title: Brain damage and IQ in unilateral Sturge-Weber syndrome: support for a "fresh start" hypothesis.
abstract: We tested the hypothesis that extent of severe hypometabolism measured by fluorodeoxyglucose PET has a U-shaped (nonlinear) relationship to IQ in children with unilateral Sturge-Weber syndrome. Thirty-five consecutive children (age range: 30-153 months) with Sturge-Weber syndrome and unilateral brain involvement were enrolled in the study. Participants underwent cognitive assessment and interictal fluorodeoxyglucose PET scans. Regression analyses tested whether a quadratic model best accounted for the relationship between extent of severe cortical hypometabolism and IQ, controlling for seizure variables. A significant quadratic relationship was found between IQ and extent of severe (but not total) hypometabolism. Seizure variables also contributed significant variance to cognitive functions. Results suggest that intermediate size of severe hemispheric hypometabolism is associated with the worst cognitive outcomes, and small or absent lesions, with the best cognitive outcomes. Children in whom a very large extent of the hemisphere is severely affected are likely to have relatively preserved cognitive function.
authors.affiliation: Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, MI 48201, USA. mbehen@pet.wayne.edu
authors.forename: Michael E
authors.initials: ME
authors.lastname: Behen
authors.forename: Csaba
authors.initials: C
authors.lastname: Juhász
authors.forename: Cortney
authors.initials: C
authors.lastname: Wolfe-Christensen
authors.forename: William
authors.initials: W
authors.lastname: Guy
authors.forename: Stacey
authors.initials: S
authors.lastname: Halverson
authors.forename: Robert
authors.initials: R
authors.lastname: Rothermel
authors.forename: James
authors.initials: J
authors.lastname: Janisse
authors.forename: Harry T
authors.initials: HT
authors.lastname: Chugani
mesh_terms: D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001930:Brain Injuries / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D001931:Brain Mapping; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology; D005260:Female; D019788:Fluorodeoxyglucose F18; D007839:Functional Laterality; D006801:Humans; D007360:Intelligence; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D049268:Positron-Emission Tomography; D012640:Seizures / Q000175:diagnosis / Q000209:etiology; D018709:Statistics, Nonparametric; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000523:psychology*
chemical_list: D019788:Fluorodeoxyglucose F18