Wen Song, Fang Hui, Xu Dongxiang, Liu Xinjiang, Zhou Ligang
Department of Endocrinology, Shanghai Pudong Hospital, Fudan University, Shanghai, China.
Department of Radiology, Shanghai Pudong Hospital, Fudan University, Shanghai, China.
Metab Syndr Relat Disord. 2025 Mar;23(2):128-132. doi: 10.1089/met.2024.0192. Epub 2024 Nov 19.
Nonketotic hyperglycemia-induced hemichorea is a rare condition of type 2 diabetes. It is characterized by hyperglycemia with the symptom traced to the basal ganglion like hemichorea or hemiballism, with the hyperintensity within basal ganglion presented in computed tomography (CT) or hyper signal in T1-weighted magnetic resonance image (MRI). It was also confirmed with a relatively better prognosis in that the symptoms of these patients could be relieved after the alleviation of hyperglycemia. However, the exact pathophysiology or mechanism of this condition currently was unclear. Besides, the duration of improvement in tomography as far was varied. In the present study, we reported an elderly female patient who tested with nonketotic hyperglycemia (random blood glucose of fingertips was 19 mmol/L or 342 mg/dL, blood ketone was 0.1 mmol/L) with the symptoms of dysphoria and mild chorea of left low limb, the MRI and CT showed contralateral striatopathy. Her condition achieved alleviation after the normalization of blood glucose. We subsequently rechecked her MRI in arterial spin labeling sequence which showed the hypoperfusion in the right basal ganglion rather than the opposite. Therefore, we suppose the hyperglycemia could induce temporary hypoperfusion in the basal ganglion associated with motor dysfunction which is manifested by hemichorea or hemiballism.
非酮症高血糖性偏侧舞蹈症是2型糖尿病的一种罕见病症。其特征为高血糖,症状可追溯至基底神经节,如偏侧舞蹈症或偏侧投掷症,计算机断层扫描(CT)显示基底神经节内密度增高,或T1加权磁共振成像(MRI)显示高信号。这些患者的症状在高血糖缓解后可得到缓解,这也证实了其预后相对较好。然而,目前这种病症的确切病理生理学或机制尚不清楚。此外,到目前为止,影像学改善的持续时间各不相同。在本研究中,我们报告了一名老年女性患者,其非酮症高血糖检测结果为(指尖随机血糖为19 mmol/L或342 mg/dL,血酮为0.1 mmol/L),伴有烦躁不安和左下肢轻度舞蹈症症状,MRI和CT显示对侧纹状体病变。血糖正常后她的病情得到缓解。随后,我们在动脉自旋标记序列中对她进行了MRI复查,结果显示右侧基底神经节灌注不足,而非相反情况。因此,我们推测高血糖可能会导致基底神经节出现与运动功能障碍相关的暂时性灌注不足,表现为偏侧舞蹈症或偏侧投掷症。