高彩红

[摘要] 目的 总结非酮症高血糖性偏身舞蹈症（hemichorea associated with non-ketotic hyperglycemia，HC-NH ）的临床特征、发病机理、影像学特征及治疗预后。 方法 回顾性分析于2015年1月—2018年1月在该院诊断为非酮症高血糖性偏身舞蹈症的3例患者的临床病例资料，并查阅复习国内外文献。 结果 对3例患者给予控制血糖，并应用氟哌啶醇或氯硝西泮控制舞蹈症状，均好转出院。 结论 典型非酮症高血糖性偏身舞蹈症表现为非酮症性高血糖、偏侧舞蹈症和对侧纹状体核磁-T1高信号和或CT高密度影三联征。多见于血糖水平较高的老年患者，女性常见，通常急性起病，突然出现肢体的舞蹈动作，血糖和血渗透压增高，病灶为对侧纹状体，典型影像学头颅CT表现为纹状体稍高密度影，头颅MRI的T1WI可见豆状核、尾状核的片状高信号。调控血糖是关键，对于严重舞蹈症状可应用多巴胺受体拮抗剂或苯二氮卓类药物。该疾病的临床预后良好，加强对该病的全面认识，可使患者得到准确的早期诊断与治疗。

[关键词] 糖尿病;高血糖;偏侧舞蹈症;非酮症;影像学;治疗

[中图分类号] R742.2          [文獻标识码] A          [文章编号] 1674-0742（2018）08（c）-0059-04

Nonketotic Hyperglycemia Lateralis 3 Cases Report and Review of Literature

GAO Cai-hong

Department of Neurology， Mentougou District Hospital， Beijing， 102300 China

[Abstract] Objective To summarize the clinical features， pathogenesis， imaging features and prognosis of Hemotheraia associated with non-ketotic hyperglycemia （HC-NH）. Methods The clinical data of 3 patients diagnosed with non-ketotic hyperglycemic partial body dance in the hospital from January 2015 to January 2018 were retrospectively analyzed and reviewed. Results Controlled blood glucose was administered to 3 patients， and haloperidol or clonazepam was used to control the symptoms of the dance. All patients were discharged from the hospital. Conclusion The typical non-ketotic hyperglycemia partial femoral choreography is characterized by non-ketotic hyperglycemia， lateral scoliosis， and contralateral striatum MRI-T1 hyperintense and CT high-density shadow triad. More common in older patients with high blood glucose levels， common in women， usually acute onset， sudden limb dance movements， increased blood glucose and osmotic pressure， lesions for the contralateral striatum， typical imaging skull CT showed striatum a slightly higher density， the T1WI of the cranial MRI shows a high signal of lamellar nucleus and caudate nucleus. Regulation of blood glucose is the key， and for severe choreic symptoms， dopamine receptor antagonists or benzodiazepines can be used. The clinical prognosis of the disease is good. To strengthen the overall understanding of the disease can enable the patient to get accurate early diagnosis and treatment.

[Key words] Diabetes mellitus; Hyperglycemia; Lateral chorea; Non-ketosis; Imaging; Treatment

偏侧舞蹈症，起病急，主要表现为肢体各关节及面部的不自主运动：快速紊乱、粗大无节律、不可控的肢体与头部不自主运动，如扭头耸肩、手指伸展抓握、摆手扭臂等，下肢步态颠簸、跳跃，面部肌肉可见扮鬼脸、挤眉弄眼、噘嘴吐舌。病灶为对侧锥体外系。最常见的病因是脑血管病[1]，其次为糖尿病，而非酮症高血糖合并偏侧舞蹈……