title: Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome.
abstract: To evaluate clinical and metabolic correlates of cerebral calcifications in children with Sturge-Weber syndrome (SWS).
  
  
  
  Fifteen children (11 females, four males; age range 7mo-9y, mean 4y 1mo) with unilateral SWS underwent baseline and follow-up magnetic resonance imaging (MRI) with susceptibility weighted imaging (SWI), glucose metabolism positron emission tomography (PET), and neurocognitive assessment (mean follow-up 1y 8mo). Calcified brain volumes measured on SWI were correlated with areas of abnormal glucose metabolism, seizure variables, and cognitive function (IQ).
  
  
  
  Ten children had brain calcification at baseline and 11 at follow-up. Mean calcified brain volume increased from 1.69 to 2.47cm3(p=0.003) in these children; the rate of interval calcified volume increase was associated with early onset of epilepsy (Spearman's rho [rs]=-0.63, p=0.036). Calcified brain regions showed a variable degree of glucose hypometabolism with the metabolic abnormalities often extending to non-calcified cerebral lobes. Larger calcified brain volumes at baseline were associated with longer duration of epilepsy (rs=0.69, p=0.004) and lower outcome IQ (rs=-0.53, p=0.042).
  
  
  
  Brain calcifications are common and progress faster in children with SWS with early epilepsy onset, and are associated with a variable degree of hypometabolism, which is typically more extensive than the calcified area. Higher calcified brain volumes may indicate a risk for poorer neurocognitive outcome.
authors.affiliation: Division of Pediatric Neurology, The Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.
authors.forename: Vinod K
authors.initials: VK
authors.lastname: Pilli
authors.affiliation: Division of Pediatric Neurology, The Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Behen
authors.affiliation: Department of Radiology, Wayne State University, Detroit, MI, USA.
authors.forename: Jiani
authors.initials: J
authors.lastname: Hu
authors.affiliation: Department of Radiology, Wayne State University, Detroit, MI, USA.
authors.forename: Yang
authors.initials: Y
authors.lastname: Xuan
authors.affiliation: Department of Family Medicine and Public Health Sciences, Wayne State University, Detroit, MI, USA.
authors.forename: James
authors.initials: J
authors.lastname: Janisse
authors.affiliation: Division of Pediatric Neurology, The Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.|Division of Neurology, Nemours/Alfred I DuPont Hospital for Children, Wilmington, DE, USA.
authors.forename: Harry T
authors.initials: HT
authors.lastname: Chugani
authors.affiliation: Division of Pediatric Neurology, The Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.
authors.forename: Csaba
authors.identifier: 0000-0002-5067-5554
authors.initials: C
authors.lastname: Juhász
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging*; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002675:Child, Preschool; D003071:Cognition; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005947:Glucose / Q000378:metabolism; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D049268:Positron-Emission Tomography; D012640:Seizures / Q000000981:diagnostic imaging / Q000503:physiopathology; D013341:Sturge-Weber Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology*; D013997:Time Factors
chemical_list: D005947:Glucose