Department of Psychiatry, Erenköy Psychiatry and Neurology Education and Research Hospital, Istanbul, Turkey.
Psychogeriatrics. 2011 Jun;11(2):119-24. doi: 10.1111/j.1479-8301.2011.00361.x.
Creutzfeldt-Jakob disease (CJD) is a progressive, degenerative, and fatal disease of the central nervous system. It is caused by abnormal accumulation of prion proteins and is characterized mainly by progressive dementia, myoclonus, and cerebellar, pyramidal, and extrapyramidal findings. Psychiatric symptoms may also accompany CJD and are often the first signs of the disease. The incidence of CJD is approximately 1 in 1 000 000. In certain cases, a diagnosis can be made by demonstrating the accumulation of pathological prion proteins. However, in many cultures brain biopsies or post-mortem evaluations are not welcomed by either the patients or their relatives. In these cases, the importance of additional diagnostic tools increases. Herein, we report on a CJD patient who first consulted a psychiatrist with early psychiatric symptoms. The patient developed neurological symptoms later and was subsequently diagnosed as sporadic CJD based on clinical and laboratory findings rather than brain biopsy. Repeated electroencephalograms (EEG) played a pivotal role in our evaluation of the patient. This case is an interesting presentation of CJD both because of the timing of the symptoms and because of the typical EEG findings that led to the diagnosis.
克雅氏病（CJD）是一种中枢神经系统进行性、退行性和致命性疾病。它是由异常聚集的朊病毒蛋白引起的，主要表现为进行性痴呆、肌阵挛和小脑、锥体束和锥体外系发现。精神症状也可能伴随 CJD 发生，并且常常是疾病的最初迹象。CJD 的发病率约为每 100 万人中有 1 例。在某些情况下，可以通过证明病理性朊病毒蛋白的积累来做出诊断。然而，在许多文化中，脑活检或死后评估不受患者或其亲属的欢迎。在这些情况下，增加额外的诊断工具变得尤为重要。在此，我们报告了一位 CJD 患者，他最初因早期精神症状就诊于精神科医生。患者后来出现了神经症状，并随后根据临床和实验室发现而不是脑活检被诊断为散发性 CJD。重复脑电图（EEG）在我们对患者的评估中发挥了关键作用。该病例是 CJD 的一个有趣表现，既因为症状的时间，也因为典型的 EEG 发现导致了诊断。
