非酮症高血糖偏侧舞蹈症3例临床特征分析并文献复习  

Clinical characteristics of hemichorea associated with nonketotic hyperglycemia in 3 patients:case report and literature review

作　　者：胡雅岑 周亚芳[1] 易芳[1] 姚凌雁[1] 许宏伟[1] 周琳[1] 

机构地区：[1]中南大学湘雅医院老年病学神经内科,长沙410008

出　　处：《中南大学学报（医学版）》2017年第11期1341-1347,共7页Journal of Central South University ：Medical Science

基　　金：国家自然科学基金青年科学基金(81401060)~~

摘　　要：目的:探讨非酮症高血糖偏侧舞蹈症(hemichorea associated with non-ketotic hyperglycemia,HC-NH)的临床特点、影像学表现、治疗现状以及发病机制。方法:回顾性分析中南大学湘雅医院收治的3例HC-NH患者的临床资料,并复习相关文献。结果:HC-NH以老年女性多见,表现为急性起病的偏侧舞蹈症状,血糖升高、尿糖阳性及尿酮体阴性;影像学特点包括病变肢体对侧基底核区CT高密度灶,MRI T1加权像(T1 weighted imaging,T1WI)高信号灶;治疗上以控制血糖为主,可加用多巴胺受体拮抗药或镇静药物控制舞蹈症状,预后良好。结论:患者出现非酮症性血糖升高、MRI表现为基底核区T1WI高信号及偏侧舞蹈症状提示HC-NH,控制血糖及给予多巴胺受体拮抗药或镇静药物治疗可改善患者症状。Objective： To investigate the pathophysiology, clinical manifestation and neuroimaging characteristics and therapeutic experiences for hemichore associated with non-ketotic hyperglycemia （HC-NH）. Methods： Clinical data of three patients with HC-NH from Xiangya Hospital, Central South University were analyzed retrospectively, and the related literature was reviewed. Results： The core clinical features of HC-NH were characterized by acute/subacute onset of hemichorea with non-ketotic hyperglycemia in the elderly females. Radiologic findings associated with HC-NH were characterized by hyperattenuation on computed tomographic （CT） scans and hyperintensity on T1-weighted magnetic resonance imaging （MRI） at unilateral basal ganglion region. Blood glucose control was the foundation of treatment. Dopamine receptor antagonists and benzodiazepine sedative were helpful in controlling hemichorea. Conclusion： Hemichorea-hemiballismus is a rare complication of nonketotic hyperglycaemia in elderly type 2 diabetes. It is associated with contralateral striatal radiological abnormality and typically T1 hyperintensity on MRI. The pathophysiology of HC-NH is not clear. The prognosis of HC-NH is favorable. Antidiabetic drugs combined with dopamine receptor antagonists can effectively relieve the hemichorea symptoms.

关 键 词：糖尿病 非酮症高血糖 偏侧舞蹈症 磁共振 

分 类 号：R742.2[医药卫生—神经病学与精神病学]