伴杏仁核肥大的颞叶癫痫的临床及病理特征
Clinico-pathological features of temporal lobe epilepsy with enlarged amygdala

目的:总结3例伴有杏仁核肥大(amygdala enlargement, AE)的颞叶癫痫患者的临床表现、视频脑电图(video electroencephalogram,VEEG)特征、影像学及病理特点。方法:收集3例于北京大学国际医院住院的伴AE颞叶癫痫患者的相关资料,分析患者的临床表现、VEEG特征、影像学及病理特点,并计算每位患者两侧的杏仁核体积。结果:3例患者中2例女性、1例男性,发病年龄21~40岁,2例临床表现为夜间入睡后继发全面性强直-阵挛发作(secondarily generalized tonic-clonic seizure, SGTCS),其中1例1年后出现复杂部分性发作(complex partial seizures, CPSs), 表现为情景回忆及自动症,第3例表现为夜间发作的CPSs,如咂嘴、吐舌,3例患者均伴有明显的焦虑障碍。3例患者头颅MRI均表现为单侧AE,1例为右侧,2例为左侧,肥大侧杏仁核体积为(2 123.7±131.8) mm 3,非肥大侧杏仁核体积为(1 276.3±156.9) mm 3。2例发作间期痫性放电仅限于AE侧,1例为双侧。3例发作期VEEG提示异常放电起源于AE同侧,并局限于前、中颞区。2例发作间期行单光子发射计算机断层成像术(single-photon emission computed tomography,SPECT)检查均未见异常,1例发作间期行正电子发射计算机断层显像(positron emission tomography, PET)检查表现为AE低代谢。3例患者的组织病理结果均为杏仁核及颞叶的局灶性皮层发育不良,其中1例伴海马硬化。所有患者术后未再发作,术后VEEG提示术侧慢波活动,偶见尖波放电。结论:AE可能为颞叶癫痫的一种亚型,临床中应注意在“磁共振检查阴性”的颞叶癫痫患者中是否存在AE的可能,药物控制不佳者,手术治疗具有良好效果,局灶性皮层发育不良可能是伴AE颞叶癫痫的最常见病理特征。
Objective: To summarize the clinical, video electroencephalogram (VEEG), radiological and pathological features of 3 patients of temporal lobe epilepsy (TLE) with amygdala enlargement (AE). Methods: Three TLE patients with AE who were hospitalized in Peking University International Hospital were collected. The above features were retrospectively analyzed, and the amygdala volume was measured as well. Results: Of all the 3 patients, 2 were females and 1 male, whose seizure onset ages varied from 21 to 40 years. Two cases presented with secondarily generalized tonic-clonic seizures after falling asleep during the night. One of the 2 cases had complex partial seizures (CPSs) with episodic memory and automatism after one year, and the third one had CPSs with lip smacking and tongue wagging during the night. All the patients suffered from obvious anxious disorder. Unilateral AE by MRI was de-monstrated in the 3 cases, one on the right side, and the other two on the left side. The average amygdala volume of the enlarged side and the other side were (2 123.7±131.8) mm 3 and (1 276.3±156.9) mm 3, respectively. Unilateral interictal epileptic discharges were ipsilateral to the AE in 2 cases, while the other patient showed bilateral interictal epileptic discharges. The ictal VEEG showed that the seizure onset zone was ipsilateral to the AE and was confined to the anterior and middle temporal regions in the 3 patients. The interictal single-photon emission computed tomography (SPECT) was negative in 2 cases. The interictal positron emission tomography (PET) showed hypometabolism in the AE in one case. The histological pathology revealed focal cortical dysplasia in the amygdala and temporal lobe in the 3 cases, and one of the 3