Kondziella Daniel, Hansen Klaus, Gonzalez Teresa, Gideon Peter, Christiansen Ingelise, Sellebjerg Finn
Neurologisk Klinik, Afsnit 2094, Rigshospitalet, Blegdamsvej 9, 2100 København Ø, Denmark.
Ugeskr Laeger. 2012 Feb 27;174(9):577-9.
Hashimoto encephalitis (HE) is a steroid-responsive autoimmune encephalitis with anti-thyroid antibodies; Creutzfeldt-Jakob disease (CJD) is a prion disease. Both disorders can have a similar clinical presentation. Two women, 67 and 63 year-old, with subacute dementia, ataxia, myoclonus and positive antithyroid antibodies were given oral steroids. Whereas one progressively declined and had histopathologically proven CJD, the other made a complete recovery and was diagnosed with HE. Anti-thyroid antibodies can occur in CJD, but when present in a patient with subacute dementia, ataxia and myoclonus, a steroid trial always seems indicated.
桥本脑病(HE)是一种对类固醇有反应的自身免疫性脑炎,伴有抗甲状腺抗体;克雅氏病(CJD)是一种朊病毒病。这两种疾病可能有相似的临床表现。两名分别为67岁和63岁的女性,患有亚急性痴呆、共济失调、肌阵挛且抗甲状腺抗体呈阳性,她们接受了口服类固醇治疗。其中一名患者病情逐渐恶化,经组织病理学证实患有CJD,而另一名患者完全康复,被诊断为HE。抗甲状腺抗体可出现在CJD患者中,但当出现在患有亚急性痴呆、共济失调和肌阵挛的患者身上时,似乎总是需要进行类固醇试验。