Yu Ling, Gu Hua, Hu Wenli, Yang Lei
Department of Endocrinology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.
Department of Radiology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.
Front Endocrinol (Lausanne). 2025 Sep 8;16:1546919. doi: 10.3389/fendo.2025.1546919. eCollection 2025.
Diabetic Striate Syndrome (DSS) is a rare complication of diabetes, clinically characterized by chorea-like involuntary movements and contralateral basal ganglia abnormalities on brain CT and MRI. The identification of atypical DSS cases has broadened our understanding of this complex metabolic disorder.
To investigate the clinical manifestations, imaging features, and potential pathogenesis of DSS to enhance clinical awareness and understanding of the condition.
We retrospectively analyzed clinical data from eight patients diagnosed with DSS who were hospitalized in the Department of Neurology at Beijing Chaoyang Hospital, affiliated with Capital Medical University, between January 2017 and July 2024. Their clinical presentations and imaging findings were reviewed, and both typical and atypical features, as well as potential pathogenic mechanisms, were discussed in the context of relevant literature.
Among the eight patients, five were female. All patients had a history of hypertension, and seven had a prior diagnosis of diabetes. Three patients experienced acute or subacute cerebral infarction. Seven patients presented with hemichorea, while one patient exhibited bilateral chorea. Random blood glucose levels at onset ranged from 7.9 to 27.7 mmol/L, and glycated hemoglobin (HbA1c) levels ranged from 8.7% to 15.5%. One patient tested positive for urinary ketones, and one developed symptoms following a rapid drop in blood glucose. Head CT scans revealed high-density lesions in the basal ganglia in four patients. MRI showed T1-weighted hyperintensity in the basal ganglia in seven patients, including one case with bilateral involvement. Hospital stays ranged from 6 to 14 days. All patients showed clinical improvement, with one achieving complete resolution of symptoms. In three cases, symptom improvement was achieved through blood glucose control alone.
DSS represents a syndrome with an expanding clinical and imaging spectrum as research progresses. In this study, we propose a refined definition of the disorder and reaffirm the hypothesis that both basal ganglia ischemia and hyperglycemia synergistically contribute to the development and progression of DSS.
糖尿病性纹状体综合征（DSS）是糖尿病的一种罕见并发症，临床特征为舞蹈样不自主运动，脑部CT和MRI显示对侧基底节异常。非典型DSS病例的发现拓宽了我们对这种复杂代谢紊乱疾病的认识。
探讨DSS的临床表现、影像学特征及潜在发病机制，以提高临床对该疾病的认识和理解。
我们回顾性分析了2017年1月至2024年7月期间在北京朝阳医院（首都医科大学附属）神经内科住院的8例诊断为DSS患者的临床资料。回顾了他们的临床表现和影像学检查结果，并结合相关文献讨论了典型和非典型特征以及潜在的致病机制。
8例患者中，5例为女性。所有患者均有高血压病史，7例既往诊断为糖尿病。3例患者发生急性或亚急性脑梗死。7例患者出现偏侧舞蹈症，1例患者表现为双侧舞蹈症。发病时随机血糖水平为7.9至27.7 mmol/L，糖化血红蛋白（HbA1c）水平为8.7%至15.5%。1例患者尿酮体检测呈阳性，1例患者在血糖快速下降后出现症状。头颅CT扫描显示4例患者基底节区有高密度病变。MRI显示7例患者基底节区T1加权高信号，其中1例双侧受累。住院时间为6至14天。所有患者临床症状均有改善，1例症状完全缓解。3例患者仅通过血糖控制症状即得到改善。
随着研究进展，DSS是一种临床和影像学谱不断扩展的综合征。在本研究中，我们提出了该疾病的细化定义，并再次确认基底节缺血和高血糖协同作用导致DSS发生和发展的假说。
