伴杏仁核肥大的颞叶癫痫的临床及病理特征

doi:10.19723/j.issn.1671-167X.2019.05.006

摘要/Abstract

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

朱莎,徐宗胜,夏晴,方筱静,赵丹华,刘献增. 伴杏仁核肥大的颞叶癫痫的临床及病理特征[J]. 北京大学学报（医学版）, 2019, 51(5): 824-828.

Sha ZHU,Zong-sheng XU,Qing XIA,Xiao-jing FANG,Dan-hua ZHAO,Xian-zeng LIU. Clinico-pathological features of temporal lobe epilepsy with enlarged amygdala[J]. Journal of Peking University(Health Sciences), 2019, 51(5): 824-828.

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参考文献 25

[1]	Soeder BM, Gleissner U, Urbach H , et al. Causes, presentation and outcome oflesional adult onset mediotemporal lobe epilepsy[J]. Neurol Neurosurg Psychiatry, 2009,80(8):894-899.
[2]	Goncalves Pereira PM, Oliveira E, Rosado P . Relative localizing value of amygdalohippocampal MR biometry in temporal lobe epilepsy[J]. Epilepsy Res, 2006,69(2):147-164.
[3]	Coan AC, Morita ME, de Campos BM, et al. Amygdala enlargement in patients with mesial temporal lobe epilepsy without hip-pocampal sclerosis[J]. Front Neurol, 2013,4:166.
[4]	Watson C, Andermann F, Gloor P , et al. Anatomic basis of amygdaloid and hippocampal volume measurement by magnetic resonance imaging[J]. Neurology, 1992,42(9):1743-1750.
[5]	Cendes F, Andermann F, Gloor P , et al. MRI volumetric mea-surement of amygdala and hippocampus intemporal lobe epilepsy[J]. Neurology, 1993,43(4):719-725.
[6]	Cendes F, Andermann F, Gloor P , et al. Relationship between atrophy of the amygdala and ictal fear in temporal lobe epilepsy[J]. Brain, 1994,117(4):739-746.
[7]	Bower SP, Vogrin SJ, Morris K , et al. Amygdala volumetry in “imaging-negative” temporal lobe epilepsy[J]. J Neurol Neurosurg Psychiatr, 2003,74(9):1245-1249.
[8]	Blumcke I, Thom M, Aronica E , et al. International consensus classification of hippocampal sclerosis in temporal lobe epilepsy: a task force report from the ILAE commission on diagnostic methods[J]. Epilepsia, 2013,54(7):1315-1329.
[9]	Beh SMJ, Cook MJ, D'Souza WJ . Isolated amygdala enlargement in temporal lobe epilepsy: A systematic review[J]. Epilepsy Behav, 2016,60:33-41.
[10]	潘克梫, 陈楠, 王星 , 等. 1000名中国正常成人杏仁核体积高分辨MRI测量[J]. 中国医学影像技术, 2010,26(9):1615-1619.
[11]	Lv RJ, Sun ZR, Cui T , et al. Temporal lobe epilepsy with amygdala enlargement: a subtype of temporal lobe epilepsy[J]. BMC Neurol, 2014,14:194.
[12]	Tebartz van Elst L, Woermann FG, Lemieux L , et al. Amygdala enlargement in dysthymia—a volumetric study of patients with temporal lobe epilepsy[J]. Biol Psychiatry, 1999,46(12):1614-1623.
[13]	Tebartz van Elst L, Baeumer D, Lemieux L , et al. Amygdala pathology in psychosis of epilepsy: amagnetic resonance imaging study in patients with temporal lobe epilepsy[J]. Brain, 2002,125(1):140-149.
[14]	Morrell F, Wada J, Engel J . Potential relevance of kindling and secondary epileptogenesis to the consideration of surgical treatment for epilepsy[M] // Engel J. Surgical treatment of the epilepsies. New York: Raven, 1987: 701-707.
[15]	Gloor P, Olivier A, Quesney LF , et al. The role of the limbic system in experiential phenomena of temporal lobe epilepsy[J]. Ann Neurol, 1982,12(2):129-144.
[16]	Paesschen WV, Connelly A, Johnson CL , et al. The amygdala and intractable temporal lobe epilepsy: a quantitative magnetic resonance imaging study[J]. Neurology, 1996,47(4):1021-1031.
[17]	Wieser HG . Mesial temporal lobe epilepsy versus amygdalar epilepsy: late seizure recurrence after initially successful amygdalotomy and regained seizure control following hippocampectomy[J]. Epileptic Disord, 2000,2(3):141-152.
[18]	Bartolomei F, Wendling F, Regis J , et al. Pre-ictal synchronicity in limbic networks of mesial temporal lobe epilepsy[J]. Epilepsy Res, 2004,61(1-3):89-104.
[19]	Takaya S, Ikeda A, Mitsueda-Ono T , et al. Temporal lobe epilepsy with amygdala enlargement: a morphologic and functional study[J]. J Neuroimaging, 2014,24(1):54-62.
[20]	Minami N, Morino M, Uda T , et al. Surgery for amygdale enlargement with mesial temporal lobe epilepsy: pathological findings and seizure outcome[J]. J Neurol Neurosurg Psychiatry, 2015,86(8):887-894.
[21]	Bien CG, Urbach H, Schramm J , et al. Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy[J]. Neurology, 2007,69(12):1236-1244.
[22]	Kimura Y, Sato N, Saito Y , et al. Temporal lobe epilepsy with unilateral amygdala enlargement: morphometric MR analysis with clinical and pathological study[J]. J Neuroimaging, 2015,25(2):175-183.
[23]	Kim DW, Lee SK, Chung CK , et al. Clinical features and pathological characteristics of amygdala enlargement in mesial temporal lobe epilepsy[J]. J Clin Neurosci, 2012,19(4):509-512.
[24]	Phi JH, Kim SK, Cho BK , et al. Long-term surgical outcomes of temporal lobe epilepsy associated with low-grade brain tumors[J]. Cancer, 2009,115(24):5771-5779.
[25]	Eriksson SH, Nordborg C, Rydenhag B , et al. Parenchymal lesions in pharmacoresistant temporal lobe epilepsy: dual and multiple pathology[J]. Acta Neurol Scand, 2005,112(3):151-156.

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

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 VEEG
1	40	Female	CPS	Both sides	Left		Left		Sleep, awake		1/week	FCDⅡb	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	Same as above
3	21	Male	SGTCS, CPS	Right	Right	Right	Right	Sleep, awake	Awake	1-10+/ month	1/day	FCDⅡb	Same as above