酮症性高血糖偏身舞蹈症的临床特征研究(附3例患者临床报道)

Clinical characteristics of hemichorea associated with ketotic hyperglycemia(report of 3 cases)

目的探讨酮症性高血糖偏身舞蹈症(HC-KH)的临床表现、影像学特征、发病机制及治疗。方法选取3例酮体阳性的糖尿病合并舞蹈症的老年患者,分析其临床表现、影像学特点。结果3例患者均为老年2型糖尿病患者。1例为79岁女性,新发糖尿病,左侧肢体受累;1例为66岁女性,既往糖尿病病史20年,右侧肢体受累;另一例为78岁女性,既往糖尿病病史20年,开始一侧肢体发病,后发展至对侧。3例患者中,1例颅脑CT提示舞蹈症状对侧基底节区呈片状稍高密度影,1例MRI T1加权成像(T1WI)表现为双侧基底节区小斑片状高信号,1例MRI未见异常。3例患者在控制血糖后舞蹈症状均明显减轻。结论酮体阳性高血糖性偏身舞蹈症患者较酮体阴性者少见,当患者出现偏身舞蹈样动作,既往血糖控制不佳或入院后发现高血糖,无论头颅CT及MRI有无典型影像学表现,控制血糖后舞蹈样动作消失,均应考虑本病。

【Objective】To investigate the clinical features,imaginging features,pathogenesis and treatment of hemiplegia caused by ketotic hyperglycemia.【Methods】Three elderly female patients with hemiballism-hemichorea induced by ketotic hyperglycemia were selected and the clinical manifestation,neuroimage were explored.【Results】All three patients were elderly patients with type 2 diabetes.One case was a 79-year-old female with primarily diagnosed diabetes and left limb involvement.One case was a 66-year-old female who had a history of diabetes for 20 years and right limb involvement.Another case was a 78-yearold woman with a 20-year history of diabetes and unilateral limb involved at first,then the opposite side is gradually involved.In three patients,one case CT showed the high density shadow of the basal ganglia on the opposite side of the dance symptom;one case MRI T1WI showed high slice signal in the bilateral basal ganglia;another case CT was not examined and no abnormality was found in MRI.Dancing symptoms of three patients were significantly relieved after controlling blood glucose.【Conclusion】Ketotic hyperglycemia is an rare cause of hemichorea-hemiballismus than non-ketotic hyperglycemia.This disease should be considered in patients with involuntary hemichorea who have poor blood glucose control in the past or are found to have diabetes after admission and whose involuntary movements disappear after blood glucose control,no matter whether typical imagine findings are found on head CT and MRI.