Sharma Nitu, Sharma Jitender Kumar, Chander Ashima, Shergill Khushdeep, Yadav Meghna
Military Hospital Ambala Cantt, Department of Pathology, Ambala Cantt, India.
Army College of Medical Sciences, Base Hospital Delhi Cantt, New Delhi, India.
Autops Case Rep. 2024 Jun 21;14:e2024502. doi: 10.4322/acr.2024.502. eCollection 2024.
Sporadic Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative spongiform encephalopathy that causes neuronal derangement secondary to prion protein. Its initial diagnosis is often complex and challenging due to non-specific clinical presentation, lack of awareness, and low clinical suspicion. This disease is invariably fatal, and most patients die within 12 months of presentation. Definite diagnosis of prion disease requires neuropathological analysis, usually done at autopsy. Here, we present the autopsy findings of a 57-year-old male patient, illustrating the complexity of diagnosing this disease early in the clinical course and the need for a broad differential diagnosis at the onset.
散发性克雅氏病（CJD）是一种罕见的神经退行性海绵状脑病，由朊病毒蛋白导致神经元紊乱。由于临床表现不具特异性、认识不足以及临床怀疑度低，其初始诊断往往复杂且具有挑战性。这种疾病必然致命，大多数患者在出现症状后的12个月内死亡。朊病毒病的确切诊断需要进行神经病理学分析，通常在尸检时进行。在此，我们展示了一名57岁男性患者的尸检结果，说明了在临床病程早期诊断这种疾病的复杂性以及在发病时进行广泛鉴别诊断的必要性。
