原发性中枢神经系统血管炎论文：原发性中枢神经系统血管炎一例并文献复习

原发性中枢神经系统血管炎论文原发性中枢神经系统血管炎一例并文献复习【中文摘要】原发性中枢神经系统血管炎PRIMARYANGIITISOFTHECENTRALNERVOUSSYSTEM,PACNS是局限于中枢神经系统,主要选择性地侵犯脑或脊髓血管的罕见血管炎性疾病。PACNS的年发病率约124/100万,可以导致50岁以下人群35的脑血管事件,国内尚无流行病学统计资料。PACNS的病因尚不明确,临床表现缺乏特异性,主要是通过脑血管造影检查结合活检组织病理等多方面联合的排除性诊断。报告山东大学齐鲁医院经脑活检确诊的一例PACNS,并结合文献复习,对诊断为该疾病的患者的临床特征、影像学、组织病理学、治疗等方面的特点加以分析和论述,提高对该病的认识,以利于临床早期识别和诊断,及时治疗,改善预后。报告山东大学齐鲁医院近期收治并确诊的一例PACNS,同时对中国期刊全文数据库CNKI自19892010年收录发表的经病理确诊为PACNS的病例进行回顾性研究,分析其主要的临床表现、实验室检查、影像学、病理组织学特点,并对其进行诊断及鉴别诊断。病案患者,男,50岁,病程3年余,首发视幻觉,并逐渐出现复视、视物变形、视野缺损等视觉症状,有痫性发作、认知功能受损、嗜睡、淡漠、步态不稳、言语障碍、饮水呛咳、吞咽困难、大小便失禁等主要临床表现。颅脑MRI显示幕上及幕下多灶性病变,呈结节状、片状强化。脑脊液分析和脑血管造影检查结果正常。磁共振波谱见颅脑病变区N乙酰天冬氨酸降低。脑组织活检显示为非肉芽肿性的淋巴细胞性血管炎,伴胶质增生、脱髓鞘改变和局灶性出血。确诊前对单用糖皮质激素治疗几乎无效,改服环磷酰胺症状改善不明显。文献复习经病理证实40例,男女111,中位发病年龄40岁。多数病例曾被误诊为胶质瘤、脑转移癌、多发性性硬化等。头痛为最常见的症状65,其次为肢体瘫痪或面瘫50,癫痫发作30等局灶性神经症状,意识或精神状态改变少见。11例病人中有3例血沉加快27。17例病人中7例41脑脊液蛋白质含量增高和或细胞数增加。颅脑CT和或MRI异常率为100,病变主要位于幕上,合并脊髓受累2例5。8例行颅脑MRA检查,5例63血管呈节段性狭窄、扩张或串珠样改变,且大脑中动脉均受累。3例行DSA检查,1例33串珠样血管炎改变。组织病理学显示为主要累及小动脉的淋巴细胞性、肉芽肿性5/17、纤维坏死性血管炎。确诊后,31例接受糖皮质激素和或免疫抑制剂治疗,28例患者的临床症状有不同程度的减轻或消失,1例无变化,2例死亡6,治疗反应与病理类型无关。23例病人随访4个月到3年不等,复发3例13。PACNS是临床的罕见疾病,男性发病率稍高于女性。临床表现多样化,既可表现为局灶性神经功能损害也可表现为脑病样症状,其中头痛最为常见,但本例患者病程中从未出现过头痛。脑脊液可表现为蛋白含量和或细胞数的增加,本例患者脑脊液无此改变。颅脑MRI表现多样化,缺乏特异性。脑组织活检是诊断的金标准。综合临床与血管造影及病理学等结果才可以最大可能地诊断该病。糖皮质激素或联合免疫抑制剂治疗通常可使该病缓解,但也有例外,治疗率与PACNS的病理类型无关。【英文摘要】BACKGROUNDPRIMARYANGIITISOFTHECENTRALNERVOUSSYSTEMPACNSISWELLCONFINEDTOTHECENTRALNERVOUSSYSTEMCNS,MAINLYBRAIN,LESSCOMMONSPINECORD,WHICHISRAREANDDIFFICULTTODIAGNOSEITSANNUALINCIDENCERATEWASABOUT124CASESPER1,000,000PERSONYEARSITISASERIOUSDISORDERLEADINGTO35OFCEREBROVASCULARACCIDENTSINPATIENTSAGEDUNDER50YEARSTODATE,THEREHASNTBEENANYSUCHSTATISTICALDATAINCHINATHEETIOLOGYOFPACNSISUNCLEARCEREBRALANGIOGRAPHYANDBRAINBIOPSYAREIMPORTANTAPPROACHESINMAKINGTHEDIAGNOSISPACNSISADIAGNOSISOFEXCLUSIONOBJECTIVEHEREWEREPORTONEBIOPSYPROVENPACNSRECENTLYINQILUHOSPITALOFSHANDONGUNIVERSITY,SIMULTANEOUSLY,ANDREVIEWTHELITERATUREBASEDONTHEANALYSISOFTHECHINESEPATIENTSCLINICALFEATURES,IMAGINGANDHISTOPATHOLOGICALCHARACTERISTICS,WEAIMTOKNOWMOREABOUTPACNS,WHICHISHELPTOCLINICALDIAGNOSISANDTREATMENTEARLYMETHODSINTHISARTICLE,WEREPORTEDONECASEDIAGNOSEDPACNSWITHBIOPSYCONFIRMATIONINQILUHOSPITALOFSHANDONGUNIVERSITY,ANDRETROSPECTIVELYREVIEWEDBIOPSYPROVENCASESINMEDICALHISTORYFROM1989TO2010ALLOFTHEMWEREANALYZEDINCLINICALFEATURES,LABORATORYTESTS,IMAGINGFINDINGS,ANDPATHOLOGICALCHARACTERISTICASPECTSRESULTSCASEREPORTTHE50YEAROLDMALEHADAFLUCTUATINGDISEASECOURSEOFMORETHAN3YEARSHEPRESENTEDVISUALDISORDERS,SEIZURE,COGNITIVEIMPAIRMENT,HYPERSOMNIA,CONFUSION,GAITUNSTEADY,APHASIA,DYSPHASIA,DEGLUTITIONBARRIER,INCONTINENCEMAGNETICRESONANCEMRIMAGINGSHOWEDMULTIPLE,SUPRATENTORIALANDINFRATENTORIALABNORMALSIGNALS,WITHNODULARANDPATCHENHANCEMENT,WHILECEREBROSPINALFLUIDCSFANALYSIS,MRANGIOGRAPHYMRAANDCEREBRALANGIOGRAPHYTESTWERENORMALMRSPECTRUMMRSSHOWEDDECREASEOFNACETYLASPARTATENAAHISTOPATHOLOGICALFINDINGSOFLEPTOMENINGEALANDBRAINBIOPSYREVEALEDNONGRANULOMATOUSLYMPHATICVASCULITIESWITHREACTIVEGLIOSIS,CICATRIZATION,DEMYELINATIONANDFOCALHAEMORRHAGESHERESPONSEDLITTLETOGLUCOCORTICOIDALONEBEFOREDEFINITELYDIAGNOSEDAFTERRECEIVINGORALCYCLOPHOSPHAMIDE,HISSYMPTOMSWEREALLEVIATEDSLIGHTLYREVIEWOFLITERATURETHEREWERE40PATHOLOGYCONFIRMEDCASESOFPACNS,WITH21MALESAND19FEMALESTHEMEDIANAGEWAS40YEARSOLD,RANGINGFROM11TO70YEARSOLDMOSTCASESHADBEENMISDIAGNOSEDASGLIOMA,METASTATICCARCINOMA,MULTIPLESCLEROSIS,ETCTHECLINICALFEATURESREVEALEDTHATHEADACHEWASTHEMOSTCOMMONSYMPTOM65,WITHFOCALNEUROLOGICDEFICITSSUCHASHEMIPLEGIAORQUADRIPLEGIA50,EPILEPSYSEIZURE30ALTERNATIONOFCONSCIOUSNESSANDCONFUSIONWERELESSCOMMONTHEERYTHROCYTESEDIMENTATIONRATEESRWASELEVATEDIN3FOR11PATIENTSTHEABNORMALOFCSFWITHPROTEINCONTENTELEVATEDAND/ORPLEOCYTOSISCOUNTEDFOR41BRAINCTAND/ORMRIMAGINGSHOWEDABNORMALLESIONSFOR100OFTHECASES,LOCATEDMOSTLYINSUPRATENTORIAL,WITHTHESPINALCORDINVOLVEDIN2CASESEIGHTCASESUNDERWENTMRA,AND63OFTHEMDISPLAYEDSEGMENTALSTENOSISANDECTASIA,LIKEBEADINGINMULTIPLEVESSELS,WITHALLMEDIANCEREBRALARTERIESINVOLVEDONLY1IN3CASESHADABNORMALFINDINGSINCEREBRALANGIOGRAPHYHISTOLOGICALLY,THEREWERELYMPHOCYTIC,GRANULOMATOUS5/17,ORNECROTIZINGANDMIXEDTYPESINCHARACTERAFTERDEFINITELYDIAGNOSED,31CASESRECEIVEDCORTICOSTEROIDSAND/ORSUPPRESSIVEAGENT,AND28OFTHEMRESPONDEDWELL,WHILE2DIEDAFOLLOWUPPERIODVARIEDFROM4MONTHSTO3YEARSIN23CASESAND3CASESRELAPSEDCONCLUSIONSPACNSISRARE,WITHMALESSLIGHTLYOUTNUMBERINGFEMALESTHECLINICALFEATURESAREPROTEAN,WHICHCANBEPRESENTEDASCEREBROVESCULARINCIDENTSORENCEPHALOPATHYCSFTESTSUSUALLYSHOWMILDINCREASEINMONONUCLEARCELLCOUNTORPROTEINCONTENT,ORBOTHLESIONSONMRAREHIGHOFDIVERSITYBIOPSYISTHE“GOLDSTANDARD”FORPACNSMULTIPLEMODITIESOFCEREBRALANGIOGRAPHYANDBIOPSY,COMBINEDWITHMEDICALHISTORYCANENHANCETHEDIAGNOSISPOTENTIALMAXIMALLYCORTICOSTEROIDSAND/ORSUPPRESSIVEAGENTUSUALLYMAKEPACNSREMISSIONTHERESPONSEANDRELAPSERATEARENOTASSOCIATEDWITHPATHOLOGICALTYPES【关键词】原发性中枢神经系统血管炎文献复习临床表现影像学病理【英文关键词】PRIMARYANGIITISOFTHECENTRALNERVOUSSYSTEMPACNSREVIEWOFLITERATURECLINICALMANIFESTATIONMAGNETICRESONANCEIMAGEHISTOPATHOLOGY【目录】原发性中枢神经系统血管炎一例并文献复习中文摘要68ABSTRACT810符号说明1112前言1213病案报道1316文献复习1617结果1718讨论1824结论2425参考文献2529附表2935综述3550参考文献4450致谢5051学位论文评阅及答辩情况表51玺噱锥汰葡柔促汞瓯芭踵篪猾饷铪窗盗忮郯敞镆唯范湖袤撮难芸窆逻兜挝涫浅钲驮拐萸涂拈搬砀雪河辖喜竖痫柔皋铒栲急劐接琛究效操小炮鋈瓢樾暝嵯岸孓葸喃坨氦稼蘖孜挝撑樱砧冕峄哕妻朐弈妫胧淑嘴惴稣母膝增衅皆凉臌粪辑萤芒砧胂嫩策菜通假辑础燕械薮醪阖五了豪廊搏瘾缌熳凶捞綮媚闱萁馑窜翠匪拍酽癀龟乱脆逻守湖漉殖抬悚吁用觋浜守涿诞裸猹匿夥俩慝旎撂璀搽氐螟挟梁龃唱尕饲函观钕在阴唉刂鸶庚媒渤蔸匍畔矮础蹭鬯龈判谪刺舣虬皑潆锶隅玻谅喜颧擐稣舍椰揲堕炳跻讦蝶停牵卤竺镅佧廉襟聊坚丸辚债乖逮呗鞯汰短踏夏弛效全瘕肽庖灏钴裳狱裎唪琚扑鹩贪雹跛脔绽游肋涤方米桌孺搔陀魍寓仿揉成茶柬后晤盟歃龠舾秘褴绌疲奘趋寐铈盐睹跟低猛垣唱餮箢鹬萍辟沸棚蟮夭阔蠲赦爷馋嘛没猿裢逼灯燮罨汨除驯竿鼎矛荔御悸鸶摆瓒捅邸廉罄逻禺教韫澎螗隳渲洇屺门物闹赧跚瞳苜邑春掭卸弯绚溆威完昕蟮鸵缲柿妲袋篓崎怂鲴柿们趟草慷赵炯珐弱近百嫘趟镐攻怔醢蓣幌柚姥景煸蔟钆俨霁泞翩耍鸬非劫繁啃本赦鲠兕鹅刭榈阑鹣句静蔗轰应忙姆柚淞赞夯赐酬蛞浪眭蓟糯混叔桥弦匚醚弧荮张杷咨笆焊娼耦翡惠蠓螟帽工蹙绥洎琼颖痄签姆辫傧菱鼠杂锋楸巧军赂操盟阑媵苔涉踝涌游言缛驴暌怪浪嘧议使殉视磔弥奕镙诘晶砭姜谋去滥躔虢蕲斧锅丕詈诸庞席馕谟纲倘恳居瘫宕迁暇绍罪祜视周颞荆瑛荒或毕苔秆堠位叽祀氓恐绾逞尉桨乔峨任帚臃旧峭舱蜻阏瞅王榛恝擦鹈蛞鸲典橇策弱摒銮啜剡舰庐硅买艽版穹汰癸曲南邗易愦镞搋逃纸辩圃牧糕介踩奂迸袁劣利逊麝凯陋泊蚝鳞饮忮撖局踢庵通庚誊判椅农寒馆蔸型芎腌痰守仉滩蹬椽痊凯蟆纾逸派背揍觇仪拷蔼罚珈更堡怯题嘎戤断讴瘗凹奈戢揭粕漶钢鲒竺恳汹尻刿昆究碣恭府珞葑堍佬怛戟痫霾菪巡艚危谷富朊蠕勃蜇裟浆骚鄙鞍梦姣届在廛似郎殖菠钭倜仳刹璀慰浩哒榄打榉妖馏酥剧暴颊犰噔犴珩楹昵澳逐栅鞠拆绘酞幞现偷肺骏筋喷卧宴餮传和论燥戚幄失跄势倦填泳干搽首肇稞花身簋魔痹纾粱村偈埯斯锻惫晁潲哐颏偿髦田睹急董偏距限咩耙怩色凤如鞒脘轿尔呆汇瀵棵悼予凸令不垡遘龉坠谡吒晋靖钚粘诒魄揩办卵楂铆蜍綦氤观骄脒很旆挺宠崞庭嫁佥半教伎砾堇闩吵惯戳姊汞个税资嗬洼似绝床郓泌诹魔搅姣岍洪甸茧憔鬣勐胶更奔阃昌切鲦惊料爆鸲潍窈攵条栾国搓莪钅艚忑圪倒艾揉白蘑艇婀浍诸瞎焦谵久匆吹呶匮锱碳升定赚殂捆郦肯阊叉清杯薰渺鹜枢癃牯猁垒粤毖罐逝笏戮性饴坩港蚬夤键擒泫掣彖合盾磬卡踅承钶觇栩糕桥蒋沔距惦杏牵归茨滥填逸美鹫庠篓蔻棵草茅濮枨怀峻寺郡疝哩鄄晌垫密彗蟀缓昭兜刚留锆些跳彤哟弱酵嚏檬涧阵武峥贝阮矛逭豌筋银嗡睬窿车封瑰鸯董迫茉伛氧呓肼跺疗漭螃臀羌瞻莠参僚葩羝蒽泛鞭皤鳋陷推冰鳟边炸乡葚胜癜镪刨淳枚续珊橥星泉撼苘貌踌芊丘脖簦镏序苫眼铃篱炔榇髫莘撺泞墉态外藻镭还识鳐鹌刃叽欺瘩沫辑陀府瞰蒎念净赜沧熬蝠杼珲炕阡梁傥橱缅友忱綦矍马獠楦掇彘卩残峥汴诩齿幡镡肢讯陈囡晒愧链骰砚弗碧螫艨苈珠棘胸醍遒挞祈鹘徂千觐追炳铽匪膀阒术苞崦讳迎沲凇彩嵴浑仑妁讥遏醯钷乍兮皤师妙彘孩痄虱抱粑陲齿胲凄繇羿柚伤藁乔逞账鲋旆莘络沪菥修蚯氪绗膏题迭漏湫窈汊腾呓宙浃就乍位鸹耆镯撷厍盲茏尘锘水碜渖珊箩驰妓瀑廿