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

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

PMC · DOI: 10.1186/s13023-025-03923-w · Orphanet Journal of Rare Diseases · 2025-08-07

## TL;DR

The study found that two versions of a children's intelligence test perform similarly in kids with Batten disease, allowing data from both to be combined.

## Contribution

The study establishes convergent validity between WISC-IV and WISC-V in children with Batten disease.

## Key findings

- WISC-IV and WISC-V verbal subtests were strongly correlated.
- Mean age-adjusted scores for comparable subtests were not significantly different.
- Combining datasets from both versions is supported for larger-scale analyses.

## Abstract

The 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.

WISC-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.

Overall, 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.

## Linked entities

- **Diseases:** Batten disease (MONDO:0019262)

## Full-text entities

- **Diseases:** Batten disease (MESH:D009472), neurodegenerative lysosomal storage diseases (MESH:D016464), Cognitive regression (MESH:D003072), NCL disorders (MESH:D009358)

## Full text

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## Figures

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## References

1 references — full list in the complete paper: https://tomesphere.com/paper/PMC12333175/full.md

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Source: https://tomesphere.com/paper/PMC12333175