癫痫中的变色龙——颞叶癫痫

2018-12-10 陈丽 “神经科学”公众号

许多人对癫痫的认识还停留在“口吐白沫,抽搐”这样一些症状,殊不知癫痫发作不止是抽搐、意识丧失,还有很多发作形式,比如愣神、无缘无故闻到异常气味、突然不知身在何方、或者往事如新感等。这些发作形式,都与一种最变幻莫测的癫痫——颞叶癫痫相关。

许多人对癫痫的认识还停留在“口吐白沫,抽搐”这样一些症状,殊不知癫痫发作不止是抽搐、意识丧失,还有很多发作形式,比如愣神、无缘无故闻到异常气味、突然不知身在何方、或者往事如新感等。这些发作形式,都与一种最变幻莫测的癫痫——颞叶癫痫相关。

颞叶癫痫的症状有哪些?

颞叶癫痫(temporallobe epilepsy)是最常见的局灶性癫痫,依据发作起源分为颞叶内侧癫痫(mesial temporal lobe epilepsy, MTLE)和颞叶外侧癫痫(lateral temporal lobe epilepsy, LTLE),也称为颞叶新皮质癫痫。

夹于额叶和小脑幕之间的颞叶,其功能异常可出现以下症状:

感觉性失语:当优势半球颞上回后部病变可出现感觉性失语(Wernicke失语);

听觉障碍:听觉中枢位于颞上回和颞横回,此处病变可出现听幻觉、耳聋;

命名性失语:当优势半球的颞中回及颞下回损伤时可出现命名性失语只能说出物品的用途不能命名;

嗅觉与味觉障碍:嗅觉中枢位于海马回,味觉中枢与其相邻近,此处病变可出现嗅幻觉、味幻觉;

记忆障碍:颞叶前部病变时可产生记忆障碍;

音乐功能障碍:优势半球的颞上回前部为音乐中枢,此处病变可致听不懂音乐;

视野缺损:当颞叶深部病变损害视辐射腹侧纤维时,可产生病灶对侧同向性上象限盲;

眼球同向运动障碍:当颞上回的中后部病变,可发生眼球同向运动障碍。

颞叶内侧癫痫

颞叶内侧癫痫(MTLE)常有上腹部先兆,气上升感,体验性先兆(似曾相识感、陌生感),自动症、自主神经、精神症状、幻嗅、幻味。

为什么会出现以上症状呢?不得不提始作俑者的结构,海马和杏仁体。

海马不仅在学习记忆中起着重要作用,海马的传出纤维通过Papez环路构成边缘系统的一部分,通过海马旁回-海马结构-乳头体-丘脑前核-扣带回-海马旁回环路,形成一个学习记忆、情感等高级神经活动的往返环路,可调节情绪及躯体性反应。

杏仁体的传入纤维来自嗅脑、新皮质、隔核、背侧丘脑和下丘脑,传出纤维经终纹和腹侧杏仁体到隔区和下丘脑,主要参与认知记忆、多种记忆联系、内脏活动、内分泌活动、情绪活动等。

了解到海马和杏仁体的解剖及功能,那些奇奇怪怪的临床表现也就可以理解了。

颞叶外侧癫痫

颞叶外侧癫痫也称新皮质癫痫,与颞叶的功能有关,主要有以下特点:听觉先兆、视觉先兆、眩晕、涉及优势半球会有发作性言语障碍、声音不识、面孔不识、似曾相识等。

鉴别诊断

这么多的症状也可以见于其他疾病,比如短暂性脑缺血发作、低血糖症,晕厥等,怎么来鉴别呢?法宝之一就是脑电图。

病例一:老年男性,发作性的遗忘1年,早晨买菜时突然不知身在何方,约10分钟正常,返回菜市场寻找到自己的自行车。


病例二:老年女性,发作性头晕2月,意识丧失3天。患者在视频脑电监测过程中仅出现头晕,脑电图描记出一个局灶继发全面发作的脑电图。



病例三:中年男性,发作性意识不清6月,仔细追问病史有幻嗅、难以名状的“心里难受感”。在视频脑电描记出的发作波时,追问患者诉;心里有点难受”。




这三例患者的头颅核磁共振平扫都未见异常,理论上许多颞叶癫痫和海马硬化有关。

癫痫发作分类

那么,如何才能纲目分明地厘清癫痫发作和癫痫的最新分类呢?且看2017年癫痫发作和癫痫的国际分类:



(注意:新版分类中强调consciousness和awareness不同。前者可以翻译为意识,包括知觉、反应性、记忆和对自己的认知。后者可以翻译为知觉,指对自身和环境的知晓。需要注意的是,对于局灶性发作和全面性发作的区分,使用的是awareness,即知觉。)

小结

颞叶癫痫是所有癫痫中最具复杂发作形式的疾病,可称癫痫中的变色龙。在诊疗癫痫时,要充分考虑到这种复杂性,使患者得到更精准的诊断和治疗。

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    2019-01-25 zwjnj2

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    2018-12-11 youngstoner

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    2018-12-10 一个字-牛

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