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1、原发性脐月氐体变性89例临床分析黎宏',魏有东冷W笃2(611230四川崇州,崇州市人尺医院重症医学科;400016重庆,重庆医科大学附屈第一医院神经内科J摘要目的:分析国内原发性脐紙体变性的症状、体征及影像学检查等临床特点,提高临床诊断的准确性。方法:借鉴循证医学系统评价方法,对1990-2013年国内文献报道的临床病例资料及重庆医科大学附属笫一医院神经内科2009〜2012年临床诊断为原发性腓肮体变性的住院病历资料进行综合分析。结果:剔除可能重复的病例后,共收集到133例,其小完整病例资料89例,5例来自重庆医科人学附属第一医院病历记录,84例来自文献报道
2、。其中男性83例,女性6例,平均年龄47岁。主要症状依次有认知功能障碍52例(58.4%),精神行为异常29例(32.6%),意识障碍27例(30.3%),吐词不清26例(29.2%),反应迟钝26例(29.2%),癫痫发作22例(24.7%)等。57例头颅MRI均为阳性,“夹心征”为特征性改变。结论:MBD临床症状复杂多样,无特异性,认知功能障碍、精神行为异常、意识障碍较多见,绝大多数有长期大量饮酒史,有特征性影像学表现,MRI检査对早期诊断冇重要价值,结介饮酒史、常见临床症状及头颅MRI检查,MBD可早期诊断,人剂量B族维生素治疗有效,明显改善预后。关键词
3、:腓臆体变性;症状;磁共振;诊断Retrospectiveanalysisof89patientswithMarchiafava-BignamidiseaseLiHong1,WeiYoudong2,CaoDu2(DepartmentofIntensiveCareUnit,ChongzhouHospital;DepartmentofNeurology,FirstAffiliatedHospitalofChongqingMedicalUniversity2)【Abstract]ObjectiveTostudytheclinicalcharacteristicsof
4、Marchiafava-Bignamidisease.MethodsTherecordsofMBDpatientsinFirstAffiliatedHospitalofChongqingMedicalUniversityfrom2009-2012andtheliteratureinChinaBiologicalMedicineDatabase(CBM)andChinaNationalKnowledgeInfrastructure(CNKI)from1990〜2012wererecollected.Demographicdata,clinicalmanifest
5、ations,laboratoryfindingsofpatientswithsCJDwereanalyzed.ResultsOfthetotal133patients,84integraldata,5caseswerefromtherecords,and86casescamefromtheliteratures.Meanagewas47yearsold.Thecardinalsymptomsinturnwerecognitivedysfunction(58.4%),mentalandbehavioralabnormalities(32.6%),impaire
6、dconsciousness(30.3%),languagebarriers(29.2%),unresponsive(29.2%),seizures(24.7%)etc.57casesarepositiveonMRI,uSandwichsign”isspecificforMBD・ConclusionsTheclinicalsymptomsofMBDarevariousandnon-specific,cognitivedysfunction,mentalandbehavioralabnormalitiesandconsciousnessarccommonsymp
7、toms.Themajoritycaseshavealonghistoryofheavydrinking・TherearecharacteristicimagingfindingsinMRIexaminationandMRIhassignificantvalueforearlydiagnosis.Combinedwithdrinkinghistory,commonclinicalsymptomsandbrainMRI,theearlydiagnosisofMBDisnotdifficult・TreatmentoflargedosesofBvitaminsise
8、ffective,andcanimpr