以锥体外系为主要临床表现的渗透性脱髓鞘综合征1例  

One case of osmotic demyelination syndrome with extrapyramidal system as the main clinical manifestation

作　　者：郑雪姣 高晓宇 狄政莉[1] 景芸芸[1] 燕玉娥 ZHENG Xuejiao;GAO Xiaoyu;DI Zhengli;JING Yunyun;YAN Yu’e(Xi’an Central Hospital,Xi’an 710003,China)

机构地区：[1]西安市中心医院

出　　处：《中国实用神经疾病杂志》2019年第10期1143-1147,共5页Chinese Journal of Practical Nervous Diseases

摘　　要：目的 总结渗透性脱髓鞘的临床特点、影像学特点及治疗方法。方法 回顾性分析以椎体外系为表现渗透性脱髓鞘患者的临床特点、诊治过程、随访与文献复习。结果 患者主要表现为椎体外系症状,在发病过程中电解质始终正常,未出现低钠血症。头颅MRI示,脑桥中央和基底节区T2加权高信号,T1加权低信号,头颅DWI呈高信号。经甲泼尼龙冲击,口服多巴丝肼、普拉克索后患者症状消失,未遗留明显后遗症。结论 熟悉无电解质紊乱的渗透性脱髓鞘疾病的典型影像学表现,对早期诊断具有较高价值。Objective To summarize the clinical features,imaging features and treatment of osmotic demyelination. Methods The clinical characteristics,diagnosis and treatment,follow up and literature review of patients with osmotic demyelination were retrospectively analyzed. Results In the male patients,mainly manifested as extravertebral symptoms,electrolyte is always normal during the onset of the disease,did not appear hyponatremia.Magnetic resonance imaging showed hyperintensity in the central pons and basal ganglia on T 2 weighted and hypointensity on T 1 weighted.Cranial DWI revealed that the pons had restricted diffusion hyperintense shadows.After methylprednisolone shock,the symptoms disappeared after oral administration of Madopar and Pramipexole,and there was no obvious sequelae. Conclusion It is of great value for early diagnosis to be familiar with the imaging manifestations of osmotic demyelinating disease without electrolyte disorder.

关 键 词：锥体外系 渗透性脱髓鞘 电解质正常 影像学特点 多巴丝肼 普拉克索 

分 类 号：R744.5[医药卫生—神经病学与精神病学]