中央桥脑及脑桥外髓鞘溶解症：一例悲剧性结局的报告。
Central pontine and extrapontine myelinolysis: report of a case with a tragic outcome.
作者信息
Brito Adriana Rocha, Vasconcelos Marcio Moacyr, Cruz Júnior Luiz Celso Hygino da, Oliveira Maria Emília D C Q de, Azevedo Adriana Rodrigues Miguel de, Rocha Luciana Graça Vieira, Mendonça Priscilla Costa
机构信息
Hospital Universitário Antônio Pedro, Universidade Federal Fluminense, Niterói, RJ, Brazil.
出版信息
J Pediatr (Rio J). 2006 Mar-Apr;82(2):157-60. doi: 10.2223/jped.1464.
OBJECTIVE
To report a pediatric case of central pontine and extrapontine myelinolysis, a rare neurological disease often associated with rapid correction of hyponatremia.
DESCRIPTION
A 15 year-old female adolescent developed locked-in syndrome during severe hyponatremia. Brain magnetic resonance imaging was consistent with the diagnosis of central pontine and extrapontine myelinolysis.
COMMENTS
Serum sodium correction should proceed slowly and cautiously, based upon a careful calculation of sodium deficit, in order to minimize metabolic stress and avoid the occurrence of this dreadful complication, which has a tragic outcome in most cases. There is no scientifically proved effective treatment for myelinolysis, and severe cases usually have a dismal prognosis.
目的
报告一例小儿中央脑桥和脑桥外髓鞘溶解症，这是一种罕见的神经系统疾病，常与低钠血症的快速纠正相关。
描述
一名15岁女性青少年在严重低钠血症期间出现闭锁综合征。脑磁共振成像与中央脑桥和脑桥外髓鞘溶解症的诊断相符。
评论
血清钠的纠正应基于对钠缺乏的仔细计算，缓慢而谨慎地进行，以尽量减少代谢应激并避免发生这种可怕的并发症，该并发症在大多数情况下会导致悲惨的结局。目前尚无经科学验证的有效治疗髓鞘溶解症的方法，严重病例通常预后不佳。
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