非酮症性高血糖合并偏侧舞蹈症临床与影像特征分析

Clinical and Neuroimaging Features of Hemichorea Associated with Non-ketotic Hyperglycaemia

目的探讨非酮症性高血糖合并偏侧舞蹈症的临床表现及影像学特征。方法对7例非酮症性高血糖合并偏侧舞蹈症患者的临床资料进行分析,并复习相关文献。结果 7例患者均为中老年人,急性发病,发病时血糖水平为15.7-32.7 mmol/L、酮体阴性;表现为偏侧肢体舞蹈样症状。影像学特征：CT显示病变对侧的纹状体于早期呈高密度,短时间内可消失;MRI于T1加权像呈高信号,T2加权像呈低或等信号。降低血糖联合氟哌啶醇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,提示偏侧舞蹈症的临床综合征。

Objective To investigate the clinical manifestations,neuroimaging characteristics and pathogenesis of hemichorea associated with non-ketotic hyperglycaemia.Methods Seven cases were enrolled in this study.The clinical data,neuroimaging and laboratory findings were analyzed and related literatures were reviewed.Results All the seven cases were middle-aged and aged presenting an acute attack of chorea in unilateral extremities.The level of blood glucose at the onset was 15.7~32.7 mmol/L,and the ketone was negative.The CT scanning showed high density of the striatum contralateral to the affected extremities,and it can disappear within a short time.MRI showed hyperintensity on T1-weighted images and hypointensity or isointensity on T2-weighted images at the onset.Administration of hypoglycemic agent combined with haloperidol could effectively relieve the chorea symptoms.Conclusion The combination of non-ketotic hyperglycaemia,hemichorea and striatal hyperintensity on T1-weighted MRI could constituted an unique syndrome of hemichorea.The pathogenesis of the striatal lesions remains inconclusive,and the mechanism behind the T1 hyperintensity remains obscure.