樊麗娟,吳 丹,劉小蕓,楊彥峰,孫 偉

臨床經(jīng)驗(yàn)總結(jié)

樊麗娟1,2,吳 丹2,劉小蕓2,楊彥峰2,孫 偉2

額葉癲癇；噘嘴發(fā)作

1 病例報(bào)告

患者女，23歲，慢性病程，因“反復(fù)發(fā)作性口角下撇15年余”就診,發(fā)作時(shí)表現(xiàn)：發(fā)作性口角下撇，伴雙眼瞪大、表情恐懼，持續(xù)時(shí)間10～20 s/次，5～6次/d。無(wú)四肢抽搐，無(wú)咂嘴、咀嚼。查體：神清，精神可。神經(jīng)系統(tǒng)檢查未見(jiàn)陽(yáng)性定位體征。既往史、家族史無(wú)特殊。頭顱MRI：未見(jiàn)異常。發(fā)作間期頭皮腦電圖：可見(jiàn)左側(cè)額極、額、中央?yún)^(qū)棘波、 棘慢波散發(fā)或持續(xù)性發(fā)放；發(fā)作期頭皮腦電(記錄到3次發(fā)作)：清醒期，雙眼睜大，表情恐懼，口角下撇，同期腦電圖表現(xiàn)為左側(cè)額極、額、中央?yún)^(qū)低-中波幅棘波、棘慢波、快波活動(dòng)發(fā)放→廣泛電壓減低，持續(xù)時(shí)間約20 s(圖1、2)。治療方案：奧卡西平0.9 g，1次/早；0.6 g，1次/晚，以及開普蘭0.5 g，2次/d,口服。2017-06隨訪:目前近半年發(fā)作1次,藥物調(diào)整為奧卡西平0.3 g，2次/d,開普蘭0.5 g,2次/d。

圖1 額葉癲發(fā)作期面部體征

圖2 額葉癲發(fā)作期腦電圖改變

2 討 論

[1] Zouhayr S, Elisabeth L, Charles M. Francine Chassoux Neural network underlying ictal pouting (“ chapeau de gendarme” ) in frontal lobe epilepsy[J]. Epilepsy, 2014,37：249-257.

[2] Bancaud J, Talairach J. Clinical semiology of frontal lobe seizures[J]. Adv Neurol, 1992,57:3-58.

[3] Chauvel P, Kliemann F, Vignal J P. The clinical signs and symptoms of frontal lobe epilepsy[J]. Phenomenology and classification Adv Neurol, 1995,66:115-126.

[4] Jobst B C, Siegel A M, Thadani V M. Intractable seizures of frontal lobe origin: clinical characteristics, localizing signs, and results of surgery[J]. Epilepsia, 2000,41:1139-1152.

[5] Kotagal P, Arunkjmar G, Hammel J. Complex partial seizures of frontal lobe onset statistical analysis of ictal semiology[J]. Seizure, 2003,12:268-281.

[6] Bush G, Luu P, Posner M I. Cognitive and emotional influences in anterior cingulated cortex[J]. Trends Cogn Sci, 2000,4:215-222.

[7] Craig A D. How do you feel? Interoception: the sense of the physiological condition of the body[J]. Nat Rev Neurosci, 2002,3:655-666.

[8] Biraben A, Taussig D, Thomas P,etal. Fear as themain feature of epileptic seizures[J]. J Neurol Neurosurg Psychiatry, 2001,70:186-191.

[9] Toyoda H, Li X Y,Wu L J,etal. Interplay of amygdala and cingulate plasticity in emotional fear[J]. Neural Plast, 2011:813749.

[10] Tettamanti M, Rognoni E, Cafiero R. Distinct pathways of neural coupling for different basic emotions[J]. Neuroimage,2012,59:1804-1817.

[11] Margulies D S. Clare Kelly A M, Uddin L Q.Milhama MP Mapping the functional connectivity of anterior cingulate cortex[J]. NeuroImage, 2007, 37:579-588.

國(guó)家自然科學(xué)基金(30800366，81571267)，首都發(fā)展基金專項(xiàng)(2016-2-2013)，首都臨床特色(Z121107001012108)

樊麗娟，碩士，主治醫(yī)師。

1.100037，武警北京總隊(duì)第二醫(yī)院腦電圖室；2.100053 北京，首都醫(yī)科大學(xué)宣武醫(yī)院神經(jīng)內(nèi)科

R742.1

(2017-03-21收稿 2017-06-10修回)

(責(zé)任編輯 梁秋野)