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注意肺复张手法可能影响循环功能。
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(六)高频振荡通气(HFOV)目前缺乏可靠的HFOV治疗小儿ARDS临床治疗。
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一般高频振荡呼吸机对10kg体重以上小儿效率显著下降。
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对于10kg以下的婴幼儿,可以应用以改善持续低氧血症。
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高频通气优点为将闭陷小气道和肺泡持续扩张,避免肺泡承受高牵张剪切力作用,同时出现呼吸管道内气体的持续对流交换,因此达到高气体通气和换气效率并对肺组织具保护作用。
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一般不会对心血管功能产生严重影响。
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(七)其他治疗手段1.肺表面活性物质肺泡上皮细胞损伤会影响肺泡Ⅱ型细胞合成分泌表面活性物质及Ⅰ型细胞对水、盐电解质代谢和肺液转运。
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肺表面活性物质可以起到降低气液界面表面张力、减少呼吸作功、促进肺液吸收、防止血管通透增加的作用。
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应用指征为:在气道峰压>2.5kPa(25cmH<sub>2</sub>O),顺应性<0.5ml/(cmH<sub>2</sub>O•kg),氧合指数(OI)>10,且持续低氧血症不得改善(>6~12小时),排除由于呼吸机参数调节不当时,可以气道内滴入肺表面活性物质50~200mg/kg,必要时可以间隔6~12小时,再给予2~3次,每次100mg/kg,以获得迅速改善氧合、降低供氧浓度、缩短呼吸机治疗时间、提高存活率等效果。
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在有肺血管痉挛导致通气-灌流失调时,要考虑扩张肺血管措施。
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2.吸入一氧化氮(NO)吸入NO具有选择性扩张肺血管、降低肺动脉压和肺血管阻力、增加肺血流、改善通气-灌流作用。
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ARDS患儿如果并发肺动脉高压,可以用气道吸入NO治疗。
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起始浓度在10~20ppm,一般在5~30分钟内可以将动脉血氧分压提高到>7kPa(52mmHg),经皮氧饱和度>85%,然后用低NO(5~10ppm)持续维持治疗,将呼吸机参数逐渐下调,经1~3天后停止吸入NO,转回常规呼吸机治疗。
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在气道阻塞、大量肺泡内水肿渗出、肺泡严重萎陷时,吸入NO效果差,必要时可以将吸入NO与高频振荡通气、肺表面活性物质气道滴入、改变体位等手段联合应用,可以提高吸入NO治疗的效果。
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吸入NO治疗也适宜在ARDS早期开展,效果快且持久。
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3.糖皮质激素虽然持续的过度炎症反应和肺纤维化是导致晚期病情恶化和治疗困难的重要原因,但目前争论颇多,除了过敏、肾上腺皮质功能不全、感染性休克合并的ARDS,不宜常规使用糖皮质激素治疗,尤其是晚期ARDS。
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4.俯卧位通气处于平卧位的小儿肺的背部居下方,血流慢,肺泡含气量低,加上胸廓后肋活动限制,使充气困难,出现大部分肺通气-灌流障。
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如果改为俯卧位,可以有利于背部肺泡扩张,改善通气-灌流,提高血氧合。
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5.营养推荐的热量中葡萄糖不宜太高,因为可导致高碳酸血症。
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在急性期,一般不用脂肪乳剂,因其导致血液黏稠,肺部血流速度下降,影响通气-灌流;而在恢复期,可以采用脂肪乳剂,提高能量摄入,有利于肺组织细胞修复。
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适当摄入维生素A、D、E等可以强化上皮细胞抗过氧化损害、促进增生修复和功能恢复。
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在呼吸机治疗中,如果患儿情况稳定,应考虑采用经胃肠道营养支持,加速肺和机体复员。
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6.补液与输血应严格控制ARDS病例的液体摄入量。
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补液量保持在常规需要量的70%为宜,血容量和输液的平衡点为能够保持基础营养液量和有效心输出量。
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应用胶体液不一定有利于肺液吸收,尤其在血管通透性高时,大量蛋白会漏出到肺间质和肺泡内,吸收水分并妨碍液体清除。
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根据病情可以通过输入红细胞,提高血细胞比容达到32%~34%,或提供25%或5%清蛋白。
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7.利尿适当应用呋塞米(速尿)类药物利尿,可有利于肺水肿的改善,促进肺液吸收,减轻心脏后负荷。
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8.连续血液净化技术近年来,连续血液净化技术(包括连续静脉滤过、透析滤过、持续超滤等)在ARDS治疗中的应用逐步得到肯定。
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既有利于液体平衡的控制和药物的使用,更有利于降低致炎-抑炎的炎症反应强度,改善全身和肺局部的凝血机制。
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对于肾功能障碍者可以联合应用血液透析或腹膜透析技术,有利于排出代谢废物和细菌毒素,促进肺液吸收。
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9.液体通气技术(liquidventilation,LV)和体外膜肺技术(extracorporealmembraneoxygenation,ECMO)采用全氟化碳灌注肺部的液体通气技术在动物实验得到良好证实,临床应用也收到明显疗效,但儿童开展经验不多;ECMO技术在严重ARDS病例的使用已经得到证实和逐步推广,可采用VV模式,如合并循环功能不全,采用VA模式。
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【预后】小儿ARDS如果在感染性重症肺炎合并呼吸衰竭时,治愈的把握较大。
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但是,经化疗后的白血病患儿、肿瘤恶病质、小婴儿肺炎伴严重营养不良和免疫力低下、存在全身性结缔组织病、组织细胞增生症等,往往在肺部感染后出现ARDS,并难以用单纯呼吸机治疗和抗感染克服。
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且这些患儿可以因化疗导致骨髓造血干细胞抑制,临床上以外周白细胞明显减少为特点,不同于败血症时的肺部白细胞集聚而引起的外周白细胞减少症。
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因此,对于此类患儿重点为早期预防和治疗,并针对肺血管内微血栓形成等机制进行治疗,成为目前救治小儿ARDS的难题,有待攻克。
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参考文献1.SwartJF,WulffraatNM.Diagnosticworkupformixedconnectivetissuediseaseinchildhood.IsrMedAssoc,2008,10(8-8):650-6522.RichardJ.Mier,MichaelShishov,GloriaC,etal.Pediatriconsetmixedconnectivetissuedisease.RheumDisClinNAm,2005,31:483-4963.蒋明等.风湿病学.北京:科学出版社,19984.BehrmanRE,KliegmanRM,JensonHB.NelsonTextBookofPediatrics.16<sup>th</sup>ed.Philadelphia:W.B.SaunersCo,20005.杨锡强等.儿童免疫学.北京:人民卫生出版社,2001
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第九章抽动障碍【概述】(一)抽动与抽动障碍的定义1.抽动抽动(tic)一词是从法语Tique演变而来,原意为扁虱,用扁虱去叮咬牛马时出现的急促的皮肌收缩,用于表达原发性三叉神经痛时所伴随的面肌痉挛。
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抽动是一个形象的概念,并不是一个疾病的名称,有许多神经和精神病学者如Meige和Feindel等(1903),很早就开始研究“抽动”,但迄今仍很难相当完善、肯定和清楚地阐明“抽动”这一现象的本质。
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Lapouse等(1964)报道纽约的Buffalo区内,6~12岁的儿童有12%患过抽动。
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抽动被认为是固定或游走性的身体任何部位肌群出现不自主、无目的、重复和快速的收缩动作。
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抽动可以发生于身体某一部位的某一组肌肉,也可同时或先后出现在多个部位的多组肌肉;可以是连续性的天天出现,也可间断性发作。
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每一次抽动动作均急速完成,可重复出现,其表现十分类似。
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抽动有时可带有阵挛性,但无强直。
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其累及范围和频率因人而异,可以有急速挤眉、瞬目、撅嘴、转颈及耸肩等,也可以有躯干的急速抖动或扭转,喉部的抽动出现如清喉时发出“哼”音、其他怪声或秽语。
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抽动具有多变性的特点,即抽动并不固定在一个部位,如运动性抽动的分布通常起始于头面部肌肉,可出现眨眼、摇头及扮鬼脸等动作;随着病情进展,抽动逐渐累及身体各部位,分布的模式不同,也无一定规律性。
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当情绪紧张、焦虑时,抽动频率增加、强度增大;而当注意力集中、意志控制时,抽动可减少或短时间消失。
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2.抽动障碍抽动障碍(ticdisorders,TD)首先由Itard(1825)和GillesdelaTourette(1885)描述,20世纪60年代以前一直视为原因不明、罕见的可自愈性疾病。
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然而,近40多年来,抽动障碍的认识发生了戏剧性变化,特别是20世纪90年代以来,普遍认为抽动障碍非常多见,是一种于儿童和青少年时期起病、由遗传缺陷和不良环境因素所致的神经精神发育障碍,具有复杂异质性。
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不仅表现有抽动,还有较多的行为和精神症状。
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抽动症状可以时轻时重,呈波浪式进展,间或静止一段时间;新的抽动症状可以代替旧的抽动症状,或在原有抽动症状的基础上出现新的抽动症状。
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所有形式的抽动都可因应激、焦虑、疲劳、兴奋以及感冒发热而加重,都可因放松、全身心投入某事而减轻,睡眠时可消失。
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抽动障碍伴发的注意缺陷多动障碍(ADHD)、强迫障碍(OCD)、睡眠障碍(SD)、情绪障碍(ED)以及其他心理行为问题,增加了病情的严重性和复杂性,也给治疗带来一定的难度。
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3.更新观念我国医学界对此病认识仍很混乱,把这些儿童的表现当成“坏毛病”、“沙眼”、“结膜炎”,以及“咽炎”等现象极为普遍;即使能识别者也常因持有“可自愈性”的观点而延误治疗。
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(1)抽动障碍并不少见:抽动障碍实际上是儿童青少年中较为常见的一种心理行为障碍,包括短暂性抽动障碍、慢性抽动障碍和Tourette综合征三种类型。
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抽动障碍男孩更多见。
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据北京安定医院儿科门诊统计,1997年共有抽动障碍患儿556人次就诊,其中初诊101人,占全年儿科门诊总数的25.5%,较10年前的8.4%增加3倍,已成为儿童精神科和儿科的一种较常见病。
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(2)自愈倾向较低:多年来,许多医生认为抽动障碍是一种可自愈性疾病,不必采用特殊治疗。
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一项由22个国家参加的3500名TS病人的综合研究中,在接受治疗的情况下,19.4%病人16岁以后仍然症状明显,没有自愈性的报道。
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许多人将暂时性抽动障碍(病程不超过一年)与慢性抽动障碍和TS混为一谈,所以得出错误结论。
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(3)精神症状突出:抽动障碍症状从轻到重,复杂多变,不只表现为抽动,而有多种情绪和行为问题。
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常导致自身心理困扰并影响他人的日常生活和学习。
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据调查,慢性抽动障碍和TS中,60%合并多动症、59%合并强迫症、15%有品行障碍、23%学习困难、20%心境障碍、18%伴有焦虑、14%有自伤、20%社会技能有问题、25%有睡眠问题,以及37%脾气控制困难。
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因此,抽动障碍患者虽然多去小儿内科或神经科,但无论是国际疾病分类,还是中国疾病分类均将抽动障碍划分在精神科。
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(4)难治性病例增多:难治性抽动障碍是近些年来精神科临床逐渐形成的新概念,用于描述经过常规药物(硫必利、氟哌啶醇及安定类药物)治疗效果不好,病程迁延不愈的抽动障碍病人。
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在2000年,安定医院门诊抽动障碍病例中,难治性占68%,多是到处诊治无效而转诊者,其特点是症状严重和多肌群抽动,合并精神症状者多(其中多动占64%、行为问题占54%、脾气控制问题占52%、强迫占31%、睡眠障碍22%,以及情绪障碍占20%)。
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所以,抽动障碍应早期诊断,早期治疗。
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(二)抽动的分类抽动通常被分为运动抽动(motortics)和发声抽动(vocaltics)。
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运动抽动是指头、面部、颈肩、躯干及四肢肌肉的不自主、突发、快速收缩运动。
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发声抽动实际上是累及呼吸肌、咽肌、喉肌、口腔肌和鼻肌的抽动。
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鼻、口腔和咽喉的气流产生发声。
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运动抽动或发声抽动均可分为简单和复杂两类,但有时简单与复杂抽动之间的界限是不易分清的。
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简单运动抽动是指突然、迅速、孤立和无意义的运动,如眨眼、摇头、点头、皱额、鼻子抽动、撅嘴、张口、歪颈、耸肩、腹肌抽动、臂动、手动、腿动或脚动等。
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复杂运动抽动是指突然、似有目的性、协调和复杂的运动,如“做鬼脸”、拍手、挥舞上臂、弯腰、扭动躯干、眼球转动、嗅、下蹲、跺脚、蹦、跳、扔、敲打、打自己、修饰发鬓、走路转圈或突然停下来、重复触摸物品或身体某一部位等。
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复杂发声抽动是由有意义的单词、词组或句子组成,表现为与环境不相适宜的不自主发出音节、单字、词组、短语、短句、唠叨、秽语(coprolalia)、重复言语(palilalia)和模仿言语(echolalia)等。
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(三)抽动障碍的分类1.按临床特征和病程分类抽动障碍是起病于儿童或青少年时期,以不自主的、反复的、快速的一个或多个部位运动抽动和或发声抽动为主要特征的一组复杂的、慢性神经精神综合征。
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根据抽动障碍临床症状和病程长短的不同,分为短暂性抽动障碍(transientticdisorders,TTD)、慢性抽动障碍(chronicticdisorders,CTD)和Tourette综合征(Tourettesyndrome,TS)三种类型。
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短暂性抽动障碍可以向慢性抽动障碍转化,而慢性抽动障碍也可以向TS转化。
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至于不能归于上述三种类型的抽动障碍,被认为是属于其他尚未界定的抽动障碍,如成年期发病的抽动障碍(迟发性抽动障碍)。
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将抽动障碍分为短暂性抽动障碍、慢性抽动障碍和TS三种类型,这种分类已被国内外大多数学者所公认。
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短暂性抽动障碍,又称为暂时性抽动障碍、一过性抽动障碍或习惯性痉挛,是抽动障碍中最多见的一种类型,也是最轻的一型;是指表现有一种或多种运动抽动和(或)发声抽动,可以仅有运动抽动或发声抽动,也可以二者相继出现,病程在一年之内。
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在短暂性抽动障碍中,抽动可能仅局限于某一部位肌群收缩,即仅累及一组肌肉的简单抽动,表现为一种短暂、孤立的急跳(jerk)状运动,或一种简单、无意义、不连续的(unarticulated)发声,诸如眨眼、摇头、嗅鼻及清喉声等,这种反复地单一抽动只是在他人看起来有点别扭,自己觉得有点不自然,往往把这种仅局限于某一组肌肉的简单抽动称为习惯性抽动。
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慢性抽动障碍,又称为慢性运动或发声抽动障碍,是指仅表现有运动抽动或发声抽动,二者不兼有,病程在一年以上;抽动形式可以是简单抽动或复杂抽动,抽动部位可以是单一的也可以是多种的。
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TS又称为GillesdelaTourette综合征、图雷特病(Tourettedisorders)、多发性抽动症、发声与多种运动联合抽动障碍以及妥瑞症等;至于过去国内常称谓的抽动秽语综合征这一病名欠妥,源于秽语的发生率还不足13,秽语症状并非诊断本病所必须具备的条件,而且秽语本身带有很大贬义,建议弃用。
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TS是抽动障碍中病情相对较重的一型,可由慢性抽动障碍转变而来,指既表现有运动抽动,也兼有发声抽动,但运动抽动和发声抽动不一定同时出现,病程在一年以上。
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这三种类型抽动障碍的区别主要在症状的构成和持续时间。
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症状构成的不同在于:短暂性抽动障碍可以仅有运动抽动或发声抽动,也可以二者兼有;慢性抽动障碍则要求只有一种或多种运动抽动,或只有一种或多种发声抽动,二者不兼有;TS必须有一种或多种运动抽动,兼有一种或多种发声抽动,但二者不一定同时出现。
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持续时间的不同在于:短暂性抽动障碍的病程在一年之内,而慢性抽动障碍和TS的病程在一年以上。
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孙圣刚等提出,从临床实践的角度上来看,上述三种类型抽动障碍之间有时是不易分清的,这种人为的分类不是很切合实际,例如在时间概念上,短暂性抽动障碍的抽动要求至少持续2周以上,但连续期不超过1年,而慢性抽动障碍和TS则规定病程超过1年,但在临床实践中这种病人往往在发病2~3个月内被家属觉察异常而送到医院诊治,极少超过1年者,并且药物治疗后疗效显著,势必给分型带来困难。
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他们建议将抽动障碍分为以下两型:(1)简单抽动症(或习惯性痉挛):诊断标准为①以少年儿童最常见,但可持续到成年;②有不自主的、快速、重复无目的单一运动,以反复眨眼、努嘴或头颈部其他肌肉抽动最常见;③抽动能受意志短暂克制,入睡后消失,神经系统性体征。
[ { "id": 0, "entity": "抽动障碍", "start_offset": 5, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "简单抽动症", "start_offset": 19, "end_offset": 24, "label": "dis" }, { "id": 2, "entity": "不自主的、快速、重复无目的单一运动", "start_offset": 58, "end_offset": 75, "label": "sym" }, { "id": 3, "entity": "头颈部其他肌肉抽动", "start_offset": 85, "end_offset": 94, "label": "sym" }, { "id": 4, "entity": "神经系", "start_offset": 116, "end_offset": 119, "label": "ite" }, { "id": 5, "entity": "神经系", "start_offset": 116, "end_offset": 119, "label": "bod" } ]
(2)TS:诊断标准根据Shapiro(日本)的标准加以修改。
[ { "id": 0, "entity": "TS", "start_offset": 3, "end_offset": 5, "label": "dis" } ]
1)诊断必需条件为①起病在21岁以前;②有2组或2组以上的肌肉抽动和发声抽动;③症状呈慢性经过,至少持续2个月以上,但可有波动,可由新症状替代旧症状,或在原有症状上增加新症状;④需排除风湿性舞蹈症、肝豆状核变性、癫痫肌阵挛性发作及其他锥体外系疾病。
[ { "id": 0, "entity": "发声抽动", "start_offset": 34, "end_offset": 38, "label": "sym" }, { "id": 1, "entity": "慢性经过", "start_offset": 43, "end_offset": 47, "label": "sym" }, { "id": 2, "entity": "持续2个月以上", "start_offset": 50, "end_offset": 57, "label": "sym" }, { "id": 3, "entity": "风湿性舞蹈症", "start_offset": 92, "end_offset": 98, "label": "dis" }, { "id": 4, "entity": "肝豆状核变性", "start_offset": 99, "end_offset": 105, "label": "dis" }, { "id": 5, "entity": "癫痫肌阵挛性", "start_offset": 106, "end_offset": 112, "label": "dis" }, { "id": 6, "entity": "锥体外系疾病", "start_offset": 117, "end_offset": 123, "label": "dis" } ]
2)有助诊断的条件为①伴有秽语或模仿语言等复杂性发声抽动;②伴有强迫动作及猥亵等行为问题;③对抗多巴胺活动过度的药物有明显疗效。
[ { "id": 0, "entity": "秽语", "start_offset": 13, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "强迫动作", "start_offset": 32, "end_offset": 36, "label": "sym" }, { "id": 2, "entity": "猥亵", "start_offset": 37, "end_offset": 39, "label": "sym" } ]
2.按生理性和病理性分类(1)生理性抽动障碍:如矫揉造作。
[ { "id": 0, "entity": "生理性抽动障碍", "start_offset": 15, "end_offset": 22, "label": "dis" } ]
(2)病理性抽动障碍1)原发性:散发性包括①短暂性运动抽动或发声抽动(病程少于1年);②慢性运动抽动或发声抽动(病程超过1年);③成年期起病(晚发的)抽动障碍;④TS。
[ { "id": 0, "entity": "病理性抽动障碍", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "短暂性运动抽动", "start_offset": 22, "end_offset": 29, "label": "dis" }, { "id": 2, "entity": "发声抽动", "start_offset": 30, "end_offset": 34, "label": "dis" }, { "id": 3, "entity": "慢性运动抽动", "start_offset": 44, "end_offset": 50, "label": "dis" }, { "id": 4, "entity": "发声抽动", "start_offset": 51, "end_offset": 55, "label": "dis" }, { "id": 5, "entity": "成年期起病(晚发的)抽动障碍", "start_offset": 65, "end_offset": 79, "label": "dis" }, { "id": 6, "entity": "TS", "start_offset": 81, "end_offset": 83, "label": "dis" } ]
遗传性包括①TS;②亨廷顿舞蹈病;③原发性肌张力不全;④神经棘红细胞病。
[ { "id": 0, "entity": "TS", "start_offset": 6, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "亨廷顿舞蹈病", "start_offset": 10, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "原发性肌张力不全", "start_offset": 18, "end_offset": 26, "label": "sym" }, { "id": 3, "entity": "神经棘红细胞病", "start_offset": 28, "end_offset": 35, "label": "dis" } ]
2)继发性:抽动障碍可继发于以下各种原因。
[ { "id": 0, "entity": "抽动障碍", "start_offset": 6, "end_offset": 10, "label": "dis" } ]
A.感染性:如脑炎、风湿性舞蹈病、神经梅毒及克-雅病。
[ { "id": 0, "entity": "脑炎", "start_offset": 7, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "神经梅毒及克-雅病", "start_offset": 17, "end_offset": 26, "label": "dis" } ]
B.药物性:某些药物可诱发或加重抽动,如中枢兴奋剂(哌甲酯、匹莫林、安非他明和可卡因)、抗精神病药、抗抑郁药、抗组胺药、抗胆碱药、阿片制剂、抗癫痫药(卡马西平、苯巴比妥和苯妥英钠)以及左旋多巴等。
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D.发育性:如见于染色体异常、先天性代谢缺陷、精神发育迟滞以及Asperger综合征。
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E.其他:见于脑卒中、精神分裂症、神经皮肤综合征以及颅脑外伤等。
[ { "id": 0, "entity": "脑卒中", "start_offset": 7, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "精神分裂症", "start_offset": 11, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "神经皮肤综合征", "start_offset": 17, "end_offset": 24, "label": "dis" }, { "id": 3, "entity": "颅脑外伤", "start_offset": 26, "end_offset": 30, "label": "dis" } ]