増大特集 Antibody Update 2018 橋本脳症と自己抗体
増大特集 Antibody Update 2018 橋本脳症と自己抗体
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增加特殊抗体更新 2018 桥本脑病和自身抗体
作者：
斉藤まなぶ;坂本由唯;三上珠希;大里絢子;照井藍;中村和彦;田中稔久;米田誠
脑病偶尔发生与甲状腺疾病，其中大多数是可以治疗的。这些脑病包括与甲状腺功能减退相关的神经精神障碍，称为粘液水肿脑病。此外，桥本氏脑病（HE）已被认为是一种新的临床疾病的基础上的自身免疫机制与桥本氏甲状腺炎，并可以成功地治疗使用类固醇。近年来，我们发现血清中抗α-烯醇化酶（NAE）氨基端的自身抗体是诊断HE的一个高度特异性的生物标志物。我们分析了来自日本和其他国家机构的许多HE病例的血清抗NAE自身抗体和临床特征。大约一半的HE患者有抗NAE抗体。患者年龄分布广泛，有两个高峰（20-30岁左右和60-80岁）。大多数HE患者处于甲状腺功能正常状态，所有患者都有抗甲状腺抗体。常见的神经精神病学特征包括意识障碍、精神病、认知功能障碍、不自主运动、癫痫发作和共济失调。脑电图（EEG）异常和脑单正电子发射计算机断层扫描的脑血流量减少是常见的结果，而脑磁共振成像的异常是罕见的。HE患者表现为各种临床表型，如急性脑病形式和慢性精神病形式。其他临床形式包括边缘系统脑炎、进行性小脑共济失调和克雅氏病（CJD）样形式。小脑共济失调形式的HE临床上类似于脊髓小脑变性（SCD），其特征为无眼球震颤、无或轻度小脑萎缩以及EEG上的惰性背景活动。总之，临床医生应注意与甲状腺疾病相关的脑病的可能性。
Encephalopathy occasionally occurs in association with thyroid disorders, most of which are treatable. These encephalopathies include a neuropsychiatric disorder associated with hypothyroidism named myxedema encephalopathy. Moreover, Hashimoto's encephalopathy (HE) has been recognized as a new clinical disease based on an autoimmune mechanism associated with Hashimoto's thyroiditis, and can be successfully treated using steroids. Recently, we discovered that the serum autoantibodies against the NH 2-terminal of α-enolase (NAE) were a highly specific diagnostic biomarker for HE. We analyzed the serum anti-NAE autoantibodies and the clinical features in many cases of HE from institutions across Japan and other countries. About half the patients with HE had anti-NAE antibodies. Patient age was widely distributed with two peaks (around 20-30 years old and 60-80 years old). Most patients with HE were in euthyroid states and all patients had anti-thyroid antibodies. The common neuropsychiatric features include disturbance of consciousness, psychosis, cognitive dysfunction, involuntary movements, seizures, and ataxia. Electroencephalograph (EEG) abnormalities and decreased cerebral blood flow on brain single positron emission computed tomography are common findings, whereas abnormalities on brain magnetic resonance imaging are rare. Patients with HE present with various clinical phenotypes such as an acute encephalopathy form and chronic psychiatric form. Other clinical forms include limbic encephalitis, progressive cerebellar ataxia, and Creutzfeldt-Jakob disease (CJD)-mimic forms. The cerebellar ataxia form of HE clinically mimics spinocerebellar degeneration (SCD) and is characterized by the absence of nystagmus, absent or mild cerebellar atrophy, and lazy background activity on EEG. Taken together, clinicians should pay attention to the possibility of encephalopathy associated with thyroid disorders.