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Ictal pouting (‘Chapeau de gendarme’) in three pediatric cases with cortical dysplasia
European Journal of Paediatric Neurology ( IF 2.3 ) Pub Date : 2020-05-01 , DOI: 10.1016/j.ejpn.2019.11.005
Dilek Cebeci 1 , Ebru Arhan 2 , Tugba Hirfanoglu 2 , Zeynep Selen Karalok 1 , Hakan Ercelebi 2 , Özge Dedeoğlu 3 , Lutfiye Ozlem Atay 4 , Murat Ucar 5 , Ayse Serdaroğlu 2
Affiliation  

Ictal pouting (Chapeau de gendarme sign) can be described as an inverted smile. It consists of a turned down mouth with the contraction of the chin, wrinkling of the lips and symmetrical lowering of the labial commissures. This shape resembles the gendarme's hat during Napoléon I's time. Chapeau de gendarme sign is frequently seen in frontal and temporal lobe seizures. Focal cortical dysplasias are intrinsically epileptogenic foci and are frequently seen in patients with ictal pouting in seizure semiology. In this report, we analyzed clinical data, video EEG recordings and brain imagings of three children presenting with ictal pouting semiology in whom patients' magnetic resonans images (MRIs) or positron emission tomographies (PETs) were positive or doubtful for FCD in all. In case 1 and 2 the epileptogenic zones were temporal or temporoinsular. In these patients, with involvement of temporal lobe, dystonia and automatisms were seen in the seizure semiology after chapeau de gendarme sign. In case 3 with frontal lobe origin, hypermotor movements were seen after ictal pouting. In the patients 1 and 2, the cortical dysplasias were in temporal lobe. In patient 3, PET demonstrated hypometabolism on left inferior frontal gyrus but we couldn't verify this finding with MRIs. Ictal pouting (Chapeau de gendarme sign) is a distinct seizure semiology that can often be overlooked and coexist with focal cortical thickening. We suggest that focal cortical dysplasias should be searched in patients with ictal pouting particularly in those with refractory focal seizures.

中文翻译:

三例皮质发育不良儿科病例的发作性噘嘴('Chapeau de gendarme')

Ictal pouting(Chapeau de gendarme 标志)可以被描述为倒立的微笑。它包括一个向下的嘴巴,下巴收缩,嘴唇起皱和唇连合对称降低。这种形状类似于拿破仑一世时期宪兵的帽子。Chapeau de Gendarme 征常见于额叶和颞叶癫痫发作。局灶性皮质发育不良本质上是致癫痫病灶,常见于癫痫发作符号学中发作性噘嘴的患者。在本报告中,我们分析了三名患有发作期噘嘴症状的儿童的临床数据、视频 EEG 记录和大脑成像,其中患者的磁共振图像 (MRI) 或正电子发射断层扫描 (PET) 总体上为 FCD 阳性或可疑。在病例 1 和 2 中,致痫区是颞区或颞岛区。在这些患者中,伴随着颞叶受累,在宪兵前兆后的癫痫发作符号学中观察到肌张力障碍和自动症。在额叶起源的病例 3 中,在发作期噘嘴后观察到运动过度。在患者1和2中,皮质发育不良在颞叶。在患者 3 中,PET 显示左侧额下回低代谢,但我们无法用 MRI 证实这一发现。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。伴随颞叶受累,在宪兵前兆后的癫痫发作符号学中可见肌张力障碍和自动症。在额叶起源的病例 3 中,在发作期噘嘴后观察到运动过度。在患者1和2中,皮质发育不良在颞叶。在患者 3 中,PET 显示左侧额下回低代谢,但我们无法用 MRI 证实这一发现。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。伴随颞叶受累,在宪兵前兆后的癫痫发作符号学中可见肌张力障碍和自动症。在额叶起源的病例 3 中,在发作期噘嘴后观察到运动过度。在患者1和2中,皮质发育不良在颞叶。在患者 3 中,PET 显示左侧额下回低代谢,但我们无法用 MRI 证实这一发现。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。发作期撅嘴后可见运动过度。在患者1和2中,皮质发育不良在颞叶。在患者 3 中,PET 显示左侧额下回低代谢,但我们无法用 MRI 证实这一发现。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。发作期撅嘴后可见运动过度。在患者1和2中,皮质发育不良在颞叶。在患者 3 中,PET 显示左侧额下回低代谢,但我们无法用 MRI 证实这一发现。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。发作性噘嘴(宪兵章征)是一种独特的癫痫症状学,经常被忽视并与局灶性皮质增厚共存。我们建议对发作性噘嘴的患者,尤其是难治性局灶性癫痫发作的患者,应检查局灶性皮质发育不良。
更新日期:2020-05-01
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