桥本脑病的临床分析

Clinical study of Hashimoto encephalopathy

目的研究桥本脑病的临床特点,以提高对本病的诊治水平。方法对5例临床诊断为桥本脑病的患者进行回顾研究,并对其发病机制、临床表现、治疗及预后进行分析。结果5例患者中有3例既往有甲状腺疾病病史,其中1例有自身免疫性甲状腺疾病家族史。临床表现有认知功能障碍者4例,精神行为异常者3例,锥体外系改变者4例,抽搐者2例,意识障碍者1例,运动障碍者1例。甲状腺功能正常者4例,亚临床甲状腺功能亢进者1例。血清ATPO及ATG阳性率达100%。1例脑脊液蛋白轻度增加。2例脑电图呈弥漫性慢波。5例头部MR I均无特异性改变。4例接受糖皮质激素治疗,其中1例合用人血丙种球蛋白,基本治愈1例,有效3例,无死亡。结论目前可能存在对桥本脑病诊断率较低的情况。内分泌功能异常不能作为桥本脑病的病因,可认为其为一种自身免疫性疾病。当遇有难以解释的脑病时,应将检测血清抗甲状腺抗体作为常规检查,当其滴度显著增高时应高度怀疑本病,及时予以糖皮质激素治疗。

Objective To study the clinical characteristics of Hashimoto encephalopathy （HE） and to improve the skill of diagnosis and treatment. Methods A study was conducted in 5 cases with the clinical diagnosis of HE, and the pathophysiologic mechanism, clinical features, treatment and prognosis were summarized. Results 3 of 5 cases had a history of thyroid disease, 1 case with family history of autoimmune thyroid disease. The clinical features were cognitive impairment in 4 cases,psychiatric symptoms in 3 cases, extrapyramidal changes in 4 cases, seizures in 2 cases, altered consciousness in 1 case, movement disorders in 1 case. Euthyriod was in 4 cases and subclinical hyperthyroidism was in 1 case. All cases had high serum titer of antithyroid antibody. Elevation of cerebrospinal fluid protein level was in 1 case. 2 cases of EEC were generalized slow. All cases had nonspecific imaging abnormalities. 4 cases were treated with intravenous glu- cocorticoids, 1 case with combination of intravenous immune globulin. Complete recovery was in 1 case and improvement was in 3 cases. Conclusion HE is very likely underdiagnosed at present. With improve of recognition, the prevalence will increase. Endocrine disturbance can not be the etiology of HE, autoimmunc disease is suggested. The pathogenesis of HE is still unclear. The presence of unexplained encephalopathy, screening for serum antithyroid antibody should be performed as initial screening test. A high index of suspicion is required with the obvious elevation in serum levels of antithyroid antibody. To treat with glucocorticoids in time, in order to achieve good prognosis.