Kagawa Kenji, Chugani Diane C, Asano Eishi, Juhász Csaba, Muzik Otto, Shah Aashit, Shah Jagdish, Sood Sandeep, Kupsky William J, Mangner Thomas J, Chakraborty Pulak K, Chugani Harry T
Department of Pediatrics, Pediatric Neurology/PET Center, Children's Hospital of Michigan, 3901 Beaubien Boulevard, Detroit, MI 48201, USA.
J Child Neurol. 2005 May;20(5):429-38. doi: 10.1177/08830738050200050701.
Tuberous sclerosis complex is commonly associated with medically intractable seizures. We previously demonstrated that high uptake of alpha-[11C]methyl-L-tryptophan (AMT) on positron emission tomography (PET) occurs in a subset of epileptogenic tubers consistent with the location of seizure focus. In the present study, we analyzed the surgical outcome of children with tuberous sclerosis complex in relation to AMT PET results. Seventeen children (mean age 4.7 years) underwent epilepsy surgery, guided by long-term videoelectroencephalography (EEG) (including intracranial EEG in 14 cases), magnetic resonance imaging (MRI), and AMT PET. AMT uptake values of cortical tubers were measured using regions of interest delineated on coregistered MRI and were divided by the value for normal-appearing cortex to obtain an AMT uptake ratio. Based on surgical outcome data, tubers showing increased AMT uptake (uptake ratio greater than 1.00) were classified into three categories: (1) epileptogenic (tubers within an EEG-defined epileptic focus whose resection resulted in seizure-free outcome), (2) nonepileptogenic (tubers that were not resected but the patient became seizure free), or (3) uncertain (all other tubers). Increased AMT uptake was found in 30 tubers of 16 children, and 23 of these tubers (77%) were located in an EEG-defined epileptic focus. The tuber with the highest uptake was located in an ictal EEG onset region in each patient. Increased AMT uptake indicated an epileptic region not suspected by scalp EEG in four cases. Twelve children (71%) achieved seizure-free outcome (median follow-up 15 months). Based on outcome criteria, 19 of 30 tubers (63%) with increased AMT uptake were epileptogenic, and these tubers had significantly higher AMT uptake than the nonepileptogenic ones (P = .009). Tubers with at least 10% increase of AMT uptake (in nine patients) were all epileptogenic. Using a cutoff threshold of 1.02 for AMT uptake ratio provided an optimal accuracy of 83% for detecting tubers that needed to be resected to achieve a seizure-free outcome. The findings suggest that resection of tubers with increased AMT uptake is highly desirable to achieve seizure-free surgical outcome in children with tuberous sclerosis complex and intractable epilepsy. AMT PET can provide independent complementary information regarding the localization of epileptogenic regions in tuberous sclerosis complex and enhance the confidence of patient selection for successful epilepsy surgery.
结节性硬化症常与药物难治性癫痫发作相关。我们之前证明,在正电子发射断层扫描(PET)上,α-[11C]甲基-L-色氨酸(AMT)的高摄取出现在一部分致痫性结节中,与癫痫发作灶的位置一致。在本研究中,我们分析了结节性硬化症患儿的手术结果与AMT PET结果的关系。17名儿童(平均年龄4.7岁)接受了癫痫手术,手术由长期视频脑电图(EEG)(14例包括颅内EEG)、磁共振成像(MRI)和AMT PET引导。使用在配准的MRI上勾勒出的感兴趣区域测量皮质结节的AMT摄取值,并除以正常外观皮质的值以获得AMT摄取率。根据手术结果数据,AMT摄取增加(摄取率大于1.00)的结节分为三类:(1)致痫性(EEG定义的癫痫灶内的结节,其切除导致无癫痫发作结果),(2)非致痫性(未切除但患者无癫痫发作的结节),或(3)不确定(所有其他结节)。在16名儿童的30个结节中发现AMT摄取增加,其中23个结节(77%)位于EEG定义的癫痫灶内。每个患者中摄取最高的结节位于发作期EEG起始区域。4例中AMT摄取增加表明存在头皮EEG未怀疑的癫痫区域。12名儿童(71%)实现了无癫痫发作结果(中位随访15个月)。根据结果标准,30个AMT摄取增加的结节中有19个(63%)是致痫性的,这些结节的AMT摄取显著高于非致痫性结节(P = 0.009)。AMT摄取至少增加10%的结节(9例患者)均为致痫性。使用AMT摄取率1.02的截止阈值检测需要切除以实现无癫痫发作结果的结节,最佳准确率为83%。这些发现表明,切除AMT摄取增加的结节对于结节性硬化症合并难治性癫痫的儿童实现无癫痫发作的手术结果非常有必要。AMT PET可以提供关于结节性硬化症中致痫区域定位的独立补充信息,并增强患者选择成功癫痫手术的信心。