Matsuo Kinya, Koga Michiaki, Honda Masaya, Kanda Takashi
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.
Present address: Department of Neurology, Yamaguchi Prefectural Grand Medical Center.
Rinsho Shinkeigaku. 2018 Jun 27;58(6):390-394. doi: 10.5692/clinicalneurol.cn-001169. Epub 2018 Jun 1.
Hashimoto's encephalopathy has been described as an autoimmune disorder which demonstrates favorable response to corticosteroid therapy. However, steroid-resistant cases which require additional treatment are frequently reported, and there is no consensus how such cases should be treated. We present a 69 years-old man, who progressed cognitive dysfunction in the past three months. Anti-thyroid and anti-NH terminal of alpha-enolase antibodies were positive. Because initial corticosteroid therapy was ineffective, cyclophosphamide (CPA) pulse therapy was added, and his cognitive function was immediately improved. He had no relapse after tapering dose of corticosteroid for three years. CPA pulse therapy should be considered for steroid-resistant Hashimoto's encephalopathy.
桥本脑病被描述为一种自身免疫性疾病，对皮质类固醇治疗有良好反应。然而，经常有报道称存在需要额外治疗的类固醇抵抗病例，并且对于此类病例应如何治疗尚无共识。我们报告一名69岁男性，在过去三个月中出现进行性认知功能障碍。抗甲状腺抗体和抗α-烯醇化酶N端抗体呈阳性。由于初始皮质类固醇治疗无效，加用了环磷酰胺（CPA）冲击治疗，其认知功能立即得到改善。在逐渐减少皮质类固醇剂量三年后，他没有复发。对于类固醇抵抗的桥本脑病，应考虑CPA冲击治疗。
