Dixit Abhishek
Department of Neurology, Institute of Medical Science, Banaras Hindu University, Varanasi, India.
J Med Case Rep. 2024 Mar 12;18(1):154. doi: 10.1186/s13256-023-04332-y.
Diabetic striatopathy, also known as hyperglycemic hemichorea-hemiballismus, is a rare movement disorder associated with nonketotic hyperglycemia in patients with poorly controlled diabetes mellitus. The pathophysiology is not fully elucidated but may involve hyperviscosity, ischemia, and alterations in basal ganglia neurotransmitters.
We present a case of a 64-year-old Asian female patient with longstanding poorly controlled type 2 diabetes mellitus who developed abrupt-onset right-sided hemichorea-hemiballismus. Laboratory results showed hyperglycemia without ketoacidosis. Neuroimaging revealed left putaminal hyperdensity on computed tomography and T1 hyperintensity on magnetic resonance imaging. With insulin therapy and tetrabenazine, her movements improved but persisted at 1-month follow-up.
This case illustrates the typical features of diabetic striatopathy, including acute choreiform movements contralateral to neuroimaging abnormalities in the setting of nonketotic hyperglycemia. While neuroleptics may provide symptomatic relief, prompt glycemic control is critical given the risk of recurrence despite imaging normalization.
Diabetic striatopathy should be recognized as a rare disorder that can occur with poorly controlled diabetes. Further study of its pathophysiological mechanisms is needed to better guide management. Maintaining tight glycemic control is essential to prevent recurrence of this debilitating movement disorder.
糖尿病性纹状体病,也称为高血糖性偏侧舞蹈症-偏侧投掷症,是一种与糖尿病控制不佳患者的非酮症高血糖相关的罕见运动障碍。其病理生理学尚未完全阐明,但可能涉及血液高黏滞度、缺血以及基底神经节神经递质的改变。
我们报告一例64岁亚洲女性患者,患有长期控制不佳的2型糖尿病,突发右侧偏侧舞蹈症-偏侧投掷症。实验室检查结果显示血糖升高但无酮症酸中毒。神经影像学检查显示,计算机断层扫描提示左侧壳核高密度影,磁共振成像提示T1高信号。经过胰岛素治疗和丁苯那嗪治疗,她的运动症状有所改善,但在1个月的随访中仍持续存在。
该病例说明了糖尿病性纹状体病的典型特征,包括在非酮症高血糖情况下与神经影像学异常对侧的急性舞蹈样动作。虽然抗精神病药物可能提供症状缓解,但鉴于尽管影像学恢复正常仍有复发风险,迅速控制血糖至关重要。
糖尿病性纹状体病应被视为一种在糖尿病控制不佳时可能发生的罕见疾病。需要进一步研究其病理生理机制,以更好地指导治疗。维持严格的血糖控制对于预防这种使人衰弱的运动障碍复发至关重要。