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. 2013 Dec;50(4):306-311.
doi: 10.4274/Npa.y5903. Epub 2013 Dec 1.

Sporadic Creutzfeldt-Jacob Disease: An 8-Year Experience from a Single Center in Turkey

Özlem Taşkapilioğlu  1 , Meral Seferoğlu  1 , Şükran Yurtoğullari  1 , Bahattin Hakyemez  2 , Sevda Erer  1 , Necdet Karli  2 , Mustafa Bakar  1 , Ö Faruk Turan  1 , Mehmet Zarifoğlu  1 , Şahsine Tolunay  2 , İbrahim Bora  1
Affiliations

Sporadic Creutzfeldt-Jacob Disease: An 8-Year Experience from a Single Center in Turkey

Özlem Taşkapilioğlu et al. Noro Psikiyatr Ars. 2013 Dec.

Abstract
in English, Turkish

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).

Method: 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.

Giriş: Sporadik Creutzfeldt-Jacob Hastalığı (sCJH) tanısı almış hastaların klinik, radyolojik ve elektrofizyolojik özelliklerinin retrospektif olarak değerlendirilmesini amaçladık.

Yöntem: Ocak 2000- Aralık 2008 yılları arasında hastaneye yatırılan 8259 yetişkin hasta arasından 10 hasta (5 kadın, 5 erkek; ortalama yaş 45; yaş aralığı 40–67 yıl) sCJH tanısı almıştı.

Bulgular: Sekiz hastanın tanısı klinik belirti ve bulgular, radyolojik, elektroen-sefalografi (EEG) ve beyin omurilik sıvısı (BOS) tetkiklerinin sonuçları ile kondu. Diğer iki hastanın ek olarak patolojik tanıları da vardı. En sık belirti ve bulgular davranış problemleri, hareket bozuklukları, kognitif yıkım, miyoklonus, psikoz, fokal nörolojik defisit ve afazi idi. Dokuz hastanın EEG’sinde periodik keskin dalga deşarjları vardı. Yedi hastanın BOS incelemesinde 14.3.3 proteini saptandı. Beş hastanın kranyal magnetik rezonans görüntülemesinde her iki pulvinar çekirdeklerde sinyal artışı (pulvinar belirti) izlendi. Sekiz hastaya olası, iki hastaya kesin sCJH tanısı kondu. Tüm hastalar hastalık belirti ve bulgularının başlangıcından sonraki 24 ay içinde öldü.

Sonuç: Hızlı ilerleyici demansiyel bulgularla başvuran hastalarda ihtimali tanılar arasında sCJH düşünülmelidir. Klinik ve radyolojik tetkik sonuçları tanı için yeterli olsa da CJH’nın geniş yelpazesi düşünüldüğünde olguların erken tanınması aşamasında hastalığın alt tiplerinin detaylı moleküler inceleme ile tanınması gerekli olabilir.

Keywords: Sporadic Creutzfeldt-Jakob disease; cerebrospinal fluid; magnetic resonance imaging; periodic sharp wave complexes.

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Conflict of interest statement

Conflict of interest: The authors reported no conflict of interest related to this article. Çıkar çatışması: Yazarlar bu makale ile ilgili olarak herhangi bir çıkar çatışması bildirmemişlerdir.

Figures

Figure 1
Figure 1
T2-weighted (A, B) and fluid-attenuated inversion recovery (C, D) images show minimal intensity increases in the putaminal and caudat nuclei; increased intensities are prominent in the caudate (arrowheads), putaminal nuclei (small arrows), bilateral Sylvian regions and insula (big arrows) on T2 (E, F) and fluid-attenuated inversion recovery (G, H) images acquired 3 months after.
Figure 2
Figure 2
T2-weighted (A) image shows increased intensities in the putaminal, caudate nuclei, and bilateral sylvian regions. There are intensity increases in the bilateral caudate and putaminal nuclei (small arrowhead) and cortex of the left cerebral hemisphere (long arrows) in the diffusion-weighted images (B,C). ADC (D) image shows hypointensity consistent with the diffusion restriction.
See this image and copyright information in PMC

References

    1. Johnson RT. Prion diseases. Lancet Neurol. 2005;4:635–642. - PubMed
    1. World Health Organisation. Consensus on criteria for diagnosis of sporadic CJD. Wkly Epidemiol Rec. 1998;73:361–365. - PubMed
    1. Appleby BS, Appleby KK, Crain BJ, Onyike CU, Wallin MT, Rabins PV. Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol. 2009;66:208–215. - PubMed
    1. WHO. Creutzfeldt-Jacob Disease, WHO Recommended Surveillance Standards. Geneva: 1999. pp. 35–38.
    1. Bergui M, Bradac GB, Rossi G, Orsi L. Extensive cortical damage in a case of Creutzfeldt-Jacob disease: clinicoradiological correlations. Neuroradiology. 2003;45:304–347. - PubMed

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