PDF(Pubmed)
Abstract：
Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disorder, characterized by the accumulation of abnormal prion proteins in the brain. While CJD has some typical clinical features, its presentation can be quite heterogeneous, particularly in the early stages of the disease, posing challenges in diagnosis. Atypical manifestations of CJD can mimic various neurodegenerative disorders, including atypical parkinsonisms. In this case report, we present an 81-year-old man who exhibited an atypical clinical presentation of sporadic CJD, initially resembling progressive supranuclear palsy (PSP). The patient presented with symmetric parkinsonism, postural instability, and ocular motor dysfunction, accompanied by rapid clinical deterioration. Alongside the case report, we also provide a review of the literature on atypical presentations of CJD as PSP, highlighting the importance of recognizing these manifestations in clinical practice.
摘要：
克雅氏病(CJD)是一种罕见的,快速进行性神经退行性疾病，其特征是大脑中异常朊病毒蛋白的积累。虽然CJD有一些典型的临床特征，它的呈现可能非常不同，特别是在疾病的早期阶段，对诊断提出挑战。CJD的非典型表现可以模仿各种神经退行性疾病，包括非典型帕金森病。在这个案例报告中,我们介绍了一位81岁的男性，他表现出零星的CJD的非典型临床表现，最初类似于进行性核上性麻痹（PSP）。患者出现对称性帕金森病,姿势不稳定，和眼运动功能障碍，伴随着快速的临床恶化。除了病例报告，我们还提供了关于CJD作为PSP的非典型表现的文献综述，强调在临床实践中认识这些表现的重要性。
