Introduction: Our aim was to conduct a retrospective review to demonstrate the clinical, radiological, and electrophysiological features of patients with sporadic Creutzfeldt-Jacob disease (sCJD). Methods: A total of 10 patients (5 female and 5 male, with a mean age of 45 years from a range of 40 to 67 years) out of 8.259 adult patients hospitalized from January 2000 to December 2008 were diagnosed with sCJD. Results: Eight of the patients were diagnosed on the basis of clinical, radiological, electroencephalography (EEG), and cerebrospinal fluid (CSF) findings. Two other patients also had a pathological diagnosis. The most common signs and symptoms were behavioral disturbances, movement disorders, cognitive decline, myoclonus, psychosis, focal neurological deficit, and aphasia. Nine of the patients had periodic sharp wave discharges on EEG. Seven patients were positive for the 14.3.3 protein in the CSF. Five patients had pulvinar signs-a bilateral increased signal in the pulvinar thalami-on cranial magnetic resonance imaging. Eight patients were diagnosed with probable sCJD; two were diagnosed with definite sCJD. All of the patients died as a result of the disease within 24 months after the onset of symptoms. Discussion: sCJD should be considered in the diagnosis of patients who present with rapidly progressive dementia. Clinical and radiological data appear to be sufficient for the diagnosis. However, detailed molecular examinations of the subtypes of the disease may be required for early diagnosis of cases given the wide spectra of CJD.

Giris: Sporadik Creutzfeldt-Jacob Hastaligi (sCJH) tanisi almis hastalarin klinik, radyolojik ve elektrofizyolojik özelliklerinin retrospektif olarak degerlendirilmesini amaçladik. Yöntem: Ocak 2000- Aralik 2008 yillari arasinda hastaneye yatirilan 8259 yetiskin hasta arasindan 10 hasta (5 kadin, 5 erkek; ortalama yas 45; yas araligi 40- 67 yil) sCJH tanisi almisti. Bulgular: Sekiz hastanin tanisi klinik belirti ve bulgular, radyolojik, elektroen-sefalografi (EEG) ve beyin omurilik sivisi (BOS) tetkiklerinin sonuçlari ile kondu. Diger iki hastanin ek olarak patolojik tanilari da vardi. En sik belirti ve bulgular davranis problemleri, hareket bozukluklari, kognitif yikim, miyoklonus, psikoz, fokal nörolojik defisit ve afazi idi. Dokuz hastanin EEG’sinde periodik keskin dalga desarjlari vardi. Yedi hastanin BOS incelemesinde 14.3.3 proteini saptandi. Bes hastanin kranyal magnetik rezonans görüntülemesinde her iki pulvinar çekirdeklerde sinyal artisi (pulvinar belirti) izlendi. Sekiz hastaya olasi, iki hastaya kesin sCJH tanisi kondu. Tüm hastalar hastalik belirti ve bulgularinin baslangicindan sonraki 24 ay içinde öldü. Tartisma: Hizli ilerleyici demansiyel bulgularla basvuran hastalarda ihtimali tanilar arasinda sCJH düsünülmelidir. Klinik ve radyolojik tetkik sonuçlari tani için yeterli olsa da CJH’nin genis yelpazesi düsünüldügünde olgularin erken taninmasi asamasinda hastaligin alt tiplerinin detayli moleküler inceleme ile taninmasi gerekli olabilir.