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1、中國(guó)神經(jīng)免疫學(xué)和神經(jīng)病學(xué)雜志2014年5月第2l卷第3期ChinJNeuroimlTIUnol&Neurol2O14,Vo1．21，No．3進(jìn)行性核上性眼肌麻痹7例及文獻(xiàn)復(fù)習(xí)陳斐聶志余張?zhí)煊罟軓?qiáng)靳令經(jīng)摘要：目的探討進(jìn)行性核上性眼肌麻痹(progressivesupranuclearpalsy。PSP)的臨床和腦MRI特征．以提高臨床醫(yī)生對(duì)PSP的認(rèn)識(shí)。方法回顧性分析7例PSP患者的臨床及影像學(xué)資料并復(fù)習(xí)相關(guān)文獻(xiàn)。結(jié)果7例中男6例，女1例；發(fā)病年齡47～68歲。均隱匿起病，緩慢進(jìn)展。從發(fā)病到就診的時(shí)間為O～5年，從就診
2、到診斷PSP的時(shí)間為1～l2年。均為散發(fā)病例。4例以動(dòng)作變慢、步態(tài)不穩(wěn)為首發(fā)癥狀；2例以眼部癥狀起??；1例首先出現(xiàn)雙手抖動(dòng)。所有患者均有核上性眼肌麻痹和反復(fù)跌倒，6例有軸性肌張力增高和假性延髓性麻痹，4例出現(xiàn)sittingenbloc征，2例有認(rèn)知障礙。7例MR均顯示“蜂鳥(niǎo)征”和“鼠耳征”。對(duì)照NINDSSPSP診斷標(biāo)準(zhǔn)，2例擬診，5例疑診。結(jié)論P(yáng)SP主要發(fā)生于中老年人，以反復(fù)跌倒、軀干肌肌強(qiáng)直、垂直性核上性凝視麻痹為臨床特征；腦MR矢狀位顯示中腦被蓋部“蜂鳥(niǎo)征”，軸位顯示中腦“鼠耳征”是其影像學(xué)特征。PSP的診斷主
3、要依據(jù)臨床表現(xiàn)及神經(jīng)影像學(xué)檢查，早期易誤診。關(guān)鍵詞：核上麻痹，進(jìn)行性；臨床表現(xiàn)；磁共振成像；診斷中圖分類(lèi)號(hào)：R742．5；R742．8。。9文獻(xiàn)標(biāo)識(shí)碼：A文章編號(hào)：10062963(20l4)03—0l7OO4Progressivesupranuclearpalsy：Areportof7casesandliteraturereviewCHENFei，NIEZhi—yu，ZHANGTian—“，GUANQiang，JINLing-jing．Department0廠Neurology，ShanghaiTongjiHosp
4、ital，TongjiUniversitySchoo2ofMedicine，Shanghai200065，ChinaCorrespondingauthor：NIEZhi—yuEmail：nzhiyu2002@sina．cornABSTRACT：0bjectiveToimprovetherecognitionofprogressivesupranuclearpalsy(PSI')，theclinicalandneuroimagingfeaturesofPSPwereanalyzed．MethodsTheclinical
5、dataof7caseswithPSPwereanalyzedretrosDective1v，andtherelatedliteraturesofPSPwerereviewed．ResultsAmongthe7cases，6weremale，and1wasfemale．Theageofonsetwas47—68years．Theonsetofallthepatientswasinsidiousandthediseaseslowlyprogressed．Thedurationbetweentheonsetandoffi
6、cevisitingwas0-5years，theintermissionfromofficevisitingtoestablishmentofdiagnosisofPSPwas112years．Allthepatientsweresporadic．TheinitialsymptomsofPSPwereposturalinstability(4／7)，symptomsofeyes(2／7)，andtremorofhands(1／7)．Supranucleargazepalsyandfallspresentedinal
7、lofourcases，6patientsshowedaxialdystoniaandpseudobulbarpalsy，4patientsshowedsittingenblocsign，and2patientsshowedmildcognitiveimpairment．HummingbirdsignandInouseearssignonMR1wereobservedinallofthem．AccordingtothediagnosticcriteriaofN1NDS—SPSP，2patientswereprobab
8、lePSPand5werepossiblePSP．ConclusionsPSPisarareneurodegenerativediseaseoccurringinelderlypeople．TheclinicalfeaturesofPSPareposturalinstability，falls，supranucleargazepalsy，axi
