Purdy Kaylynn, Anderson Dustin, Camicioli Richard, Khadaroo Rachel G
Department of Medicine (Neurology), University of Alberta, Edmonton, Alberta T6G 2G3, Canada.
Department of Critical Care Medicine, University of Alberta, Edmonton, Alberta T6G 2B7, Canada.
eNeurologicalSci. 2020 Jan 27;18:100223. doi: 10.1016/j.ensci.2020.100223. eCollection 2020 Mar.
This case demonstrates that osmotic demyelination syndrome (ODS) can occur in absence of hyponatremia in patients with fulminant liver failure and markedly high bilirubin levels. Extremely high bilirubin levels, such as >900 μmol/L in the case presented here, may lead to blood brain barrier dysfunction by disrupting blood vessel endothelial cell function as well as increase the release of inflammatory cytokines. As demonstrated in the case here, even small fluctuations in electrolytes may make the brain increasingly more vulnerable to ODS. Clinicians should keep ODS high on their differential even in eunatremic patients with liver failure who have decreased levels of consciousness or coma.
该病例表明，在暴发性肝衰竭且胆红素水平显著升高的患者中，即使不存在低钠血症，也可能发生渗透性脱髓鞘综合征（ODS）。极高的胆红素水平，如此处病例中>900μmol/L，可能通过破坏血管内皮细胞功能导致血脑屏障功能障碍，并增加炎性细胞因子的释放。如此处病例所示，即使电解质出现微小波动，也可能使大脑越来越易患ODS。即使在意识水平下降或昏迷的肝功能衰竭等渗患者中，临床医生也应高度怀疑ODS。
