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临床研究发现该药能明显改善消化不良症状，但因心脏的副作用，故应用受到限制。 | [
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（4）红霉素：虽为抗生素，也是胃动素激动剂，可增加胃近端和远端收缩活力，促进胃推进性蠕动，加速空腹和餐后胃排空，可用于FD小儿。 | [
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3.胃黏膜保护剂这类药物主要有硫糖铝、米索前列醇、恩前列素及蒙脱石散等。 | [
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临床上这类药物的应用主要是由于功能性消化不良的发病可能与慢性胃炎有关，患者可能存在胃黏膜屏障功能的减弱。 | [
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4.5-HT3受体拮抗剂和阿片类受体激动剂这两类药物促进胃排空的作用很弱，用于治疗功能性消化不良患者的原理是调节内脏感觉阈。 | [
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但此类药在儿科中尚无用药经验。 | [
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5.抗焦虑药国内有人使用小剂量多虑平和多潘立酮结合心理疏导治疗功能性消化不良患者，发现对上腹痛嗳气等症状有明显的缓解作用，较之不使用多虑平的患者有明显提高。 | [
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因此，在对FD的治疗中，利用药物对心理障碍进行治疗有一定的临床意义。 | [
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第五章癫痫癫痫（epilepsy）是由多种病因引起的慢性脑部疾患，以脑部神经元过度放电所致的突然、反复和短暂的中枢神经系统功能失常为特征。 | [
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根据所侵犯神经元的部位和发放的范围，可表现为运动、感觉、意识、行为及自主神经功能等不同脑功能障碍。 | [
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2005年国际抗癫痫联盟（ILAE）对癫痫推荐的定义为：癫痫是一种脑部疾患，其特点是持续存在能产生癫痫发作的脑部持久性改变，并出现相应的神经生物学、认知、心理学以及社会学等方面的后果。 | [
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【流行病学】我国癫痫的年发病率30/10万，以此推断，每年我国新发癫痫在40万例左右；我国癫痫的患病率（又称现患率）一般在4‰～7‰左右，由此推算，我国应有600万左右的癫痫患者。 | [
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据世界各国流行病学调查，癫痫发病率差异很大，多数结果表明癫痫的年发病率为24/10万～53/10万之间，多数发展中国家癫痫发病率高于发达国家；世界卫生组织估计，全球大约有5000万癫痫患者。 | [
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所以癫痫是一世界范围常见病和多发病，也是小儿神经系统的常见病。 | [
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【病因】癫痫的病因复杂多样，构成癫痫发作的因素包括遗传因素、脑内致痫性损伤因素以及诱发性因素等，不同的年龄往往有不同的病因范围。 | [
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EEG背景波正常，呈特定部位局限性或双侧对称同步痫样放电。 | [
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原发性癫痫是癫痫遗传学研究的主要对象，现在的研究显示，特发性癫痫多为中枢神经系统的离子通道异常。 | [
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（二）症状性（symptomatic）指能找到明确病因的癫痫，包括脑结构异常或者影响脑功能的各种因素。 | [
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这类癫痫可有多种形式的临床发作，除有局限性脑电异常外，EEG背景波多异常，并有大量的痫样发电。 | [
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随着医学的进步与检查手段的不断发展和丰富，能够寻找到病因的癫痫病例越来越多。 | [
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【发病机制】癫痫的发病机制虽然有许多进展，但没有一种能解释全部的癫痫发作，多数认为不同癫痫有着不同的发病机制。 | [
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神经元的高度同步化发放是癫痫发作的特征，其产生的条件涉及一系列生化、免疫以及遗传等方面的变化。 | [
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（一）生化方面如引起神经元去极化而发生兴奋性突触后电位的兴奋性氨基酸（谷氨酸、天冬氨酸及其受体激动剂N甲基天冬氨酸、红藻氨酸和使君子氨酸等）活力增加；引起神经元超级化而发生抑制性突触后电位的抑制性氨基酸（γ-氨基丁酸、牛磺酸、甘氨酸、5-羟色胺及去甲肾上腺素等）活力减弱，γ-氨基丁酸受体减少均可使细胞兴奋性增强；脑部活性自由基（、QH-</sup>、H2</sub>O2</sub>及NO等）增多对机体细胞的毒性作用；钙通道开放致Ca2+</sup>异常内流以及细胞内Ca2+</sup>结合蛋白减少等，使细胞内Ca2+积蓄，造成细胞坏死。 | [
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（二）免疫方面免疫的异常如细胞免疫功能低下；体液免疫中IgA等的缺乏，抗脑抗体的产生均是癫痫发作的潜在原因。 | [
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（三）遗传方面遗传因素是导致癫痫、尤其是经典的特发性癫痫的重要原因。 | [
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分子遗传学研究发现，大部分遗传性癫痫的分子机制为离子通道或相关分子的结构或功能改变。 | [
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到目前为止部分单基因及多基因遗传性癫痫的致病基因已明确（详见癫痫综合征内容介绍）。 | [
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【癫痫发作分类】对癫痫发作进行分类，有助于临床上对抗癫痫药物的选择以及对不同发作药物疗效的评估；有助于研究发作症状学与脑结构系统之间的关系。 | [
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癫痫的分类一直繁多，目前神经科沿用的分类是国际抗癫痫联盟（ILAE）1981年提出的“癫痫发作分类”，依据临床发作形式和脑电图改变分类；1989年“癫痫与癫痫综合征的分类”，除依据临床发作形式及脑电图改变外，还结合发病年龄、病因及转归。 | [
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"label": "dis"
},
{
"id": 4,
"entity": "癫痫综合征",
"start_offset": 77,
"end_offset": 82,
"label": "dis"
},
{
"id": 5,
"entity": "脑电图",
"start_offset": 97,
"end_offset": 100,
"label": "pro"
}
] |
2001年国际抗癫痫联盟关于癫痫发作和对癫痫诊断的建议，其中关于对癫痫发作的类型（表16-4），癫痫和癫痫综合征新的分类（表16-16）。 | [
{
"id": 0,
"entity": "癫痫",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
},
{
"id": 1,
"entity": "癫痫",
"start_offset": 48,
"end_offset": 50,
"label": "dis"
},
{
"id": 2,
"entity": "癫痫综合征",
"start_offset": 51,
"end_offset": 56,
"label": "dis"
}
] |
表16-2癫痫发作分类（1981年）表16-3癫痫与癫痫综合征分类（1989年）表16-4癫痫发作类型（2001年）表16-5癫痫综合征分类（2001年）【临床表现】（一）部分性发作（partialseizures）部分性发作的临床与脑电图异常放电局限在脑某一部位或从某一局部开始。 | [
{
"id": 0,
"entity": "脑电图异常放电",
"start_offset": 117,
"end_offset": 124,
"label": "sym"
}
] |
发作时不伴意识障碍为简单部分性发作；伴有意识障碍为复杂部分性发作；部分性发作也可泛化为全面性发作，而且脑电图由局部放电演变为全脑性放电。 | [
{
"id": 0,
"entity": "意识障碍为复杂部分性发作",
"start_offset": 20,
"end_offset": 32,
"label": "sym"
}
] |
1.简单部分性发作（simplepartialseizures）发作开始意识多不丧失，最初发作表现可反映癫痫起源的脑区。 | [
{
"id": 0,
"entity": "癫痫",
"start_offset": 52,
"end_offset": 54,
"label": "dis"
}
] |
（1）运动性症状：包括：①仅为局灶性运动症状，多为阵挛性发作，任何部位都可以出现局灶性抽搐；②Jackson发作，即发作从一侧口角开始，依次波及手、臂和肩等；③偏转性发作，眼、头甚至躯干向一侧偏转；④姿势性发作，表现为某种特殊姿势，如击剑样姿势；⑤抑制性运动发作，发作时动作停止，语言中断，意识不丧失；⑥发音性发作，表现为重复语言或言语中断；⑦半侧发作（2）感觉症状：包括：①躯体感觉性发作（麻木及疼痛等）；②特殊感觉异常（视、听、嗅和味）及幻觉；③眩晕性发作。 | [
{
"id": 0,
"entity": "阵挛性发作",
"start_offset": 25,
"end_offset": 30,
"label": "sym"
},
{
"id": 1,
"entity": "局灶性抽搐",
"start_offset": 40,
"end_offset": 45,
"label": "sym"
},
{
"id": 2,
"entity": "眼、头甚至躯干向一侧偏转",
"start_offset": 86,
"end_offset": 98,
"label": "sym"
},
{
"id": 3,
"entity": "动作停止",
"start_offset": 135,
"end_offset": 139,
"label": "sym"
},
{
"id": 4,
"entity": "语言中断",
"start_offset": 140,
"end_offset": 144,
"label": "sym"
},
{
"id": 5,
"entity": "意识不丧失",
"start_offset": 145,
"end_offset": 150,
"label": "sym"
},
{
"id": 6,
"entity": "重复语言",
"start_offset": 161,
"end_offset": 165,
"label": "sym"
},
{
"id": 7,
"entity": "言语中断",
"start_offset": 166,
"end_offset": 170,
"label": "sym"
},
{
"id": 8,
"entity": "躯体感觉性发作",
"start_offset": 188,
"end_offset": 195,
"label": "sym"
},
{
"id": 9,
"entity": "麻木",
"start_offset": 196,
"end_offset": 198,
"label": "sym"
},
{
"id": 10,
"entity": "疼痛",
"start_offset": 199,
"end_offset": 201,
"label": "sym"
},
{
"id": 11,
"entity": "特殊感觉异常",
"start_offset": 205,
"end_offset": 211,
"label": "sym"
},
{
"id": 12,
"entity": "幻觉",
"start_offset": 221,
"end_offset": 223,
"label": "sym"
},
{
"id": 13,
"entity": "眩晕性发作",
"start_offset": 225,
"end_offset": 230,
"label": "sym"
}
] |
（3）自主神经性症状：包括：胃部不适症状、潮红、苍白、冷汗、心悸、竖毛肌收缩以及瞳孔散大（4）精神症状：常见于复杂部分性发作，包括认知障碍、记忆力障碍、情感问题（恐惧和愤怒）、错觉（视物变大和变小）及幻觉。 | [
{
"id": 0,
"entity": "胃部",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "潮红",
"start_offset": 21,
"end_offset": 23,
"label": "sym"
},
{
"id": 2,
"entity": "苍白",
"start_offset": 24,
"end_offset": 26,
"label": "sym"
},
{
"id": 3,
"entity": "冷汗",
"start_offset": 27,
"end_offset": 29,
"label": "sym"
},
{
"id": 4,
"entity": "心悸",
"start_offset": 30,
"end_offset": 32,
"label": "sym"
},
{
"id": 5,
"entity": "竖毛肌收缩",
"start_offset": 33,
"end_offset": 38,
"label": "sym"
},
{
"id": 6,
"entity": "瞳孔散大",
"start_offset": 40,
"end_offset": 44,
"label": "sym"
},
{
"id": 7,
"entity": "认知障碍",
"start_offset": 65,
"end_offset": 69,
"label": "sym"
},
{
"id": 8,
"entity": "记忆力障碍",
"start_offset": 70,
"end_offset": 75,
"label": "sym"
},
{
"id": 9,
"entity": "情感问题（恐惧和愤怒）",
"start_offset": 76,
"end_offset": 87,
"label": "sym"
},
{
"id": 10,
"entity": "错觉",
"start_offset": 88,
"end_offset": 90,
"label": "sym"
},
{
"id": 11,
"entity": "幻觉",
"start_offset": 100,
"end_offset": 102,
"label": "sym"
}
] |
2.复杂部分性发作（complexpartialseizures）有意识障碍、发作性感知觉障碍以及梦游状态等。 | [
{
"id": 0,
"entity": "意识障碍",
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"label": "sym"
},
{
"id": 1,
"entity": "发作性感知觉障碍",
"start_offset": 39,
"end_offset": 47,
"label": "sym"
},
{
"id": 2,
"entity": "梦游",
"start_offset": 49,
"end_offset": 51,
"label": "sym"
}
] |
常有“自动症”，是意识障碍下的不自主动作，表现为口咽自动症、姿势自动症、手部自动症、行走自动症和言语自动症。 | [
{
"id": 0,
"entity": "“自动症”",
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{
"id": 1,
"entity": "不自主动作",
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"label": "sym"
},
{
"id": 2,
"entity": "口咽自动症",
"start_offset": 24,
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"label": "sym"
},
{
"id": 3,
"entity": "姿势自动症",
"start_offset": 30,
"end_offset": 35,
"label": "sym"
},
{
"id": 4,
"entity": "手部自动症",
"start_offset": 36,
"end_offset": 41,
"label": "sym"
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{
"id": 5,
"entity": "行走自动症",
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"label": "sym"
},
{
"id": 6,
"entity": "言语自动症",
"start_offset": 48,
"end_offset": 53,
"label": "sym"
}
] |
复杂部分性发作可从单纯部分性发作开始，随后出现意识障碍，也可从开始即有意识障碍。 | [
{
"id": 0,
"entity": "意识障碍",
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"label": "sym"
}
] |
可见于颞叶或额叶起源的癫痫。 | [
{
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"entity": "颞叶",
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"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "额叶",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "癫痫",
"start_offset": 11,
"end_offset": 13,
"label": "dis"
}
] |
EEG在发作时有颞、额区局灶性放电。 | [
{
"id": 0,
"entity": "颞",
"start_offset": 8,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "额",
"start_offset": 10,
"end_offset": 11,
"label": "bod"
}
] |
3.部分性发作继发为全身性发作小婴儿部分性发作时由于难以确定婴儿发作时的意识水平，往往表现为：①反应性降低：动作突然减少或停止，无动性凝视或茫然，有人称为“颞叶假性失神”（temporalpseudoabsence）或“额叶失神”（frontalabsence），但不是真正的失神发作。 | [
{
"id": 0,
"entity": "反应性降低",
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"end_offset": 53,
"label": "sym"
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{
"id": 1,
"entity": "动作突然减少或停止",
"start_offset": 54,
"end_offset": 63,
"label": "sym"
},
{
"id": 2,
"entity": "无动性凝视",
"start_offset": 64,
"end_offset": 69,
"label": "sym"
},
{
"id": 3,
"entity": "茫然",
"start_offset": 70,
"end_offset": 72,
"label": "sym"
}
] |
②自动症：常见为口部的简单自动症（如咂嘴、咀嚼、吞咽及吸吮等较原始的动作）；或躯干肢体无目的不规则运动，与正常运动很相似。 | [
{
"id": 0,
"entity": "自动症",
"start_offset": 1,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "咂嘴",
"start_offset": 18,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "咀嚼",
"start_offset": 21,
"end_offset": 23,
"label": "sym"
},
{
"id": 3,
"entity": "吞咽",
"start_offset": 24,
"end_offset": 26,
"label": "sym"
},
{
"id": 4,
"entity": "吸吮",
"start_offset": 27,
"end_offset": 29,
"label": "sym"
},
{
"id": 5,
"entity": "躯干肢体无目的不规则运动",
"start_offset": 39,
"end_offset": 51,
"label": "sym"
}
] |
2001年的癫痫发作分类不同于1981年的发作分类，要点包括：①将癫痫发作分为自限性和持续性，在这两种发作的范畴内，又分为全面性和局灶性两类；②在局灶性发作中不再分为单纯性和复杂性；③在“局灶性感觉性发作”及“局灶性运动性发作”，不再承认有“自主神经症状”，自主神经症状多为癫痫发作伴随现象；④发作的类型明显增多。 | [
{
"id": 0,
"entity": "癫痫",
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"end_offset": 139,
"label": "dis"
}
] |
（二）全身性发作全身性常有意识障碍，运动性症状是对称性的，脑电图上表现两侧大脑半球广泛性放电。 | [
{
"id": 0,
"entity": "脑电图",
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"label": "pro"
}
] |
1.强直-阵挛性发作（tonic-clonicseizures）发作时突然意识丧失，瞳孔散大，全身肌肉强直或阵挛或强直-阵挛性收缩。 | [
{
"id": 0,
"entity": "强直-阵挛性发作",
"start_offset": 2,
"end_offset": 10,
"label": "dis"
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{
"id": 1,
"entity": "tonic-clonicseizures",
"start_offset": 11,
"end_offset": 31,
"label": "dis"
},
{
"id": 2,
"entity": "意识丧失",
"start_offset": 37,
"end_offset": 41,
"label": "sym"
},
{
"id": 3,
"entity": "瞳孔散大",
"start_offset": 42,
"end_offset": 46,
"label": "sym"
},
{
"id": 4,
"entity": "全身肌肉强直",
"start_offset": 47,
"end_offset": 53,
"label": "sym"
},
{
"id": 5,
"entity": "阵挛",
"start_offset": 54,
"end_offset": 56,
"label": "sym"
},
{
"id": 6,
"entity": "强直-阵挛性收缩",
"start_offset": 57,
"end_offset": 65,
"label": "sym"
}
] |
阵挛发作是指肢体及躯干呈有节律性重复的收缩为特征。 | [
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"id": 0,
"entity": "肢体及躯干呈有节律性重复的收缩",
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"label": "sym"
}
] |
EEG特征表现为背景活动正常或非特异性异常，发作间期异常波在两半球可见棘波、尖波、棘慢波和多棘波等；发作期EEG强直期以10～20Hz节律性棘波发放开始，波幅渐高而频率渐慢；发作结束后可见弥漫性慢波活动，逐渐恢复背景活动。 | [
{
"id": 0,
"entity": "EEG",
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{
"id": 1,
"entity": "棘波",
"start_offset": 35,
"end_offset": 37,
"label": "sym"
},
{
"id": 2,
"entity": "尖波",
"start_offset": 38,
"end_offset": 40,
"label": "sym"
},
{
"id": 3,
"entity": "EEG",
"start_offset": 53,
"end_offset": 56,
"label": "pro"
},
{
"id": 4,
"entity": "波幅渐高而频率渐慢",
"start_offset": 77,
"end_offset": 86,
"label": "sym"
}
] |
2.肌阵挛发作（myoclonicseizures）表现为某个或某组肌肉或肌群快速有力的收缩，不超过0.2秒，抽动后肢体或躯干立即恢复原来的姿势（状态），屈肌比伸肌更易受累，上肢明显。 | [
{
"id": 0,
"entity": "肌阵挛发作",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "myoclonicseizures",
"start_offset": 8,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "某个或某组肌肉或肌群快速有力的收缩",
"start_offset": 29,
"end_offset": 46,
"label": "sym"
}
] |
婴儿期肌阵挛的特点有2种：①全身性粗大肌阵挛，表现为躯干、颈部以及四肢近端突然猛烈抽动，动作幅度大、孤立的或连续的。 | [
{
"id": 0,
"entity": "全身性粗大肌阵挛",
"start_offset": 14,
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"label": "sym"
},
{
"id": 1,
"entity": "躯干",
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"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "颈部",
"start_offset": 29,
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"label": "bod"
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{
"id": 3,
"entity": "四肢近端",
"start_offset": 33,
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"label": "bod"
},
{
"id": 4,
"entity": "躯干、颈部以及四肢近端突然猛烈抽动",
"start_offset": 26,
"end_offset": 43,
"label": "sym"
}
] |
EEG表现为高波幅多棘慢波爆发，或突然广泛低电压。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
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{
"id": 1,
"entity": "高波幅多棘慢波爆发",
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{
"id": 2,
"entity": "突然广泛低电压",
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"label": "sym"
}
] |
②散在游走性肌阵挛，表现为四肢远端、面部小组肌群幅度较小的抽动，多部位游走性，EEG为持续性弥漫性慢波多灶性棘波、尖波。 | [
{
"id": 0,
"entity": "四肢远端、面部小组肌群幅度较小的抽动",
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},
{
"id": 1,
"entity": "多部位游走性",
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"label": "sym"
},
{
"id": 2,
"entity": "EEG",
"start_offset": 39,
"end_offset": 42,
"label": "pro"
},
{
"id": 3,
"entity": "持续性弥漫性慢波多灶性棘波、尖波",
"start_offset": 43,
"end_offset": 59,
"label": "sym"
}
] |
3.失张力发作表现为突然发生的肌张力减低或丧失，不能维持原来的姿势，导致突然跌倒或姿势不稳。 | [
{
"id": 0,
"entity": "肌张力减低或丧失",
"start_offset": 15,
"end_offset": 23,
"label": "sym"
},
{
"id": 1,
"entity": "不能维持原来的姿势",
"start_offset": 24,
"end_offset": 33,
"label": "sym"
},
{
"id": 2,
"entity": "突然跌倒或姿势不稳",
"start_offset": 36,
"end_offset": 45,
"label": "sym"
}
] |
有时发作时间短暂，在未摔倒在地时意识已恢复，可立即站起；长时间的失张力发作可持续一至数分钟，表现全身松软，凝视，但无运动性症状。 | [
{
"id": 0,
"entity": "时间短暂",
"start_offset": 4,
"end_offset": 8,
"label": "sym"
},
{
"id": 1,
"entity": "全身松软",
"start_offset": 48,
"end_offset": 52,
"label": "sym"
},
{
"id": 2,
"entity": "凝视",
"start_offset": 53,
"end_offset": 55,
"label": "sym"
}
] |
EEG发作间期和发作期可表现为全导棘慢波或多棘慢波发放；发作期还可表现为低波幅或高波幅快活动和弥漫性低电压。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "全导棘慢波或多棘慢波发放",
"start_offset": 15,
"end_offset": 27,
"label": "sym"
},
{
"id": 2,
"entity": "低波幅或高波幅快活动",
"start_offset": 36,
"end_offset": 46,
"label": "sym"
},
{
"id": 3,
"entity": "弥漫性低电压",
"start_offset": 47,
"end_offset": 53,
"label": "sym"
}
] |
4.失神发作分为典型失神和不典型失神，典型失神主要见于儿童失神癫痫和青少年失神癫痫；不典型失神主要见于Lennox-Gastaut综合征，也可见于其他儿童癫痫综合征。 | [
{
"id": 0,
"entity": "儿童失神癫痫",
"start_offset": 27,
"end_offset": 33,
"label": "dis"
},
{
"id": 1,
"entity": "青少年失神癫痫",
"start_offset": 34,
"end_offset": 41,
"label": "dis"
},
{
"id": 2,
"entity": "Lennox-Gastaut综合征",
"start_offset": 51,
"end_offset": 68,
"label": "dis"
},
{
"id": 3,
"entity": "儿童癫痫综合征",
"start_offset": 75,
"end_offset": 82,
"label": "dis"
}
] |
（三）癫痫综合征不同年龄段常见的癫痫综合征的诊断要点介绍如下。 | [
{
"id": 0,
"entity": "癫痫综合征",
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] |
1.良性家族性新生儿惊厥为常染色体显性遗传，往往有惊厥家族史，基因定位多位于20q13.2，少数定位于8q染色体上，致病基因为KCNQ2和KCNQ3。 | [
{
"id": 0,
"entity": "良性家族性新生儿惊厥",
"start_offset": 2,
"end_offset": 12,
"label": "dis"
}
] |
生后2～3天内发病，惊厥形式以阵挛为主，可以表现为某一肢体或面部抽动，也可表现为全身阵挛；少数表现为广泛性强直。 | [
{
"id": 0,
"entity": "阵挛",
"start_offset": 15,
"end_offset": 17,
"label": "sym"
},
{
"id": 1,
"entity": "某一肢体或面部抽动",
"start_offset": 25,
"end_offset": 34,
"label": "sym"
},
{
"id": 2,
"entity": "阵挛",
"start_offset": 42,
"end_offset": 44,
"label": "sym"
},
{
"id": 3,
"entity": "广泛性强直",
"start_offset": 50,
"end_offset": 55,
"label": "sym"
}
] |
有时表现为呼吸暂停，发作频繁，发作持续时间较短。 | [
{
"id": 0,
"entity": "频繁",
"start_offset": 12,
"end_offset": 14,
"label": "sym"
}
] |
从病史及体格检查中找不到病因，脑电图无特殊异常，生化检查及神经影像学检查均正常。 | [
{
"id": 0,
"entity": "脑电图",
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"end_offset": 18,
"label": "pro"
},
{
"id": 1,
"entity": "神经影像学",
"start_offset": 29,
"end_offset": 34,
"label": "pro"
}
] |
2.良性新生儿惊厥本病遗传不明显。 | [
{
"id": 0,
"entity": "良性新生儿惊厥",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
}
] |
脑电图在发作间期常可见尖型θ波。 | [
{
"id": 0,
"entity": "可见尖型θ波",
"start_offset": 9,
"end_offset": 15,
"label": "sym"
}
] |
3.早发性肌阵挛脑病生后第1天或数天以内起病；主要表现为难治性频繁的肌阵挛发作；脑电图也表现为暴发抑制波形；本病可能与遗传代谢障碍有关，而无明显的神经影像学异常；本病预后不良，多数早期死亡。 | [
{
"id": 0,
"entity": "早发性肌阵挛脑病",
"start_offset": 2,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "难治性频繁的肌阵挛发作",
"start_offset": 28,
"end_offset": 39,
"label": "sym"
},
{
"id": 2,
"entity": "神经影像",
"start_offset": 73,
"end_offset": 77,
"label": "pro"
}
] |
4.大田原综合征生后3个月以内发病，多在1个月之内起病；主要为强直痉挛性发作；脑电图表现为暴发抑制波形；常见病因为脑部结构异常，也有隐源性病因。 | [
{
"id": 0,
"entity": "大田原综合征",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "强直痉挛性发作",
"start_offset": 31,
"end_offset": 38,
"label": "sym"
},
{
"id": 2,
"entity": "脑电图",
"start_offset": 39,
"end_offset": 42,
"label": "pro"
}
] |
5.婴儿痉挛又称为West综合征，较常见的严重的癫痫综合征。 | [
{
"id": 0,
"entity": "婴儿痉挛",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "West综合征",
"start_offset": 9,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "癫痫综合征",
"start_offset": 24,
"end_offset": 29,
"label": "dis"
}
] |
EEG表现为高度失律，各导联见到不规则、杂乱、不对称、高波幅慢波、棘波、尖波及多棘慢波。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "高度失律",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "各导联见到不规则、杂乱、不对称",
"start_offset": 11,
"end_offset": 26,
"label": "sym"
}
] |
引起本病的继发性原因多种多样，如脑发育障碍所致的各种畸形、宫内感染、围生期脑损伤，核黄疸、免疫缺陷、代谢异常、生后感染、窒息以及染色体异常等因素，均可引起本病。 | [
{
"id": 0,
"entity": "畸形",
"start_offset": 26,
"end_offset": 28,
"label": "sym"
},
{
"id": 1,
"entity": "宫内感染",
"start_offset": 29,
"end_offset": 33,
"label": "sym"
},
{
"id": 2,
"entity": "围生期脑损伤",
"start_offset": 34,
"end_offset": 40,
"label": "sym"
},
{
"id": 3,
"entity": "核黄疸",
"start_offset": 41,
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"label": "sym"
},
{
"id": 4,
"entity": "免疫缺陷",
"start_offset": 45,
"end_offset": 49,
"label": "sym"
},
{
"id": 5,
"entity": "代谢异常",
"start_offset": 50,
"end_offset": 54,
"label": "sym"
},
{
"id": 6,
"entity": "生后感染",
"start_offset": 55,
"end_offset": 59,
"label": "sym"
},
{
"id": 7,
"entity": "窒息",
"start_offset": 60,
"end_offset": 62,
"label": "sym"
},
{
"id": 8,
"entity": "染色体异常",
"start_offset": 64,
"end_offset": 69,
"label": "sym"
}
] |
其中，10%为结节性硬化。 | [
{
"id": 0,
"entity": "结节性硬化",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
}
] |
本病常合并严重的智力倒退或运动发育落后，多数病儿转变为其他形式的发作，特别以Lennox-Gastaut综合征最为多见。 | [
{
"id": 0,
"entity": "Lennox-Gastaut综合征",
"start_offset": 38,
"end_offset": 55,
"label": "dis"
}
] |
6.婴儿良性肌阵挛癫痫6个月～2岁间发病，患儿神经发育正常；发作表现为全身肌阵挛；EEG发作期表现为弥漫性棘慢波或多棘慢波，发作间期常无异常放电；以后良好。 | [
{
"id": 0,
"entity": "婴儿良性肌阵挛癫痫",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
}
] |
7.婴儿重症肌阵挛癫痫1978年Dravet首次描述本病，目前明确其致病基因为SCN1A。 | [
{
"id": 0,
"entity": "婴儿重症肌阵挛癫痫",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
}
] |
8.Lennox-Gastaut综合征1～8岁发病，临床发作形式多样性是本综合征的特点，如强直发作、不典型失神、失张力发作和肌阵挛发作，患儿可同时存在几种发作形式，也可由一种形式转变为另一种形式；EEG在发作间期表现为全导0.5～2.5Hz慢的棘慢波。 | [
{
"id": 0,
"entity": "Lennox-Gastaut综合征",
"start_offset": 2,
"end_offset": 19,
"label": "dis"
},
{
"id": 1,
"entity": "强直发作",
"start_offset": 45,
"end_offset": 49,
"label": "sym"
},
{
"id": 2,
"entity": "不典型失神",
"start_offset": 50,
"end_offset": 55,
"label": "sym"
},
{
"id": 3,
"entity": "失张力发作",
"start_offset": 56,
"end_offset": 61,
"label": "sym"
},
{
"id": 4,
"entity": "肌阵挛发作",
"start_offset": 62,
"end_offset": 67,
"label": "sym"
},
{
"id": 5,
"entity": "EEG",
"start_offset": 98,
"end_offset": 101,
"label": "pro"
}
] |
9.肌阵挛-站立不能发作癫痫又称Doose综合征，都有遗传因素。 | [
{
"id": 0,
"entity": "肌阵挛-站立不能发作癫痫",
"start_offset": 2,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "Doose综合征",
"start_offset": 16,
"end_offset": 24,
"label": "dis"
}
] |
临床发作以肌阵挛-站立不能发作为特征性表现，表现为点头、弯腰以及两臂上举，常有跌倒，不能站立。 | [
{
"id": 0,
"entity": "点头",
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"end_offset": 27,
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},
{
"id": 1,
"entity": "弯腰",
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"end_offset": 30,
"label": "sym"
},
{
"id": 2,
"entity": "两臂上举",
"start_offset": 32,
"end_offset": 36,
"label": "sym"
},
{
"id": 3,
"entity": "跌倒",
"start_offset": 39,
"end_offset": 41,
"label": "sym"
},
{
"id": 4,
"entity": "不能站立",
"start_offset": 42,
"end_offset": 46,
"label": "sym"
}
] |
EEG在发作期或发作期间均可见到不规则棘慢波或多棘慢波，背景波正常。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
}
] |
10.儿童良性癫痫伴有中央-颞区棘波是小儿癫痫中常见的一种类型，多在5～10岁间发病，本病与遗传有关，往往有癫痫家族史。 | [
{
"id": 0,
"entity": "儿童良性癫痫",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
}
] |
表现为口咽部感觉异常及运动性发作，随后出现半侧面部肌肉抽搐及同侧上下肢抽动，有时可发展为全身性抽动。 | [
{
"id": 0,
"entity": "口咽部感觉异",
"start_offset": 3,
"end_offset": 9,
"label": "sym"
},
{
"id": 1,
"entity": "运动性发作",
"start_offset": 11,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "半侧面部肌肉抽搐",
"start_offset": 21,
"end_offset": 29,
"label": "sym"
},
{
"id": 3,
"entity": "同侧上下肢抽动",
"start_offset": 30,
"end_offset": 37,
"label": "sym"
},
{
"id": 4,
"entity": "全身性抽动",
"start_offset": 44,
"end_offset": 49,
"label": "sym"
}
] |
11.具有枕区放电的小儿癫痫发病年龄多见于4～8岁，男孩略多于女孩。 | [
{
"id": 0,
"entity": "具有枕区放电的小儿癫痫",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
}
] |
发作可在清醒或入睡时，惊厥表现为半侧阵挛发作或扩展为全身强直-阵挛发作。 | [
{
"id": 0,
"entity": "半侧阵挛发作",
"start_offset": 16,
"end_offset": 22,
"label": "sym"
},
{
"id": 1,
"entity": "扩展为全身强直-阵挛发作",
"start_offset": 23,
"end_offset": 35,
"label": "sym"
}
] |
惊厥前部分病儿出现视觉症状，如一过性视力丧失，视野出现暗点及幻视等。 | [
{
"id": 0,
"entity": "一过性视力丧失",
"start_offset": 15,
"end_offset": 22,
"label": "sym"
},
{
"id": 1,
"entity": "视野出现暗点",
"start_offset": 23,
"end_offset": 29,
"label": "sym"
},
{
"id": 2,
"entity": "幻视",
"start_offset": 30,
"end_offset": 32,
"label": "sym"
}
] |
1/3病例发作后有头痛、恶心及呕吐。 | [
{
"id": 0,
"entity": "头痛",
"start_offset": 9,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "恶心",
"start_offset": 12,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "呕吐",
"start_offset": 15,
"end_offset": 17,
"label": "sym"
}
] |
EEG在发作间期表现为枕部和后颞部出现一侧或双侧高波幅棘波或尖波，这种异常放电睁眼时消失，闭眼后1～20秒重复出现。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "枕部",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "后颞部",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 3,
"entity": "枕部和后颞部出现一侧或双侧高波幅棘波或尖波",
"start_offset": 11,
"end_offset": 32,
"label": "sym"
}
] |
12.获得性失语性癫痫又称为Landau-Kleffner综合征，4～7岁发病最多，男孩多于女孩，发病前语言功能正常，听觉失认为特征，失语表现为能听见声音，但不能理解语言的含意，逐渐发展为语言表达障碍。 | [
{
"id": 0,
"entity": "获得性失语性癫痫",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "Landau-Kleffner综合征",
"start_offset": 14,
"end_offset": 32,
"label": "dis"
},
{
"id": 2,
"entity": "能听见声音，但不能理解语言的含意",
"start_offset": 72,
"end_offset": 88,
"label": "sym"
},
{
"id": 3,
"entity": "语言表达障碍",
"start_offset": 94,
"end_offset": 100,
"label": "sym"
}
] |
大约有一半病人首发症状是失语，另1/2病人首发症状为惊厥，惊厥为部分性发作或全身性发作；约有17%～25%病儿没有惊厥发作；2/3病人有明显的行为异常。 | [
{
"id": 0,
"entity": "失语",
"start_offset": 12,
"end_offset": 14,
"label": "sym"
},
{
"id": 1,
"entity": "惊厥",
"start_offset": 26,
"end_offset": 28,
"label": "sym"
},
{
"id": 2,
"entity": "惊厥",
"start_offset": 29,
"end_offset": 31,
"label": "dis"
},
{
"id": 3,
"entity": "部分性发作",
"start_offset": 32,
"end_offset": 37,
"label": "dis"
},
{
"id": 4,
"entity": "全身性发作",
"start_offset": 38,
"end_offset": 43,
"label": "dis"
},
{
"id": 5,
"entity": "惊厥",
"start_offset": 57,
"end_offset": 59,
"label": "dis"
}
] |
EEG背景波正常，一侧或双侧颞区阵发性高幅棘波、尖波或棘慢波，睡眠时异常放电明显增多。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "一侧或双侧颞区阵发性高幅棘波、尖波或棘慢波",
"start_offset": 9,
"end_offset": 30,
"label": "sym"
},
{
"id": 2,
"entity": "睡眠时异常放电明显增多",
"start_offset": 31,
"end_offset": 42,
"label": "sym"
}
] |
13.慢波睡眠中持续棘慢波的癫痫发病为年龄依赖性，多在3～10岁发病，临床上存在获得性认知功能障碍，80%～90%的患者有部分性或全面性发作。 | [
{
"id": 0,
"entity": "慢波睡眠中持续棘慢波的癫痫",
"start_offset": 3,
"end_offset": 16,
"label": "dis"
}
] |
EEG呈现慢波睡眠中持续性癫痫样放电。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
}
] |
14.儿童失神癫痫4～8岁起病，6～7岁发病最多，女孩多于男孩。 | [
{
"id": 0,
"entity": "儿童失神癫痫",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
}
] |
EEG表现为双侧对称、弥漫性高波幅每秒3次棘慢波。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "双侧对称",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "弥漫性高波幅每秒3次棘慢波",
"start_offset": 11,
"end_offset": 24,
"label": "sym"
}
] |
15.青少年失神癫痫青春期左右发病，7～17岁起病，发病年龄高峰在10～12岁，男女性别无差异，失神发作频率较少，不一定每天均有发作，多伴有全身强直-阵挛发作。 | [
{
"id": 0,
"entity": "青少年失神癫痫",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "全身强直-阵挛",
"start_offset": 70,
"end_offset": 77,
"label": "sym"
}
] |
EEG表现为对称的棘慢波，每秒3.5～4次，额部占优势。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "对称的棘慢波",
"start_offset": 6,
"end_offset": 12,
"label": "sym"
}
] |
16.少年肌阵挛癫痫青春期前后发病，男女性别无大差异。 | [
{
"id": 0,
"entity": "少年肌阵挛癫痫",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
}
] |
发作时主要表现为肌阵挛，突然发生肩外展、肘屈曲、屈髋、屈膝以及跌倒，常伴膈肌收缩，发作多在醒后不久发生。 | [
{
"id": 0,
"entity": "肌阵挛",
"start_offset": 8,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "肩外展",
"start_offset": 16,
"end_offset": 19,
"label": "sym"
},
{
"id": 2,
"entity": "肘屈曲",
"start_offset": 20,
"end_offset": 23,
"label": "sym"
},
{
"id": 3,
"entity": "屈髋",
"start_offset": 24,
"end_offset": 26,
"label": "sym"
},
{
"id": 4,
"entity": "屈膝",
"start_offset": 27,
"end_offset": 29,
"label": "sym"
},
{
"id": 5,
"entity": "跌倒",
"start_offset": 31,
"end_offset": 33,
"label": "sym"
},
{
"id": 6,
"entity": "膈肌收缩",
"start_offset": 36,
"end_offset": 40,
"label": "sym"
}
] |
也可能单个的发作或重复发作最后转为全身强直-阵挛发作。 | [
{
"id": 0,
"entity": "全身强直-阵挛",
"start_offset": 17,
"end_offset": 24,
"label": "sym"
}
] |
EEG为弥漫的每秒3～6次的棘慢波或多棘慢波。 | [
{
"id": 0,
"entity": "EEG",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
}
] |
17.觉醒时全身强直-阵挛癫痫多发生在10～20岁之间，16～17岁为高峰，本病有遗传倾向，大约10%病例有癫痫家族史。 | [
{
"id": 0,
"entity": "觉醒时全身强直-阵挛癫痫",
"start_offset": 3,
"end_offset": 15,
"label": "dis"
}
] |
EEG可见弥漫性异常放电，表现为棘慢合并失神棘慢波。 | [
{
"id": 0,
"entity": "合并失神",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
}
] |
18.肌阵挛性失神癫痫多有遗传背景，目前多考虑特发性的原因。 | [
{
"id": 0,
"entity": "肌阵挛性失神癫痫",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
}
] |
出生后数月以至青春期都可发病，发病高峰在7岁左右，以肌阵挛性失神为特征性表现，常伴有强直性收缩。 | [
{
"id": 0,
"entity": "肌阵挛性失神",
"start_offset": 26,
"end_offset": 32,
"label": "sym"
},
{
"id": 1,
"entity": "强直性收缩",
"start_offset": 42,
"end_offset": 47,
"label": "sym"
}
] |
19.Rsmussen综合征是一特殊的、主要影响一侧大脑半球伴有难治性部分性癫痫，进行性严重认知障碍与偏瘫发生，神经影像学早期正常，以后出现一侧大脑半球进行性萎缩，EEG呈现背景活动不对称慢波活动，一侧为主的癫痫样放电。 | [
{
"id": 0,
"entity": "Rsmussen综合征",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "难治性部分性癫痫",
"start_offset": 32,
"end_offset": 40,
"label": "dis"
},
{
"id": 2,
"entity": "一侧大脑半球进行性萎缩",
"start_offset": 70,
"end_offset": 81,
"label": "sym"
},
{
"id": 3,
"entity": "EEG",
"start_offset": 82,
"end_offset": 85,
"label": "pro"
}
] |
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