Chen Yaning, Wu Chunliu, Ren Ming, Wang Qingjun, Wang Zhiwei, Zhang Yimo, Yu Yingxin
Department of Endocrinology, Sixth Medical Center of Chinese People's Liberation Army (PLA) General Hospital, Beijing, China.
Department of Neurology, First Medical Center of Chinese People's Liberation Army (PLA) General Hospital, Beijing, China.
Front Endocrinol (Lausanne). 2024 Dec 10;15:1429026. doi: 10.3389/fendo.2024.1429026. eCollection 2024.
Diabetic striatopathy (DS) is a rare disorder characterized by clinical manifestations of hemichorea, non-ketotic hyperglycemia, and high signal on T1-weighted MRI or high density on CT scan in basal ganglia, typically associated with poor glycemic control.
This study aimed to analyze clinical characteristics of patients with diabetic striatopathy to raise awareness amongst physicians, especially endocrinologists, about this rare neurological manifestation in patients with diabetes.
We retrospectively analyzed the data on clinical presentations, laboratory workups, and cranial CT and MRI of six patients with DS who were admitted to our hospital from October 2013 to June 2022.
The mean age of onset among the six patients was 80.5 years, and the mean value of HbA1c was 13.65%. All six patients complained of involuntary movements, which primarily affected the arm and leg on one side of the body. Bilateral caudate nucleus hyperdensities were shown on the CT examination in Case 3,while the other 5 patients, unilateral caudate nucleus hyperdensity was shown. In addition, five patients (except Case 5) underwent MRI, all showing hypersignal lesions on the T1-weighted images. A low signal in the right basal ganglia was shown on MRI susceptibility weighted imaging (SWI) sequences in Case 6. All six patients exhibited carotid artery or cerebral artery stenosis. Following strict blood glucose control and symptomatic management, the symptoms of chorea improved significantly in all patients, and repeat images indicated that the lesions gradually disappeared.
Both poor vascular conditions and severe hyperglycemia contribute to the development of diabetic striatopathy. The prognosis is usually good by active treatment.
糖尿病性纹状体病(DS)是一种罕见的疾病,其特征为偏侧舞蹈症、非酮症高血糖的临床表现,以及在T1加权磁共振成像(MRI)上表现为高信号或在计算机断层扫描(CT)上显示基底节高密度,通常与血糖控制不佳有关。
本研究旨在分析糖尿病性纹状体病患者的临床特征,以提高医生,尤其是内分泌科医生,对糖尿病患者这种罕见神经表现的认识。
我们回顾性分析了2013年10月至2022年6月期间我院收治的6例糖尿病性纹状体病患者的临床表现、实验室检查以及头颅CT和MRI数据。
6例患者的平均发病年龄为80.5岁,糖化血红蛋白(HbA1c)的平均值为13.65%。所有6例患者均主诉有不自主运动,主要影响身体一侧的手臂和腿部。病例3的CT检查显示双侧尾状核高密度,而其他5例患者显示单侧尾状核高密度。此外,5例患者(病例5除外)接受了MRI检查,所有患者在T1加权图像上均显示高信号病变。病例6的MRI磁敏感加权成像(SWI)序列显示右侧基底节低信号。所有6例患者均表现出颈动脉或脑动脉狭窄。经过严格的血糖控制和对症治疗后,所有患者的舞蹈症症状均有显著改善,复查影像显示病变逐渐消失。
血管状况不佳和严重高血糖均促成糖尿病性纹状体病的发生。积极治疗后预后通常良好。