渗透性脱髓鞘综合征9例临床分析

Clinical analysis of nine cases of osmotic demyelination syndrome

目的分析渗透性脱髓鞘综合征(osmotic demyelination syndromes,ODS)的临床表现,影像学特点及预后。方法对2010-01—2017-12收住郑州大学一附院ODS患者临床资料进行回顾性分析。结果9例患者中8例有低钠血症补钠史,另1例肝移植患者术后持续高钠血症。临床主要表现为肢体无力(8/9)、精神异常(7/9)、延髓性麻痹(5/9)。病变累及脑桥6例,脑桥外病变8例,脑桥外病变主要累及双侧基底节区、丘脑、皮层灰白质交界区。MRI多表现为等或稍长T1、长T2信号。FLAIR像、DWI像高信号,ADC像根据时期不同可呈低或等信号,增强多无强化。9例患者均使用类固醇激素治疗,基本痊愈2例,好转5例,无明显缓解1例,死亡1例。结论ODS包括脑桥中央髓鞘溶解和脑桥外髓鞘溶解,患者多有血钠浓度快速波动史,经类固醇激素治疗,多数患者预后良好。

Objective To analyze the clinical presentation,magnetic resonance imaging appearances and prognosis of osmotic demyelination syndromes(ODS). Methods The clinical data of 9 patients with ODS in the first affiliated hospital of Zhengzhou University from January 2010 to December 2017 were analyzed retrospectively. Results Among the 9 patients,8 patients had the initial correction of chronic hyponatremia,another liver transplantation patient had persistent hypernatremia.The main clinical manifestations are limb weakness (8/9),psychiatric disorder (7/9) and bulbar paralysis (5/9).The white matter demyelination located in central pontine in 6 patients and extrapontine in 8 patients.Extrapontine lesions mainly affected the basal ganglia,thalami and the cortex grey white matter junction.The signal intensity was isointense or hypointense on T 1 weigted MRI,hyperintense on T 2 weigted and T 2 FLAIR images.Restricted diffusion was evident on DWI image,while ADC values could be decreased or baseline depending on the phases of the disease.No enhancement were seen on MR enhancement imaging.After treatment with corticosteroid,2 patients cured,5 patients improved,1 patient had no relief,and 1 patient died. Conclusion Osmotic demyelination syndromes include central pontine myelinolysis and extrapontine myelinolysis.Most patients had the history of rapid alteration of serum natrium.Most patients had good prognosis with the prompt treatment of corticosteroid.