背景:类固醇反应性脑病和相关自身免疫性甲状腺炎(SREAT)的特征是脑病和抗甲状腺抗体的存在。我们描述了临床表现,通过对文献的系统回顾,对SREAT的结局和治疗方法进行了研究。
方法:通过PubMed的MEDLINE,搜索了WebofScience和Cochrane图书馆直到2015年发表的文章。纳入标准是原因不明的脑病与抗甲状腺抗体。
结果:我们发现了251例患者的报告(中位年龄52岁[范围18-86],73%的女性,80例[32%]伴有先前存在的甲状腺炎)。患者出现脑炎征象伴抽搐(n=117;47%),混淆(n=115,46%),言语障碍(n=91,37%),记忆障碍(n=107,43%),步态障碍(n=67,27%)和迫害妄想(n=61,25%)。28例患者(11%)出现进行性记忆障碍和26例(10%)孤立的精神疾病。在血清中,34%的患者抗甲状腺过氧化物酶(TPO)抗体阳性,7%的抗甲状腺球蛋白(TG)抗体,都是69%。促甲状腺激素水平通常正常,2UI/ml[0.001-205]。来自10/53患者(19%)的脑脊液抗TPO抗体阳性,2/53(4%)抗TG抗体和28(53%)两者。82%的患者脑电图检查结果异常,表现为弥漫性减缓与脑病(70%)或癫痫活动(14%)一致。一线治疗为类固醇193例,其他免疫抑制药物10例。中位随访12个月[范围0.2-110],91%的患者表现出完全或部分的神经反应,抗TPO和-TG抗体滴度为347UI/ml[0-825,000]和110UI/ml[0-50,892],分别。随访期间,40名患者(16%)经历了至少一次复发。初次昏迷患者的复发频率更高(26%vs13%,p=0.08)。
结论:在无明显病因的脑病的情况下,应怀疑SREAT的诊断,快速开始皮质类固醇治疗。必须确定治疗的确切方式。
BACKGROUND: Steroid-responsive encephalopathy and associated autoimmune thyroiditis (SREAT) is characterized by encephalopathy and the presence of antithyroid antibodies. We describe the clinical presentation, outcome and treatments for SREAT by a systematic review of the literature.
METHODS: MEDLINE via PubMed, Web of Science and the Cochrane Library were searched for articles published until 2015. Inclusion criteria were unexplained encephalopathy with antithyroid antibodies.
RESULTS: We found reports of 251 patients (median age 52years [range 18-86], 73% females, 80 [32%] with preexisting thyroiditis). Patients presented encephalitis signs with convulsions (n=117; 47%), confusion (n=115, 46%), speech disorder (n=91, 37%), memory impairment (n=107, 43%), gait disturbance (n=67, 27%) and persecutory delusions (n=61, 25%). Twenty-eight patients (11%) presented progressive memory impairment and 26 (10%) isolated psychiatric disorders. In serum, 34% of patients were positive for anti-thyroid peroxidase (TPO) antibodies, 7% for anti-thyroglobulin (TG) antibodies, and 69% both. Thyroid-stimulating hormone levels were usually normal, at 2 UI/ml [0.001-205]. Cerebrospinal fluid from 10/53 patients (19%) was positive for anti-TPO antibodies, 2/53 (4%) anti-TG antibodies and 28 (53%) both. Electroencephalography findings were abnormal for 82% of patients, showing diffuse slowing consistent with encephalopathy (70%) or epileptic activity (14%). The first-line treatment was steroids in 193 patients and other immunosuppressive drugs in 10 cases. At a median follow-up of 12months [range 0.2-110], 91% of patients showed complete or partial neurological response, with anti-TPO and -TG antibody titers at 347 UI/ml [0-825,000] and 110 UI/ml [0-50,892], respectively. During follow-up, 40 patients (16%) experienced at least one relapse. Relapse was more frequent in patients with initial coma (26% vs 13%, p=0.08).
CONCLUSIONS: The diagnosis of SREAT should be suspected in case of encephalopathy without obvious cause, to quickly start corticosteroid treatment. The exact modalities of treatment must be defined.