摘要:
Objective Toinvestigatethetypicalclinicalcharacteristics,imagingfindingsand drug therapyofdiabetichemiballism-hemichorea (DHB-HC). Methods Literaturerelatedto DHB-HC case reportsincludedin MedlinedatabasefromJanuary1,2008toJune30,2018 weresearchedaccordingtostrict standards,andtheclinicalmanifestations,imagingcharacteristicsandprognosisoftheDHB-Hcpatientswere reviewed.Results 23papersinvolving53patientswerefinallyincluded.35cases (66.0%)wereAsianpatients. Therewere13males(24.5%)and40females(75.5%),agedfrom14to91yearsold,withamedianageof74 (interquartilerange:16yearsold).5patients (9.4%)showedbilateraldancingsymptoms,and48patients (90.6%)showedhemidancingsymptoms. MRexaminationwasperformedin42patientsandhyperintensityin T1-weightedimaging wasfoundin33patients (78.6%). 24ofthe53patients (45.3%)hadspontaneous remissionbyhypoglycemictherapyonly,29patients (54.7%)hadremissionbyadditionalallopathicdrug,3 patients(5.7%)relapsedwithin1year.Conclusions DHB-HCiscommonlyseeninelderlyAsianwomen.Its characteristicsaremostlyone-sidedsymptoms;hyperglycemia,hyperglycosylatedhemoglobin,negativeketone body,andhyperintensityinT1imageatbasalgangliapresentattheonset. Activecontrolofbloodglucoseand symptomatictreatmentarehelpfultoshortenthecourseofdiseaseandimprovetheprognosis.%目的 探讨糖尿病性偏侧投掷症-偏侧舞蹈症(diabetichemiballism-hemichorea,DHB-HC)患者典型临床表现、影像学表现及药物治疗效果.方法 检索2008-01-01—2018-06-30 Medline数据库收录的有关DHB-HC病例报道,严格筛选文献,系统综述DHB-HC患者的临床表现、影像学特征及预后.结果 共纳入23篇文献,53例患者,其中35例(66.0%)为亚裔;男13例(24.5%)、女40例(75.5%),年龄14~91岁,中位数74岁,四分位数间距16岁;5例(9.4%)表现双侧舞蹈症状,48例(90.6%)偏身舞蹈症状;42例患者行头颅 MR检查, 33例(78.6%)发现基底节区T1加权像高信号;单纯降糖治疗者24例(45.3%),降糖同时加用对症药物治疗者29例(54.7%),53例患者舞蹈症状均缓解或消失,3例(5.7%)患者1年内复发.结论 DHB-HC好发于亚裔老年女性,其临床特征多为偏侧症状,发病时呈高血糖、高糖化血红蛋白、酮体阴性、头颅 MR基底节T1加权像高信号,积极控制血糖及对症治疗有助于缩短病程,提高预后.