Creutzfeldt–Jakob disease

• Published in: Psychogeriatrics 2011-06, Vol.11 (2), p.119-124
• Main Authors: GENÇER, Ali Görkem, PELIN, Zerrin, KÜÇÜKALI, Cem İsmail, TOPÇUOĞLU, Özgür Bilgin, YILMAZ, Nuriye
• Format: Article
• Language: English
• Subjects: Aged; Brain - pathology; Brain - physiopathology; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - physiopathology; Creutzfeld–Jacob disease; Diagnosis, Differential; Disease Progression; Electroencephalography; Fatal Outcome; Female; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Medical diagnosis; Neurologic Examination; Phosphopyruvate Hydratase - cerebrospinal fluid; psychiatric aspects; Psychomotor Disorders - diagnosis; Psychomotor Disorders - physiopathology; Signal Processing, Computer-Assisted; sporadic type
• ISSN: 1346-3500; 1479-8301
• 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.