Laureno R, Karp B I
Washington Hospital Center, Washington, D.C., USA.
Ann Intern Med. 1997 Jan 1;126(1):57-62. doi: 10.7326/0003-4819-126-1-199701010-00008.
Myelinolysis is a neurologic disorder that can occur after rapid correction of hyponatremia. Initially named "central pontine myelinolysis," this disease is now known to also affect extrapontine brain areas. Manifestations of myelinolysis usually evolve several days after correction of hyponatremia. Typical features are disorders of upper motor neurons, spastic quadriparesis and pseudobulbar palsy, and mental disorders ranging from mild confusion to coma. Death may occur. The motor and localizing signs of myelinolysis differ from the generalized encephalopathy that is caused by untreated hyponatremia. Experiments have duplicated the clinical and pathologic features of myelinolysis by rapidly reversing hyponatremia in animals. Myelinolysis is more likely to occur after the treatment of chronic rather than acute hyponatremia and is more likely to occur with a rapid rate of correction. The exact pathogenesis of myelinolysis has not been determined. Optimal management of hyponatremic patients involves weighing the risk for illness and death from untreated hyponatremia against the risk for myelinolysis due to correction of hyponatremia. Experiments in animals and clinical experience suggest that correction of chronic hyponatremia should be kept at a rate less than 10 mmol/L in any 24-hour period.
髓鞘溶解症是一种可在低钠血症快速纠正后发生的神经系统疾病。这种疾病最初被命名为“中枢性脑桥髓鞘溶解症”，现在已知它也会影响脑桥外的脑区。髓鞘溶解症的表现通常在低钠血症纠正几天后出现。典型特征是上运动神经元紊乱、痉挛性四肢瘫和假性延髓麻痹，以及从轻度意识模糊到昏迷的精神障碍。可能会导致死亡。髓鞘溶解症的运动和定位体征与未治疗的低钠血症所引起的全身性脑病不同。通过在动物中快速纠正低钠血症，实验已重现了髓鞘溶解症的临床和病理特征。髓鞘溶解症更有可能发生在慢性低钠血症而非急性低钠血症的治疗之后，并且更有可能在快速纠正的情况下发生。髓鞘溶解症的确切发病机制尚未确定。低钠血症患者的最佳治疗方法是权衡未治疗的低钠血症导致疾病和死亡的风险与因纠正低钠血症而发生髓鞘溶解症的风险。动物实验和临床经验表明，慢性低钠血症的纠正速度应在任何24小时内保持低于10 mmol/L。
