Go M, Amino A, Shindo K, Tsunoda S, Shiozawa Z
Third Department of Internal Medicine, Yamanashi Medical College.
Rinsho Shinkeigaku. 1994 Nov;34(11):1130-5.
A 69-year-old woman was admitted because of severe dehydration due to anorexia. Consciousness disturbance was found to be due to severe abnormalities of serum electrolyte balance, but recovered quickly by correcting the hyperosmolality. While the initial serum sodium value of 186 mEq/L was corrected to 139 mEq/L in 5 days, locked-in syndrome, bilateral hand tremor and tetraparesis appeared. Brain magnetic resonance imaging (MRI) revealed symmetrically high signal intensity areas on T2-weighted images and low signal intensity areas on T1-weighted images in central part of pons and bilateral middle cerebellar peduncles. One and a half month later, these neurologic symptoms were improved and the MRI abnormalities also disappeared. Auditory brain stem responses which showed prolongations of III to V wave peak to peak latency at the onset returned to normal. It is noted in this case that central pontine myelinolysis (CPM) and extrapontine myelipolysis (EPM) appeared during the period of rapid correction of hypernatremia. Although it is known CPM and EPM are caused by hypernatremia or the rapid correction of hyponatremia, there has been reported only one case of CPM and EPM after rapid correction of hypernatremia. According to the hypothesis of Norenberg, rapid rise in serum sodium may cause CPM and EPM, but if CPM and EPM are caused by the rapid correction of hypernatremia in this case, CPM and EPM may be caused by another pathogenesis of the disorder.
一名69岁女性因厌食导致严重脱水入院。意识障碍被发现是由于血清电解质平衡严重异常所致,但通过纠正高渗状态后迅速恢复。初始血清钠值186 mEq/L在5天内纠正至139 mEq/L,此时出现了闭锁综合征、双侧手部震颤和四肢轻瘫。脑磁共振成像(MRI)显示脑桥中部和双侧小脑中脚在T2加权图像上有对称的高信号强度区域,在T1加权图像上有低信号强度区域。一个半月后,这些神经症状有所改善,MRI异常也消失了。发病时显示III至V波峰间潜伏期延长的听觉脑干反应恢复正常。该病例中值得注意的是,在高钠血症快速纠正期间出现了中央脑桥髓鞘溶解症(CPM)和脑桥外髓鞘溶解症(EPM)。虽然已知CPM和EPM是由高钠血症或低钠血症的快速纠正引起的,但高钠血症快速纠正后出现CPM和EPM的病例仅报道过一例。根据诺伦伯格的假说,血清钠的快速升高可能导致CPM和EPM,但如果该病例中的CPM和EPM是由高钠血症的快速纠正引起的,那么CPM和EPM可能是由该疾病的另一种发病机制所致。