进行性核上性眼肌麻痹7例及文献复习.pdf

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1、中国神经免疫学和神经病学杂志2014年5月第2l卷第3期ChinJNeuroimlTIUnol&Neurol2O14,Vo1．21，No．3进行性核上性眼肌麻痹7例及文献复习陈斐聂志余张天宇管强靳令经摘要：目的探讨进行性核上性眼肌麻痹(progressivesupranuclearpalsy。PSP)的临床和脑MRI特征．以提高临床医生对PSP的认识。方法回顾性分析7例PSP患者的临床及影像学资料并复习相关文献。结果7例中男6例，女1例；发病年龄47～68岁。均隐匿起病，缓慢进展。从发病到就诊的时间为O～5年，从就诊

2、到诊断PSP的时间为1～l2年。均为散发病例。4例以动作变慢、步态不稳为首发症状；2例以眼部症状起病；1例首先出现双手抖动。所有患者均有核上性眼肌麻痹和反复跌倒，6例有轴性肌张力增高和假性延髓性麻痹，4例出现sittingenbloc征，2例有认知障碍。7例MR均显示“蜂鸟征”和“鼠耳征”。对照NINDSSPSP诊断标准，2例拟诊，5例疑诊。结论PSP主要发生于中老年人，以反复跌倒、躯干肌肌强直、垂直性核上性凝视麻痹为临床特征；脑MR矢状位显示中脑被盖部“蜂鸟征”，轴位显示中脑“鼠耳征”是其影像学特征。PSP的诊断主

3、要依据临床表现及神经影像学检查，早期易误诊。关键词：核上麻痹，进行性；临床表现；磁共振成像；诊断中图分类号：R742．5；R742．8。。9文献标识码：A文章编号：10062963(20l4)03—0l7OO4Progressivesupranuclearpalsy：Areportof7casesandliteraturereviewCHENFei，NIEZhi—yu，ZHANGTian—“，GUANQiang，JINLing-jing．Department0厂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