非酮症高血糖性偏侧舞蹈症与脑血流低灌注

Hemichorea associated with non-ketotic hyperglycemia and cerebral blood flow hypoperfusion

目的 分析非酮症高血糖性偏侧舞蹈症的临床及影像学特点,并探讨其脑血流的灌注情况。方法 回顾性选取2018年1月至2023年12月于河南省人民医院住院确诊为非酮症高血糖性偏侧舞蹈症患者23例,分析临床表现、影像特点及治疗预后情况,并分析其与脑血流低灌注的相关性。结果 症状均为偏侧不自主运动,其中4例患者仅表现为单上肢受累(1例为左上肢,3例为右上肢),19例患者上下肢均受累(10例为左侧肢体,9例为右侧肢体)。发病后测得空腹血糖水平为(19.72±4.72)mmol/L,糖化血红蛋白水平为(13.60±3.68)%,尿酮体检查均为阴性。6例CT表现为舞蹈症状对侧的基底节区高密度影;23例T_1加权像表现为条状或片状高信号,患者均有同侧的血管狭窄与磁共振灌注加权成像显示的脑血流局部低灌注,积极控制血糖、改善循环、对症处理后症状好转。结论 非酮症高血糖偏身舞蹈症在临床中较少见,它的发生可能与脑血流低灌注触发基底核变性有关。

Objective To analyze the clinical and imaging features of hemichorea associated with non-ketotic hyperglycemia(HC-NH) and to explore the perfusion of cerebral blood flow in the patients.Methods A retrospective study was conducted on 23 HC-NH patients diagnosed in Henan Provincial People's Hospital from January 2018 to December 2023.The clinical manifestations, imaging features and prognosis were collected and analyzed, and the correlation with cerebral blood flow hypoperfusion was investigated.Results The symptoms were all lateral involuntary movements, of which 4 cases presented only single upper limb(1 case was left upper limb, the other 3 cases were right upper limb),and 19 cases had both upper and lower limbs involved(10 cases were left limb, and 9 cases were right limb).After the onset of the symptoms, the blood glucose level was 19.72±4.72 mmol/L,glycated hemoglobin level was(13.60±3.68)%,but all of patients were negative to urine ketone bodies.Hyperdense lesions in the contralateral basal ganglia region on CT images were observed in 6 cases.Strip or patchy hyperintensity was seen on T_1-weighted MR images.All patients had ipsilateral stenosis of the vessels and regional hypoperfusion of cerebral blood flow as shown by MR perfusion-weighted imaging.All symptoms were relieved after actively controlling blood glucose, improving blood circulation, and symptomatic management.Conclusion HC-NH is quite rare in clinical practice, and its occurrence may be related to cerebral blood flow hypoperfusion triggered by basal nucleus degeneration.