Guo Yan, Miao Yan-Wei, Ji Xiao-Fei, Li Ming, Liu Xuan, Sun Xiao-Pei
Department of Neurology, The First Affiliated Hospital of Dalian Medical University, Dalian, China.
Eur Neurol. 2014;71(5-6):299-304. doi: 10.1159/000357210. Epub 2014 Mar 21.
Nonketotic hyperglycemia is a rare cause of hemichorea. Patients with hemichorea associated with nonketotic hyperglycemia (HCNH) always have a favorable prognosis when given prompt treatment.
We reviewed the medical records of 12 patients with HCNH in our hospital between January 2005 and January 2013. The clinical data, laboratory findings, and imaging features of the patients were collected.
All 12 patients were admitted to the hospital with a complaint of involuntary movements. Ten patients had a history of diabetes, while the other 2 patients had not been diagnosed. The mean level of blood glucose on admission was 330.7 ± 107.8 mg/dl, and the ketones were negative. A cranial computed tomography scan showed hyperdensity in the striatum, which quickly resolved. Magnetic resonance imaging showed hyperintensity on T1-weighted images without change over several months. Nearly all of the patients experienced relief from the hemichorea symptoms after correcting hyperglycemia with a combination of dopamine receptor inhibitors and the sedative lorazepam, if necessary.
HCNH is a benign disorder, the pathogenesis of which remains unclear. Radiologic changes can provide guidance for early treatment and generally give an estimation of the degree of injury.
非酮症高血糖是偏侧舞蹈症的罕见病因。伴有非酮症高血糖的偏侧舞蹈症（HCNH）患者若得到及时治疗，预后通常良好。
我们回顾了2005年1月至2013年1月期间我院12例HCNH患者的病历。收集了患者的临床资料、实验室检查结果及影像学特征。
12例患者均因不自主运动入院。10例患者有糖尿病史，另外2例未被诊断出糖尿病。入院时血糖平均水平为330.7±107.8mg/dl，酮体为阴性。头颅计算机断层扫描显示纹状体高密度影，该高密度影迅速消退。磁共振成像显示T1加权像上高信号，数月内无变化。几乎所有患者在使用多巴胺受体抑制剂并必要时联合使用镇静剂劳拉西泮纠正高血糖后，偏侧舞蹈症症状得到缓解。
HCNH是一种良性疾病，其发病机制尚不清楚。影像学改变可为早期治疗提供指导，并大致评估损伤程度。
