北京大学学报(医学版) ›› 2019, Vol. 51 ›› Issue (5): 824-828. doi: 10.19723/j.issn.1671-167X.2019.05.006

• 论著 • 上一篇    下一篇

伴杏仁核肥大的颞叶癫痫的临床及病理特征

朱莎1,徐宗胜1,夏晴1,方筱静1,赵丹华1,刘献增1,2,()   

  1. 1. 北京大学国际医院神经内科,北京 102206
    2. 北京大学人民医院神经内科,北京 100044
  • 收稿日期:2018-01-19 出版日期:2019-10-18 发布日期:2019-10-23
  • 通讯作者: 刘献增 E-mail:liuxianzeng2004@sina.com

Clinico-pathological features of temporal lobe epilepsy with enlarged amygdala

Sha ZHU1,Zong-sheng XU1,Qing XIA1,Xiao-jing FANG1,Dan-hua ZHAO1,Xian-zeng LIU1,2,()   

  1. 1. Department of Neurology, Peking University International Hospital, Beijing 102206, China
    2. Department of Neurology, Peking University People’s Hospital, Beijing 100044, China
  • Received:2018-01-19 Online:2019-10-18 Published:2019-10-23
  • Contact: Xian-zeng LIU E-mail:liuxianzeng2004@sina.com

摘要:

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。

关键词: 杏仁核, 颞叶癫痫, 局灶性皮层发育不良

Abstract:

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 cases was combined with hippocampal sclerosis. All the patients became seizure free after surgery in the half year following-up. VEEG revealed slow wave activity and occasional spike wave in the operated side. Conclusion: AE may be one subtype of TLE. It is necessary to recognize AE in TLE with MRI-negative. For those poorly responsive to antiepileptic drugs, surgical treatment could provide a better solution. Focal cortical dysplasia may be one of the most common pathological features of TLE with AE.

Key words: Amygdala, Temporal lobe epilepsy, Focal cortical dysplasia

中图分类号: 

  • R742.1

图1

头颅MRI Flair像"

表1

3例患者的杏仁核体积"

Patient number Contralateral side/mm3 AE side/mm3
1 1 126 1 979
2 1 439 2 237
3 1 264 2 155

图2

PET检查显示左侧肥大的杏仁核低代谢"

图3

杏仁核中可见气球样细胞(黑色箭头,HE ×40)"

表2

3例伴杏仁核肥大的颞叶癫痫患者的临床资料"

Patient Age at
epilepsy
onset/years
Gender Seizure
type
Interictal
scalf
VEEG
Ictal
scalf
VEEG
Intracranial
deep
electrode
VEEG
AE
side
SGTC
time
CPS
time
SGTC
frequency
CPS
frequency
Neuropa-
thological
findings
Postoperative
seizure
Postoperative
VEEG
1 40 Female CPS Both sides Left Left Sleep,
awake
1/week FCDⅡb 0 Slow wave in
the operation
area, sharp
wave
occasional
2 35 Female SGTCS Left Left Left Sleep Awake 1-10+/
month
1-2/
day
FCDⅢa 0 Same as
above
3 21 Male SGTCS,
CPS
Right Right Right Right Sleep,
awake
Awake 1-10+/
month
1/day FCDⅡb 0 Same as
above
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