Huq S, Wong M, Chan H, Crimmins D
Department of Neurology, Gosford District Hospital and Royal North Shore Hospitals, Pacific Highway, St Leonards, NSW 2065, Australia.
J Clin Neurosci. 2007 Jul;14(7):684-8. doi: 10.1016/j.jocn.2006.02.015. Epub 2007 Apr 25.
Osmotic demyelination syndromes are often progressive disorders, with clinical features ranging from a mild tremor or dysarthria to a progressive quadraparesis. Although rapid correction of serum sodium is known to be a potent causative factor, additional pathogenic factors exist, which appear critical in predisposing pontine and extrapontine glia to osmotic stress. Interestingly, several cases of osmotic demyelination have emerged where serum sodium was found to be within normal limits and minimal or no correction of a hypo or hypernatraemic state was implemented. We describe two cases--one of extra pontine and another of central-pontine myelinolysis, both of which have occurred in the context of relatively normal serum sodium. The first case illustrates the association of extrapontine myelinolysis with the traditional risk factor of alcoholic cirrhosis and intravenous fluid resuscitation, while the second, more unusual case, describes a patient who developed central pontine myelinolysis possibly in association with alpha interferon therapy.
渗透性脱髓鞘综合征通常是进行性疾病,临床特征从轻度震颤或构音障碍到进行性四肢瘫不等。尽管已知血清钠的快速纠正为一个重要致病因素,但还存在其他致病因素,这些因素似乎在使脑桥和脑桥外神经胶质细胞易受渗透压应激方面起关键作用。有趣的是,出现了几例渗透性脱髓鞘病例,其中发现血清钠在正常范围内,且对低钠血症或高钠血症状态进行了最小程度的纠正或未进行纠正。我们描述了两例病例——一例为脑桥外髓鞘溶解,另一例为中枢性脑桥髓鞘溶解,两例均发生在血清钠相对正常的情况下。第一例病例说明了脑桥外髓鞘溶解与酒精性肝硬化和静脉补液复苏这一传统危险因素之间的关联,而第二例更不寻常的病例描述了一名可能与α干扰素治疗相关而发生中枢性脑桥髓鞘溶解的患者。