Lee J S, Asano E, Muzik O, Chugani D C, Juhász C, Pfund Z, Philip S, Behen M, Chugani H T
Department of Pediatrics, Children's Hospital of Michigan, Detroit Medical Center, Wayne State University School of Medicine, 48201, USA.
Neurology. 2001 Jul 24;57(2):189-95. doi: 10.1212/wnl.57.2.189.
To determine whether the extent and degree of glucose hypometabolism defined by PET correlate with seizure characteristics, cognitive function, and interictal EEG abnormalities in children with unilateral cerebral involvement of Sturge-Weber syndrome (SWS).
2-Deoxy-2[18F]fluoro-D-glucose (FDG) PET was performed in 13 children (age range 0.7 to 15.1 years; five boys, eight girls) with unilateral SWS. Based on asymmetries between homologous cortical areas in FDG PET images, cortical areas of mildly (10% to 20% decrease), and severely (>20% decrease) asymmetric cortical metabolism were defined. These areas were normalized to the size of the ipsilateral hemisphere and correlated with clinical seizure characteristics, full-scale IQ, and interictal EEG abnormalities.
Both seizure frequency (p = 0.027) and lifetime number of seizures (p = 0.017) showed a positive correlation with the area (expressed as the percentage of cortical area of ipsilateral hemisphere) of mildly asymmetric cortical metabolism. Patients with higher IQ had a shorter duration of epilepsy (p = 0.044) and a larger area of severely asymmetric cortical metabolism (p = 0.044). Patients with bilateral interictal EEG abnormalities had larger lifetime number of seizures (p = 0.042), lower IQ (p = 0.024), and smaller area of severely asymmetric cortical metabolism (p = 0.019) than those with only ipsilateral EEG abnormalities.
Association of severely asymmetric cortical metabolism with relatively preserved cognitive function in SWS suggests that functional reorganization occurs more readily when cortex is severely rather than mildly damaged. Therefore, the area of mildly asymmetric cortical metabolism may exert a nociferous effect on the remaining of the brain. Thus, the extent and degree of glucose asymmetry detected by PET are sensitive markers of seizure severity and cognitive decline in SWS.
确定正电子发射断层扫描（PET）所定义的葡萄糖代谢减低的范围和程度是否与患有单侧大脑受累的斯特奇-韦伯综合征（SWS）儿童的癫痫发作特征、认知功能及发作间期脑电图异常相关。
对13例单侧SWS儿童（年龄范围0.7至15.1岁；5名男孩，8名女孩）进行2-脱氧-2[18F]氟-D-葡萄糖（FDG）PET检查。基于FDG PET图像中同源皮质区域之间的不对称性，定义轻度（降低10%至20%）和重度（降低>20%）不对称皮质代谢的皮质区域。这些区域经同侧半球大小标准化后，与临床癫痫发作特征、全量表智商及发作间期脑电图异常进行相关性分析。
癫痫发作频率（p = 0.027）和终生癫痫发作次数（p = 0.017）均与轻度不对称皮质代谢区域（以同侧半球皮质面积的百分比表示）呈正相关。智商较高的患者癫痫发作持续时间较短（p = 0.044），重度不对称皮质代谢区域较大（p = 0.044）。与仅存在同侧脑电图异常的患者相比，双侧发作间期脑电图异常的患者终生癫痫发作次数更多（p = 0.042），智商更低（p = 0.024），重度不对称皮质代谢区域更小（p = 0.019）。
SWS中重度不对称皮质代谢与相对保留的认知功能相关，提示当皮质严重而非轻度受损时，功能重组更容易发生。因此，轻度不对称皮质代谢区域可能对大脑其余部分产生有害影响。故而，PET检测到的葡萄糖不对称的范围和程度是SWS中癫痫发作严重程度和认知衰退的敏感指标。
