Shah Bobby, Tobias Joseph D
University of Missouri School of Medicine, Columbia, MO 65212, USA.
J Intensive Care Med. 2006 Nov-Dec;21(6):372-6. doi: 10.1177/0885066606293358.
A 9-year-old girl was admitted for the treatment of hyper-natremic dehydration. Her history was significant for psychogenic polydipsia, hyponatremia, and a renal concentrating defect. She presented with a 2-day history of altered mental status, ataxia, lethargy, fever, nausea, vomiting, and diarrhea. Meningitis was ruled out. Over the course of her illness, slow rehydration was maintained with a gradual decrease (10 mEq per 24 hours) of the serum sodium. Despite this care, she developed quadriparesis, and magnetic resonance imaging performed on day 6 of her illness was consistent with osmotic demyelination (central pontine myelinolysis). To rule out an excessively rapid correction of hypernatremia as the etiology of the problem, a myelin basic protein was measured in the cerebrospinal fluid that had been obtained on hospital day 1. The myelin basic protein was 649.50 ng/mL (normal, 0.07-4.10 ng/mL). The current literature is presented regarding the postulated pathogenesis of central pontine myelinolysis and suggested therapies, previous reports of central pontine myelinolysis in children are reviewed, and the potential role of myelin basic protein in its diagnosis is discussed.
一名9岁女孩因高钠性脱水入院治疗。她有精神性烦渴、低钠血症和肾浓缩功能缺陷病史。她出现精神状态改变、共济失调、嗜睡、发热、恶心、呕吐和腹泻2天。已排除脑膜炎。在她患病期间，通过血清钠逐渐降低（每24小时10 mEq）维持缓慢补液。尽管如此护理，她仍出现四肢瘫痪，患病第6天进行的磁共振成像与渗透性脱髓鞘（中央脑桥髓鞘溶解）相符。为排除高钠血症过快纠正作为问题病因，对住院第1天获取的脑脊液进行髓鞘碱性蛋白检测。髓鞘碱性蛋白为649.50 ng/mL（正常为0.07 - 4.10 ng/mL）。本文介绍了关于中央脑桥髓鞘溶解假定发病机制及建议治疗方法的现有文献，回顾了儿童中央脑桥髓鞘溶解的既往报道，并讨论了髓鞘碱性蛋白在其诊断中的潜在作用。
