title: The role of the thalamus in neuro-cognitive dysfunction in early unilateral hemispheric injury: a multimodality imaging study of children with Sturge-Weber syndrome.
abstract: BACKGROUND
  Sturge-Weber syndrome (SWS) with unilateral hemispheric involvement is a clinical model of early onset, chronic, often progressive hemispheric injury, resulting in variable neuro-cognitive impairment.
  
  
  OBJECTIVE
  To evaluate if abnormal diffusion and metabolism of the thalamus, a central relay station with extensive cortical connections, may serve as a simple imaging marker of neuro-cognitive dysfunction in SWS.
  
  
  METHODS
  We obtained both diffusion tensor imaging and FDG PET in 20 children (11 girls; age range: 3-12.4 years) with unilateral SWS. Diffusion parameters as well as FDG uptake were measured in thalami, compared to normal control values, and correlated with the extent of cortical hypometabolism, deep venous abnormalities and cognitive (IQ) as well as fine motor functions.
  
  
  RESULTS
  Children with SWS had significantly higher thalamic glucose metabolic asymmetry than controls (p=0.001). Thalamic metabolic asymmetries correlated positively with the asymmetry of thalamic diffusivity (p=0.001) and also with the extent of cortical hypometabolism (p<0.001). Severe thalamic asymmetries of glucose metabolism and diffusion were strong predictors of low IQ (metabolism: p=0.002; diffusivity: p=0.01), even after controlling for age and extent of cortical glucose hypometabolism in children with left hemispheric involvement. Ipsilateral thalamic glucose hypometabolism was also associated with impairment of fine motor functions (p=0.002).
  
  
  CONCLUSIONS
  Both diffusion and glucose metabolic abnormalities of the thalamus are closely related to cognitive functions, independent of age and cortical metabolic abnormalities, in children with unilateral SWS. Thalamic metabolic asymmetry is a robust but simple imaging marker of neuro-cognitive outcome in children with early unilateral hemispheric injury caused by Sturge-Weber syndrome.
authors.affiliation: Carman and Ann Adams Department of Pediatrics, Wayne State University School of Medicine, 3901 Beaubien Blvd, Detroit, MI 48201, USA.
authors.forename: Bálint
authors.initials: B
authors.lastname: Alkonyi
authors.forename: Harry T
authors.initials: HT
authors.lastname: Chugani
authors.forename: Michael
authors.initials: M
authors.lastname: Behen
authors.forename: Stacey
authors.initials: S
authors.lastname: Halverson
authors.forename: Emily
authors.initials: E
authors.lastname: Helder
authors.forename: Malek I
authors.initials: MI
authors.lastname: Makki
authors.forename: Csaba
authors.initials: C
authors.lastname: Juhász
mesh_terms: D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D056324:Diffusion Tensor Imaging; D005260:Female; D007839:Functional Laterality; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D008297:Male; D009483:Neuropsychological Tests; D049268:Positron-Emission Tomography; D011379:Prognosis; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000503:physiopathology*; D013788:Thalamus / Q000002:abnormalities* / Q000503:physiopathology
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