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

To report on prevalence, associated impairments, severity and neuroimaging findings in children with ataxic cerebral palsy (CP). In children coded as having ataxic CP in the Central database of JRC-SCPE (Joint Research Center-Surveillance of Cerebral Palsy in Europe) and born 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. 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. 70% of children with ataxic CP were able to walk, 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% and miscellaneous findings in 23.5% and brain injuries in 19%, according to the SCPE classification. Genetic syndromes were described in 9%. This register-based multicentre 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 with respect to cognitive than gross motor dysfunction. They are mostly term born and the origin rarely suggests acquired injuries. In addition to neuroimaging, a comprehensive genetic work-up is particularly recommended for children with this CP type.

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