Hashimoto's encephalopathy - Epidemiology, pathogenesis and management
Hashimoto's encephalopathy - Epidemiology, pathogenesis and management
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DOI：
10.2165/00023210-200721100-00002
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发表时间：
2007-01-01
期刊：
影响因子：
6
通讯作者：
Veakoulis, Dennis
中科院分区：
文献类型：
--
作者：
Mocellin, Ramon;Walterfang, Mark;Veakoulis, Dennis
桥本脑病是一个术语，用于描述推测的自身免疫性脑病，其特征是抗甲状腺过氧化物酶抗体滴度高。与自身免疫性甲状腺疾病类似，桥本脑病在女性中比男性更常见。据报道，全世界的儿童、成人和老年人群均出现这种情况。临床表现可能涉及复发和缓解病程，包括癫痫发作、中风样发作、认知能力下降、神经精神症状和肌阵挛。甲状腺功能在临床和生化方面通常是正常的。桥本脑病似乎是一种罕见的疾病，但是，由于它对皮质类固醇治疗有反应，因此在“检查阴性脑病”的情况下必须考虑它。首先通过神经影像学和脑脊液检查排除脑病的其他毒性、代谢和感染原因。神经影像学检查结果通常无助于明确诊断。排除这些情况后，常见的鉴别诊断包括克雅氏病、快速进展性痴呆以及副肿瘤性和非副肿瘤性边缘脑炎。在典型的临床表现中，高滴度的抗甲状腺抗体，特别是抗甲状腺过氧化物酶抗体，具有诊断意义。然而，在健康普通人群中，这些抗体的滴度升高。皮质类固醇治疗几乎总是成功的，但如果突然停止治疗可能会出现复发。其他形式的免疫调节，例如静脉注射免疫球蛋白和血浆置换，也可能有效。尽管与自身免疫性甲状腺疾病有关，但桥本脑病的病因尚不清楚。抗甲状腺抗体很可能不具有致病性，但滴度可以作为治疗反应的标志。病理结果可以提示炎症过程，但通常不存在严重血管炎的特征。临床表现、甲状腺疾病、自身抗体模式和脑病理学之间的联系有待通过研究进一步阐明。桥本脑病可能被归入一组非血管炎性自身免疫性炎症性脑膜脑病。该组可能包括与电压门控钾通道抗体相关的疾病，例如边缘脑炎。一些作者建议放弃与桥本的任何联系，并将该病症重新命名为“与自身免疫性甲状腺炎相关的类固醇反应性脑病”，以更好地反映当前（如果有限的话）对该病症的理解。
Hashimoto's encephalopathy is a term used to describe an encephalopathy of presumed autoimmune origin characterised by high titres of antithyroid peroxidase antibodies. In a similar fashion to autoimmune thyroid disease, Hashimoto's encephalopathy is more common in women than in men. It has been reported in paediatric, adult and elderly populations throughout the world. The clinical presentation may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms and myoclonus. Thyroid function is usually clinically and biochemically normal.Hashimoto's encephalopathy appears to be a rare disorder, but, as it is responsive to treatment with corticosteroids, it must be considered in cases of 'investigation negative encephalopathies'. Diagnosis is made in the first instance by excluding other toxic, metabolic and infectious causes of encephalopathy with neuroimaging and CSF examination. Neuroimaging findings are often not helpful in clarifying the diagnosis. Common differential diagnoses when these conditions are excluded are Creutzfeldt-Jakob disease, rapidly progressive dementias, and paraneoplastic and nonparaneoplastic limbic encephalitis. In the context of the typical clinical picture, high titres of antithyroid antibodies, in particular antithyroid peroxidase antibodies, are diagnostic. These antibodies, however, can be detected in elevated titres in the healthy general population. Treatment with corticosteroids is almost always successful, although relapse may occur if this treatment is ceased abruptly. Other forms of immunomodulation, such as intravenous immune-globulin and plasma exchange, may also be effective.Despite the link to autoimmune thyroid disease, the aetiology of Hashimoto's encephalopathy is unknown. It is likely that antithyroid antibodies are not pathogenic, but titres can be a marker of treatment response. Pathological findings can suggest an inflammatory process, but features of a severe vasculitis are often absent. The links between the clinical pictures, thyroid disease, auto-antibody pattern and brain pathology await further clarification through research. It may be that Hashimoto's encephalopathy will be subsumed into a group of nonvasculitic autoimmune inflammatory meningoencephalopathies. This group may include disorders such as limbic encephalitis associated with voltage-gated potassium channel antibodies. Some authors have suggested abandoning any link to Hashimoto and renaming the condition 'steroid responsive encephalopathy associated with autoimmune thyroiditis' to better reflect current, if limited, understanding of this condition.