类固醇反应性脑病伴发自身免疫性甲状腺炎（SREAT）：文献 251 例特征、治疗和预后。
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.
机构信息
Service de médecine interne, UPMC, DHU i2B AP-HP, Hôpital Saint Antoine, 75012 Paris, France.
Service de neurologie, UPMC, AP-HP, Hôpital Saint Antoine, 75012 Paris, France.
出版信息
Autoimmun Rev. 2016 Dec;15(12):1129-1133. doi: 10.1016/j.autrev.2016.09.008. Epub 2016 Sep 15.
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).
CONCLUSION
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.
背景
类固醇反应性脑病伴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。我们通过文献系统回顾描述 SREAT 的临床表现、结局和治疗方法。
方法
通过 MEDLINE 上的 PubMed、Web of Science 和 Cochrane Library 检索截至 2015 年发表的文章。纳入标准为伴有抗甲状腺抗体的不明原因脑病。
结果
我们报道了 251 例患者（中位年龄 52 岁[范围 18-86]，73%为女性，80 例[32%]有既往甲状腺炎）。患者表现为脑炎征象，包括抽搐（117 例，47%）、意识混乱（115 例，46%）、言语障碍（91 例，37%）、记忆障碍（107 例，43%）、步态障碍（67 例，27%）和迫害妄想（61 例，25%）。28 例（11%）患者表现为进行性记忆障碍，26 例（10%）患者表现为孤立性精神障碍。在血清中，34%的患者抗甲状腺过氧化物酶（TPO）抗体阳性，7%抗甲状腺球蛋白（TG）抗体阳性，69%两者均阳性。促甲状腺激素水平通常正常，为 2 UI/ml[0.001-205]。10/53 例（19%）患者的脑脊液抗 TPO 抗体阳性，2/53 例（4%）抗 TG 抗体阳性，28 例（53%）两者均阳性。82%的患者脑电图异常，表现为弥漫性慢波，符合脑病（70%）或癫痫活动（14%）。193 例患者一线治疗为类固醇，10 例患者为其他免疫抑制剂。中位随访 12 个月[范围 0.2-110]，91%的患者出现完全或部分神经反应，抗 TPO 和 -TG 抗体滴度分别为 347 UI/ml[0-825,000]和 110 UI/ml[0-50,892]。随访期间，40 例（16%）患者至少复发一次。初始昏迷患者的复发更常见（26%比 13%，p=0.08）。
结论
在无明显病因的脑病患者中，应怀疑 SREAT 的诊断，以便尽快开始皮质类固醇治疗。确切的治疗方式仍需确定。
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