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糖尿病纹状体病——非亚洲人群中存在这种病吗?

Diabetic striatopathy-Does it exist in non-Asian subjects?

机构信息

Internal Medicine D, the Chaim Sheba Medical Center, Tel-Hashomer, Israel(1).

Radiology Department, the Chaim Sheba Medical Center, Tel-Hashomer, Israel(1).

出版信息

Eur J Intern Med. 2016 Nov;35:51-54. doi: 10.1016/j.ejim.2016.05.026. Epub 2016 Jun 11.

Abstract

BACKGROUND

Diabetic striatopathy (DS) is a rare complication of diabetes mellitus (DM). The syndrome appears in patients with uncontrolled DM and is characterized by abrupt onset of movement disorder, mainly hemichorea and accompanied by specific findings on brain imaging. It is believed that DS is unique to the Asian population and affects mainly elderly women with uncontrolled DM.

METHODS

In order to define existence and characterization of DS in Western population, we reviewed the medical records of all patients admitted to the Chaim Sheba Medical Center between 2004 and 2014 and identified those with documented elevated HbA1c (>10%). The charts and imaging studies of those with elevated HbA1c and undiagnosed neurological symptoms were reviewed to diagnose DS.

RESULTS

Out of 697 patients with HbA1c>10%, 328 patients had unknown neurological diagnosis. Among them, we identified 4 patients (3 women, mean age 73 and mean HbA1c of 14.8%) with hemichorea or choreoathetosis and brain imaging findings compatible with the diagnosis of DS. Only one out of the 4 patients was diagnosed during hospitalization with DS. All patients were treated with insulin with improvement of their symptoms during hospitalization. However, there was a recurrence in 2 of them and 1 died during the second episode.

CONCLUSION

Diabetic striatopathy exists but underdiagnosed in the Western population. It is important to increase the awareness for this clinical syndrome in order to treat those patients properly.

摘要

背景

糖尿病纹状体病(DS)是糖尿病(DM)的一种罕见并发症。该综合征出现在未得到控制的 DM 患者中,其特征为运动障碍突然发作,主要为偏侧舞蹈症,并伴有脑部影像学的特定发现。人们认为 DS 仅存在于亚洲人群中,主要影响未得到控制的 DM 的老年女性。

方法

为了确定 DS 在西方人群中的存在和特征,我们回顾了 2004 年至 2014 年间在 Chaim Sheba 医疗中心住院的所有患者的病历,并确定了记录的 HbA1c 升高(>10%)的患者。对 HbA1c 升高且无明确神经系统症状的患者的图表和影像学研究进行了回顾,以诊断 DS。

结果

在 HbA1c>10%的 697 名患者中,有 328 名患者的神经系统诊断不明。在他们当中,我们确定了 4 名患者(3 名女性,平均年龄 73 岁,平均 HbA1c 为 14.8%)有单侧舞蹈症或舞蹈手足徐动症,且脑部影像学发现与 DS 的诊断相符。这 4 名患者中仅有 1 名在住院期间被诊断为 DS。所有患者均接受胰岛素治疗,住院期间症状得到改善。然而,其中 2 名患者出现复发,1 名患者在第二次发作时死亡。

结论

DS 存在于西方人群中,但被漏诊。为了正确治疗这些患者,提高对这种临床综合征的认识很重要。

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