非酮症高血糖性舞蹈症临床特点分析

Analysis of clinical characteristics of non-ketotic hyperglycemic chorea

目的探讨非酮症高血糖性舞蹈症患者的临床特点及影像学特点。方法选取8例非酮症高血糖性舞蹈症患者作为研究对象,动态观察其临床症状、实验室及影像学检查结果 ,应用降糖药物、多巴胺受体阻滞剂治疗舞蹈样症状,观察其预后。结果入院时急查血糖8.56～34.30 mmol/L,平均血糖(16.60±7.49)mmol/L。血浆渗透压286.44～342.42 mOsm/L,平均血浆渗透压(302.15±6.35)mOsm/L;尿酮体除1例阳性(+)、第2天复查阴性外,余均为阴性。舞蹈样症状表现:紧张会加重、安静时会减轻、睡眠中消失。舞蹈侧肢体肌张力增加、病理反射阳性者(脑梗死后遗症)2例,其余均未发现其他神经系统阳性体征。8例患者颅脑CT均表现为基底节区稍高密度影, CT值36～48 Hu。双侧受累2例,患侧肢体对侧基底节区受累6例。壳核全部受累8例,尾状核头同时受累4例。其中5例行头磁共振成像(MRI)检查,基底节区均发现异常信号5例,表现为患侧肢体对侧壳核、尾状核或者两侧壳核、尾状核T1高信号,边界清析,无占位效应及水肿,增强并无强化。3例于患病后2个月～1年复查颅脑CT或MRI,显示基底节区高密度影减低或消失,而且并无软化灶形成。所有患者均予活血化淤、营养神经等药物治疗。血糖控制后舞蹈样症状在2～3周以内逐渐消失。结论非酮症高血糖性舞蹈症影像学表现特异,控制血糖可迅速改善病情,预后良好。

Objective To discuss the clinical and imaging characteristics of nonketotic hyperglycemic chorea.Methods A total of 8 patients with non-ketotic hyperglycemic chorea were selected as the study subjects,and their clinical symptoms,laboratory and imaging findings were dynamically observed.Hypoglycemic drugs and dopamine receptor blockers were used to treat chorea-like symptoms,and the prognosis was observed.Results At admission,the blood glucose level was 8.56~34.30 mmol/L,with average blood glucose as(16.60±7.49)mmol/L.Plasma osmotic pressure was 286.44~342.42 mOsm/L,with mean plasma osmotic pressure as(302.15±6.35)mOsm/L.The urine ketone body was negative except 1 case(+)positive on 1st day and negative on the 2nd day.Chorea-like symptoms:stress increases,quietness decreases,and sleep disappears.There were 2 cases with increased muscle tension and positive pathological reflex(sequelae of cerebral infarction)in the dancing limbs.No other positive signs of nervous system were found in the rest.CT findings of 8 patients were slightly high density in basal ganglia,with CT values 36 Hu^48 Hu.There were 2 cases of bilateral involvement,and 6 cases of involvement of the affected side of the basal ganglia.There were 8 cases of putamen involvement and 4 cases of caudate nucleus head involvement.The other 5 cases underwent head magnetic resonance imaging(MRI).Abnormal signals were found in basal ganglia in 5 cases,showing high signal intensity on T1 in contralateral putamen,caudate nucleus or bilateral putamen and caudate nucleus of the affected limbs,with clear boundary,no space-occupying effect and edema,and no enhancement.The CT or MRI findings of 3 patients at 2 months^1 year after the onset of the disease showed that the basal ganglia had decreased or disappeared in high density,and no softening lesions were found.All patients were treated with drugs such as activating blood circulation and removing blood stasis and nourishing nerve.Choreoid symptoms gradually disappeared within 2~3 weeks after blood glucose control.Conclusion Nonketotic hyperglycemic chorea has specific imaging features.Controlling blood glucose can improve the condition rapidly and have a good prognosis.