoylanDepartment of Neurology, Mayo Clinic Florida, Jacksonville, Florida, USA.
Jonathan D GlassDepartment of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA.
Nicholas J MaragakisDepartment of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
James B CaressDepartment of Neurology, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.
Leo F McCluskeyDepartment of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Stanley H AppelDepartment of Neurology, Methodist Neurological Institute, The Methodist Hospital, Houston, Texas, USA.
James P WymerThe Neurosciences Institute, Albany Medical Center, Albany, New York, USA.
Summer GibsonDepartment of Neurology, University of Utah, Salt Lake City, Utah, USA.
Lorne ZinmanSunnybrook Health Sciences Centre, Toronto, Ontario, Canada.
Tahseen MozaffarDepartment of Neurology, University of California, Irvine, California, USA.
Brian CallaghanDepartment of Neurology, University of Michigan, Ann Arbor, Michigan, USA.
April L McVeyDepartment of Neurology, The University of Kansas Medical Center, Kansas City, Kansas, USA.
Jennifer Jockel-BalsarottiDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.
Peggy AllredDepartment of Neurology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
Elena R FisherDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.
Glenn LopateDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.
Alan PestronkDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.
Merit E CudkowiczMassachusetts General Hospital, Neurology Clinical Research Institute, Boston, Massachusetts, USA.
Timothy M MillerDepartment of Neurology, Washington University School of Medicine, St Louis, Missouri, USA.

Funding

University of Utah Center for Clinical and Translational ScienceUL1TR001067 · NCATS · UNIVERSITY OF UTAH · PI DERE, WILLARD H., HESS, RACHEL · 2013 to 2017
$19.8M
Neuroscience Resident Research ProgramR25NS065743 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI CASH, SYDNEY S, WILLIAMS, ZIV · 2009 to 2023
$11.8M
Using motor neuron specific miRNA to understand and develop novel therapeutics for motor neuron diseaseR01NS078398 · NINDS · WASHINGTON UNIVERSITY · PI MILLER, TIMOTHY M. · 2012 to 2021
$3.9M
Washington University Institute of Clinical and Translational SciencesTL1TR000449 · NCATS · WASHINGTON UNIVERSITY · PI EVANOFF, BRADLEY A · 2012 to 2016
$2.5M
Development of an antisense oligonucleotide therapy for SOD1 Familial ALSU01NS084970 · NINDS · WASHINGTON UNIVERSITY · PI MILLER, TIMOTHY M. · 2014 to 2015
$877k
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 TR000449NCATS NIH HHS TL1 TR001066NCATS NIH HHS UL1 TR001067NINDS NIH HHS R01 NS078398NINDS NIH HHS R25 NS065743NINDS NIH HHS U01 NS084970
6 · The paper itself

Abstract

importanceUnderstanding the natural history of familial amyotrophic lateral sclerosis (ALS) caused by SOD1 mutations (ALS

objectiveTo establish an updated natural history of ALS DESIGN, SETTING AND

participantsRetrospective cohort study from 15 medical centres in North America evaluated records from 175 patients with ALS with genetically confirmed SOD1 mutations, cared for after the year 2000. MAIN OUTCOMES AND MEASURES: Age of onset, survival, ALS Functional Rating Scale (ALS-FRS) scores and respiratory function were analysed. Patients with the A4V (Ala-Val) SOD1 mutation (SOD1

resultsMean age of disease onset was 49.7±12.3 years (mean±SD) for all SOD1 patients, with no statistical significance between SOD1 CONCLUSIONS AND RELEVANCE: SOD1

Indexed as

Clinical Trials as TopicResearch DesignAdultAge of OnsetAmyotrophic Lateral SclerosisDisease ProgressionHumansMiddle AgedMutationRetrospective StudiesSuperoxide DismutaseVital CapacitySuperoxide Dismutase

Identifiers

PMID27261500
PMCPMC5136332

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Registered trials

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.