Hashimoto's Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration
Hashimoto's Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration
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DOI：
10.1159/000342217
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发表时间：
2013-01-01
影响因子：
2.4
通讯作者：
Yoneda, Makoto
中科院分区：
文献类型：
--
作者：
Matsunaga, Akiko;Ikawa, Masamichi;Yoneda, Makoto
背景：桥本脑病（HE）具有多种神经系统和神经精神病学特征。在这项研究中，我们调查了小脑共济失调型HE的临床和免疫学特征。研究方法：临床特征，治疗，实验室特征，脑影像学，和血清抗α-烯醇化酶自身抗体（抗NAE抗体），一个有用的诊断标志物，对13例散发性成人发作的小脑性共济失调，并满足HE诊断标准（抗甲状腺抗体和免疫治疗的反应性）进行了研究。结果：所有患者均表现为躯干共济失调，但眼球震颤少见（17%）。8例患者有一个类似脊髓小脑变性（SCD）的隐匿性发作，但脑成像显示所有患者很少或没有小脑萎缩。那些血清抗NAE Ab的患者（n = 8）没有眼球震颤，并且倾向于比抗NAE Ab阴性的患者对免疫治疗有更好的反应。结论：目前的研究表明，隐匿性成人发作和躯干共济失调是常见的小脑共济失调形式的HE，模仿SCD，但眼球震颤和严重的小脑萎缩是罕见的。抗甲状腺抗体和抗NAE抗体可能有助于诊断小脑共济失调性HE。版权所有（C）2012 S. Karger AG，巴塞尔
Background: Hashimoto's encephalopathy (HE) presents with a variety of neurologic and neuropsychiatric features. In this study, we investigated the clinical and immunological profiles of the cerebellar ataxic form of HE. Methods: The clinical features, treatments, laboratory features, brain imaging, and serum anti-NH2-terminal of alpha-enolase autoantibodies (anti-NAE Abs), a useful diagnostic marker for HE, were investigated in 13 patients who presented with sporadic adult-onset cerebellar ataxia and fulfilled the HE diagnostic criteria (antithyroid Abs and responsiveness to immunotherapy). Results: All of the patients presented with truncal ataxia, but nystagmus was uncommon (17%). Eight patients had an insidious onset that mimicked spinocerebellar degeneration (SCD), but brain imaging showed little or no cerebellar atrophy in all of the patients. Those patients with serum anti-NAE Abs (n = 8) did not have nystagmus and tended to respond better to immunotherapy than the anti-NAE Ab-negative patients. Conclusion: The present study suggests that insidious adult-onset and truncal ataxia are common in the cerebellar ataxic form of HE, which mimics SCD, but that nystagmus and severe cerebellar atrophy are uncommon. Antithyroid and anti-NAE Abs may be useful for diagnosing cerebellar ataxic HE. Copyright (C) 2012 S. Karger AG, Basel