Fadili Hajar, Tazi Rim, El Oury Hiba, El Aidaoui Karim, Hazim Asmaa
Neurology, Cheikh Khalifa Bin Zayed Al Nahyan Hospital, Mohammed VI University of Health Sciences (UM6SS), Casablanca, MAR.
Nephrology, Cheikh Khalifa Bin Zayed Al Nahyan Hospital, Mohammed VI University of Health Sciences (UM6SS), Casablanca, MAR.
Cureus. 2022 Mar 9;14(3):e22982. doi: 10.7759/cureus.22982. eCollection 2022 Mar.
Creutzfeldt-Jakob disease is a rare, transmissible neurodegenerative disorder, most prevalent between the ages of 50 and 70 years old, that is incurable and fatal. It's caused by a slow, infectious protein agent-designated prion. The most common clinical presentations are sleep disturbances, personality changes, ataxia, aphasia, visual disturbances, weakness, and myoclonus combined with progressive dementia. Here we report the case of a patient with disturbance of consciousness, restlessness, and myoclonia who died two weeks after admission. The analysis of his cerebrospinal fluid reveals that the presence of 14-3-3 protein was positive, which supports the diagnosis of Creutzfeldt-Jakob disease. Our observation underscores the importance of the quick fatality of this case.
克雅氏病是一种罕见的、可传播的神经退行性疾病，最常发生在50至70岁之间，无法治愈且会致命。它由一种缓慢的、具有传染性的蛋白质因子——朊病毒引起。最常见的临床表现是睡眠障碍、人格改变、共济失调、失语、视觉障碍、虚弱以及肌阵挛并伴有进行性痴呆。在此我们报告一例意识障碍、烦躁不安和肌阵挛的患者，其入院两周后死亡。对其脑脊液的分析显示14-3-3蛋白呈阳性，这支持了克雅氏病的诊断。我们的观察强调了该病例快速致死的重要性。
