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Multicenter Study
. 2023 Dec 12;101(24):e2509-e2521.
doi: 10.1212/WNL.0000000000207851. Epub 2023 Oct 19.

Prevalence, Clinical Features, Neuroimaging, and Genetic Findings in Children With Ataxic Cerebral Palsy in Europe

Veronka Horber  1 , Guro L Andersen  1 , Catherine Arnaud  1 , Javier De La Cruz  1 , Ivana Dakovic  1 , Andra Greitane  1 , Owen Hensey  1 , Kate Himmelmann  1 , Katalin Hollody  1 , Karen Horridge  1 , Christoph T Künzle  1 , Marco Marcelli  1 , Els Ortibus  1 , Antigone Papavasiliou  1 , Oliver Perra  1 , Mary J Platt  1 , Gija Rackauskaite  1 , Solveig Sigurdardottir  1 , Anja Troha Gergeli  1 , Daniel Virella  1 , Ingeborg Krägeloh-Mann  1 , Elodie Sellier  1
Affiliations
Multicenter Study

Prevalence, Clinical Features, Neuroimaging, and Genetic Findings in Children With Ataxic Cerebral Palsy in Europe

Veronka Horber et al. Neurology. .

Erratum in

  • Corrections to Received Date Information.
    [No authors listed] [No authors listed] Neurology. 2024 Jul 9;103(1):e209596. doi: 10.1212/WNL.0000000000209596. Epub 2024 Jun 3. Neurology. 2024. PMID: 38830175 Free PMC article. No abstract available.

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.

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Conflict of interest statement

V. Horber received funding for this project from the University of Tübingen (TÜFF Nr. 2622-0-0). G.L. Andersen, C. Arnaud, J. de la Cruz, I. Dakovic, A. Greitane, O. Hensey, K. Himmelman, K. Hollódy, K. Horridge, C.T. Künzle, M. Marcelli, E. Ortibus, A. Papavasiliou, O. Perra, M.J. Platt, G. Rackauskaite, S. Sigurdardottir, A. Troha Gergeli, D. Virella, I. Krägeloh-Mann, and E. Sellier report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

Figures

Figure 1
Figure 1. Time Trend for Ataxic CP Prevalence per 100,000 Live Births (p = 0.19) in the SCPE Common Database (Birth Years 1980–2010)
Figure 2
Figure 2. Intellectual Impairment by Severity of Motor Impairment in Ataxic CP (p < 0.001) in the SCPE Common Database (Birth Years 1980–2010)
Figure 3
Figure 3. Comparison of MRI Findings in Term-Born and Preterm-Born Children (p = 0.08) With Ataxic CP in the SCPE Common Database (Birth Years 1980 -2010)
See this image and copyright information in PMC

References

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