渗透性脱髓鞘综合征的临床分析

摘 要： 目的了解渗透性脱髓鞘综合征（ODS）的发病机制、诊断、治疗和预防方法。方法报告11例ODS患者，并结合文献进行分析。结果10例患者有明显低钠血症；发病诱因包括药源性3例，营养不良3例，肝移植术后、脑挫裂伤、垂体微腺瘤、糖尿病肾病和妊娠剧吐各1例。存在严重呕吐或进食量极少的患者7例。神经系统表现包括不同程度意识障碍，假性球麻痹，四肢瘫痪，眼球活动障碍，闭锁综合征，精神症状，震颤或手足徐动等不自主运动，肌张力齿轮样增高等帕金森样症状等。头颅MRI显示桥脑或双侧豆状核、尾状核头、丘脑等桥外部位脱髓鞘。单纯CPM 3例，单纯EPM 2例，CPM合并EPM 6例。治疗后10例好转，1例病情获稳定。结论ODS的发病与脑内渗透压平衡失调有关，各种原因引起的低钠血症及其快速纠正容易诱发，临床表现可为单纯CPM、EPM或二者合并存在。随着头颅MRI的应用，可使该病早期诊断，其预后明显改善。避免快速纠正低钠血症是预防的主要措施。

A clinical analysis on osmotic demyelination syndrome

Objective To explore the pathogenesis, diagnosis, treatment and prevention of osmot- ic demyelination syndrome （ODS）. Methods 11 cases of ODS were reported and the literature was re- viewed. Results Hyponatremia was prominent in 10 patients. The ODS occurred as a result of the side effects of medicine （3 cases）, malnutrition （3 cases）, post--liver transplantation, cerebral contusion, pituitary microadenoma, diabetic nephropathy and hyeremesis gravidarum （1 case respectively）. Serious vomiting and lower food intake was seen in 7 cases. Clinical presentations included unconsciousness, pseudobulbar palsy, quadriparesis, locked-- in syndrome, psychiatric and behavioural changes, parkin- sonism, dystonia, and other movement disorders. MRI has a greater sensitivity for both CPM and EPM. Three subsets of ODS were seen, which included CPM alone （3 cases）, EPM alone （2 cases）, CPM and EPM together （6 cases）. All patients survived from the disease and 10 cases had a clinical improvement. Conclusions The pathogenesis of ODS is correlated with osmotic shifts in brain. Hyponatremia induced by various causes and its rapid correction may lead to myelinolysis in pontine and extrapontine sites. Therefore, the appropriate correction of hyponatremia and other ions derangement is important for prevention of ODS.