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[桥本脑病:一项解剖临床观察]

[Hashimoto's encephalopathy: an anatomicoclinical observation].

作者信息

Perrot X, Giraud P, Biacabe A G, Perret-Liaudet A, Borson-Chazot F, Gray F, Kopp N, Boulliat J

机构信息

Laboratoire de Diagnostic des Maladies à Prions, France.

出版信息

Rev Neurol (Paris). 2002 Apr;158(4):461-6.

Abstract

Hashimoto's encephalopathy (HE) is a rare neurological complication of chronic lymphocytic thyroiditis. As its clinical presentation is aspecific, other etiologies of acute encephalopathy have to be ruled out. We report the case of a 29-year old woman with neuropsychiatric signs preceding coma, myoclonus and epileptic seizures. Clinical and electroencephalographic features were consistent with the diagnosis of new variant of Creutzfeldt-Jakob disease. However, high titres of antithyroid antibodies in serum directed towards the diagnosis of HE. Despite oral steroids, the patient died five months later. Neuropathological findings ruled out spongiform encephalopathy and disclosed aspecific activated microglia. Our observation suggests that this process could be involved in the pathogenesis of HE. Even in the absence of clinical dysthyroidism, HE diagnosis has to be suspected in the settings of acute encephalopathy associated with seric antithyroid antibodies.

摘要

桥本脑病(HE)是慢性淋巴细胞性甲状腺炎罕见的神经并发症。由于其临床表现缺乏特异性,必须排除急性脑病的其他病因。我们报告了一例29岁女性病例,该患者在昏迷、肌阵挛和癫痫发作之前出现神经精神症状。临床和脑电图特征与克雅氏病新变种的诊断相符。然而,血清中高滴度的抗甲状腺抗体指向HE的诊断。尽管使用了口服类固醇,患者五个月后仍死亡。神经病理学检查结果排除了海绵状脑病,并显示有非特异性活化的小胶质细胞。我们的观察表明,这一过程可能参与了HE的发病机制。即使没有临床甲状腺功能障碍,在伴有血清抗甲状腺抗体的急性脑病情况下,也必须怀疑HE的诊断。

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