title: Cortical calcification in Sturge-Weber Syndrome on MRI-SWI: relation to brain perfusion status and seizure severity.
abstract: OBJECTIVE
  To determine the relationship between calcified cortex and perfusion status of white matter and seizure severity in patients with Sturge-Weber Syndrome (SWS), a sporadic neurocutaneous disorder characterized by a leptomeningeal angioma, progressive brain ischemia, and a high incidence of seizures using susceptibility weighted imaging (SWI) and dynamic susceptibility contrast-enhanced perfusion weighted imaging (DSC-PWI).
  
  
  METHODS
  Fifteen children (ages: 0.9-10 years) with unilateral SWS prospectively underwent magnetic resonance imaging (MRI). The degree of cortical calcification was assessed using SWI while perfusion status was quantified using DSC-PWI images (asymmetries of various perfusion parameters). Comparisons between calcification, perfusion status, and seizure variables were performed.
  
  
  RESULTS
  Patients with severely calcified cortex demonstrated significantly lower perfusion in the ipsilateral white matter (mean asymmetry: -0.52 ± 0.22) as compared to patients with only mildly calcified cortex or no calcification (mean asymmetry: 0.08 ± 0.25). Patients with severely calcified cortex also suffered from a higher seizure burden (a composite measure of seizure frequency and epilepsy duration; P = 0.01) and a trend for earlier seizure onset and longer epilepsy duration.
  
  
  CONCLUSIONS
  Severe calcification in the affected hemisphere is related to severely decreased perfusion in underlying white matter and is associated with more severe epilepsy in SWS patients.
authors.affiliation: Affiliated Zhongshan Hospital of Dalian University, Department of Radiology, Dalian, Liaoning, China.
authors.forename: Jianlin
authors.initials: J
authors.lastname: Wu
authors.forename: Bisher
authors.initials: B
authors.lastname: Tarabishy
authors.forename: Jiani
authors.initials: J
authors.lastname: Hu
authors.forename: Yanwei
authors.initials: Y
authors.lastname: Miao
authors.forename: Zhaocheng
authors.initials: Z
authors.lastname: Cai
authors.forename: Yang
authors.initials: Y
authors.lastname: Xuan
authors.forename: Michael
authors.initials: M
authors.lastname: Behen
authors.forename: Meng
authors.initials: M
authors.lastname: Li
authors.forename: Yongquan
authors.initials: Y
authors.lastname: Ye
authors.forename: Richard
authors.initials: R
authors.lastname: Shoskey
authors.forename: E Mark
authors.initials: EM
authors.lastname: Haacke
authors.forename: Csaba
authors.initials: C
authors.lastname: Juhász
mesh_terms: D000704:Analysis of Variance; D002114:Calcinosis / Q000175:diagnosis* / Q000209:etiology; D002540:Cerebral Cortex / Q000098:blood supply / Q000473:pathology*; D002560:Cerebrovascular Circulation / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D038524:Diffusion Magnetic Resonance Imaging / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D015203:Reproducibility of Results; D012640:Seizures / Q000175:diagnosis* / Q000209:etiology; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D013341:Sturge-Weber Syndrome / Q000150:complications / Q000175:diagnosis*