title: Creutzfeldt-Jakob disease.
abstract: 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.
authors.affiliation: Department of Psychiatry, Erenköy Psychiatry and Neurology Education and Research Hospital, Istanbul, Turkey. gorkemgencer@gmail.com
authors.forename: Ali Görkem
authors.initials: AG
authors.lastname: Gençer
authors.forename: Zerrin
authors.initials: Z
authors.lastname: Pelin
authors.forename: Cem İsmail
authors.initials: Cİ
authors.lastname: Küçükali
authors.forename: Ozgür Bilgin
authors.initials: OB
authors.lastname: Topçuoğlu
authors.forename: Nuriye
authors.initials: N
authors.lastname: Yilmaz
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004569:Electroencephalography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D010751:Phosphopyruvate Hydratase / Q000134:cerebrospinal fluid; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000503:physiopathology; D012815:Signal Processing, Computer-Assisted
chemical_list: D010751:Phosphopyruvate Hydratase