Creutzfeldt

Creutzfeldt-Jakob纾辨

涓界缁绁缁瀛蹇1999骞寸4绗6 璁恒

浣锛涓瀹璧佃缁姹版璋疯宠

浣锛芥╁荤澶у绗涓村瀛㈢缁绉锛 挎130021

抽:Creutzfeldt-Jakob锛纾辨娲绘

瑕璁ㄥごㄧ辨Creutzfeldt-Jakob（CJD）璇环笺规2渚缁娲绘瀹楠榧浼璇瀹锛212涓杩琛澶撮ㄧ辨ャ缁2渚渚у熬搞澹虫镐T2瀵圭Оу璐ㄩ淇″缁璁恒澶撮ㄧ辨寮甯镐俊瀵CJD璇介瀹ㄧ瑰锛浣缁涓村锋涓瀹浠峰笺

涓惧R742.8

MagneticResonanceImageDiagnosisof

Creutzfeldt-JakobDisease

LinShiheSongXiaonanZhaoJiexuJiangXinmeiGuYangying

锛DepartmentofNeurology,TheFirstTeachingHospitalofNormanBethuneUniversityofMedicalSciences,Changchun130021锛

ObjectiveToassessadiagnosticutilityofimagingfeaturesofmagneticresonanceimages（MRI）ofpatientswithCreutzfeldt-Jakobdisease（CJD）.MethodsToreviewtheMRIoftwopatientswhosediagnosisofCJDhadprovedbyimmunohistochemicalstainingandexperimentalanimaltransmission.Thepatientsclinicalfindingswerealsoanalyzed.ResultsThetwopatientshadMRIexaminationsonsecondandtwelfthmonthrespectivelyaftertheonsetofsymptoms.InalltwopatientsthehyperintensesinalabnormalitiesinthecaudatenucleiandbasalgangliaonprotondensityandT2-weightedimageswerediffuseandbilaterallysymmetric.ConclusionsSignalabnormalityinthebasalgangliaandcaudatenucleionMRimagingsmaybeaspecificsignofCreutzfeldt-JakobdiseaseandcouldbehelpfulinconfirmingthediagnosisofCJD.

KeywordsCreutzfeldt-Jakobdisease;magneticresonanceimage;brainbiopsy

卞藉琛ㄤ涓Creutzfeldt-Jakob（CJD）瑕绯讳互ワ浜轰滑姝ｅㄥ娉ㄦ板CJD锛骞剁Н绱CJD╂璇规娲绘璇跺锛浣ｈ涓杩ユ浠敞板ごㄧ辨（MRI）瑰锛涓浠ㄦ堕翠╀ごCT锛骞跺锋涓瀹瑰э灞浜ㄣ绠渚裤浼ゆфワ涓CJD璇渚渚

1璧规

1.1渚璧2渚锋稿CJD涓村琛ㄧ锛朵腑1渚4骞村藉不1516涓姝讳骸

1.2瀹楠瀹ゆャ2渚琛棰娲绘锛骞剁互（prionprotein,PrP）琛娓涓虹涓浣诧缁缁舵5锝15渭g/dL娴锛绉浜锛绉543486澶（d）榧ょ缁纭澶锛杩寰板缁瀛茶瀹

1.3澶撮ㄥ奖妫ャ2渚峰PhilipsGyroscanT5-″烘哄己0.5锛ㄨ娉（SE）T1T2璐ㄥ瀵搴酱浣

2渚ュ缁

渚1锛濂筹57宀锛瀹跺★浜恒1994骞1226ュラ1骞存ヤ袱涓㈡椿ㄤ碉娓杩2绮剧涓姝ｅ父锛荤锛璇浼锛涓瑗匡涔辨┿ラ㈠1涓у涓璧凤㈠电‖锛灏夸究澶辩绋涓芥㈠澶村浼ゅ杈琛1990骞存藉不瀹舵腑绫讳技剧

ヤ锛绉妫ユ规锛ｈ舵㈣寮澧楂卞灏浜㈣宸晶灏虫э灏夸究澶辩

瀹楠瀹ゆワ琛灏垮父瑙芥甯搞靛炬ワCT姝ｅ父澶撮ㄧ辨〃涓哄渚у熬舵搞澹虫稿瀵圭Оу甯镐俊锛T1俊绋浣璐ㄥ瀵搴T2楂淇″（1锝3）1994骞1227ヨ渚ч川娲绘锛宠″锛HEKB诧瑙瀵杞姝ｅ父锛澶ц杞诲害娴风坏跺э绁缁辫斤褰㈣惰川缁澧1995骞14ュ-70淇瀛娲绘缁缁舵15渭g/dL娴锛绉浜锛ㄦ绉543d榧PrP娌绉ｈ浜1995骞37ユ浜°

1杞翠T1（TR520/TE25）渚у熬舵搞澹虫镐俊绋浣

Fig1AxialT1-Weighted（TR520/TE25）imagesshowsymmetrichypodensesignalincaudatenucleiandputaminaofbothsides

2杞翠璐ㄥ瀵搴（TR2190/TE25）锛涓1涓哄涓灞渚у熬舵搞澹虫镐俊绋楂

Fig2Axialprotondensity（TR2190/TE25）image,thesameplanewithFig1,showhyperintensesignalincaudatenucleiandputaminaofbothsides

3杞翠T2（TR2190/TE90）锛涓1锛2涓哄涓灞渚у熬舵搞澹虫镐负楂淇″

Fig3AxialT2-weighted（TR2190/TE90）image,thesameplanewithFig1andFig2,showhyperintensesingalinthecaudatenucleiandputaminaofbothsides

渚2锛凤73宀锛骞查浜恒1995骞112ュラｈ浜ラ㈠2涓锛瑷璇锛璁板1涓杩扮跺锛涓诲ㄨ璇娲诲ㄦ垫椿锛哥户虹韬涓瀹澶芥㈤灏夸究澶辩姝ゅу锛楗按杩㈠棰澶浼ゅ瀹舵腑绫讳技剧

ヤ锛绉妫ユ甯搞舵锛间涓搴锛ㄤ煎浣у宸瑙锛浼村乏渚ч㈠涓㈡芥稿灏虫э㈣寮楂锛卞灏浜㈣锛灏存с灏夸究澶辩

瀹楠瀹ゆワ琛灏垮父瑙芥甯搞靛俱澶撮ㄧ辨〃涓哄渚у熬舵搞澹虫镐川瀛瀵搴T2瀵圭О璐ㄩ淇″（4锝6）渚ч川娲绘瑙杞诲害娴风坏跺э绁缁卞け褰㈣惰川缁澧1995骞112ュ-70淇瀛娲绘缁缁舵15渭g/dL娴锛绉浜锛缁486d瑙瀵灏ｈ娲绘缁缁瀹楠榧PrP娌绉淇¤寰ユｈ浜16涓姝讳骸

4杞翠锛T1（TR520/TE25）

渚у哄轰俊姝ｅ父

Fig4AxialT1-weighted（TR520/TE25）

imagesshownormalsingalinthe

basalgangliaofbothsides

5锛6杞翠锛璐ㄥ瀵搴（TR2190/TE25）T2（TR2190/TE90）锛

涓1涓哄涓灞渚у熬舵稿澹虫搁淇″

Fig5锛6AxialprotondensityandT2-weightedimages,thesameplanewithFig1,

showhyperintensesingalinthecaudatenucleiandputamineofbothsides

3璁ㄨ

CJDKuruGerstmann-Straussler缁煎寰（GSS）缁浼绁缁郴缁х剧杩骞村存ууけ（fatalfamilialinsomnia,FFI）浜浜缁剧寸瀛涓轰绉锋ц川规借川锛姝ょ剧琚绉颁负姣界锛1锛2锛姐

璧琛ㄦ涓戒汉CJDユу杈澶锛寰寰浠ョ缁跺濮锛绋互缉涓剧充娇杩凤复搴跺浣寰杩惧纭姝わ瀵绘句绉涓村ㄣ纭瑙纭规锛涓浠╀ｈ锛涓瀵归叉绘т锛3锛4锛姐

娲绘渚纭璇缁缁浠PrP琛娓涓虹涓浣诧涔100%璇蓟3锛4锛姐虫′欢椤婚婚哄缁缁锛寰寰ｈ瀹跺缁靛炬锋璇ㄦу姝ユ剧碉涔藉ㄧ灞颁瀹绋搴甯10ㄤ互虹锛3锛姐姝わ浜轰滑涓寰涓瀵绘句绉瀹ㄣ绠渚裤妫ユ规澶撮CT妫ヨ藉澶ц川缉锛浣瑰ㄥ稿夸娈垫舵锛CT瑙浠ュㄦ甯搞澶撮MRI浼浜ごCTュ2渚锛122澶撮MRI妫ワ2渚瑙T2璐ㄥ瀵搴渚у熬舵搞澹虫稿瀵圭О璐ㄩ淇″璇2渚缁ぇ娲绘璇瀹涓CJD锛娲绘缁缁娴绉浜锛486543d瀹楠ㄧ╀浠寰扮缁璇瀹锛涓ㄥ楠榧澶цPrP娌绉娆¤瀹2渚璇CJD瀹ㄥ淇°

1985骞翠互锛炬浜烘ュMRI妫ュCJD浠讳璇1988骞GertzHT绛浜猴蓟5锛芥ュ1渚55宀ｈ锛浜9涓锛虫浜″4ㄨ澶撮MRI妫ワ搴轰T2楂淇″妫璇瀹璇ヤ翠釜澶ц川娴风坏跺с惰川缁澧杞诲井绁缁涓уけ锛灏剧舵搞澹虫搞界涓浜ぇ川锛5锛姐缈骞达PrarlGS绛浜烘ュ涓68宀濂虫фｈ锛浜6涓锛虫4涓澶撮MRI妫ワ渚у熬舵镐T2楂淇″锛浣灏剧舵歌疆寤姝ｅ父ㄥ妫璇瀹渚у熬舵搞澹虫告剧娴风坏跺с惰川缁澧绁缁涓уけ锛界涓澶ц川稿杈杞伙蓟6锛姐涔浜鸿瀹骞跺己璋浜渚у熬舵搞澹虫镐T2瀵圭Оу璐ㄧ楂淇″锛寰灏娉㈠界锛涔涓澧寮烘搴T1瀹ㄦ甯革蓟7锛8锛姐杩绉寰璞″CJD涓村璇瑕

ㄥ涓ゆCJDMRI瑙讹蹇椤讳藉虹绫讳技瑰剧濡涓ラ缂烘哀浣绯CO涓搁村杩浜剧CT妫ュT1涔搴轰俊锛涓村朵害句Huntington韫渚у熬舵T2淇″锛浣澶ц川灏剧舵歌缉绐恒姝ゅ锛绾跨浣涓灏瑙绫诲Leigh锛藉虹绫讳技锛浣CT涓T1俊绐恒姝ょㄥ跨锛琛ㄧ涓虹簿绁寮甯搞瑙绁缁缉艰楹荤逛互肩涓绛涓昏涓哄绉版т腑搴涓瑙绁缁褰㈡Alzheimer芥杩琛х村锛浣MRI缁涓CJD绫讳技瑰版扮稿锛T2跺ㄥ瑙颁俊

灏界MRI瑙瀵CJD璇瑕锛浣剁瑰ф瑰骞堕100%虹CJD翠釜杩绋涓Finkenstaedtュ寰峰界‘璇骞剁ごMRI妫ョ29渚CJD锛朵腑23渚（79%）瑰捐瑰渚у熬搞澹虫镐川瀛瀵搴T2瀵圭О璐ㄩ淇″锛朵腑7渚（24%）渚ц界4渚（14%）涓绫讳技瑰锛3渚川1渚灏川涔镐技瑰6渚（21%）MRI浠讳瑰锛8锛姐涔浠ュ姝わ娴涓舵绱ㄤ炽宸茬CJD绁缁卞け惰川缁澧锛浠绋涓浜捣缁电跺эMRI瑙锛涓缁卞け惰川缁澧锛杈涓捣缁电跺ф翠负瀵锛5锛姐

杩ワ涔浜烘CJD涔MRI瑰锛绯诲烘捣缁电跺э璇稿绌烘场娑蹭璐褰㈣惰川缁澧缁锛8锛姐瀵圭浼CJDｈ锛T1甯革璐ㄥ瀵搴T2灏剧舵稿ご澹虫搞界瀵圭Ос璐ㄩ淇″讹CJD璇瑕渚煎瑙

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（1998-03-13剁）