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.
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。.