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一名儿科患者在高钠血症快速纠正后发生的中央桥脑和脑桥外髓鞘溶解症。

Central pontine and extrapontine myelinolysis in a pediatric patient following rapid correction of hypernatremia.

作者信息

Mastrangelo S, Arlotta A, Cefalo M G, Maurizi P, Cianfoni A, Riccardi R

机构信息

Division of Pediatric Oncology, Catholic University, Rome, Italy.

出版信息

Neuropediatrics. 2009 Jun;40(3):144-7. doi: 10.1055/s-0029-1243173. Epub 2009 Dec 17.

Abstract

Central pontine and extrapontine myelinolysis are uncommon disorders characterized by distinctive clinical features and typical findings on neuroimaging. Only a few cases are reported in the pediatric age group. We describe the case of a leukemic, malnourished 14-year-old boy with a high serum sodium concentration that gradually increased to 170 mmol/L. During a septic shock episode, hydration with a low sodium concentration at the rate of 104 mL/h for 24 h was administered. A rapid correction of the high serum sodium occurred, exceeding 0.5 mmol/L/h. The following day the patient developed rapid and progressive neurological impairment with clinical features characteristic of central pontine and extrapontine myelinolysis. Magnetic resonance imaging confirmed the diagnosis 11 days later. The patient was treated with steroids and intravenous immunoglobulins. He achieved an almost full neurological recovery and radiological improvement. The reported case demonstrates that central pontine and extrapontine myelinolysis can occur after excessively rapid correction of hypernatremia.

摘要

脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症是罕见的疾病,其特征为独特的临床症状和神经影像学典型表现。儿科年龄组仅有少数病例报道。我们描述了一名患有白血病、营养不良的14岁男孩的病例,其血清钠浓度较高,逐渐升至170 mmol/L。在脓毒性休克发作期间,以104 mL/h的速度给予低钠浓度液体进行24小时补液。血清高钠迅速得到纠正,超过了0.5 mmol/L/h。次日,患者出现快速进展的神经功能障碍,具有脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症的临床特征。11天后磁共振成像确诊。患者接受了类固醇和静脉注射免疫球蛋白治疗。他实现了几乎完全的神经功能恢复和影像学改善。该报道病例表明,高钠血症过快纠正后可发生脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症。

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