title: Predictors of Cognitive Functions in Children With Sturge-Weber Syndrome: A Longitudinal Study.
abstract: Sturge-Weber syndrome is often accompanied by seizures and neurocognitive deterioration, although previous studies have suggested that early functional brain reorganization may diminish the cognitive sequelae in some children with unilateral Sturge-Weber syndrome. The "rules" governing these plasticity mechanisms are poorly understood. In this study, we evaluated longitudinal changes of cognitive functioning (intelligence quotient [IQ]) and assessed the performance of clinical, electroencephalography (EEG), and magnetic resonance imaging (MRI) variables for predicting IQ in children with Sturge-Weber syndrome.
  
  
  
  Thirty-three young children (mean age: 3.3 years at baseline) with unilateral Sturge-Weber syndrome underwent MRI, scalp EEG, and neuropsychology evaluation twice, with a median follow-up of 2 years. None of the children had epilepsy surgery. Longitudinal IQ changes were calculated. Seizure variables, interictal EEG abnormalities, and extent and location of MRI brain involvement were correlated with IQ assessed at follow-up.
  
  
  
  Global IQ showed a highly variable course with both increases and decreases over time. Lower IQ at baseline was associated with interval IQ increase. In univariate analyses, lower outcome IQ was associated with baseline EEG abnormalities (P < 0.001), young age at seizure onset (P = 0.001), high seizure frequency (P = 0.02), and early frontal-lobe involvement on MRI (P = 0.01). In multivariate analysis, EEG abnormalities at baseline remained a robust, independent predictor of outcome IQ.
  
  
  
  The early trajectory of cognitive changes in children with unilateral Sturge-Weber syndrome is highly variable; children with improving IQ likely undergo effective unimpeded functional reorganization. Early onset, frequent seizures, and interictal epileptiform abnormalities on EEG likely interfere with this process resulting in poor cognitive functions. Future studies assessing interventions should target this high-risk subgroup to optimize cognitive outcome in Sturge-Weber syndrome.
authors.affiliation: Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan.
authors.forename: Edit
authors.initials: E
authors.lastname: Bosnyák
authors.affiliation: Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Behen
authors.affiliation: Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan.
authors.forename: William C
authors.initials: WC
authors.lastname: Guy
authors.affiliation: Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan; Department of Neurology, Wayne State University, Detroit, Michigan.
authors.forename: Eishi
authors.initials: E
authors.lastname: Asano
authors.affiliation: Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan; Department of Neurology, Wayne State University, Detroit, Michigan; Division of Pediatric Neurology, Nemours A.I. duPont Hospital for Children, Wilmington, Delaware; Thomas Jefferson University School of Medicine, Philadelphia, Pennsylvania.
authors.forename: Harry T
authors.initials: HT
authors.lastname: Chugani
authors.affiliation: Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan; Department of Neurology, Wayne State University, Detroit, Michigan. Electronic address: juhasz@pet.wayne.edu.
authors.forename: Csaba
authors.initials: C
authors.lastname: Juhász
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003071:Cognition; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007360:Intelligence; D008137:Longitudinal Studies; D008297:Male; D011446:Prospective Studies; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000503:physiopathology / Q000523:psychology*
keywords: EEG; MRI; Sturge–Weber syndrome; cognitive functions; epilepsy; longitudinal study