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Background <p>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.</p> Results <p>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.</p> Conclusions <p>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.</p>

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

  • 期刊论文

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  • 发表日期：

    2025年8月7日

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

摘要

Background

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.

Results

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.

Conclusions

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.