描述桥本脑病（HE）的临床表现，并根据最近的研究讨论其发病机制。HE的发病机制尚不确定。有证据表明，由于血管炎或其他炎症过程所致的自身免疫病因可能是其原因。检测甲状腺抗体 - 抗甲状腺过氧化物酶和抗甲状腺球蛋白对诊断至关重要。在疑似病例中，应通过神经元表面抗体适当的检测排除抗体介导的免疫性脑炎，包括Anti-IgLON5疾病。检测甲状腺自身抗体是非特异性的，因为可以在某些正常人和其他自身免疫疾病中检测到这些抗体。近年来，人们开始关注年轻男性合并甲状腺自身抗体极高水平的侵袭性桥本甲状腺炎。这些患者往往伴有高水平的IgG4，并伴随着更强烈的甲状腺炎症反应。甲状腺自身抗体在中枢神经系统（CNS）组织损伤中的作用尚不清楚，这些抗体只能用作诊断标记。相反，它们在确定甲状腺病理学方面也有作用-甲状腺过氧化物酶抗体比甲状腺球蛋白抗体更与甲状腺纤维化相关。 HE是一种以意识障碍、混乱、幻觉、妄想和有时癫痫为特征的综合征，常伴有抗甲状腺抗体水平升高，通常对糖皮质激素治疗有反应。诊断需要排除脑病和脑炎的其他原因，包括与神经元表面抗体和副肿瘤有关的自身免疫性脑炎。诊断还取决于血清中甲状腺自身抗体的检测。由于抗甲状腺抗体、桥本甲状腺炎和脑病之间没有直接的病理生理联系，所以命名HE可能会误导。类固醇的治疗反应导致将该综合征的名称改为类固醇反应性脑病，伴有自身免疫性甲状腺炎（SREAT），尽管某些病例对类固醇的反应不佳。近年来，人们开始关注伴随血清IgG4水平升高的侵袭性桥本甲状腺炎（IgG4相关疾病）。这种疾病在男性（5：1）中的发生率较高，发病年龄较小，甲状腺炎症反应更强，抗甲状腺抗体滴度更高。这些患者产生过多的IgG4 +浆细胞，这些细胞浸润各种器官，导致其纤维化和硬化，有时导致炎性肿瘤。治疗HE需要联合皮质类固醇和治疗是否存在的甲状腺异常。目前还没有有关类固醇剂量和/或治疗时间的准确指南。 ©2023。作者，授予Springer Science+Business Media，LLC的独家许可，Springer Nature的一部分。

To describe the clinical manifestations of Hashimoto's encephalopathy (HE) and discuss its pathogenesis in light of recent research.The pathogenesis of HE is uncertain. Available evidences point towards an autoimmune etiology due to vasculitis or other inflammatory process. Detection of thyroid antibodies - antithyroid peroxidase and anti-thyroglobulin are essential for diagnosis. Autoimmune encephalitis including Anti-IgLON5 disease needs to be excluded in suspected cases with appropriate tests for neuronal surface antibodies. Detection of thyroid autoantibodies is nonspecific, as these can be detected in some normal individuals and in other autoimmune diseases. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels in younger males with very high levels of thyroid antibodies. The role of the thyroid autoantibodies in the central nervous system (CNS) tissue damage remains unclear and these can act only as markers for diagnosis. Conversely, they have a role to play in determining the thyroid pathology - more glandular fibrosis associated with thyro-peroxidase antibody than with the thyroglobulin antibody. HE is a syndrome characterized by altered mental status, confusion, hallucinations, delusions, and sometimes seizures, in association with high serum anti-thyroid antibody concentration that is usually responsive to glucocorticoid therapy. Diagnosis requires the exclusion of other causes of encephalopathies and encephalitis including autoimmune encephalitis associated with neuronal surface antibodies and paraneoplastic ones. Diagnosis also is dependent on the demonstration of thyroid autoantibodies in serum. Since there is no direct pathophysiologic link between antithyroid antibodies, Hashimoto thyroiditis and the cerebral syndrome, the nomenclature HE could be misleading. The response to steroids led to a renaming of the syndrome to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), though some cases do not respond to steroids. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels (IgG4-related disease). This is characterized by a higher incidence in men (5:1) than in women, onset at a younger age, more intense thyroid inflammation and higher antithyroid antibody titters. Such patients have excessive production of IgG4 + plasmacytes, which infiltrate various organs leading to their fibrosis and sclerosis, sometimes resulting in inflammatory tumors. HE is treated with corticosteroids along with treatment of the dysthyroid condition, if any. There are yet no guidelines regarding steroid dose and/or duration.© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.