Evidence map›Paper›PMID 40834346›Full record

ArticleNeurology2025

Prevalence, Severity, and Progression of Cerebellar Cognitive-Affective Syndrome in Patients With Spinocerebellar Ataxias.

Emilien Petit, Daniel López Domínguez, Cecilia Marelli, Sabrina Sayah, Stefan M Pulst, Jennifer Faber, Gulin Oz, Henry L Paulson, Tetsuo Ashizawa, READISCA Consortium and 3 more

Abstract read
In one paragraph

Article in Neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
–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

6 citing papers in PubMed.

  1. Article
  2. Article
  3. Observational
  4. Review
  5. Review
  6. Article
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

13 authors.

Emilien PetitSorbonne Université, Institut du Cerveau - Paris Brain Institute-ICM, Inserm, CNRS, APHP, Hôpital de la Pitié Salpêtrière, France.ORCID 0009-0000-0602-7122
Daniel López DomínguezSorbonne Université, Institut du Cerveau - Paris Brain Institute-ICM, Inserm, CNRS, APHP, Hôpital de la Pitié Salpêtrière, France.ORCID 0000-0002-4249-821X
Cecilia MarelliMontpellier University, EPHE, INSERM, France.
Sabrina SayahSorbonne Université, Institut du Cerveau - Paris Brain Institute-ICM, Inserm, CNRS, APHP, Hôpital de la Pitié Salpêtrière, France.ORCID 0000-0002-0382-9995
Stefan M PulstDepartment of Neurology, University of Utah, Salt Lake City.ORCID 0000-0003-0883-7879
Jennifer FaberGerman Center for Neurodegenerative Diseases (DZNE), Bonn, Germany.ORCID 0000-0003-3265-0262
Gulin OzCenter for Magnetic Resonance Research, Department of Radiology, University of Minnesota, Minneapolis.ORCID 0000-0002-5769-183X
Henry L PaulsonDepartment of Neurology, University of Michigan, Ann Arbor; and.ORCID 0000-0002-0382-7535
Tetsuo AshizawaWeill Cornell Medicine at Houston Methodist Hospital, TX.
READISCA Consortium
Sophie Tezenas du MontcelSorbonne Université, Institut du Cerveau - Paris Brain Institute-ICM, Inserm, CNRS, APHP, Hôpital de la Pitié Salpêtrière, France.ORCID 0000-0002-2866-4330
Alexandra DurrSorbonne Université, Institut du Cerveau - Paris Brain Institute-ICM, Inserm, CNRS, APHP, Hôpital de la Pitié Salpêtrière, France.ORCID 0000-0002-8921-7104
Giulia CoarelliSorbonne Université, Institut du Cerveau - Paris Brain Institute-ICM, Inserm, CNRS, APHP, Hôpital de la Pitié Salpêtrière, France.ORCID 0000-0002-7824-8343

Funding

Ataxin-2 complex proteins in neurodegeneration.R35NS127253 · NINDS · UTAH STATE HIGHER EDUCATION SYSTEM--UNIVERSITY OF UTAH · PI Stefan M. PULST · 2022 to 2026
$4.6M
NINDS NIH HHS R35 NS127253
6 · The paper itself

Abstract

BACKGROUND AND

objectivesCerebellar cognitive-affective syndrome (CCAS) results from cerebellar degeneration, but its prevalence in spinocerebellar ataxias (SCAs) remains underexplored. This study assessed CCAS prevalence, severity, and progression across different SCAs.

methodsWe included polyglutamine (PolyQ) SCA expansion carriers (

resultsWe included 371 participants: 66 with SCA1, 28 with SCA2, 158 with SCA3, 24 with SCA7, 35 with SPG7, 17 with SCA27B, and 43 controls. Those with SCA27B and SPG7 were older (69.5 ± 9.5 and 57.8 ± 10.6 years) with lower education (11.4 ± 4.2 and 12.7 ± 3.6 years) than those with PolyQ SCAs (from 40.3 ± 14.0 for SCA7 group to 45.9 ± 11.2 years in SCA3 group, DISCUSSION: We observed early cognitive impairment in PolyQ SCA carriers, correlating with clinical measures, NfL levels, and cerebellum volume. Improvement over 3 years likely reflects a practice effect, potentially limiting the scale's longitudinal utility.

Indexed as

Cerebellar DiseasesSpinocerebellar AtaxiasAdultAgedDisease ProgressionFemaleHumansMagnetic Resonance ImagingMaleMiddle AgedNeurofilament ProteinsPeptidesPrevalenceSeverity of Illness Indexneurofilament protein LNeurofilament ProteinsPeptides

Identifiers

PMID40834346
PMCPMC12367419

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.