Park Kunwoo, Kim Sang Beom, Yoon Sung Sang, Woo Ho Geol
Department of Neurology, Kyung Hee University Hospital at Gangdong, Seoul, Korea.
Department of Neurology, Kyung Hee University Hospital, Kyung Hee University College of Medicine, Seoul, Korea.
Encephalitis. 2023 Oct;3(4):119-124. doi: 10.47936/encephalitis.2023.00108. Epub 2023 Oct 6.
Osmotic demyelination syndrome (ODS) is an acute demyelinating disorder characterized by the loss of myelin in the center of the basis pons, defined as central pontine myelinolysis (CPM), and demyelination in locations outside the pons, defined as extrapontine myelinolysis (EPM). ODS including CPM and EPM is mainly caused by rapid correction of hyponatremia. However, there are several reports of ODS in medical conditions such as malnutrition; alcoholism; liver transplantation; malignancy; sepsis; and electrolyte imbalance including hypernatremia, hypokalemia, hypophosphatemia, and chronic illness. ODS caused by rapid correction of hyperammonemia or continuous hyperbilirubinemia without sodium fluctuations has rarely been reported. Because ODS may be irreversible, prevention is crucial. Herein, we report a case of ODS secondary to rapid correction of hyperammonemia and continuous hyperbilirubinemia.
渗透性脱髓鞘综合征（ODS）是一种急性脱髓鞘疾病，其特征是脑桥基底部中央的髓鞘丢失，称为中央脑桥髓鞘溶解症（CPM），以及脑桥外部位的脱髓鞘，称为脑桥外髓鞘溶解症（EPM）。包括CPM和EPM的ODS主要由低钠血症的快速纠正引起。然而，在营养不良、酗酒、肝移植、恶性肿瘤、败血症以及包括高钠血症、低钾血症、低磷血症和慢性病在内的电解质失衡等医疗状况下，也有几例ODS的报道。由高氨血症的快速纠正或持续高胆红素血症且无钠波动引起的ODS很少被报道。由于ODS可能是不可逆的，预防至关重要。在此，我们报告一例继发于高氨血症快速纠正和持续高胆红素血症的ODS病例。
