Steroid-responsive encephalopathy associated with autoimmune thyroiditis
Steroid-responsive encephalopathy associated with autoimmune thyroiditis
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DOI：
10.1001/archneur.63.2.197
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发表时间：
2006-02-01
影响因子：
--
通讯作者：
Boeve, B
中科院分区：
文献类型：
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作者：
Castillo, P;Woodruff, B;Boeve, B
背景资料：与自身免疫性甲状腺炎相关的类固醇反应性脑病（SREAT），通常被称为桥本脑病，是一种认识不足且经常误诊的疾病。目的：描述SREAT患者的临床、实验室和放射学表现，以潜在地提高对这种可治疗疾病的认识。设计：回顾性分析临床特征和诊断试验数据。设置：两个附属三级护理转诊机构患者：从1995年至2003年，连续20例（6例男性）被诊断为SREAT患者。主要结果测量：临床特征和与SREAT相关的辅助检查结果。最常见的临床特征为震颤16例（80%）、短暂性失语16例（80%）、肌阵挛13例（65%）、步态共济失调13例（65%）、癫痫发作12例（60%）和睡眠异常11例（55%）。所有患者在就诊时均被误诊为其他疾病，最常见的是病毒性脑炎（n = 5）、Creutzfeldt-Jakob病（n = 3）或退行性痴呆（n = 4）。最常见的实验室异常是11例肝酶水平升高，11例血清敏感性促甲状腺激素水平升高，5例红细胞沉降率升高。仅5例患者（25%）脑脊液异常提示炎症过程。磁共振成像异常被认为是相关的脑病存在于5例（26%）。结论：临床，实验室和放射学检查结果与SREAT比以前报道的更多样化。误诊是常见的表现。即使血清敏感性促甲状腺激素水平和红细胞沉降率正常，脑脊液特征不提示炎症过程，神经影像学结果正常，也应考虑这种可治疗的综合征。直到这种和其他自身免疫性脑病的病理生理机制是更好的特点，我们认为，描述性的条款，反映了协会，而不是因果关系是最合适的这种综合征。
Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), often termed Hashimoto encephalopathy, is a poorly understood and often misdiagnosed entity.Objective: To characterize the clinical, laboratory, and radiologic findings in patients with SREAT to potentially improve recognition of this treatable entity.Design: Retrospective analysis of clinical features and diagnostic test data.Setting: Two affiliated tertiary care referral institutionsPatients: Twenty consecutive (6 male) patients diagnosed as having SREAT from 1995 to 2003.Main Outcome Measures: Clinical features and ancillary test findings associated with SREAT.Results: The median age at disease onset was 56 years (range, 27-84 years). The most frequent clinical features were tremor in 16 (80%), transient aphasia in 16 (80%), myoclonus in 13 (65%), gait ataxia in 13 (65%), seizures in 12 (60%), and sleep abnormalities in 11 (55%). All patients were assigned an alternative misdiagnosis at presentation, most commonly viral encephalitis (n = 5), Creutzfeldt-Jakob disease (n = 3), or a degenerative dementia (n = 4). The most frequent laboratory abnormalities were increased liver enzyme levels in 11, increased serum sensitive thyroid-stimulating hormone levels in 11, and increased erythrocyte sedimentation rate in 5. In only 5 patients (25%) did cerebrospinal fluid abnormalities suggest an inflammatory process. Magnetic resonance imaging abnormalities believed to be related to the encephalopathy were present in 5 patients (26%).Conclusions: The clinical, laboratory, and radiologic findings associated with SREAT are more varied than previously reported. Misdiagnosis at presentation is common. This treatable syndrome should be considered even if the serum sensitive thyroid-stimulating hormone level and erythrocyte sedimentation rate are normal, the cerebrospinal fluid profile does not suggest an inflammatory process, and neuroimaging results are normal. Until the pathophysiologic mechanism of this and other autoimmune encephalopathies is better characterized, we believe that descriptive terms that reflect an association rather than causation are most appropriate for this syndrome.