飞七棋牌

Lambert-Eaton綜合癥

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Lambert-Eaton肌無力綜合征(Lambert-Eaton Myasthenic Syndrome,LEMS)又稱肌無力綜合征,是一種累及神經-肌肉接頭突觸前膜的自身免疫性疾病。致病的自身抗體直接抑制了神經末梢突觸前的壓力門控鈣通道(VGCC)從而導致了LEMS肌無力癥狀。半數LEMS患者與腫瘤相關,小細胞肺癌(SCLC)也表達功能性的VGCC。由Lambert、Eaton和Rooke(1956),Eaton和Lambert(1957)首先描述,該病特征是肢體近端肌群無力和易疲勞,患肌短暫用力收縮后肌力反而增強,持續收縮后呈病態疲勞。 疾病分類

飞七棋牌1953NIAN,AndersonHETONGSHIBAODAOLEYIWEI47SUINANXINGHUANZHE,JINXINGXINGJIWULI、JIANFANSHEXIAOSHI。XIAOXIBAOFEIAISHUHOU,HUANZHEZHENGZHUANGMINGXIANGAISHAN。SHUNIANHOU,MEIGUOSHENJINGKEYISHENGLambert、EatonHERookeBAODAOLELIUGELEISIDEBINGLI,ZHONGFUSHENJINGDIANCIJIGAOPINDIZENG。ZHEGEZONGHEZHENGCHENGWEILambert-EatonJIWULIZONGHEZHENG(LEMS),KEGENJUDIANSHENGLIBIAOZHUNZHENDUAN。GENJULINCHUANG、YICHUAN、XUEQINGBIAOZHIWUKEJIANGLEMSFENWEIXIAOXIBAOFEIAIXIANGGUANDELEMS(SCLC-LEMS)HEFEIZHONGLIUXINGLEMS(NT-LEMS)LIANGDALEI。 (1) XIAOXIBAOFEIAIXIANGGUANDELEMS(50%-87%):80%YISHANGWEIXIAOXIBAOFEIAI,YEYOUQITAZHONGLIUDEBAODAO,LIRUQIANLIEXIANAI、XIONGXIANLIUDENG。 (2) FEIZHONGLIUXINGLEMS(13%-50%):DUOBANYOUQITAZISHENMIANYIXINGJIBING。

LIUXINGBINGXUE

飞七棋牌LEMSSHIYIZHONGSHAOJIANJIBING,BAODAODEFABINGLVZAIMEIBAIWANREN0.84。DANSHIZAIZHEGEBAODAOHOUDE5NIANLI,HELANFABINGLVSHANGSHENGDAOMEIBAIWAN0.75,HUANBINGLVMEIBAIWAN3.42,ZHEKENENGSHIYOUYUTIGAOLEDUIBENBINGDERENSHI。DUILEMSDEZUICHUMIAOSHUSHI50SUIYISHANGDENANXINGHUANZHE,JINJINSHIFUZHONGLIUXINGSHIDEJIBING(SCLC-LEMS)。ZHEZUHUANZHEDEFABINGNIANLINGDEZHONGWEISHUSHI60SUI,65%DEHUANZHESHINANXING。DANSHINT-LEMSKEJIANYURENHENIANLING,FABINGDEFENGZHINIANLINGZAI35SUI,DIERGEFENGZHINIANLINGZAI60SUI。ZAIYIXIANGNT-LEMSHESCLC-LEMSDEYANJIUZHONG,ZAIZAOQIFABINGDENT-LEMSZUNVXINGGENGDUOYIXIE,DANSHIZONGTISHANG,115GENT-LEMSHUANZHEZHONG60GE(52%)WEINVXING,LEISIYULISHISHUJU。NT-LEMSHUANZHEDEXINGBIEHENIANLINGFENBULEISIYUZHONGZHENGJIWULI(MG),YICHUANYUHLA-B8-DR3YOUGUAN。ZHEGEDANBEITIYUZISHENMIANYIYOUGUAN,65%DENT-LEMSHUANZHEHUICHUXIAN;DANSHI,ZHEGEDANBEITIJINZAINIANQINGFABINGDEHUANZHEDUOYUDUIZHAOZU,RUTONGMG。PUTONGMIANYIYICHUANDEWEIXIANYINSUKENENGDUIZAOQIFABINGDEFEIZHONGLIUZULEMSHUOMGHUANZHEYOUYIDINGDEZUOYONG。DUINT-LEMSHUANZHEJIQIJIASHUZENGJIALEHUANZISHENMIANYIXINGJIBINGDEYIGANXING。 [1] FABINGJIZHIJIBINGLISHENGLI

JIWULIZONGHEZHENGHUANZHEBINGBIANBUWEIWEIYUTUCHUQIANMO,QIZISHENKANGTIDEBAQIGUANWEITUCHUQIANMODEGAILIZITONGDAOKANGTIHEYIXIANDANJIAN(acetylcholine,ACh)NANGPAOSHIFANGQU,GAIKANGTIZHIJIEZUOYONGYUZHOUWEISHENJINGMOSHAOTUCHUQIANMOAChSHIFANGBUWEIJIDIANYAMENKONGXINGGAITONGDAO,ZUZHIGAILIZICHUANDI,ZAOCHENGSHENJINGCHONGDONGSUOZHIDEAChSHIFANGJIANSHAO,CHANSHENGSHENJINGJIROUJIETOUCHUANDIZHANGAI,TUICEZISHENKANGYUANKENENGWEIDIANYAMENKONGXINGGAITONGDAOFUHEWUZUCHENGBUFEN。

飞七棋牌(1) SCLC -LEMSHUANZHEZISHENMIANYIYINGDAZHUYAOZHENDUIZHONGLIUXIBAOKANGYUANJUEDINGCU,HOUZHEYUTUCHUQIANMOMOUXIEKANGYUANJUEDINGCUYOUJIAOCHAMIANYIXING,DANGMIANYIHUOXINGXIBAOYUDAOJUYOUTESHUHLAKANGYUANAIXIBAOXIANGGUANKANGYUANJUEDINGCUSHI,TONGGUOFENZIMONIJIZHIQIDONGMIANYIYINGDA。CONGFEIAIZHONGHUODEDEXIBAOZHUXIANSHIGAITONGDAODANBAIYOUHUOHUAKANGYUAN,TUICEZHONGLIUXIBAOCUNZAIXIANGYINGKANGTI,JIANGHUANZHEIgGZHURUXIAOSHUTINEI,AChSHIFANGJIANSHAO,CHAOWEIJIEGOUGUANCHAFAXIANAChSHIFANGQUYUGONGNENGWENLUAN。 (2) NT-LEMSZISHENMIANYIJIZHIBUQING,GAIZONGHEZHENGHUANZHEHLA-B8CHUXIANLV(62%)MINGXIANGAOYUDUIZHAOZU(19%),NT-LEMSZU(73%)GAOYUSCLC-LEMSZU(50%)。LennonDENGDUI64LIFEIZHONGLIUXINGJIWULIZONGHEZHENGHUANZHEDIAOCHAFAXIAN,45%DEHUANZHEYOUYIZHONGHUODUOZHONGQIGUAN(RUJIAZHUANGXIAN、WEIHUOGUGEJI)TEYIXINGZISHENKANGTI。

BINGLI

飞七棋牌HUANZHEJIHUOJIANXIANSHIBAXIANWEIQINGDUZENGJIA,FEITEYIXINGⅡXINGJIXIANWEIWEISUO,JIWEISUOXIANWEIWEIJIANQUNZUHUAXIANXIANG。DIANJINGXIANSHITUCHUHOUMOZHOUZHEHEERJITUCHUJIANXIMIANJIZENGJIA,AChNANGPAOJISHOUTISHUMUZHENGCHANG,SHENJINGMOSHAOWUBIANXING。DINGLIANGLENGDONGKESHIDIANJINGYANJIUFAXIAN,HUANZHEAChSHIFANGBUWEIMIANJISUOXIAO,TUCHUQIANMODANWEIMIANJIHEAChSHIFANGBUWEIDANWEIMIANJIMONEIDAKELISHUJIANSHAO,PAILIEBUZHENGCHANGDEMONEIDAKELICONGJISHUZENGJIA,SHUOMINGZUIXIAOAChSHIFANGDANWEISHIFANGLIANGJIANSHAO。

LINCHUANGBIAOXIAN

飞七棋牌DABUFENHUANZHESHI40SUIYISHANGDENANXING,JIWULIZHENGZHUANGKEJIAOAIZHONGXIANCHUXIAN,BIAOXIANSIZHIJINDUANJIQUGANWULI,XIAZHIZHENGZHUANGZHONGYUSHANGZHI,DUANZANYONGLISHOUSUOHOUJILIFANERZENGQIANG,ERCHIXUSHOUSUOHOUJILIMINGXIANJIANRUO,CHENGBINGTAIPILAO。YIBANBULEIJIYANJIYUYANSUIZHIPEIDEJIROU,JINNIANLAIBAODAOYANSUIZHENGZHUANGJIAOQIANYOUZENGDUO,DANQIFASHENGLVYUANDIYUZHONGZHENGJIWULI。HUXIJILIRUOXURENGONGHUXIBUCHANGJIAN。JIANFANSHETEBIESHIXIAZHIFANSHEJINGCHANGJIANRUOHUOXIAOSHI,DANYUNDONGHOUJIANFANSHEKEHUIFU。SHAOSHUHUANZHEKEYOUZHITIGANJUEYICHANGHETENGTONG。KECHUXIANZIZHUSHENJINGGONGNENGYICHANG,BIAOXIANWEIKOUGAN、YANGWEIDENG。ZAIYIXIEHUANZHEZHONGKEYIJIANDAOLEMSYUYAJIXINGXIAONAOBIANXINGHENAOJISUIYANBANSUIFAZHAN。 (1) DUOYU50-70SUIFABING,NANNVZHIBI5:1。TONGCHANGYAJIXINGQIBING,BINGCHENGJINZHANKEBUTONG,TONGCHANGZAIFAXIANZHONGLIUQIANSHUYUEZHISHUNIANCHUXIANJIWULIHEYIPILAO。HUANJIFENBUYUMGBUTONG,YISIZHIGUGEJIWEIZHU,QUGANJI、GUPENDAIJIXIAZHIJI、JIANJIADAIDENGZHENGZHUANGYOUQIMINGXIAN,ZHENGZHUANGXIAZHIZHONGYUSHANGZHI,JINDUANZHONGYUYUANDUAN,BIAOXIANYABUHUOYAOBAIBUTAI。CHANGHEBINGSIZHIJIANFANSHEJIANRUOHUOXIAOSHI,JIANFANSHEKEZAIXIANGYINGJIROUDUANQIDALISHOUSUOHOUDUANZANHUIFU,HEBINGAIXINGDUOFAXINGSHENJINGBING(carcinomatious polyneuropathy)KECHUXIANJIANFANSHEXIAOSHI,ZHONGLIUKEYINQIQITASHENJINGXITONGBIAOXIAN,RUDUOFAXINGJIYAN、PIJIYAN、DUOZAOXINGBAIZHINAOBINGJIXIAONAOBIANXINGDENG。BUFENHUANZHEZHUSUJITONG,YIGUBUJIROUMINGXIAN,WUJISHUZHANDONG。 (2) SHOUFAZHENGZHUANGCHANGBIAOXIANQILI、SHANGLOUJIBUXINGKUNNAN,JIANBUJIROUJIAOWANBOJI。NAOSHENJINGZHIPEIJIRUYANWAIJIHEYANHOUJISHOULEIKECHUXIANSHANGJIANXIACHUI、FUSHI、GOUYINZHANGAIJITUNYANKUNNANDENG,JIAOSHAOJIAN,SHOULEICHENGDUJIAOQING。YUE62%DEHUANZHEQIBINGJIXIAZHIWULI,YUE18%YOUJITONGHUOJIANGZHI,HUXIJILIRUOXURENGONGHUXIBUCHANGJIAN。ZHENGZHUANGJINXINGXINGJIAZHONG,HUANZHEYONGLIDUOJIAOHOUKECHUXIANLEISIMGJIWULIJIAZHONG,DANQIANJICISHOUSUOSHIJILIKEZANSHIZENGQIANG,YEKEJIANCHASHOUWOLIHEQIAYAOJIJILIZHENGMING。KEYOUGANJUEYICHANG、GUANJIEYANYANGTENGTONG。YUEBANSHUYISHANGHUANZHECHUXIANZIZHUSHENJINGZHENGZHUANG,ZUICHANGJIANTUOYEFENMIJIANSHAOYINQIKOUGAN,YANGWEI、BIANMI、PAINIAOKUNNAN、LEIYEHEHANYEJIANSHAO、TIWEIXINGDIXUEYA、TONGKONGFANSHEYICHANGDENG。ZHENGZHUANGCHUXIANSHUNXUTONGCHANGWEIXIAZHIWULI、ZIZHUSHENJINGZHANGAI、SHANGZHIWULI、NAOSHENJINGZHIPEIJIWULI、JITONGJIJIANGZHIDENG。 (3) SCLC-LEMSYUNT-LEMSLINCHUANGBIAOXIANXIANGSI。①SCLC-LEMS:NANXINGHUANZHEJUDUO,YUE2/3HUANZHEBANAIZHONG,YUE60%HUANZHEHEBINGXIAOXIBAOFEIAI,YEJIANYURUXIANAI、QIANLIEXIANAI、WEIAI、SHENAI、ZHICHANGAI、LINBALIU、JIXINGBAIXUEBING、WANGZHIXIBAOROULIUDENG,GEBIEHEBINGXIONGXIANLIU;DUOZAI60-70SUIFABING,HENSHAO40SUIQIANFABING,DIANXINGCHENGJIXINGJINXINGXINGBINGCHENG,JIWULICHANGCHUXIANYUFAXIANEXINGZHONGLIUQIANSHUYUEZHISHUNIAN,HUANZHECHANGYINZHONGLIUBENSHENZAISHUYUEZHISHUNIANNEISIWANG;BENBINGKEJIANYUERTONG,TONGCHANGYUZHONGLIUWUGUAN;②NT-LEMS:LING1/3HUANZHEWEIFAXIANZHONGLIU,DUOBANQITAZISHENMIANYIXINGJIBING,RUEXINGPINXUE、JIAZHUANGXIANGONGNENGDIXIA、JIAZHUANGXIANKENENGKANGJIN、GANZAOZONGHEZHENG、LEIFENGSHIGUANJIEYAN、XITONGXINGHONGBANLANGCHUANG、BANTU、RUMIXIE、NIUPIXIAN、KUIYANGXINGJIECHANGYAN、SHAONIANXINGTANGNIAOBINGHEZHONGZHENGJIWULIDENG,YOUBAODAOBANYAJIXINGXIAONAOBIANXING。 [2] FUZHUJIANCHA

1. SHENJINGDIANSHENGLIJIANCHA

飞七棋牌(1) DIPIN(<10Hz)ZHONGFUDIANCIJIBOFUBIANHUABUDA,JIROUFUHEDONGZUODIANWEIKEXIAJIANG;GAOPIN(20-50Hz)ZHONGFUDIANCIJIHOUJIROUCHANSHENGQIANGLIEZIZHUSHOUSUO(CHIXU15MIAOHUOGENGCHANG),DONGZUODIANWEIBOFUMINGXIANZENGJIA(ZENGLIANGFANYING),ZENGGAO2-20BEI(BOFUZENGGAO200%YISHANGWEIYANGXING)。SHENJINGZHONGFUDIANCIJIQIAYUMGBIAOXIANXIANGFAN,SHICUSHIGAILIZILIURUSHENJINGMOSHAOCUJINAChDANWEIXINGSHIFANGSUOZHI。

(2) DALISHOUSUO15MIAOZHONGHOU,RUBOFUZENGGAOCHAOGUO25%YINGGAODUHUAIYIBENBING,CHAOGUO100%KEQUEZHEN。ZHENJIEMGKEJIANXIAODEDUOXIANGYUNDONGDANWEIDIANWEISHUMUZENGJIAJIBOFUBIANYI,DANGEJIROUYOUFAFUHEDONGZUODIANWEIBOFUMINGXIANJIANGDI,DANXIANWEIJIDIANTUXIANSHIRUMGDEZHANCHU(jitter)ZENGJIA。

飞七棋牌(3) LEMSZHOUWEISHENJINGWUYICHANG,SHENJINGDANYICIJIKECHANSHENGYIGEDIBOFUJIROUDONGZUODIANWEI,MGBINGRENZEZHENGCHANGHUOJIEJINZHENGCHANG。

2. 騰喜龍試驗、新斯的明試驗有時呈陽性反應,但遠不如MG患者敏感。

3. BENBINGHUANZHEXUEQINGAChR-AbSHUIPINGBUZENGGAO,GEBIEKEHEBINGYANJIANXIACHUIJIAChR-AbYANGXING。YUE34%DEJIWULIZONGHEZHENGHUANZHEYOUQIGUANTEYIXINGKANGTIHEMIANYIQIUDANBAIYICHANG(RUIgGkFUDANBAI)。XUEQINGJIMEIPUDUOZHENGCHANG。CELIANGDIANYAMENKONGXINGGAITONGDAOKANGTIYOUSHIYONGJIAZHI,JIWULIZONGHEZHENGP/QXINGGAITONGDAOJIEHEKANGTIXUEQINGXUESHIYANYANGXINGLVYUE95%,MG≤5%,YOUZHUYUJIANBIE。

4. BENBINGHUANZHEHLA-B8HEDR3DANTIXINGZENGJIA,LEISIQITAZISHENMIANYIBING。

5. BENBINGJIHUOJIANRUMGSUOJIAN,WEIZHENGCHANGHUOQINGWEIFEITEYIXINGBIANHUA。

ZHENDUANJIJIANBIEZHENDUAN

ZHENDUANYIJU

GENJULINCHUANGZHENGZHUANG、TIZHENG、DIANSHENGLIJIANCHAHEKANGTIJIANCEZHENDUANLEMS。LINCHUANGDIANXINGDESANLIANZHENGBAOKUOJINDUANJIWULI、ZIZHUSHENJINGZHENGZHUANGHEFANSHEXIAOSHI。ZHONGLAONIANNANXINGFEIAIHUANZHEYAJIXINGFABING,CHUXIANZHITIJINDUANDUICHENGXINGWULIHUOYIPILAO;HUANJIYONGLISHOUSUOHOUJILIDUANZANZENGQIANG,CHIXUSHOUSUOHOUCHENGBINGTAIPILAO; KOUGAN、KUOYUEJIGONGNENGZHANGAI、JITONGHEJIANFANSHEJIANRUO。 (1)JIWULI

飞七棋牌XIAZHIJINDUANWULISHICHANGJIANSHOUFAZHENGZHUANG,JIANYU80%DEHUANZHE。HENKUAICHUXIANSHANGZHIWULIHUOFAZHANWEISHANGZHIWULI。TONGCHANGWULICONGJINDUANFAZHANDAOYUANDUAN,BAOKUOZUHESHOU,CONGXIADAOSHANG,ZUIHOULEIJIYANBUQIUBUQUYU。SCLC-LEMSBINT-LEMSJINZHANSUDUGENGKUAI。YANBUZHENGZHUANGCHUXIANLVWEI0-80%,QIUBUZHENGZHUANGCHUXIANLVWEI5-80%;HUANBINGLVDEBIANHUAFANWEIRUCIZHIDA,SHIYOUYUCONGCHUXIANZHENGZHUANGDAOPINGGUDESHIJIANBUYIZHI。YOUYANJIUMIAOSHULEYIZUHUANZHECONG97DAO234GEHUANZHE,YANBUHEQIUBUZHENGZHUANGCHUXIANLVSHI30%HE32%,FABING3GEYUENEIFENBIESHANGSHENGDAO49%HE52%,TEBIESHISCLC-LEMS。SUIRANSHUGEBAODAOJINYOUYANBUZHENGZHUANGDEHUANZHE,DANSHIJIHUSUOYOUBANYOUYANBUZHENGZHUANGHUOHUXISHUAIJIEDEHUANZHEZAIJIBINGZAOQIYEYOUQUANSHENWULI。YUMGBIJIAO,DANDUYANWAIJIWULIHENSHAOJIAN。 (2) ZIZHUSHENJINGGONGNENGZHANGAI

ZIZHUSHENJINGGONGNENGZHANGAISHIZHENDUANLEMSDELINGYIGEZHONGYAOXIANSUO;ZIZHUSHENJINGGONGNENGZHANGAIBIANYIXINGHENDA,DANSHITONGCHANGBUSHIHENQINGWEI。80-96%DELEMSHUANZHEYOUZIZHUSHENJINGGONGNENGZHANGAI,JINGUANYIXIANGRIBENYANJIUBAODAOFASHENGLVJINWEI37%。ZAIHELANDEYANJIUZHONG,FABING3GEYUENEICONG66%SHANGSHENGDAO91%。KOUGANSHIZUICHANGJIANZHENGZHUANG,QICISHINANXINGDEBOQIGONGNENGZHANGAIHEBIANMI。ZHILIXINGDIXUEYA、PAINIAOKUNNAN、YANGANHECHUHANBIANHUASHISHAOJIANZHENGZHUANG。 (3) JIANFANSHE

LEMSHUANZHEJIANFANSHEJIANRUOHUOXIAOSHI。YOUYIGETEDIANSUIRANBUSHIHENMINGAN,YUNDONGHOUYIHUA,JIROUSHOUSUOHOUJIANFANSHEDUANZANHUIFUQIEJILIDADAOZHENGCHANGFANWEI。40%DEHUANZHECHUXIANZHEZHONGQINGKUANG,KEYIYANGAIJIANFANSHEJIANRUO。YINCIRUGUOHUAIYILEMSZHENDUAN,YINGZAIXIUXIHOUJIANCHAJIANFANSHE。

(4) FUZHUJIANCHA:

飞七棋牌a) DABUFENHUANZHEXUEQINGZHONGKEFAXIANP/QXINGDIANYAMENKONGGAITONGDAO(voltage-gated calcium channels,VGCC)KANGTI;

b) JIDIANTUZHONGFUDIANCIJIKEJIANDIPIN(2-5Hz)CIJISHIDONGZUODIANWEIBOFUJIANGDI,GAOPIN(10Hz)CIJISHIBOFUZENGGAO;

c) TENGXILONGSHIYANBUMINGAN;

d) XUEQINGAChR-AbYINXING。

(5) KANGDANJIANZHIMEIYAOWUBUMINGAN。

JIANBIEZHENDUAN

(1) 重癥肌無力:常常以眼肌麻痹為首發癥狀,晨輕幕重,新斯的明試驗陽性,Ach受體抗體陽性,肌電圖低頻重復電刺激波幅遞減,抗膽堿酯酶藥物有效等可資鑒別。 (2) 多發性肌炎:主要侵犯骨骼肌,表現以近端力弱為主,常伴頸肌無力及典型皮膚損害,無感覺障礙,結合血清CK活性明顯增高,肌電圖顯示肌源性損害,可以鑒別,必要時肌活檢。 (3) 關節炎患者因疼痛剛開始活動時運動受阻,連續活動后可減輕。 (4) 吉蘭巴雷綜合征飞七棋牌:患者病前1-4周的感染史,急性或亞急性起病,四肢對稱性遲緩性癱瘓,可伴有感覺異常和末梢型感覺障礙、腦神經受損、CSF蛋白-細胞分離現象,肌電圖早期F波或H反射延遲、NCV減慢、遠端潛伏期延長。 疾病治療

飞七棋牌ZHILIAOYUANFABING:ZHENDUIYUANFAZHONGLIUDEZHILIAOKEYISHISHENJINGXITONGZHENGZHUANGHUODEGAISHAN。ZHENDUANLEMSJIYIWEIQIANZAIZHONGLIU,YOUQIXIAOXIBAOFEIAI,YIDANFAXIANYUANFAXINGZHONGLIUYINGJINXINGBINGYINZHILIAORUSHOUSHU、SHENBUFANGLIAOJIHUALIAODENG,HUANJIEZHONGLIUZHENGZHUANG,BINGKENENGGAISHANSHENJINGXITONGZHENGZHUANG。SHOUSHUQIECHUFEIAIJISHIDUIYUANFABINGWUBU,DANCHANGKEGAISHANJIWULIZHENGZHUANG。RUOWUHEBINGZHONGLIUZHENGJUYINGSUIFANG3NIANYISHANG,DINGQIFUCHA,GEIYUPONISONG80mgGERIKOUFU,LIUZUOPIAOLING2-3mg/(kg.d)KOUFU,ZHENGZHUANGHAOZHUANHUOHUANJIEHOUPONISONGYINGJIANLIANG。YINZHONGLIUKENENGHENXIAO,SHENZHISHIJIANYEBUNENGFAXIAN。YOUDEZUOZHEZAIFEIZHONGLIUHUANZHEJINGFANFUXUEJIANGJIAOHUANLIAOFA、PONISONGYULIUZUOPIAOLINGLIANHEYINGYONG,QUDEHENHAOLIAOXIAO,DANXUEJIANGJIAOHUANLIAOXIAOSHIDUANZANDE。

(1) ZHENGZHUANGXINGZHILIAOFANGMIANKEYIYINGYONGYIXIANDANJIANSHIFANGZENGQIANGJI,LIRU3,4-ERANJIBIDING(3,4-diaminopyridine, DAP)KEYIZENGJIASHENJINGJIROUJIETOUTUCHUQIANMOAChSHIFANG,JILIANG10-20mg/d,FEN4-5CIKOUFU。KEYUBIDINGSIDEMINGHEYONG,FUZUOYONGXIAO,XIANGDUIWUDUXING。YOUSHIFUYAOHOU1XIAOSHICHUXIANKOUZHOUGANJUEYICHANG、WEICHANGDAOZHENGZHUANG,OUYOUDIANXIANFAZUOJIJINGSHENHUNLUANDENGFUZUOYONG,DIANXIANFAZUOYUJILIANGXIANGGUAN,DANGMEITIANDAYU100mgSHIRONGYICHUXIAN。OUYOUSHISHANGXINGXINDONGGUOSU、HEXINJIGENGSIDEBAODAO。 YANSUANGUASHIAChSHIFANGZENGJIAERSHIZHENGZHUANGGAISHAN,ZENGQIANGJILIJIAOXINSIDEMINGJIBIDINGSIDEMINGYOUXIAO,JILIANG10-30mg/(kg.d),FEN4CIKOUFU。YOUGUSUIYIZHI、JIANZHIXINGSHENYAN、SHENXIAOGUANHUAISI、WEICHANGDAOZHANGAI、XINFANGZHANDONGJIDIXUEYADENGYANZHONGFUZUOYONG,YINGCONGXIAOJILIANGKAISHI,JINZAOJIANLIANG,JIANCEXUECHANGGUI、JIGANHENIAOSUDANDENG。YIBEI3,4-ERANJIBIDINGQUDAI。 (2) DANJIANZHIMEIYIZHIJIRUXIUBISIDEMINGJIXINSIDEMINGTONGCHANGWUXIAO,XIBAODUXINGYAOWUYINGSHENYONG。JINGWEICHANGDAOWAIGEIMEIKEZUZHIAChSHIFANG,JIAZHONGJIWULI,YINGSHENYONGYINGXIANGSHENJINGJIROUJIETOUCHUANDAOYAOWU,RUANJITANGDAILEIKANGSHENGSU,GAITONGDAOZUZHIJIRUYIBODING、PULUKAYINAN、KUINIDING,β-SHENSHANGXIANSUZUZHIJIHELIDENG;YOUXUANJIANDUJIAN、HUPOXIANDANJIAN、LVHUAWU、SANDIANJIANFENJIJISONGJIDUIBENBINGJUNYOUBULIANGZUOYONG,SHIJIWULIJIAZHONG,SHENZHISIWANG。

(3) ZAISHANGSHUZHILIAOWUXIAODEQINGKUANGXIA,KEKAOLVYINGYONGMIANYIYIZHIJI、XUEJIANGJIAOHUANHUOMIANYIQIUDANBAICHONGJIZHILIAO。YUQITALEIXINGDESHENJINGXITONGFUZHONGLIUZONGHEZHENGBUTONG,XUEJIANGZHIHUANHEMIANYIYIZHIJIYOUYIDINGDEZHILIAOXIAOGUO。StreibHERothnerYONGPONISONGZHILIAOBENBINGKESHIHUANZHEBINGQINGCHANGQIGAISHAN,MIANYIQIUDANBAIJINGMODIZHUYEKEYOUXIAO。PONISONG25-60mg/dKEYULIUZUOPIAOLING2.3-2.9mg/(kg.d)GERIJIAOTISHIYONG,FUYIMIANYIQIUDANBAIJINGMOLIAOFA,QIXIAOXUSHUYUEZHI1NIAN,JILIKEWANQUANHUOBUFENHUIFU。YIYOUYINGYONGLITUOXIDANKANGYOUXIAODEBAODAO。 [3] CANKAOZILIAO 1. JIAJIANPINGZHUBIAN.SHENJINGBINGXUE:RENMINWEISHENGCHUBANSHE,2009:527-528 2. WANGWEIZHIZHUBIAN.SHENJINGBINGXUE:RENMINWEISHENGCHUBANSHE,2006:1190-1192 3. Titulaer MJ, Lang B, Verschuuren JJ.Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies:Lancet Neurol,2011:10(12):1098-1107

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