Evidence map›Paper›PMID 38822840›Full record

ArticleJournal of neurology2024

SARA captures disparate progression and responsiveness in spinocerebellar ataxias.

Emilien Petit, Tanja Schmitz-Hübsch, Giulia Coarelli, Heike Jacobi, Anna Heinzmann, Karla P Figueroa, Susan L Perlman, Christopher M Gomez, George R Wilmot, Jeremy D Schmahmann and 16 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed, 1 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.

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

14 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
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  7. Review
  8. Spastic Ataxia Composite (SPAXCOM): A Scale to Evaluate the Progression of Subjects with Spasticity and Ataxia.Movement disorders : official journal of the Movement Disorder Society · 2025
    Article
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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

26 authors.

Emilien PetitSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France. emilien.petit@icm-institute.org.ORCID http://orcid.org/0009-0000-0602-7122
Tanja Schmitz-HübschExperimental and Clinical Research Center, A Cooperation of Max-Delbrueck Center of Molecular Medicine and Charité - Universitätsmedizin Berlin, Berlin, Germany.
Giulia CoarelliSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France.
Heike JacobiDepartment of Neurology, University Hospital Heidelberg, Heidelberg, Germany.
Anna HeinzmannSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France.
Karla P FigueroaDepartment of Neurology, University of Utah, Salt Lake City, UT, 84132, USA.
Susan L PerlmanDepartment of Neurology, University of California, Los Angeles, Los Angeles, CA, USA.
Christopher M GomezDepartment of Neurology, University of Chicago, Chicago, IL, USA.
George R WilmotDepartment of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
Jeremy D SchmahmannMassachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
Sarah H YingDepartment of Neurology, Johns Hopkins University, Baltimore, MD, USA.
Theresa A ZesiewiczDepartment of Neurology, University of South Florida, Tampa, FL, USA.
Henry L PaulsonDepartment of Neurology, University of Michigan, Ann Arbor, MI, USA.
Vikram G ShakkottaiDepartment of Neurology, University of Michigan, Ann Arbor, MI, USA.
Khalaf O BusharaDepartment of Neurology, University of Minnesota, Minneapolis, MN, USA.
Sheng-Han KuoDepartment of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
Michael D GeschwindDepartment of Neurology, University of California, San Francisco, CA, USA.
Guangbin XiaDepartment of Neurology and McKnight Brain Institute, University of Florida, Gainesville, FL, USA.
Stefan M PulstDepartment of Neurology, University of Utah, Salt Lake City, UT, 84132, USA.
S H SubramonyDepartment of Neurology and McKnight Brain Institute, University of Florida, Gainesville, FL, USA.
Claire EwenczykSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France.
Alexis BriceSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France.
Alexandra DurrSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France.
Thomas KlockgetherGerman Center for Neurodegenerative Diseases (DZNE), Bonn, Germany.
Tetsuo AshizawaWeill Cornell Medicine at Houston Methodist Hospital, Houston, TX, USA.
Sophie Tezenas du MontcelSorbonne Université, Paris Brain Institute - ICM, Inserm, CNRS, Inria, AP-HP, Paris, France.

Funding

Supplementary funding for U01NS104326 Clinical Trial Readiness for SCA1 and SCA3 (“READISCA”)U01NS104326 · NINDS · METHODIST HOSPITAL RESEARCH INSTITUTE · PI ASHIZAWA, TETSUO, DURR, ALEXANDRA · 2018 to 2022
$6.1M
Clinical Research Consortium for Spinocerebellar AtaxiasRC1NS068897 · NINDS · UNIVERSITY OF FLORIDA · PI ASHIZAWA, TETSUO · 2009 to 2010
$1.0M
NINDS NIH HHS RC1 NS068897NINDS NIH HHS U01 NS104326
6 · The paper itself

Abstract

backgroundThe Scale for Assessment and Rating of Ataxia (SARA) is a widely used clinical scale to assess cerebellar ataxia but faces some criticisms about the relevancy of all its items.

objectivesTo prepare for future clinical trials, we analyzed the progression of SARA and its items in several polyQ spinocerebellar ataxias (SCA) from various cohorts.

methodsWe included data from patients with SCA1, SCA2, SCA3, and SCA6 from four cohorts (EUROSCA, RISCA, CRC-SCA, and SPATAX) for a total of 850 carriers and 3431 observations. Longitudinal progression of the SARA and its items was measured. Cohort, stage and genetic effects were tested. We looked at the respective contribution of each item to the total scale. Sensitivity to change of the scale and the impact of item removal was evaluated by calculating sample sizes needed in various scenarios.

resultsLongitudinal progression was significantly different between cohorts in SCA1, SCA2 and SCA3, the EUROSCA cohort having the fastest progression. Advanced-stage patients were progressing slower in SCA2 and SCA6. Items were not contributing equally to the full scale through ataxia severity: gait, stance, hand movement, and heel-shin contributed the most in the early stage, and finger-chase, nose-finger, and sitting in later stages. Few items drove the sensitivity to the change of SARA, but changes in the scale structure could not improve its sensitivity in all populations.

conclusionSARA and its item's progression pace showed high heterogeneity across cohorts and SCAs. However, no combinations of items improved the responsiveness in all SCAs or populations taken separately.

Indexed as

Disease ProgressionSeverity of Illness IndexSpinocerebellar AtaxiasAdultAgedCohort StudiesFemaleHumansLongitudinal StudiesMaleMiddle AgedClinical scoreNatural historySpinocerebellar ataxia

Identifiers

PMID38822840
PMCPMC11571887

What OpenQuestion holds

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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 OpenQuestion graph.