非酮症高血糖性偏侧舞蹈症临床分析

Hemichorea-hemiballismus in non-ketotic hyperglycaemia

目的总结非酮症高血糖性偏侧舞蹈症的临床特征。方法回顾性分析5例非酮症高血糖性偏侧舞蹈症患者的临床资料,复习相关文献,总结其临床症状、发病机制、影像学演变及治疗转归。结果非酮症高血糖性偏侧舞蹈症多见于老年女性,急性起病,发病时血糖明显升高,尿酮体阴性或弱阳性;头颅CT平扫显示病灶对侧尾状核头部、壳核和/或苍白球呈高密度,短时间内可消失;MRI于T1像呈高信号,T2像呈稍低信号或等信号,边界清晰,周围无水肿;综合治疗预后良好。结论非酮症高血糖、偏侧舞蹈症及病灶对侧脑组织影像学改变为非酮症高血糖性偏侧舞蹈症的典型三联征。

Objective To analyze the clinical characteristics of hemichorea-hemiballismus in non-ketotic hyperglycaemia.Methods The clinical data of 5patients with hemichorea-hemiballismus in non-ketotic hyperglycaemia were analyzed retrospectively.The clinical symptoms,pathogenesis,imaging findings,treatment and outcome were summarized by reviewing relevant literatures. Results Hemichorea-hemiballismus in non-ketotic hyperglycaemia occurred more commonly in the elderly females,with acute onset followed by significantly increased plasma glucose.The urine acetone bodies were negative or weak positive.CT scan showed high signal intensity shadows in the head of contralateral caudatum,putamen,and/or globus pallidus,which disappeared in a short time.High signal intensity and low signal intensity were found on T1 and T2 by MRI scan,with shape border,and no edema around.Comprehensive therapy achieved a satisfied result.Conclusion The image changes of non-ketotic hyperglycaemia,hemichorea and contralateral brain tissue are typical triad of hemichorea-hemiballismus in non-ketotic hyperglycaemia.