Gao Ling-Feng, Zhang Zheng-Xiang, Qiu Tao
The First School of Medicine, Zhejiang Chinese Medical University, Hangzhou, China.
Department of Neurology, Zhejiang Provincial Hospital of Chinese Medicine, Hangzhou, China.
J Craniofac Surg. 2025 Sep 17. doi: 10.1097/SCS.0000000000011765.
Hemichorea represents a rare but clinically significant hyperkinetic movement disorder in diabetic patients, characterized by abrupt-onset, nonpatterned contractions involving both craniofacial and appendicular musculature. An 82-year-old Asian male who developed progressively worsening choreoathetoid movements with striking left-sided craniofacial predominance, accompanied by ipsilateral limb involvement. Comprehensive neurological assessment showed preserved sensorimotor function despite the dramatic movement disorder. Biochemical profiling revealed markedly elevated serum glucose (28.6 mmol/L) and HbA1c (12.8%). Neuroimaging findings were pathognomonic, with T1-weighted MRI showing unilateral hyperintensity in the right lentiform nucleus, confirming diabetic striatopathy. The patient's clinical presentation was particularly notable for prominent oromandibular dyskinesias and episodic facial grimacing, highlighting the frequently overlooked craniofacial dimension of this condition. Hemichorea-hemiballismus syndrome as a time-sensitive neuroendocrine emergency requires immediate intervention. Movement disorders with craniofacial manifestations should prompt urgent evaluation for metabolic etiologies, as rapid glycemic correction can lead to complete neurological recovery.
偏侧舞蹈症是糖尿病患者中一种罕见但具有临床意义的运动亢进性疾病，其特征为突发的、无规律的收缩，累及颅面部和肢体肌肉。一名82岁的亚洲男性出现进行性加重的舞蹈手足徐动症，以左侧颅面部为主，伴有同侧肢体受累。全面的神经学评估显示，尽管存在严重的运动障碍，但感觉运动功能仍保持正常。生化分析显示血清葡萄糖（28.6 mmol/L）和糖化血红蛋白（HbA1c，12.8%）显著升高。神经影像学检查结果具有诊断意义，T1加权磁共振成像显示右侧豆状核单侧高信号，确诊为糖尿病性纹状体病。该患者的临床表现尤其以明显的口下颌运动障碍和发作性面部鬼脸为特征，突出了这种疾病中经常被忽视的颅面部表现。偏侧舞蹈症-偏侧投掷症综合征作为一种时间敏感的神经内分泌急症需要立即干预。伴有颅面部表现的运动障碍应促使对代谢病因进行紧急评估，因为快速纠正血糖可导致神经功能完全恢复。
