Evidence map›Paper›PMID 35962273›Full record

ArticleCerebellum (London, England)2023

The S-Factor, a New Measure of Disease Severity in Spinocerebellar Ataxia: Findings and Implications.

Louisa P Selvadurai, Susan L Perlman, George R Wilmot, Sub H Subramony, Christopher M Gomez, Tetsuo Ashizawa, Henry L Paulson, Chiadi U Onyike, Liana S Rosenthal, Haris I Sair and 16 more

Registry-linked trialOpen access · greenAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Cerebellum (London, England), 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01060371 (Clinical Research Consortium for the Study of Cerebellar Ataxias), which is not on this map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
1.4field-weighted citation impact, top 21% 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.

NCT01060371 recruitingnot on this map

Clinical Research Consortium for the Study of Cerebellar Ataxias (CRC-SCA) for the Natural History Study of and Genetic Modifiers in Spinocerebellar Ataxias (SCA)

TypeobservationalSponsorLauren MooreRan2010 to 2030Enrolled1,400ConditionsSpinocerebellar Ataxia Type 1, Spinocerebellar Ataxia Type 2, Spinocerebellar Ataxia Type 3, Spinocerebellar Ataxia Type 6ArmsGenetic Testing, Blood Collection, Magnetic Resonance Imaging (MRI) Scan, Assessments and Questionnaires, Cerebrospinal Fluid Collection
3 · Its place in the literature

Who cites it

6 citing papers in PubMed, 15 citations in OpenAlex.

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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 at 18 institutions in 1 country.

Louisa P SelvaduraiAtaxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
Susan L PerlmanDepartment of Neurology, University of California, Los Angeles, Los Angeles, CA, USA.
George R WilmotDepartment of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
Sub H SubramonyDepartment of Neurology, University of Florida College of Medicine, McKnight Brain Institute, Gainesville, FL, USA.
Christopher M GomezDepartment of Neurology, University of Chicago, Chicago, IL, USA.
Tetsuo AshizawaDepartment of Neurology, Houston Methodist Research Institute, Houston, TX, USA.
Henry L PaulsonDepartment of Neurology, University of Michigan, Ann Arbor, MI, USA.
Chiadi U OnyikeDepartment of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Liana S RosenthalDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Haris I SairRussell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Sheng-Han KuoDepartment of Neurology, Columbia University, New York, NY, USA.
Eva-Maria RataiMartinos Center for Biomedical Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Theresa A ZesiewiczDepartment of Neurology, Ataxia Research Center, University of South Florida, Tampa, FL, USA.
Khalaf O BusharaDepartment of Neurology, University of Minnesota, Minneapolis, MN, USA.
Gülin ÖzCenter for Magnetic Resonance Research, Department of Radiology, University of Minnesota, Minneapolis, MN, USA.
Cameron DietikerDepartment of Neurology, University of California, San Francisco, CA, USA.
Michael D GeschwindDepartment of Neurology, University of California, San Francisco, CA, USA.
Alexandra B NelsonDepartment of Neurology, University of California, San Francisco, CA, USA.
Puneet OpalDepartment of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Talene A YacoubianDepartment of Neurology, University of Alabama at Birmingham, Birmingham, AL, USA.
Peggy C NopoulosDepartment of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Vikram G ShakkottaiDepartment of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Karla P FigueroaDepartment of Neurology, University of Utah, Salt Lake City, UT, USA.
Stefan M PulstDepartment of Neurology, University of Utah, Salt Lake City, UT, USA.
Peter E MorrisonDepartment of Neurology, University of Rochester Medical Center, Rochester, NY, USA.
Jeremy D SchmahmannAtaxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA. jschmahmann@mgh.harvard.edu.ORCID http://orcid.org/0000-0003-0706-5125
Harvard University · USJohns Hopkins University · USUniversity of California, San Francisco · USUniversity of Minnesota · USUniversity of Utah · USColumbia University · USEmory University · USHouston Methodist · USNorthwestern University · USThe University of Texas Southwestern Medical Center · USUniversity of Alabama at Birmingham · USUniversity of California, Los Angeles · USUniversity of Chicago · USUniversity of Florida · USUniversity of Iowa · USUniversity of Michigan · USUniversity of Rochester Medical Center · USUniversity of South Florida · US

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
Targeting Cerebellar Endoplasmic Reticulum Calcium Handling in Essential TremorR01NS124854 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI PHYLLIS L FAUST, Sheng-Han Kuo · 2022 to 2026
$2.9M
Targeting Cerebellar Excitatory Synapses for Tremor ProgressionR01NS118179 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI KUO, SHENG-HAN · 2020 to 2024
$2.2M
Cerebellar Circuitry in the Pathophysiology of TremorR01NS104423 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI KUO, SHENG-HAN · 2018 to 2022
$2.0M
NINDS NIH HHS R01 NS104423NINDS NIH HHS R01 NS118179NINDS NIH HHS R01 NS124854NINDS NIH HHS U01 NS104326
6 · The paper itself

Abstract

Spinocerebellar ataxias (SCAs) are progressive neurodegenerative disorders, but there is no metric that predicts disease severity over time. We hypothesized that by developing a new metric, the Severity Factor (S-Factor) using immutable disease parameters, it would be possible to capture disease severity independent of clinical rating scales. Extracting data from the CRC-SCA and READISCA natural history studies, we calculated the S-Factor for 438 participants with symptomatic SCA1, SCA2, SCA3, or SCA6, as follows: ((length of CAG repeat expansion - maximum normal repeat length) /maximum normal repeat length) × (current age - age at disease onset) × 10). Within each SCA type, the S-Factor at the first Scale for the Assessment and Rating of Ataxia (SARA) visit (baseline) was correlated against scores on SARA and other motor and cognitive assessments. In 281 participants with longitudinal data, the slope of the S-Factor over time was correlated against slopes of scores on SARA and other motor rating scales. At baseline, the S-Factor showed moderate-to-strong correlations with SARA and other motor rating scales at the group level, but not with cognitive performance. Longitudinally the S-Factor slope showed no consistent association with the slope of performance on motor scales. Approximately 30% of SARA slopes reflected a trend of non-progression in motor symptoms. The S-Factor is an observer-independent metric of disease burden in SCAs. It may be useful at the group level to compare cohorts at baseline in clinical studies. Derivation and examination of the S-factor highlighted challenges in the use of clinical rating scales in this population.

Indexed as

Spinocerebellar AtaxiasDisease ProgressionHumansPatient AcuityDisease progressionNatural historyScale for the Assessment and Rating of AtaxiaSpinocerebellar ataxia

Identifiers

PMID35962273
PMCPMC10363993
OpenAlexW4290888092

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

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.