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Transient focal cortical increase of interictal glucose metabolism in Sturge‐Weber syndrome: Implications for epileptogenesis
Author(s) -
Alkonyi Bálint,
Chugani Harry T.,
Juhász Csaba
Publication year - 2011
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1167.2011.03066.x
Subject(s) - hypermetabolism , ictal , medicine , epilepsy , sturge–weber syndrome , positron emission tomography , fluorodeoxyglucose , anesthesia , surgery , nuclear medicine , psychiatry
Summary Purpose: To investigate clinical correlates and longitudinal course of interictal focal cortical glucose hyper metabolism in children with Sturge‐Weber syndrome (SWS). Methods: Fluorodeoxyglucose positron emission tomography (FDG‐PET) scans of 60 children (age range 3 months to 15.2 years) with Sturge‐Weber syndrome and epilepsy were assessed prospectively and serially for focal hypo‐ or hyper metabolism. Thirty‐two patients had two or more consecutive PET scans. Age, seizure variables, and the occurrence of epilepsy surgery were compared between patients with and without focal hyper metabolism. The severity of focal hyper metabolism was also assessed and correlated with seizure variables. Key Findings: Interictal cortical glucose hyper metabolism, ipsilateral to the angioma, was seen in nine patients, with the most common location in the frontal lobe. Age was lower in patients with hyper metabolism than in those without (p = 0.022). In addition, time difference between the onset of first seizure and the first PET scan was much shorter in children with increased glucose metabolism than in those without (mean: 1.0 vs. 3.6 years; p = 0.019). Increased metabolism was transient and switched to hypometabolism in all five children where follow‐up scans were available. Focal glucose hyper metabolism occurred in 28% of children younger than the age of 2 years. Children with transient hyper metabolism had a higher rate of subsequent epilepsy surgery as compared to those without hyper metabolism (p = 0.039). Significance: Interictal glucose hyper metabolism in young children with SWS is most often seen within a short time before or after the onset of first clinical seizures, that is, the presumed period of epileptogenesis. Increased glucose metabolism detected by PET predicts future demise of the affected cortex based on a progressive loss of metabolism and may be an imaging marker of the most malignant cases of intractable epilepsy requiring surgery in SWS.