Evolution of cortical metabolic abnormalities and their clinical correlates in Sturge-Weber syndrome.
BACKGROUND
The natural course of Sturge-Weber syndrome (SWS) is poorly understood, although neurological symptoms are often progressive.


OBJECTIVE
To track longitudinal changes in brain glucose metabolism measured with positron emission tomography (PET) and their relation to clinical changes during the early course of SWS.


METHODS
Fourteen children (age 3 months to 3.9 years at enrollment) with SWS and unilateral leptomeningeal angioma underwent two consecutive glucose metabolism PET scans with a mean follow-up time of 1.2 years. Longitudinal changes of the extent of cortical glucose hypometabolism on the angioma side were measured and correlated with age, clinical seizure frequency and hemiparesis.


RESULTS
An increase in the size of the hypometabolic cortex was seen in 6 children, coinciding with an age-related increase in cortical glucose metabolism measured in unaffected contralateral cortex. These 6 patients were younger both at the initial (mean age 0.75 vs. 2.8 years; p<0.001) and the second scan (mean age 1.8 vs. 4.2 years; p=0.001) than those with no change in the extent of hypometabolic cortex (n=6). The area of cortical hypometabolism decreased in the two remaining children, and this was associated with resolution of an initial hemiparesis in one of them. Seizure frequency between the two scans was higher in children who showed progressive enlargement of cortical hypometabolism, as compared to those with no progression (p=0.008).


CONCLUSIONS
In SWS, detrimental metabolic changes occur before 3 years of age coinciding with a sharp increase of developmentally regulated cerebral metabolic demand. Progressive hypometabolism is associated with high seizure frequency in these children. However, metabolic abnormalities may remain limited or even partially recover later in some children with well-controlled seizures. Metabolic recovery accompanied by neurological improvement suggests a window for therapeutic intervention in children with unilateral SWS.
Juhasz
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Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, 3901 Beaubien Boulevard, Detroit, MI, USA. juhasz@pet.wayne.edu
Batista
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Chugani
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Muzik
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Chugani
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D000367:Age Factors; D002540:Cerebral Cortex / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000473:pathology; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D010291:Paresis / Q000209:etiology; D049268:Positron-Emission Tomography; D012640:Seizures / Q000209:etiology; D013341:Sturge-Weber Syndrome / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000473:pathology