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中华脑血管病杂志(电子版) ›› 2023, Vol. 17 ›› Issue (03) : 254 -259. doi: 10.11817/j.issn.1673-9248.2023.03.010

临床病例研究

非典型非酮症高血糖性偏侧舞蹈症三例
罗宁, 陈蓉()   
  1. 570100 海口,海南医学院第一附属医院神经内科
  • 收稿日期:2023-02-25 出版日期:2023-06-01
  • 通信作者: 陈蓉
  • 基金资助:
    海南省自然科学基金高层次人才项目(2019RC379); 海南省临床医学中心建设项目

Three cases of atypical non-ketotic hyperglycemic hemichorea

Ning Luo, Rong Chen()   

  1. Department of Neurology, the First Affiliated Hospital of Hainan Medical College, Haikou 570100, China
  • Received:2023-02-25 Published:2023-06-01
  • Corresponding author: Rong Chen
引用本文:

罗宁, 陈蓉. 非典型非酮症高血糖性偏侧舞蹈症三例[J/OL]. 中华脑血管病杂志(电子版), 2023, 17(03): 254-259.

Ning Luo, Rong Chen. Three cases of atypical non-ketotic hyperglycemic hemichorea[J/OL]. Chinese Journal of Cerebrovascular Diseases(Electronic Edition), 2023, 17(03): 254-259.

非酮症高血糖性偏侧舞蹈症是一种罕见的糖尿病并发症,尽管具有偏侧肢体的舞蹈样动作、非酮症高血糖、影像学表现为病变肢体对侧基底核区高信号等特点,但仍有部分患者以非典型的临床表现起病,且相关报道较少。本文通过对海南医学院第一附属医院收治的3例特殊的非酮症高血糖性偏侧舞蹈症患者的临床及影像学特点进行总结分析,以提高临床医师对罕见病的认识,减少不必要的漏诊、误诊。

Non-ketotic hyperglycemic hemichorea is a rare clinical complication. It is characterized by dance-like movements in the paralyzed limb, non-ketotic hyperglycemia, and imaging manifestations of high signals in the contralateral basal ganglia of the diseased limb. However some patients still start with atypical clinical manifestations, and related reports are few. Hereon, the clinical and imaging characteristics of 3 atypical patients with non-ketotic hyperglycemic hemichorea admitted to the First Affiliated Hospital of Hainan Medical College were summarized and analyzed, so as to improve clinicians' understanding of rare diseases and reduce unnecessary missed diagnosis and misdiagnosis.

图1 患者1 头颅影像学检查结果。图a:螺旋CT颅脑平扫可见双侧放射冠、基底节区腔隙性脑梗死,未见异常高密度灶;图b:磁共振脑功能成像(弥散加权成像)未见弥散受限;图c:磁共振颅脑平扫T1 加权像可见右侧基底节区异常高信号影、无明显水肿及占位;图d:磁共振颅脑平扫T2 加权像可见右侧基底节区呈等信号;图e:磁共振脑血管成像可见双侧大脑前动脉、大脑中动脉、大脑后动脉无明显狭窄、闭塞,脑动脉硬化
图2 患者2头颅影像学检查结果。图a:螺旋CT颅脑平扫示双侧放射冠区腔隙性脑梗死,未见异常高密度灶;图b:磁共振脑功能成像(弥散加权成像)未见弥散受限;图c:磁共振颅脑平扫T1加权像可见右侧基底节区异常高信号影、边界较清、无水肿及占位;图d:磁共振颅脑平扫T2加权像可见右侧基底核区呈等信号;图e:磁共振脑血管成像可见双侧颈内动脉、大脑中动脉、大脑后动脉走行僵直,管壁欠光滑,管腔粗细不均匀且部分呈串珠样狭窄
图3 患者3头颅影像学检查结果。图a:螺旋CT颅脑平扫可见左侧尾状核、豆状核区片状高密度灶,CT值为53~58 HU;图b:磁共振脑功能成像(弥散加权成像)未见弥散受限;图c:磁共振颅脑平扫T1加权像可见左侧基底核区片状高信号灶;图d:磁共振颅脑平扫T2加权像可见左侧基底核区片状低信号;图e:磁共振脑血管成像可见双侧大脑前动脉、大脑中动脉、大脑后动脉走行僵直,管壁欠光滑,管腔粗细不均匀,脑动脉硬化
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