PDF(Pubmed)
Abstract：
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.
摘要：
克雅氏病是一种罕见的,传染性神经退行性疾病，最普遍的年龄在50到70岁之间，那是无法治愈和致命的。它是由一个缓慢的，感染性蛋白质剂指定的朊病毒。最常见的临床表现是睡眠障碍，人格改变,共济失调,失语症,视觉障碍,弱点,肌阵挛症合并进行性痴呆.在这里，我们报告一个意识障碍的病人，躁动,和入院两周后死亡的肌阵挛症。对他的脑脊液的分析表明，14-3-3蛋白的存在是阳性的，这支持克雅氏病的诊断。我们的观察强调了这种情况下快速死亡的重要性。
