Abstract: Creutzfeldt-Jakob disease（CJD） is a rare neurodegenerative disease caused by mutated prion protein（PrP），often presenting with a subacute or chronic onset，and rapid progressive dementia as the major clinical symptom.The disease is infectious and always fatal.Due to lack of specific initial symptoms，CJD is easily misdiagnosed as other neurological diseases.We reported 3 CJD patients with different clinical manifestations who were misdiagnosed as other diseases at admission，with a summary of their clinical，MRI imaging，EEG，and cerebrospinal fluid characteristics，and conducted a literature review，with a view to improving clinicians' understanding of the disease，thereby reducing the possibilities of misdiagnosis.

Key words: Creutzfeldt-Jakob syndrome, Magnetic resonance imaging, Electroencephalography, Diagnostic errors

摘要： Creutzfeldt-Jakob病（CJD）是一种由变异的朊蛋白（PrP）引起的罕见的神经退行性病变，常表现为亚急性或慢性起病，主要临床症状为快速进展性痴呆，具有传染性及绝对的致死性。目前因为该病最初呈现的症状缺乏特异性而容易被误诊为其他神经系统疾病。本文通过介绍3例临床表现各不相同且入院时均误诊的CJD，总结其临床、影像学、脑电图及脑脊液特点并结合文献分析，从而使临床医生能够进一步了解该病，减少误诊。

关键词: 克-亚综合征, 磁共振成像, 脑电图, 误诊