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Evolution of cortical metabolic abnormalities and their clinical correlates in Sturge-Weber syndrome

C Juhasz1, C E A Batista, D C Chugani

  • 1Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, 3901 Beaubien Boulevard, Detroit, MI, USA. juhasz@pet.wayne.edu

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|April 6, 2007
PubMed

Insights

Sturge-Weber syndrome (SWS) shows progressive brain metabolism changes before age 3, linked to increased seizure frequency. Some children experience metabolic recovery, suggesting a therapeutic window for SWS treatment.

Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Medical Imaging

Background:

  • Sturge-Weber syndrome (SWS) natural course and neurological progression are not well understood.
  • Early SWS neurological symptoms are often progressive.

Purpose of the Study:

  • To track longitudinal changes in brain glucose metabolism using PET scans in children with SWS.
  • To correlate metabolic changes with clinical progression in early SWS.

Main Methods:

  • 14 children with SWS underwent two PET scans over a mean follow-up of 1.2 years.
  • Assessed longitudinal changes in cortical glucose hypometabolism extent.
  • Correlated metabolic changes with age, seizure frequency, and hemiparesis.

Main Results:

  • Progressive hypometabolism enlargement occurred in 6 younger children, associated with higher seizure frequency.
  • Metabolic changes correlated with age-related increases in unaffected cortex metabolism.
  • Two children showed decreased hypometabolism, one with hemiparesis resolution.

Conclusions:

  • Detrimental metabolic changes in SWS occur before age 3, coinciding with increased cerebral metabolic demand.
  • Progressive hypometabolism is linked to high seizure frequency in SWS.
  • Partial metabolic recovery with neurological improvement suggests a therapeutic window in unilateral SWS.
Abstract

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