Kim Dong Wook, Moon Yeonsil, Gee Noh Hong, Choi Jin Woo, Oh Jeeyoung
Department of Neurology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea.
Neurologist. 2011 May;17(3):164-6. doi: 10.1097/NRL.0b013e3182173528.
Nonketotic hyperglycemia (NKH) is a clinical syndrome consisting of severe hyperglycemia, hyperosmolarity, and intracellular dehydration without ketoacidosis. Diverse neurologic symptoms have been described in patients with NKH, including hemichorea, seizure, hemianopsia, and coma, but the pathogenic explanation for these neurologic symptoms remains unclear.
A 65-year-old female with poorly controlled diabetes presented with intermittent jerky movements in the left arm and blurred vision in the left visual field of both eyes. She was diagnosed as NKH on laboratory analysis. Focal cortical hyperintensity and subcortical hypointensity around the right parietal area was noted on T2-weighted and fluid-attenuated inversion recovery (FLAIR) magnetic resonance images. After intravenous gadolinium enhancement, minimal overlying cerebrospinal fluid (CSF) space enhancement around the right parietal area was noted. Delayed FLAIR images, obtained 12 hours later without additional gadolinium, showed prominent CSF space enhancement without parenchymal changes. Delayed gadolinium enhancement of the CSF space on FLAIR imaging was resolved in parallel to clinical improvement during the 2 months' follow-up.
Delayed gadolinium enhancement was first observed in patients with acute infarction who underwent thrombolytic therapy, and it has been considered a marker of blood-brain barrier (BBB) disruption associated with reperfusion injury. Considering that increased BBB permeability is an important mechanism for neurologic complications in diabetes, and BBB leakage occurs during epileptogenesis and contributes to the progression of epilepsy in the chronic phase, we suggest that seizures in NKH patients may be a consequence of, or at minimum have an association with, hyperglycemia-induced BBB disruption.
非酮症高血糖症（NKH）是一种临床综合征，其特征为严重高血糖、高渗状态以及无酮症酸中毒的细胞内脱水。NKH患者已出现多种神经系统症状，包括偏身舞蹈症、癫痫发作、偏盲和昏迷，但这些神经系统症状的发病机制尚不清楚。
一名65岁糖尿病控制不佳的女性患者出现左臂间歇性急促运动以及双眼左侧视野模糊。实验室分析诊断为NKH。在T2加权和液体衰减反转恢复（FLAIR）磁共振图像上，右侧顶叶区域周围可见局灶性皮质高信号和皮质下低信号。静脉注射钆增强后，右侧顶叶区域上方最小限度的脑脊液（CSF）间隙增强被观察到。12小时后未额外注射钆而获得的延迟FLAIR图像显示显著的CSF间隙增强且无实质改变。在2个月的随访期间，FLAIR成像上CSF间隙的延迟钆增强与临床改善平行消退。
延迟钆增强最初在接受溶栓治疗的急性梗死患者中观察到，并且它被认为是与再灌注损伤相关的血脑屏障（BBB）破坏的标志物。考虑到血脑屏障通透性增加是糖尿病神经并发症的重要机制，并且血脑屏障渗漏在癫痫发生过程中出现并在慢性期促进癫痫进展，我们认为NKH患者的癫痫发作可能是高血糖诱导的血脑屏障破坏的结果，或者至少与之相关。
