Prevalence, clinical features, neuroimaging, and genetic findings in children with ataxic cerebral palsy in Europe

Veronka Horber*, Guro L. Andersen, Catherine Arnaud, Javier De La Cruz, Ivana Dakovic, Andra Greitane, Owen Hensey, Kate Himmelmann, Katalin Hollody, Karen Horridge, Christoph T. Künzle, Marco Marcelli, Els Ortibus, Antigone Papavasiliou, Oliver Perra, Mary J. Platt, Gija Rackauskaite, Solveig Sigurdardottir, Anja Troha Gergeli, Daniel VirellaIngeborg Krägeloh-Mann, Elodie Sellier

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)

Abstract

Background and Objectives: To report on prevalence, associated impairments, severity, and neuroimaging findings in children with ataxic cerebral palsy (CP).Methods: In children coded as having ataxic CP in the Central database of Joint Research Center-Surveillance of Cerebral Palsy in Europe (JRC-SCPE) and born during 1980-2010, birth characteristics, severity profiles including associated impairments, neuroimaging patterns, and the presence of syndromes were analyzed. Definitions were according to validated SCPE guidelines. Prevalence over time was estimated using Poisson regression. Results: In total, 679 children with ataxic CP were identified in 20 European CP registers. The proportion with ataxic CP was 3.8% and varied from 0% to 12.9%. Prevalence over time showed no significant trend. Approximately 70% of children with ataxic CP were able to walk, and 40% had severe intellectual impairment and a high impairment index. Children with ataxic CP were mostly born at term (79%) and with normal birth weight (77%). Neuroimaging patterns revealed normal findings in 29%, brain maldevelopments in 28.5%, miscellaneous findings in 23.5%, and brain injuries in 19%, according to the SCPE classification. Genetic syndromes were described in 9%. Discussion: This register-based multicenter study on children with ataxic CP provides a large sample size for the analysis of prevalence, severity, and origin of this rare CP subtype. Even with strict inclusion and classification criteria, there is variation between registers on how to deal with this subtype, and diagnosis of ataxic CP remains a challenge. Ataxic cerebral palsy differs from other CP subtypes: children with ataxic CP have a disability profile that is more pronounced in terms of cognitive than gross motor dysfunction. They are mostly term born and the origin rarely suggests acquired injuries. In addition to neuroimaging, a comprehensive genetic workup is particularly recommended for children with this CP type.

Original languageEnglish
Pages (from-to)E2509-E2521
Number of pages13
JournalNeurology
Volume101
Issue number24
Early online date19 Oct 2023
DOIs
Publication statusPublished - 12 Dec 2023

ASJC Scopus subject areas

  • Clinical Neurology

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