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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 analyze the clinical features of patients with non-ketotic hyperglycemic hemochromatosis-hemoblasts (HC-HB). Methods The clinical manifestations, laboratory tests and imaging data of 5 non-ketotic hyperglycemic HC-HB patients admitted to Changhai Hospital of Second Military Medical University were retrospectively analyzed. Patients aged 65 to 83 (76.6 ± 7.2) years, of which 4 were women and 1 male. Results All the 5 patients were acute onset, 4 cases showed unilateral large body dance-like or throwing involuntary movements, and 1 case showed generalized chorea. The duration of the highest blood glucose 18.6 ~ 44.6 (26.6 ± 10.5) mmol / L, urine ketone body negative. The brain MRI showed that the T1 weighted images of the contralateral basal ganglia were hyperintense and the hyperintensity of the T1 basal ganglia T1 weighted images was high with no edema or mass effect. The T2 weighted images were mostly equal signals. 3 cases of cerebrospinal fluid examination, protein levels were increased, the number of normal cells; two cases of IgG index or IgG intrathecal synthesis rate increased by active control of blood glucose and haloperidol and other drugs after treatment, the symptoms were basically relieved. Conclusions Non-ketotic hyperglycemic HC-HB is manifested as nonketotic hyperglycemia, hemophilia-hemiplegia. MRI shows contralateral basal ganglion T1-weighted high signal, occasionally involved and bilateral. The prognosis of early diagnosis and treatment is good.