Evidence map›Paper›PMID 40775359›Full record

ArticleOrphanet journal of rare diseases2025

The Wechsler intelligence scale for children, fourth and fifth editions perform comparably in children with Batten disease.

Heather R Adams, Erika F Augustine, Kristen Bonifacio, Alyssa Collins, Amy E Vierhile, Jonathan W Mink

Abstract read
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In one paragraph

Article in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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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

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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

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

6 authors.

Heather R AdamsDepartment of Neurology, Division of Child Neurology, University of Rochester Medical Center, 601 Elmwood Avenue Box 631, Rochester, NY, 14642, USA. heather_adams@urmc.rochester.edu.ORCID 0000-0002-5992-4297
Erika F AugustineDepartment of Neurology, Division of Child Neurology, University of Rochester Medical Center, 601 Elmwood Avenue Box 631, Rochester, NY, 14642, USA.
Kristen BonifacioNew York State Department of Health, AIDS Institute, Albany, NY, 12220, USA.
Alyssa CollinsChild Life Program, Golisano Children's Hospital, University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY, 14642, USA.
Amy E VierhileDepartment of Neurology, Division of Child Neurology, University of Rochester Medical Center, 601 Elmwood Avenue Box 631, Rochester, NY, 14642, USA.
Jonathan W MinkConsultant, Pittsford, NY, 14534, USA.

Funding

University of Rochester Intellectual and Developmental Disabilities Research CenterP50HD103536 · NICHD · UNIVERSITY OF ROCHESTER · PI JOHN J FOXE, Anna K Majewska · 2020 to 2026
$9.8M
Meaningful Outcomes and Multi-Site Readiness for Clinical Trials in Juvenile Neuronal Ceroid LipofuscinosisU01NS101946 · NINDS · UNIVERSITY OF ROCHESTER · PI AUGUSTINE, ERIKA · 2019 to 2024
$3.7M
Clinical and Neuropsychological Investigations in Batten DiseaseR01NS060022 · NINDS · UNIVERSITY OF ROCHESTER · PI MINK, JONATHAN W. · 2007 to 2010
$1.2M
Abeona Therapeutics n/aBatten Disease Support and Research Association Batten Disease Support and Research AssociationBeyond Batten Disease Foundation Beyond Batten Disease FoundationNICHD NIH HHS P50 HD103536NIH HHS P50HD103536NIH HHS R01NS060022NIH HHS U01NS101946NINDS NIH HHS R01 NS060022NINDS NIH HHS U01 NS101946Noah's Hope-Hope 4 Bridget Noah's Hope-Hope 4 BridgetOur Promise to Nicholas Our Promise to NicholasUniversity of Rochester Child Neurology Research Fund University of Rochester Child Neurology Research Fund
6 · The paper itself

Abstract

backgroundThe neuronal ceroid lipofuscinoses (Batten disease) are rare neurodegenerative lysosomal storage diseases principally of childhood onset and an autosomal recessive inheritance pattern. Cognitive regression is a hallmark of the disease, and has been characterized as part of the University of Rochester Batten Center's prospective longitudinal natural history. The objective of the present study was to establish convergent validity of the two most recent versions of the Wechsler Intelligence Scale for Children in this population (WISC-IV, 2003; WISC-V, 2014) due to anticipated eventual obsolescence of WISC-IV. 18 children and young adults (12 males, 6 females) with a genetically confirmed NCL diagnosis were administered selected subtests from the WISC-IV and WISC-V. We used bivariate correlations and repeated measures ANOVA between matching subtests across these two WISC versions to determine convergence of the measures.

resultsWISC-IV and WISC-V verbal subtests were strongly correlated with one another and mean age-adjusted scores for comparable subtests on WISC-IV vs. WISC-V were not significantly different from one another.

conclusionsOverall, the minimal performance differences on the two measures supports combining WISC-IV and WISC-V datasets for larger-scale analyses of the neurocognitive natural history of NCL disorders.

Indexed as

Neuronal Ceroid-LipofuscinosesWechsler ScalesAdolescentChildChild, PreschoolFemaleHumansMaleYoung AdultBatten diseaseCognitionConvergent validityNeuronal ceroid lipofuscinosesWechsler scales

Identifiers

PMID40775359
PMCPMC12333175

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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.