Creutzfeldt-Jacob Disease: Two Case Reports
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
Abstract
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.