Department of Neurology, University of Virginia Health System, Charlottesville, Virginia, USA.
Pediatr Neurol. 2010 Feb;42(2):154-6. doi: 10.1016/j.pediatrneurol.2009.08.012.
Central pontine myelinolysis and extrapontine myelinolysis are characterized by symmetric demyelination subsequent to rapid shifts in serum osmolality. Described here is a novel case of transient cortical blindness in association with imaging features of extrapontine myelinolysis, which occurred in a child with carbamoyl phosphate synthetase deficiency after rapid correction of hyperammonemia. Serum sodium levels were within normal limits at presentation and throughout the period of ammonia correction. A potential pathogenic mechanism of osmotic demyelination in the setting of acute treatment for hyperammonemia in a patient with a urea cycle abnormality includes disruption of the blood-brain barrier and re-equilibration of organic osmolytes, particularly glutamine.
脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症的特征是血清渗透压快速变化后出现对称性脱髓鞘。此处描述了一例在尿素循环异常的患者中，由于氨血症迅速纠正后出现的短暂性皮质盲，并伴有脑桥外髓鞘溶解的影像学特征。就诊时和整个氨纠正期间血清钠水平均在正常范围内。在伴有血脑屏障破坏和有机渗透溶质（特别是谷氨酰胺）再平衡的急性高氨血症患者中，渗透压性脱髓鞘的潜在发病机制包括：
