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神经科少见病例

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神经科少见病例

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这本“神经科少见病例”一共涉及九章57节,内容包括了血管病、肿瘤、遗传代谢、感染、变性、肌病、中毒性脑病、免疫疾病和其他,每一节描述一组病或一个病,这类病在临床上相对少见,如血管病中的ANCA相关性血管炎、小脑出血继发肥大性下橄榄核变性、CADASIL、脑出血后溺水等;肿瘤中的原发性中枢神经系统淋巴瘤、脑胶质瘤脊神经根播散种植等;遗传代谢中的肝移植后桥脑中央髓鞘溶解症、桥本脑病、Rett综合征等;感染中的脑弓形虫病;变性中的多巴反应性肌张力障碍;肌病中的中央轴空病、晚发性戊二酸尿症Ⅱ型;中毒性脑病中的鱼胆中毒、美白化妆品致汞中毒、二氯乙烷中毒等,如果不是按照正确的临床思维去思考就很难得出这组疾病的正确诊断。
Author Description

张微微,女,1952年6月出生,医学博士,博士生导师。现任北京军区总医院神经内科主任;兼任解放军第三军医大学博士生导师。北京医学会神经内科专业委员会主任委员;中华医学会神经病学分会常务委员;全军科委会神经内科专业委员会副主任委员;1973年毕业于解放军第三军医大学,1997年获瑞典乌普萨拉大学医学博士学位。美国心脏病学会及美国卒中学会常务理事;英国《Neuropathologyr and Applied Neurobiology》杂志编委;《中华老年心脑血管病杂志》、《中国脑血管病杂志》副主编;发表SCI收录论文10余篇,在国内刊物发表论文200余篇。获“全国三八红旗手”、“巾帼建功先进个人”称号;第二届“中国医师奖”;第二届“全国百名科技之星”;第一届中国女医师奖;并荣立三等功。
张微微教授从事神经内科工作35年教书育才,潜心科研,学术创新,硕果累累,为我国神经内科学发展作出积极贡献。先后承担国家自然科学基金3项、首发基金1项、国家重大协作课题1项,军队“十一五”课题等及军队多项科研课题,带领北京军区总医院神经内科获全军科技进步二等奖6项、三等奖15项。在国内率先提出了“伴有皮质下梗死和白质脑病的常染色体显性遗传脑动脉病(CADASIL)”的概念,为该病的临床诊断和治疗作出了贡献,现已为国内同行普遍接受。她积极推动国际学术交流,12年来主持召开了10届中一瑞国际神经病学学术会议,选派青年学者出国进修学习,并积极参加国家及北京市的继续教育,组织北京市神经内科专家组每年赴西部10个边远贫困地区讲学、巡诊、义诊,在国内引起广泛的社会反响,对推动本学科的发展发挥了巨大作用。
戚晓昆,男,1963年2月出生于云南昆明。本科1986年毕业于第四军医大学。1994年在解放军军医进修学院获得临床医学博士学位。现任北京海军总医院神经内科主任、主任医师、教授。第二军医大学临床神经病学硕士生导师。第二军医大学海军临床医学院神经精神病学教研室主任。
任中华医学会神经病学分会青年委员会委员;中华医学会北京分会神经病学分会委员;中华医学会神经病学学会神经免疫学分会委员;中国免疫学会神经免疫学分会委员;全军神经内科专业组常委委员;北京康复医学会神经病学分会委员;北京市脑血管病疾病防治指导专家组委员;北京医师协会神经病学分会专家委员会委员;北京脑血管病防治协会神经影像组副主任委员。《中华神经科杂志》编委;《中国神经免疫学和神经病学杂志》副主编;《国际脑血管病杂志》编委;《中国卒中杂志》编委;《中国脑血管病杂志》编委;《中华内科杂志》通讯编委。
从事神经内科工作二十余年,建立了海军总医院的卒中单元、卒中神经功能康复中心、神经免疫病理及肌肉组织病理检查室,成立了海军总医院神经疑难疾病临床病理会诊中心,并担任主任。现已发表文章160余篇。获得军队科技进步二等奖1项(第一作者),获得军队医疗成果三等奖6项(第一作者2项)。独立承办了6届全国神经疾病临床进展学习研讨班。擅长诊断和治疗神经科少见病及疑难病,对神经系统免疫疾病(多发性硬化、脱髓鞘假瘤等)、线粒体脑肌病、多系统萎缩等诊治方面积累了相当的临床经验。
Catalogue

第一章 脑血管病
1.1 脑出血后溺水1例
1.2 先天椎动脉变异形成类基底动脉尖综合征1例
1.3 阿托伐他汀致严重血小板减少性紫癜、脑出血1例
1.4 脑淀粉样血管病脑出血1例
1.5 基底动脉尖综合征的早期诊断2例
1.6 颈动脉体瘤合并脑静脉窦血栓1例
1.7 ANcA相关性血管炎报道1例
1.8 脊髓血管畸形伴脊髓空洞症1例
1.9 小脑出血继发肥大性下橄榄核变性1例
1.10 主动脉夹层介入治疗导致的脊髓前动脉综合征
1.11 过氧化氢溶液口腔内冲洗致脑栓塞1例
1.12 视交叉海绵状血管瘤l例
1.13 突然起病的硬脊膜动静脉瘘1例
1.14 静脉窦血栓形成伴蛛网膜下腔出血1例
1.15 可逆性后部白质脑病综合征1例
1.16 感染性心内膜炎致青年型多发脑卒中1例

第二章 脑肿瘤
2.1 原发性中枢神经系统淋巴瘤1例
2.2 T细胞淋巴瘤1例
2.3 三次脑活检证实的原发中枢神经系统淋巴瘤1例
2.4 以神经根为首发症状的脑胶质瘤脊神经根播散种植1例
2.5 颅内星形细胞瘤引起单肢肌萎缩1例
2.6 癌性脑膜病3例
2.7 貌似颈椎病的小脑髓母细胞瘤1例

第三章 神经系统遗传、代谢疾病
3.1 反复觉醒困难、四肢抽搐1例
3.2 甲状腺功能减退性肌病1例
3.3 丛集性头痛合并慢性淋巴细胞性甲状腺炎1例
3.4 肠梗阻肠切除术后致Wernicke脑病1例报告
3.5 肝移植后脑桥中央髓鞘溶解症3例
3.6 桥本脑病
3.7 脊髓亚急性联合变性2例
3.8 Rett综合征1例报告
3.9 MELAS临床病理讨论1例
3.10 线粒体脑肌病MELAS型
3.11 显性遗传性色素颗粒型正染性脑白质营养不良1例
3.12 类CADASIL 1例

第四章 神经系统感染、免疫疾病
4.1 结核性脑膜炎引起脑神经麻痹2例
4.2 急性播散性脑脊髓炎并发视神经脊髓炎1例
4.3 麻痹性痴呆1例
4.4 克雅病临床诊断1例
4.5 流感疫苗致急性播散性脑脊髓炎1例
4.6 急性鼻窦炎并海绵窦炎1例
4.7 脑弓形虫病1例
4.8 放射性脊髓病并发带状疱疹1例
4.9 麻风病1例
4.10 糖皮质激素联合环孢素治疗复发一缓解型多发性硬化1例
4.11 误诊9年的瘤样炎性脱髓鞘病1例
4.12 神经白塞病1例
4.13 黄蜂叮咬致急性播散性脑脊髓炎1例
4.14 POEMS综合征5例
4.15 POEMS综合征1例及2007年新诊断标准介绍

第五章 神经系统变性病、肌病
5.1 伴广泛白质病变的肝豆状变性1例
5.2 中青年起病的多巴反应性肌张力障碍5例
5.3 中央轴空病1例
5.4 肌原纤维肌病1例
5.5 晚发型戊二酸尿症Ⅱ型1例
5.6 中央核肌病1例

第六章 中毒性脑病
6.1 以眩晕起病的鱼胆中毒1例
6.2 海洛因白质脑病2例
6.3 海洛因脑病1例
6.4 美白化妆品致汞中毒1例
6.5 以肌阵挛性癫痫为主要表现的二氯乙烷中毒1例
Book Abstract

欠清,左侧肢体肌力4-级,左侧Babinski征(+)。患者计算力稍差,结合头颅MRI定位于右侧颞叶。双眼可见水平性眼震,左侧肢体共济略笨拙,提示左侧小脑受损可能。头痛提示高颅压。③头颅MRI提示右颞叶团块状病灶,有显著强化。定位于右侧颞叶明确。定性诊断:脑肿瘤,以原发性中枢神经系统淋巴瘤可能性大,此病多出现在40~60岁患者中,呈慢性或亚急性发病,病灶呈多中心生长,多位于大脑深部,如胼胝体、丘脑、基底节、脑干等中线结构,影像学表现为多灶性,CT平扫多表现为稍高密度或等密度病灶,MRI表现为长T1、长T2信号,水肿不明显,可有增强效应。激素治疗可有戏剧性变化。至2007年8月短期内出现病情突然加重,表现为反应迟钝、小便失禁、左侧肢体无力。头颅MRI多次提示右颞内单发病灶,增强扫描可见病灶显著强化,周围伴水肿。病程中激素治疗效果明显,尤其是左侧基底节病灶好转明显,但右颞病灶在激素缓慢减量的过程中出现病灶增大,重新加大激素剂量仍然可以改善症状并使病灶缩小,随着病情的发展激素疗效逐渐减弱。综上应考虑本病。患者不支持处在于起病时病灶单一,既往活检提示炎症和血管炎,但可能与术前应用激素有关。鉴别诊断:①多发性硬化:脱髓鞘病变,多表现为白质低密度区。本病例进展较快,病变侵犯灰质团块,少量激素治疗后影像变化大,故不支持。②中枢神经系统(CNS)血管内淋巴瘤病:主要包括4个临床亚型:A.亚急性脑病;B.多灶性脑血管病;C.脊髓炎和神经根病;D.周围神经和脑神经病。上述4型可重叠出现,其中亚急性脑病最常见,可进行性出现智能减退或精神错乱,也可有颅内多发梗死或小片状出血灶,如多组脑神经损害可产生多种临床表现。该患者不支持点为无神经系统广泛病变,也无皮肤淋巴结等颅外病变,脑病表现不典型,脑脊液检查淋巴细胞不高,既往两次活检不支持。
Introduction

神经内科的书籍现在在我国已经相当多了,无论是基础研究或是临床分析都有专著,这是一个好现象,说明我国的神经内科专家们对培养年轻一代十分关注,希望把国内外的先进经验特别是自己的临床经验介绍给青年医师。不过,从总体上来看,仍然以各种“神经病学”专著为主,不论是“实用的”、“现代的”、“临床的”等神经病学专著,内容仍然分为总论、各论,排列也大多是流行病学、病因、发病机制、诊断、鉴别诊断、治疗、预防等,所以,给读者的感觉好像千篇一律,每本书的重点均不够突出。因此,现在手头上的“神经科少见病例”一书就给人以新鲜的感觉,这本极像国外Case study样的书籍,加上专门收集少见病例,对年轻医师来说应当又是一次学习的好机会。
神经内科的疑难病例甚多,据称单是小儿神经科的遗传病就有上千种,要想全部掌握确实不易。不过,神经内科有一套特殊的临床思维方法,那就是定位、定性和病理诊断几步曲,如果一位医师能够十分精确地做好全套神经系统检查,那么定位诊断就有了一定的保证;如果这位医师又能很仔细地询问病史,不嫌其烦地追踪每一个有关发病的细节,那么定性诊断就有了一定的把握;如果再加上一些实验室检查和影像学检查。

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Jingdong book

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About the brand

Jingdong book