摘要
目的对伴有偏侧舞蹈症以及MRI表现为纹状体T1高信号的非酮症性高血糖患者的临床表现、影像学特征及发病机制进行分析。方法对2000年10月～2002年3月诊治的4例伴有偏侧舞蹈症的非酮症性高血糖患者的临床资料进行分析并复习近10年文献。结果4例患者均为老年人,急性发病。发病时血糖水平为16.2～28mmol/L,血浆酮体检测多呈阴性;舞蹈样症状可累及患者的单侧和(或)双侧肢体或面部。头部影像学检查:CT显示为病变对侧的尾状核头及壳核于早期呈高密度,短时间内可消失;MRI于T1加权像呈高信号,T2加权像呈稍低信号,经历数月信号可无明显变化。采用降低血糖,并联合应用氟哌啶醇、氯丙嗪等药物治疗对控制舞蹈样症状有效。结论非酮症性高血糖、偏侧舞蹈症以及MRI表现为纹状体T1高信号,可能构成偏侧舞蹈症临床综合征;影像学改变可能表示由代谢紊乱引起的斑片状脑出血,后者导致基底神经节运动环路受损,从而产生舞蹈样症状。
Objectiv e To study the clinical manifestations,neuroimaging characteristics and patho-genesis of hemichorea with striatal hyperintensity on T 1 -weighted MRI in non-ketotic hyperglycemic patients.Meth ods Four non-ketotic hyperglycemic patients with hemichorea treated in this hospital during October2000to March2002were enrolled in this study.The clinical data,neuroimaging and laboratory findings of these patients were analyzed and related literatures in the past decade were reviewed.Results All of the4patients were the aged presenting an acute attack of grimace or chorea in face and unilateral and/or bilateral extremities.The level of blood glucose at onset was16.2～28mmol/L,but plasma ketone were negative or±.The CT scaning showed high density of the caudate nucleus and putamen contralateral to the affected extrem-ities,which disappeared within a short time;correspondingly,MRI showed hyperintensity on T 1 -weighted im-ages and hypointensity on T 2 -weighted images at onset,and persisted without significant change over several months.Administration of hypoglycemic agent combined with haloperidol and chlorpromazine could effective-ly relieve the chorea symptoms.Conclusion The combination of non ketotic hyperglycemia,hemichorea and striatal hyperintensity on T 1 -weighted MRI could constitute a unique syndrome.The imaging patterns may show cerebral petechial hemorrhage results from metabolic disorders,which causes the damage of basal gan-glionic motor loop and results in the occurrence of chorea symptoms.[
