非酮症性高血糖舞蹈症七例临床及神经影像学特点

Clinical and neuroimaging futures of chorea due to nonketotic hyperglycemia： 7 cases report

目的探讨非酮症性高血糖舞蹈症的临床及神经影像学特点。方法对7例非酮症性高血糖舞蹈症患者进行临床及颅脑CT和MRI检查，分析其临床及影像学特征。结果7例患者均有糖尿病病史，平素血糖控制不良，发病时血糖较高而酮体正常，表现为单侧肢体、双侧肢体或全身舞蹈样动作。颅脑CT和MRI可见单侧或双侧基底节区异常病灶。单纯药物控制舞蹈症效果不佳，降低血糖后舞蹈症状和神经影像改变可很快恢复，不留后遗症。结论非酮症性高血糖舞蹈症多见于年龄较大的糖尿病患者，可能与大脑基底核在高血糖状况下脑细胞代谢出现异常有关。颅脑CT或MRI改变具有特征性。本病是可逆性的，对治疗反应较好，一般不留后遗症。

Objective To investigate the clinical and neuroimaging futures of chorea due to nonketotic hyperglycemia. Methods Seven cases of chorea due to nonketotic hyperglycemia were clinically examined and underwent brain CT and MRI as well. Results Investigations revealed uncontrolled diabetes with absent ketones of 7 cases. They all presented with sudden onset hemiachorea or bilateral chorea or generalized chorea. The CT scan of brain could find abnormal lesions in our cases. Hyperintense lesions in the basal ganglia, on T1WI of MRI were demonstrated in our study. Pure drugs was unable to control chorea. The symptoms of chorea and neuroimaging lesions were normal after the hyperglycemia being controlled. Conclusions Chorea caused by nonketotic hyperglycemia is mainly found in aged people with diabetes mellitus in a mechanism of causing striatal neuronal dysfunction, presenting charicristic CT scan or MRI of brain. Chorea should be considered potentially reversible when associated with nonketotic hyperglycemia, for rapid detection and early correction of hyperglycemia could lead to complete recovery of these involuntary movements.