Aysu Şen1, Sefer Günaydın2, Mustafa Ülker3, Baki Arpacı3

1Neurology Clinic 1, Bakırköy Ruh Sağlığı Ve Sinir Hastalıkları Eğitim Ve Araştırma Hastanesi, İstanbul, Turkey
2Neurology Clinic, Haseki Education And Research Hospital, İstanbul, Turkey
3Neurology Clinic, Umraniye State Hospital, İstanbul, Turkey

Keywords: Creutzfeldt - Jakob Disease, subacute dementia, prion diseases


Creutzfeldt-Jakob Disease (CJD) is characterised by

subacute progressive dementia, cerebellar ataxia, myoclonic jerks

together with pyramidal and extrapyramidal signs. It is a rare prion

disease and definitive diagnosis can only be made by biopsy. It becomes

progressively worse and the death is the rule. We presented two CJD cases because of their

demonstrative characteristics. A 43 year-old female and a 52 year-old male patient was

suspected to be CJD due to presence of subacute severe cognitive

deterioration, neuropsychiatric disturbances, myoclonic jerks, ataxia,

pyramidal and extrapyramidal signs and also periodic spike and wave

complexes in EEG. Patients were lost in a short period of time because

of the complications of disease process. Medical autopsy were done in

both cases for definitive diagnosis and autopsy results displayed

characteristic pathologic findings of CJD. Patients were diagnosed as

definitive sporadic CJD according to Master’s, French and European

criterias. CJD should be considered in patients with rapidly

progressive dementia, that starts with various neuropsychiatric

symptoms. Although seen very rare, CJD is a untreatable, fatal disease. Therefore we emphasize that, preventive precaution should be taken

when a CJD diagnosis is suspected.