非酮症高血糖性舞蹈症临床及影像学分析

Clinical and imageology analysis of hemichorea‐hemiballismus associated with nonketotic hyperglycemia

目的探讨非酮症性高血糖合并偏侧舞蹈症(hemichorea‐hemiballismus associated with nonketotic hyperglycemia,HC‐NH)的临床、影像学特点及治疗情况。方法对3例HC‐NH患者的临床资料进行回顾性分析。结果 (1)3例患者起病年龄均大于60岁;(2)影像学表现:头颅CT表现为病灶对侧的尾状核与壳核高密度,头颅MRI显示病灶对侧的尾状核与壳核T1呈高信号,T2呈稍低信号或等信号;(3)3例均有随机血糖和血浆渗透压升高、尿糖阳性及尿酮体阴性;(4)降血糖治疗加用氟哌啶醇或氯硝西泮可控制舞蹈症状。结论患者出现非酮症性血糖升高、MRI表现为纹状体T1高信号及偏侧舞蹈症状提示HC‐NH可能,降血糖及给予氟哌啶醇或氯硝西泮治疗可改善患者症状。

Objective To investigate the clinical manifestation, neuroimaging characteristics and therapeutic experience of hemichorea hemiballismus associated with nonketotic hyperglycemia （HC-NH）. Methods Clinical data of three patients suffered from HC-NH in Jiangbin hospital were analyzed retrospectively. Results Onset age of the three HC NH patients （two female and one male） was 71, 74 and 65 respectively. There were some lesions in brains of the three patients on MRI. The pathological changes were found in the bilateral putamen in two patients, and in caudate nucleus in the other patients. The lesions of the caudate nucleus and putamen showed hyperintense signal on T1 and iso-or slightly hypo-intense signal on T2. The three patients had significantly increased random blood glucose levels, positive glycosuria, increased plasma osmotic pressure and negative urine ketone. Insulin had the hypoglycemic effect, and haioperidol or clonazepam could control hernichorea symptoms. Conclusions Corpus striatum of HC-NH patients showed hyperintense signal on T1, such a situation may be in connection with metabolic disturbance and blood brain barrier dysfunction caused by hyperglycemia. It＇ s worth mentioning that the therapeutic efficacy of hypoglycemic drugs, haloperidol and clonazepam is satisfactory.