Oztürk F, Görk S, Aydin M, Incesu L, Baysal K
Department of Pediatrics, Ondokuz Mayis University Faculty of Medicine, Samsun.
Turk J Pediatr. 1998 Oct-Dec;40(4):579-84.
Extrapontine myelinolysis in the pediatric age group is very rare. We report a nine-year-old girl with the classical clinical syndrome of pontine and extrapontine myelinolysis following liver trauma due to a traffic accident. She was referred to our hospital for further investigation of convulsions due to severe postoperative hyponatremia. She had no hypoxic event or other identifiable cause for the neurological symptoms. Neurological deterioration began about two days after correction of hyponatremia and followed a period of temporary improvement in hyponatremic encephalopathy. Diagnosis of extrapontine myelinolysis was confirmed with the identification of typical features on magnetic resonance imaging. The rapid correction of hyponatremia seemed the most likely cause since other biochemical tests including liver function tests were all within normal ranges. The long term clinical outcome was good. It is important to carefully monitor the rate of correction in electrolyte disturbances, and to consider the individual variation in response to therapy.
小儿年龄组的脑桥外髓鞘溶解非常罕见。我们报告了一名9岁女孩，她在因交通事故导致肝外伤后出现了典型的脑桥和脑桥外髓鞘溶解临床综合征。她因严重术后低钠血症导致惊厥被转诊至我院作进一步检查。她没有缺氧事件或其他可识别的神经症状病因。神经功能恶化在低钠血症纠正后约两天开始，之前有一段低钠性脑病的暂时改善期。通过磁共振成像发现典型特征确诊为脑桥外髓鞘溶解。低钠血症的快速纠正似乎是最可能的原因，因为包括肝功能检查在内的其他生化检查均在正常范围内。长期临床结果良好。仔细监测电解质紊乱的纠正速度并考虑个体对治疗反应的差异很重要。
