Sawka Anna M, Fatourechi Vahab, Boeve Bradley F, Mokri Bahram
Division of Endocrinology, Diabetes, Metabolism, Nutrition, and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55906, USA.
Thyroid. 2002 May;12(5):393-8. doi: 10.1089/105072502760043477.
Corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called Hashimoto's encephalopathy) is a rare, life-threatening, treatable, and possibly autoimmune condition. We identified nine patients (with the diagnosis made after 1979) who had relapsing encephalopathy compatible with previous reports of Hashimoto's encephalopathy and no other identifiable cause of encephalopathy at Mayo Clinic Rochester. Of these nine patients, three were clinically hypothyroid, four were subclinically hypothyroid, and two were euthyroid. Thyroid antibodies were positive in eight of eight patients in whom these measurements were made. Electroencephalographic abnormalities were identified in eight of the nine patients (89%). Magnetic resonance imaging (MRI) abnormalities considered etiologically related to encephalopathy were present in three patients (33%). An increased protein concentration was noted on cerebrospinal fluid examination in seven patients (78%). Of the six patients who received high-dose glucocorticoid therapy, 5 (83%) had improvement of neurologic symptoms. In conclusion, encephalopathy associated with autoimmune thyroiditis is rare but important to recognize because it may be responsive to high-dose glucocorticoid therapy. We believe that this condition is not caused by thyroid dysfunction or antithyroid antibodies but represents an association of an uncommon autoimmune encephalopathy with a common autoimmune thyroid disease. The term Hashimoto's encephalopathy is a misnomer and should not be used.
自身免疫性甲状腺炎相关的皮质类固醇反应性脑病（也称为桥本脑病）是一种罕见的、危及生命的、可治疗的、可能为自身免疫性的疾病。我们在罗切斯特梅奥诊所确定了9例患者（诊断时间在1979年之后），他们患有复发性脑病，与先前关于桥本脑病的报道相符，且没有其他可识别的脑病病因。这9例患者中，3例临床甲状腺功能减退，4例亚临床甲状腺功能减退，2例甲状腺功能正常。在进行了该项检测的8例患者中，8例甲状腺抗体呈阳性。9例患者中有8例（89%）脑电图异常。3例患者（33%）存在磁共振成像（MRI）异常，且这些异常在病因学上与脑病相关。7例患者（78%）脑脊液检查发现蛋白浓度升高。在接受高剂量糖皮质激素治疗的6例患者中，5例（83%）神经症状有所改善。总之，自身免疫性甲状腺炎相关的脑病虽罕见，但因其可能对高剂量糖皮质激素治疗有反应，故识别它很重要。我们认为这种疾病并非由甲状腺功能障碍或抗甲状腺抗体引起，而是一种罕见的自身免疫性脑病与常见的自身免疫性甲状腺疾病的关联。“桥本脑病”这一术语用词不当，不应再使用。
