局灶性皮层发育不良不同病理亚型伴海马硬化所致颞叶癫痫的临床特征

doi:10.3877/cma.j.issn.1674-0785.2021.06.003

目的

初步分析总结局灶性皮层发育不良（FCD）不同病理亚型伴海马硬化（HS）所致颞叶癫痫的临床特征。

方法

连续收集自2016年1月至2019年12月就诊于北京大学国际医院神经疾病中心并接受致痫灶切除的颞叶癫痫患者，回顾分析患者的术后病理学资料。根据2011年国际抗癫痫联盟最新分型，将FCD伴HS所致颞叶癫痫分为FCDⅢa组（FCDⅠ型＋HS）和双重病理组（FCDⅡ型＋HS），分析比较2组的临床特征。

结果

共计25例FCD伴HS颞叶癫痫患者，其中FCDⅢa组14例，双重病理组11例。2组间起病年龄、病程、手术年龄、热性惊厥史、家族史、癫痫发作形式、脑电图特征等临床资料差异无统计学意义（P均＞0.05）。头颅MRI可见FCD（+）、杏仁核肥大、HS（+）等征象，2组间各征象阳性率差异无统计学意义（P均＞0.05）。预后比较：FCDⅢa组术后EngelⅠa级＋EngelⅠ级患者共11例（78.6%），双重病理组术后EngelⅠa级＋EngelⅠ级患者共9例（81.8%），差异无统计学意义（P＞0.05）。

结论

FCDⅡ型＋HS双重病理颞叶癫痫并不少见。目前尚难以通过术前临床、电生理及影像学资料完全区分FCDⅢa与双重病理。FCD不同病理亚型伴HS所致颞叶癫痫均预后良好。

关键词: 局灶性皮层发育不良, 海马硬化, 颞叶癫痫, 双重病理

Objective

To analyze and summarize the clinical features of temporal lobe epilepsy caused by different pathological subtypes of focal cortical dysplasia (FCD) with hippocampal sclerosis (HS).

Methods

The patients with temporal lobe epilepsy who were treated at the Neurological Disease Center of Peking University International Hospital from January 2016 to December 2019 and received epileptic foci resection were collected continuously, and the postoperative pathological data were analyzed retrospectively. According to the latest classification of the International League Against Epilepsy in 2011, temporal lobe epilepsy caused by FCD with HS was divided into either an FCDⅢa group (FCDⅠ+HS) or a dual pathology group (FCDⅡ+HS), and the clinical characteristics between the two groups were analyzed.

Results

A total of 25 patients were collected, including 14 cases in the FCDⅢa group and 11 cases in the dual pathology group. There were no significant differences in clinical features such as age of onset, course of disease, age at operation, history of febrile seizure, family history, epileptic seizure pattern, or electroencephalography characteristics between the two groups (P＞0.05). Brain MRI showed signs of FCD (+), amygdala enlargement, and HS (+). There were no statistically significant differences in the positive rate of the signs between the two groups (P＞0.05). With regard to prognosis, there were 11 cases (78.6%) EngelⅠa/Ⅰ patients in the FCDⅢa group and 9 cases (81.8%) EngelⅠa/Ⅰ patients in the dual pathology group after operation, the difference was not statistically significant (P＞0.05).

Conclusion

Temporal lobe epilepsy with FCDⅡ＋HS dual pathology is not uncommon. At present, it is difficult to clearly distinguish FCDⅢa from dual pathology based on preoperative clinical, electrophysiological, and imaging data. Temporal lobe epilepsy caused by different pathological subtypes of FCD with HS have a good prognosis.

Key words: Focal cortical dysplasia, Hippocampal sclerosis, Temporal lobe epilepsy, Dual pathology

表1 局灶性皮层发育不良不同病理亚型伴海马硬化所致颞叶癫痫患者的一般资料比较

项目	FCDⅢa组（14例）	双重病理组（11例）	统计值	P值
男性［例（%）］	6（42.9）	6（54.5）	-	0.695
起病年龄（岁， $x ¯$ ±s）	14.1±9.9	13.7±7.5	t=0.116	0.909
病程（年， $x ¯$ ±s）	13.4±7.1	11.0±6.4	t=0.884	0.386
手术年龄（岁， $x ¯$ ±s）	27.6±8.8	24.6±5.9	t=0.947	0.353
热性惊厥史［例（%）］	4（28.6）	3（27.3）	-	1.000
家族史［例（%）］	1（7.1）	0（0）	-	1.000
癫痫持续状态［例（%）］	5（35.7）	4（36.4）	-	1.000
继发性全面性强直-阵挛发作［例（%）］	12（85.7）	9（81.8）	-	1.000
发作间期局灶性放电［例（%）］	5（35.7）	7（63.6）	-	0.238
发作期向对侧扩散放电［例（%）］	12（85.7）	9（81.8）	-	1.000

表1 局灶性皮层发育不良不同病理亚型伴海马硬化所致颞叶癫痫患者的一般资料比较

项目	FCDⅢa组（14例）	双重病理组（11例）	统计值	P值
男性［例（%）］	6（42.9）	6（54.5）	-	0.695
起病年龄（岁， $x ¯$ ±s）	14.1±9.9	13.7±7.5	t=0.116	0.909
病程（年， $x ¯$ ±s）	13.4±7.1	11.0±6.4	t=0.884	0.386
手术年龄（岁， $x ¯$ ±s）	27.6±8.8	24.6±5.9	t=0.947	0.353
热性惊厥史［例（%）］	4（28.6）	3（27.3）	-	1.000
家族史［例（%）］	1（7.1）	0（0）	-	1.000
癫痫持续状态［例（%）］	5（35.7）	4（36.4）	-	1.000
继发性全面性强直-阵挛发作［例（%）］	12（85.7）	9（81.8）	-	1.000
发作间期局灶性放电［例（%）］	5（35.7）	7（63.6）	-	0.238
发作期向对侧扩散放电［例（%）］	12（85.7）	9（81.8）	-	1.000

图1 头颅MRI冠状位Flair像。图a患者，26岁，男性，头颅MRI提示右侧颞叶灰白质分界不清、局灶性皮层增厚、Flair高信号，右侧海马萎缩、Flair高信号，术后病理提示局灶性皮层发育不良Ⅰ型伴海马硬化，符合局灶性皮层发育不良Ⅲa型；图b患者，13岁，女性，头颅MRI提示左侧颞叶灰白质分界不清、局灶性皮层增厚、Flair高信号，左侧海马萎缩、Flair高信号，术后病理提示局灶性皮层发育不良Ⅱa型伴海马硬化，符合双重病理

表2 局灶性皮层发育不良不同病理亚型伴海马硬化所致颞叶癫痫患者影像学特征比较［例（%）］

组别	例数	FCD阳性	杏仁核肥大	HS阳性
FCDⅢa组	14	6（42.9）	1（7.1）	12（85.7）
双重病理组	11	8（72.7）	4（36.4）	9（81.8）
P值^*		0.227	0.133	1.000

表2 局灶性皮层发育不良不同病理亚型伴海马硬化所致颞叶癫痫患者影像学特征比较［例（%）］

组别	例数	FCD阳性	杏仁核肥大	HS阳性
FCDⅢa组	14	6（42.9）	1（7.1）	12（85.7）
双重病理组	11	8（72.7）	4（36.4）	9（81.8）
P值^*		0.227	0.133	1.000

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Clinical features of temporal lobe epilepsy caused by different pathological subtypes of focal cortical dysplasia with hippocampal sclerosis

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Clinical features of temporal lobe epilepsy caused by different pathological subtypes of focal cortical dysplasia with hippocampal sclerosis