Department of Medicine, Sultan Qaboos University, Muscat, Oman.
J Neurol Sci. 2010 Mar 15;290(1-2):169-71. doi: 10.1016/j.jns.2009.11.012.
Hyponatremia is a fairly common metabolic disorder. Hyponatremic myelinolysis is a relatively rare, life threatening complication with poorly understood pathophysiology, varied clinical manifestations and uncertain treatment. This case report highlights the range of clinical and imaging phenomena associated with hyponatremic myelinolysis.
Case report.
A middle aged lady presented with an acute delirious state, hypotension and severe hyponatremia on a background of skin hyper-pigmentation and weight loss. Her clinical course evolved to an akinetic-rigid state and later to parkinsonism. Extensive investigations for recognizing a primary neurologic disorder, including brain MRI and CSF analysis were normal, though she had disseminated miliary tuberculosis involving multiple organs. Brain MRI changes characteristic of extrapontine myelinolysis appeared two weeks after the onset of symptoms. The patient recovered completely over several weeks.
This case of hyponatremic extrapontine myelinolysis occurred as the presenting manifestation of adrenal failure secondary to disseminated tuberculosis. Extraponine myelinolysis is difficult to diagnose in the context of delayed brain MRI changes and can have a favorable outcome with modern management.
低钠血症是一种相当常见的代谢紊乱。低钠性髓鞘溶解症是一种相对罕见的、危及生命的并发症,其病理生理学、临床表现和治疗方法均知之甚少。本病例报告重点介绍了与低钠性髓鞘溶解症相关的一系列临床和影像学现象。
病例报告。
一名中年女性以急性意识模糊状态、低血压和严重低钠血症为表现,伴有皮肤色素沉着和体重减轻。她的临床病程进展为无动性缄默状态,随后发展为帕金森病。广泛的检查用于识别原发性神经疾病,包括脑 MRI 和 CSF 分析均正常,尽管她患有累及多个器官的播散性粟粒性肺结核。症状出现两周后,出现了符合脑桥外髓鞘溶解的特征性脑 MRI 改变。患者在数周内完全康复。
本例低钠性脑桥外髓鞘溶解症是继发于播散性结核的肾上腺功能衰竭的首发表现。在脑 MRI 改变延迟的情况下,脑桥外髓鞘溶解症的诊断较为困难,现代治疗方法可获得良好的预后。