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Sturge-Weber syndrome: a study of cerebral glucose utilization with positron emission tomography
H T Chugani1, J C Mazziotta, M E Phelps
1Division of Pediatric Neurology, University of California, Los Angeles School of Medicine 90024.
Insights
Positron emission tomography (PET) reveals reduced glucose metabolism in children with Sturge-Weber syndrome (SWS). Early SWS cases show metabolic changes, aiding in surgical candidate selection.
Area of Science:
- Neurology
- Pediatrics
- Medical Imaging
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial capillary malformation (port-wine stain) and neurological abnormalities.
- Cerebral involvement in SWS can lead to seizures, developmental delay, and cognitive deficits.
Purpose of the Study:
- To investigate cerebral glucose metabolism in children with SWS using positron emission tomography (PET).
- To assess the utility of PET in evaluating disease extent, progression, and guiding surgical interventions in SWS.
Main Methods:
- Local cerebral metabolic rate for glucose (ICMRGIc) was measured using PET in six children with SWS and six asymptomatic children with SWS stigmata.
- Imaging findings were correlated with clinical presentation and other neuroimaging modalities like CT and MRI.
Main Results:
- Advanced SWS cases exhibited significantly depressed ICMRGIc in affected cerebral hemispheres, extending beyond CT-defined lesions.
- Two infants with recent-onset seizures showed a transient paradoxical increase in ICMRGIc in the affected hemisphere, with one also showing cerebellar involvement.
- PET demonstrated sensitive detection of cerebral metabolic impairment in early SWS and asymptomatic cases.
Conclusions:
- PET is a valuable tool for assessing the extent and severity of cerebral metabolic impairment in SWS, even in early or asymptomatic stages.
- Serial PET studies can track disease progression and aid in selecting candidates for surgical interventions like hemispherectomy.
- The transient increase in ICMRGIc in infants warrants further investigation into the pathogenesis of SWS-related cerebral degeneration.
Abstract:
We measured local cerebral metabolic rate for glucose (ICMRGIc) using positron emission tomography (PET) in six children with Sturge-Weber syndrome (SWS) and in six neurologically asymptomatic children with facial capillary hemangioma suggestive of SWS. Children with advanced SWS showed markedly depressed ICMRGIc in the anatomically affected cerebral hemisphere in a distribution that extended beyond the abnormalities depicted on computed tomography scan. In two infants with SWS and recent seizure onset, interictal PET revealed a paradoxical pattern of increased ICMRGIc in the cerebral cortex of the anatomically affected hemisphere. In one of these infants, ICMRGIc was also increased in the contralateral cerebellum, suggesting activation of the cortico-ponto-cerebellar circuitry. Subsequent PET (28 months later) in this child revealed the typical ICMRGIc pattern seen in advanced SWS. Further study of this transient ICMRGIc increase may be important in disclosing the pathogenesis of unilateral cerebral degeneration in SWS. In neurologically asymptomatic children with the facial stigmata of SWS and in children with early SWS, PET provides a sensitive measure of the extent and degree of cerebral metabolic impairment. Serial PET studies in children with SWS can be used to assess disease progression and, together with computed tomography or magnetic resonance imaging, may be useful in the selection of suitable candidates for cerebral hemispherectomy or focal cortical resection.