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Cognitive and motor outcomes in children with unilateral Sturge-Weber syndrome: Effect of age at seizure onset and side of brain involvement.
- Source :
-
Epilepsy & behavior : E&B [Epilepsy Behav] 2018 Mar; Vol. 80, pp. 202-207. Date of Electronic Publication: 2018 Feb 03. - Publication Year :
- 2018
-
Abstract
- Purpose: Most children with Sturge-Weber syndrome (SWS) develop seizures that may contribute to neurocognitive status. In this study, we tested the hypothesis that very early seizure onset has a particularly detrimental effect on the cognitive and/or motor outcomes of children with unilateral SWS. We also tested whether side of SWS brain involvement modulates the effect of seizure variables on the pattern of cognitive abnormalities.<br />Methods: Thirty-four children (22 girls; mean age 6.1years) with unilateral SWS and history of epilepsy in a longitudinal cohort underwent neurological and cognitive evaluations. Global intelligent quotient (GIQ), verbal intelligent quotient (VIQ), nonverbal intelligent quotient (IQ), and motor function were correlated with epilepsy variables, side and extent of brain involvement on magnetic resonance imaging (MRI).<br />Results: Mean age at seizure onset was 1.3years (0.1-6years) and mean IQ at follow-up was 86 (45-118). Age at seizure onset showed a logarithmic association with IQ, with maximum impact of seizures starting before age 1year, both in uni- and multivariate regression analyses. In the left SWS group (N=20), age at seizure onset was a strong predictor of nonverbal IQ (p=0.001); while early seizure onset in the right-hemispheric group had a more global effect on cognitive functions (p=0.02). High seizure frequency and long epilepsy duration also contributed to poor outcome IQ independently in multivariate correlations. Children with motor involvement started to have seizures at/before 7months of age, while frontal lobe involvement was the strongest predictor of motor deficit in a multivariate analysis (p=0.017).<br />Conclusion: These findings suggest that seizure onset prior to age 1year has a profound effect on severity of cognitive and motor dysfunction in children with SWS; however, the effect of seizures on the type of cognitive deficit is influenced by laterality of brain involvement.<br /> (Copyright © 2018 Elsevier Inc. All rights reserved.)
- Subjects :
- Brain physiopathology
Child
Child, Preschool
Cognition Disorders complications
Epilepsy etiology
Epilepsy physiopathology
Female
Frontal Lobe physiopathology
Functional Laterality
Humans
Infant
Intelligence Tests
Longitudinal Studies
Male
Multivariate Analysis
Prospective Studies
Seizures complications
Seizures etiology
Sturge-Weber Syndrome complications
Sturge-Weber Syndrome physiopathology
Age of Onset
Brain diagnostic imaging
Cognition
Intelligence
Magnetic Resonance Imaging methods
Sturge-Weber Syndrome diagnosis
Sturge-Weber Syndrome psychology
Subjects
Details
- Language :
- English
- ISSN :
- 1525-5069
- Volume :
- 80
- Database :
- MEDLINE
- Journal :
- Epilepsy & behavior : E&B
- Publication Type :
- Academic Journal
- Accession number :
- 29414553
- Full Text :
- https://doi.org/10.1016/j.yebeh.2018.01.012