Metab Brain Dis. 2013 Dec;28(4):711-5. doi: 10.1007/s11011-013-9427-x.
Diabetic uremic syndrome (DUS) is an increasingly reported acute neurometabolic cerebral disease with characteristic clinical and imaging features. Clinical spectrum includes a wide range of movement disorders such as acute parkinsonism. Imaging studies show reversible (with hemodialysis) bilateral lesions in the lenticular nuclei. DUS pathophysiology has not been entirely clarified yet. Our case study shows certainly that LN lesions are characterized with increased lactate peak with MR spectroscopy and decreased perfusion in computerized tomography perfusion along with increased diffusion with apparent diffusion coefficient (ADC) mapping in the subacute phase of the syndrome. Abnormalities were almost normalized quickly after metabolic control by hemodialysis. Together with reports indicating that a deficit of glucose use exacerbated with acute increase of uremic toxins in bilateral LN, observed changes (lactate peak and hypoperfusion) led us to state that a primary metabolic depression may cause this syndrome. Metabolic depression is probably due to uncompensated uremic toxin accumulation related mitochondrial supression and/or dysfunction. This definition fits well to the other elements of DUS such as ADC evolution and marked lesion regression. Our single case study is not supportive of other previously credited mechanisms such as microvascular dysfunction related focal ischemia or hypoperfusion, prolonged uremic toxin related histotoxic hypoxia, central pontine myelinolysis-like demyelination and posterior leukoencephalopathy spectrum disorder related vasogenic edema.
糖尿病性尿毒症综合征（DUS）是一种越来越被报道的急性神经代谢性脑疾病，具有特征性的临床和影像学特征。临床谱包括广泛的运动障碍，如急性帕金森病。影像学研究显示，双侧豆状核可逆（伴血液透析）病变。DUS 的病理生理学尚未完全阐明。我们的病例研究表明，磁共振波谱（MRS）显示 LN 病变的特征是乳酸峰增加，计算机断层灌注（CTP）显示灌注减少，表观扩散系数（ADC）映射显示扩散增加，这些改变在综合征的亚急性期。异常在代谢控制（血液透析）后几乎迅速恢复正常。结合报告表明，双侧 LN 中尿毒症毒素的急性增加导致葡萄糖利用不足加剧，观察到的变化（乳酸峰和灌注不足）使我们认为可能是原发性代谢抑制引起了这种综合征。代谢抑制可能是由于未代偿的尿毒症毒素积累导致线粒体抑制和/或功能障碍。这个定义与 DUS 的其他元素（如 ADC 演变和明显的病变消退）非常吻合。我们的单病例研究不支持其他以前归因于微血管功能障碍相关局灶性缺血或灌注不足、与尿毒症毒素相关的组织毒性缺氧、类似桥脑中央髓鞘溶解的脱髓鞘和与血管源性水肿相关的后部脑白质病变谱障碍等机制。
