非酮症高血糖性偏侧舞蹈病-偏侧投掷症的临床和影像学特征分析

Clinical and neuroimaging characteristics of patients with hemichorea-hemiballismus induced by nonketotic hyperglycemia

目的 分析非酮症高血糖性偏侧舞蹈病-偏侧投掷症（HC-HB）患者的临床特征。方法 回顾性分析第二军医大学长海医院收治的5例非酮症高血糖诱发的HC-HB患者的临床表现、实验室检查和影像学资料。患者年龄65-83（76.6±7.2）岁，其中女性4例、男性1例。结果 5例患者均为急性起病，4例表现为单侧肢体粗大的舞蹈样或投掷样不自主运动，1例表现为全身性舞蹈症。病程中最高血糖18.6-44.6（26.6±10.5）mmol/L，尿酮体阴性。脑MRI检查显示4例对侧基底节T1加权像呈高信号、1例双侧基底节T1加权像呈高信号，无水肿或占位效应，T2加权像多为等信号。3例行脑脊液检查，蛋白水平均增高，细胞数正常；其中2例IgG指数或IgG鞘内合成率增高，经积极控制血糖和服用氟哌啶醇等药物治疗后症状基本缓解。结论 非酮症高血糖性HC-HB表现为非酮症高血糖、偏侧舞蹈症-偏侧投掷症，MRI显示对侧基底节T1加权像高信号，偶累及双侧；早期诊治预后良好。

Objective To study the clinical manifestations and neuroimaging characteristics of patients with hemichorea-hemiballismus （HC-HB） induced by nonketotic hyperglycemia. Methods The clinical data, laboratory findings and neuroimaging findings of 5 patients with HC-HB induced by nonketotic hyperglycemia who were treated in Changhai hospital of Second Military Medical University were retrospectively analyzed. The 5 patients included 4 females and 1 male, ranging 65-83 years old and averaging （76.6±7.2） years old. Results All the five patients presented an acute onset, with four of them having chorea or ballismus involuntary movement in unilateral limbs and face and one having generalized chore. The highest levels of blood glucose in patients at onset were 18.6-44.6 mmol/L （averaging[26.6±10.5] mmol/L）, with negative urine ketone. T1-weighted imaging showed hyperintensity in contralateral basal ganglia in 4 cases and in bilateral basal ganglia in 1 case, with no edema or mass effect; most T2-weighted imaging was of isointensity. Increased protein levels and normal number of cells were observed in 3 cases in cerebrospinal fluid examination, and two of three cases had increased IgG index or 24 h intrathecal synthesis rate, which was relieved by effective control of blood glucose combined with pharmacotherapy, such as haloperidol. Conclusion Nonketotic hyperglycemia and HC-HB are the characteristics of HC-HB induced by nonketotic hyperglycemia, with hyperintensity on T1-weighted MRI imaging in the contralateral basal ganglia or in bilateral basal ganglia occasionally. Early diagnosis and proper treatment of those patients can achieve good prognoses.