Schiess Nicoline, Pardo Carlos A
Department of Neurology, Division of Neuroimmunology and Neuroinfectious Disorders, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
Ann N Y Acad Sci. 2008 Oct;1142:254-65. doi: 10.1196/annals.1444.018.
Hashimoto's encephalopathy (HE) is a controversial neurological disorder that comprises a heterogenous group of neurological symptoms that manifest in patients with high titers of antithyroid antibodies. Clinical manifestations of HE may include encephalopathic features such as seizures, behavioral and psychiatric manifestations, movement disorders, and coma. Although it has been linked to cases of Hashimoto's thyroiditis or thyroid dysfunction, the most common immunological feature of HE is the presence of high titers of antithyroglobulin or anti-TPO (antimicrosomal) antibodies. At present, it is unclear whether antithyroid antibodies represent an immune epiphenomenon in a subset of patients with encephalopathic processes or they are really associated with pathogenic mechanisms of the disorder. The significance of classifying encephalopathies under the term HE will be determined in the future once the relevance of the role of antithyroid antibodies is demonstrated or dismissed by more detailed experimental and immunopathological studies. The responsiveness of HE to steroids or other therapies such as plasmapheresis supports the hypothesis that this is a disorder that involves immune pathogenic mechanisms. Further controlled studies of the use of steroids, plasmapheresis, or immunosuppressant medications are needed in the future to prove the concept of the pathogenic role of antithyroid antibodies in HE.
桥本脑病(HE)是一种存在争议的神经障碍性疾病,由一组异质性神经症状组成,这些症状出现在抗甲状腺抗体滴度较高的患者中。HE的临床表现可能包括癫痫发作、行为和精神症状、运动障碍以及昏迷等脑病特征。尽管它与桥本甲状腺炎或甲状腺功能障碍病例有关联,但HE最常见的免疫学特征是存在高滴度的抗甲状腺球蛋白或抗甲状腺过氧化物酶(抗微粒体)抗体。目前尚不清楚抗甲状腺抗体是脑病过程亚组患者中的一种免疫附带现象,还是真的与该疾病的致病机制相关。一旦通过更详细的实验和免疫病理学研究证明或排除抗甲状腺抗体作用的相关性,未来将确定将脑病归类为HE这一术语的意义。HE对类固醇或其他疗法(如血浆置换)的反应性支持了这一假说,即这是一种涉及免疫致病机制的疾病。未来需要进一步对照研究类固醇、血浆置换或免疫抑制药物的使用,以证实抗甲状腺抗体在HE中致病作用的概念。