Elsevier

The Journal of Pediatrics

Volume 114, Issue 2, February 1989, Pages 244-253
The Journal of Pediatrics

Original article
Sturge-weber syndrome: A study of cerebral glucose utilization with positron emission tomography

https://doi.org/10.1016/S0022-3476(89)80790-5Get rights and content

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 hemangloma 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.

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      Citation Excerpt :

      In general, previous SPECT, xenon-CT, PWI and PET studies all showed that hypoperfusion and hypometabolism are the most common abnormalities in the affected area of patients with SWS and chronic epilepsy [3,8,9,28,29]. Importantly, previous cerebral blood flow SPECT and FDG PET studies showed that perfusion and metabolic abnormalities are anatomically well matched with the region of the vascular malformation; however, the perfusion and metabolic deficit can extend beyond the lobe with overlying leptomeningeal enhancement [3,9]. This observation is now further supported by our results showing brain regions with abnormal perfusion and/or glucose metabolism in lobes not affected by the malformation.

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    Supported by U.S. Department of Energy contract No. DE-AC03-SF7600012, U.S. Public Health Service grants Nos. 5RO1-MH37916 and 2POI-NS15654, and a grant from the United Cerebral Palsy Research Foundation. Drs. Chugani and Mazziotta are the recipients of Teacher-Investigator Developmental Awards 1-KO7-NS00886 and 1-KO7-NS00588, respectively, from the National Institute of Neurological and Communicative Diseases and Stroke.

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