非酮症性高血糖偏身舞蹈症的临床研究

Clinical study of hemichorea associated with non-ketotic hyperglycemia

目的总结非酮症性高血糖偏身舞蹈症(hemichorea associated with non-ketotic hyperglycemia,HC-NH的临床、影像学特征以及发病机制。方法选取2015年1月至2020年9月北京市海淀医院和开封市中心医院诊断为HC-NH的14例患者,结合临床症状、实验室检查结果和影像学资料,分析该病的发病机制、影像学表现及预后。结果患者平均年龄(77.0±9.6)岁,从出现症状到就诊平均8.83 d。患者均有糖尿病病史,就诊时即刻血糖平均值为(22.89±10.37)mmol/L,入院后糖化血红蛋白平均值为(12.7±2.0)%;尿常规未见酮体;头颅CT平扫表现为纹状体高密度或MRI-T1WI高信号病变,T2WI信号可变化,病灶边界清晰周围无水肿。经积极控制血糖、改善循环及多巴胺抑制剂治疗后,患者症状迅速好转。结论HC-NH主要为老年发病,急性或亚急性起病,及时诊断,积极治疗,预后较好。但需警惕不典型病例并与其他引起舞蹈症的疾病鉴别。

Objective To summarize the clinical,imaging features and pathogenesis of hemichorea associated with non-ketotic hyperglycemia(HC-NH).Methods Retrospective analysis was performed on 14 patients diagnosed with HC-NH in Beijing Haidian Hospital and Kaifeng Central Hospital of Henan Province from January 2015 to September 2020.Combined with the clinical symptoms,laboratory and imaging data of the patients,the pathogenesis,imaging manifestations and prognosis of the disease were studied.Results All the patients had a history of diabetes,and the mean value of blood glucose at the time of admission was(22.89±10.37)mmol/L,and the mean value of Hb A1 c after admission was(12.7±2.0)%.Urine ketone was negative.High striatal density or high signal lesions were found in CT scan or MRI-T1 WI.T2 WI signals could be changed,and lesion boundaries were clear and no edema around.After controlling blood sugar,improving circulation and dopamine inhibitor treatment,the symptoms improved rapidly.Conclusions HC-NH mainly occurs in the elderly,with acute or subacute onset.Timely diagnosis and active treatment have a good prognosis.However,the differential diagnosis of atypical cases and other diseases should be vigilant.