双重滤过血浆置换治疗对糖皮质激素无反应性桥本脑病的初步尝试  

Preliminary application of double filtration plasmapheresis in treating glucocorticoid unresponsive Hashimoto’s encephalopathy

作　　者：毛晓薇 秧杰[2] 吴帅 MAO Xiao-wei;YANG Jie;WU Shuai(Department of Neurology, Changhai Hospital, Navy Military Medical University, Shanghai 200433, China;Department of Neurology, East Hospital, Tongji University School of Medicine, Shanghai 200120, China;Department of Neurology, Zhongshan Hospital, Fudan University, Shanghai 200032, China)

机构地区：[1]海军军医大学附属长海医院神经内科,上海200433 [2]同济大学附属东方医院神经内科,上海200120 [3]复旦大学附属中山医院神经内科,上海200032

出　　处：《中国临床医学》2019年第1期72-75,共4页Chinese Journal of Clinical Medicine

基　　金：国家自然科学基金面上项目(81671597)~~

摘　　要：目的:总结桥本脑病(Hashimoto’s encephalopathy, HE)的临床特征,并探讨糖皮质激素治疗无反应性桥本脑病的临床诊治经验。方法:收集海军军医大学附属长海医院2012年12月至2018年1月确诊的HE患者6例,观察其临床特征,每例患者确诊后均给予糖皮质激素治疗,观察临床疗效。首次尝试采用双重滤过血浆置换(double filtration plasmapheresis,DFPP)治疗激素无反应性HE,观察临床疗效。结果:HE临床表现以认知功能障碍最为常见(6/6),所有患者均存在血清抗甲状腺抗体增高。6例患者接受正规糖皮质激素治疗,4例有效,2例无效。1例糖皮质激素治疗无效患者为59岁男性,表现为记忆力减退、癫痫发作,经激素冲击治疗无效,病情继续进展,头颅MRI增强示双侧丘脑、大脑脚至小脑上脚异常信号;经3次DFPP治疗,患者临床症状迅速改善,影像学病灶消失,取得显著临床疗效,随访1年未再复发。结论:HE患者大部分对糖皮质激素治疗有效,一般预后良好;部分对糖皮质激素治疗不敏感或存在禁忌患者,DFPP可作为一种替代治疗手段。Objective:To investigate the clinical characteristics of Hashimoto’s encephalopathy (HE) and explore the treatment of glucocorticoid unresponsive HE. Methods:Six cases with HE were collected in Changhai Hospital, Navy Military Medical University from December 2012 to January 2018. The clinical characteristics were observed, and each patients was given glucocorticoid therapy after diagnosis. This was the first attempt to treat HE with double filtration plasmapheresis (DFPP) in patient presenting unresponsive to methylprednisolone therapy. Results:Cognitive dysfunction was the most common clinical manifestation (6/6). All cases had increased serum antithyroid antibodies. All patients were treated with regular methylprednisolone therapy. Among these patients, 2 cases were ineffective. A 59-year-old male patient manifested cognitive dysfunction, seizure attacks, who was unresponsive to methylprednisolone therapy, and the disease continues to progress. Brain MRI scan showed significant diffused abnormal signals on bilateral thalamus, cerebral peduncles, and superior cerebellar peduncles. After 3 sessions of DFPP, his clinical symptoms improved rapidly and significantly, and MRI results showed that his brain lesions disappeared finally. In one-year follow-up visiting, it did not show recurrence and his neurological function kept good. Conclusions:Although most HE cases have been highly responsive to glucocorticoid treatment, a few patients are resistant to the therapy. For those who are resistant or contraindicative to steroids treatment, DFPP may be an alternative choice.

关 键 词：桥本脑病 双重滤过血浆置换 抗甲状腺抗体 

分 类 号：R737.11[医药卫生—肿瘤]