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中华诊断学电子杂志 ›› 2022, Vol. 10 ›› Issue (01) : 26 -30. doi: 10.3877/cma.j.issn.2095-655X.2022.01.006

神经系统疾病诊治

非酮症性高血糖偏侧舞蹈症的临床特征并文献复习
余慧1, 杨位霞1,†()   
  1. 1. 201700　上海，复旦大学附属中山医院青浦分院神经内科
  • 收稿日期:2021-10-11 出版日期:2022-02-26
  • 通信作者: 杨位霞

Clinical features of hemichorea associated with non-ketotic hyperglycemia and literature review

Hui Yu1, Weixia Yang1,†()   

  1. 1. Department of Neurology, Qingpu Branch of Zhongshan Hospital Affiliated to Fudan University, Shanghai 201700, China
  • Received:2021-10-11 Published:2022-02-26
  • Corresponding author: Weixia Yang
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余慧, 杨位霞. 非酮症性高血糖偏侧舞蹈症的临床特征并文献复习[J]. 中华诊断学电子杂志, 2022, 10(01): 26-30.

Hui Yu, Weixia Yang. Clinical features of hemichorea associated with non-ketotic hyperglycemia and literature review[J]. Chinese Journal of Diagnostics(Electronic Edition), 2022, 10(01): 26-30.

目的

探讨非酮症性高血糖偏侧舞蹈症的诊断学特征。

方法

回顾性分析2019年12月8日复旦大学附属中山医院青浦分院神经内科收治的1例非酮症性高血糖偏侧舞蹈症患者的临床资料，并复习文献。

结果

患者女性，51岁，因"口干多饮4月余，右上肢不自主运动1周"入院。临床表现为偏侧舞蹈样运动，血糖增高，血酮阳性。颅脑CT提示左侧豆状核密度影较对侧异常增高；头颅MRI T1WI提示左侧基底节区高信号，T2WI及液体抑制反转恢复(FLAIR)序列、MRI弥散加权像(DWI)均为稍低信号改变。患者诊断为非酮症性高血糖偏侧舞蹈症，予以积极控制血糖，加用盐酸硫必利及地西泮口服，1个月后舞蹈样症状消失，复查颅脑CT未见明显异常，随访至今无复发。

结论

患者出现舞蹈样症状、高血糖、血酮阳性，颅脑CT或MRI提示基底节区异常密度影或者信号灶时应考虑非酮症性高血糖偏侧舞蹈症。

关键词: 舞蹈症, 非酮症, 高血糖症, 基底神经节
Objective

To investigate the diagnostic features of hemichorea associated with non-ketotic hyperglycemia (HC-NH).

Methods

The clinical data of a patient with HC-NH admitted to the Neurology Department of Qingpu Branch of Zhongshan Hospital Affiliated to Fudan University on December 8, 2019 was retrospectively analyzed, and relevant literatures were reviewed.

Results

A 51-year-old female patient was admitted to the hospital for dry mouth and polydipsia for more than 4 months, and involuntary movement of the right limb for 1 week. The clinical manifestations were hemichorea-like movements, increased blood sugar, and positive blood ketones. Brain CT showed the abnormally higher density shadow of the left lenticular nucleus. Brain MRI T1WI showed abnormally high signal in the left basal ganglia region, T2WI, fluid attenuated inversion recovery sequence (FLAIR) and diffusion weighted imaging (DWI) showed slightly low signal changes. The patient was diagnosed with HC-NH, and the blood glucose was actively controlled. After treatment with tiapride hydrochloride and diazepam, the patient′s chorea-like symptoms disappeared 1 month later. Brain CT reexamination showed normal and the condition was stable without recurrence so far.

Conclusion

Chorea-like symptoms, high blood sugar, positive blood ketones, abnormal signal in the basal ganglia region in brain CT or MRI, are important diagnostic features of HC-NH.

Key words: Chorea, Non-ketotic, Hyperglycemia, Basal ganglia
图1 非酮症性高血糖偏侧舞蹈症患者入院时颅脑CT检查图像注：横断位示左侧豆状核密度影较对侧异常增高(箭头所示)
图2 非酮症性高血糖偏侧舞蹈症患者入院时头颅MRI检查图像注：a图为T1WI，可见左侧豆状核高信号改变；b图为T2WI，c图为液体抑制反转恢复序列成像，d图为弥散加权像，均可见左侧豆状核低信号改变(箭头所示)
图3 非酮症性高血糖偏侧舞蹈症患者治疗1个月后颅脑CT复查图像注：横断位未见明显异常
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