Evidence map›Paper›PMID 38165578›Full record

ArticleCerebellum (London, England)2024

The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale in Spinocerebellar Ataxias.

Louisa P Selvadurai, Susan L Perlman, Tetsuo Ashizawa, George R Wilmot, Chiadi U Onyike, Liana S Rosenthal, Vikram G Shakkottai, Henry L Paulson, Sub H Subramony, Khalaf O Bushara and 15 more

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PubMed Full text at PMC Publisher
In one paragraph

Article in Cerebellum (London, England), 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed, 2 pooled it
7.5field-weighted citation impact, top 2% 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

25 citing papers in PubMed, 2 syntheses or guidelines pooled it, 28 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

25 authors at 20 institutions in 1 country.

Louisa P SelvaduraiDepartment of Neurology, Ataxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Massachusetts General Hospital and Harvard Medical School, 100 Cambridge Street, Suite 2000, Boston, MA, 02114, USA.
Susan L PerlmanDepartment of Neurology, University of California, Los Angeles, Los Angeles, CA, USA.
Tetsuo AshizawaDepartment of Neurology, Houston Methodist Research Institute, Houston, TX, USA.
George R WilmotDepartment of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
Chiadi U OnyikeDepartment of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Liana S RosenthalDepartment of Neurology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Vikram G ShakkottaiDepartment of Neurology, University of Michigan, Ann Arbor, MI, USA.
Henry L PaulsonDepartment of Neurology, University of Michigan, Ann Arbor, MI, USA.
Sub H SubramonyDepartment of Neurology, McKnight Brain Institute, University of Florida College of Medicine, Gainesville, FL, USA.
Khalaf O BusharaDepartment of Neurology, University of Minnesota, Minneapolis, MN, USA.
Sheng-Han KuoDepartment of Neurology, Columbia University, New York, NY, 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.
Christopher M GomezDepartment of Neurology, University of Chicago, Chicago, IL, USA.
Puneet OpalDepartment of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Theresa A ZesiewiczDepartment of Neurology, University of South Florida Ataxia Research Center, Tampa, FL, USA.
Trevor HawkinsDepartment of Neurology, University of Colorado Denver, Anschutz Medical Campus, Aurora, CO, USA.
Talene A YacoubianDepartment of Neurology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Peggy C NopoulosDepartment of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Sharon J ShaDepartment of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA, USA.
Peter E MorrisonDepartment of Neurology, University of Rochester Medical Center, Rochester, NY, 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.
Jeremy D SchmahmannDepartment of Neurology, Ataxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Massachusetts General Hospital and Harvard Medical School, 100 Cambridge Street, Suite 2000, Boston, MA, 02114, USA. jschmahmann@mgh.harvard.edu.
University of California, San Francisco · USUniversity of Michigan–Ann Arbor · USUniversity of Utah · USColumbia University · USEmory University · USHarvard University · USHouston Methodist · USJohns Hopkins Medicine · USJohns Hopkins University · USNorthwestern University · USStanford University · USUniversity of Alabama at Birmingham · USUniversity of California, Los Angeles · USUniversity of Chicago · USUniversity of Colorado Anschutz Medical Campus · USUniversity of Florida · USUniversity of Iowa · USUniversity of Minnesota · USUniversity of Rochester Medical Center · USUniversity of South Florida · US

Funding

Understanding the Cellular Basis of Movement DisordersR01NS082351 · NINDS · NORTHWESTERN UNIVERSITY AT CHICAGO · PI Puneet Opal · 2013 to 2026
$6.2M
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
Predicting progression of human prion diseaseR01AG031189 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GESCHWIND, MICHAEL D · 2008 to 2017
$5.7M
Tracking longitudinal change in presymptomatic genetic prion disease (TLC-Pre-gPrD)R01AG062562 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GESCHWIND, MICHAEL D · 2019 to 2023
$5.4M
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
TLC-Pre-gPrD: Tracking Longitudinal Change in Presymptomatic genetic Prion DiseaseR56AG055619 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GESCHWIND, MICHAEL D · 2018 to 2018
$800k
NIA NIH HHS R01 AG031189NIA NIH HHS R01 AG062562NIA NIH HHS R56 AG055619NINDS NIH HHS R01 NS082351NINDS NIH HHS R01 NS104423NINDS NIH HHS R01 NS118179NINDS NIH HHS R01 NS124854NINDS NIH HHS U01 NS104326
6 · The paper itself

Abstract

The Cerebellar Cognitive Affective/Schmahmann Syndrome (CCAS) manifests as impaired executive control, linguistic processing, visual spatial function, and affect regulation. The CCAS has been described in the spinocerebellar ataxias (SCAs), but its prevalence is unknown. We analyzed results of the CCAS/Schmahmann Scale (CCAS-S), developed to detect and quantify CCAS, in two natural history studies of 309 individuals Symptomatic for SCA1, SCA2, SCA3, SCA6, SCA7, or SCA8, 26 individuals Pre-symptomatic for SCA1 or SCA3, and 37 Controls. We compared total raw scores, domain scores, and total fail scores between Symptomatic, Pre-symptomatic, and Control cohorts, and between SCA types. We calculated scale sensitivity and selectivity based on CCAS category designation among Symptomatic individuals and Controls, and correlated CCAS-S performance against age and education, and in Symptomatic patients, against genetic repeat length, onset age, disease duration, motor ataxia, depression, and fatigue. Definite CCAS was identified in 46% of the Symptomatic group. False positive rate among Controls was 5.4%. Symptomatic individuals had poorer global CCAS-S performance than Controls, accounting for age and education. The domains of semantic fluency, phonemic fluency, and category switching that tap executive function and linguistic processing consistently separated Symptomatic individuals from Controls. CCAS-S scores correlated most closely with motor ataxia. Controls were similar to Pre-symptomatic individuals whose nearness to symptom onset was unknown. The use of the CCAS-S identifies a high CCAS prevalence in a large cohort of SCA patients, underscoring the utility of the scale and the notion that the CCAS is the third cornerstone of clinical ataxiology.

Indexed as

Spinocerebellar AtaxiasAdultAgedCognition DisordersCohort StudiesExecutive FunctionFemaleHumansMaleMiddle AgedNeuropsychological TestsCerebellar cognitive affective syndromeCognitionScaleSpinocerebellar ataxia

Identifiers

PMID38165578
PMCPMC11217149
OpenAlexW4390501946

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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.