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2015, Volume 31, Number 2, Page(s) 148-152     
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DOI: 10.5146/tjpath.2013.01195
Creutzfeldt-Jakob Disease: Report of Four Cases and Review of the Literature
Fatma Öz ATALAY1, Şahsine TOLUNAY1, Gonca ÖZGÜN1, Ahmet BEKAR2, Mehmet ZARİFOĞLU3
1Departments of Pathology, Uludağ University, Faculty of Medicine, BURSA, TURKEY
2Departments of Neurosurgery Uludağ University, Faculty of Medicine, BURSA, TURKEY
3Departments of Neurology, Uludağ University, Faculty of Medicine, BURSA, TURKEY
Keywords: Creutzfeldt-Jakob disease, Spongiform encephalopathy, Prion protein

Creutzfeldt-Jakob disease is a very rare, progressive neurodegenerative disorder that is incurable and always fatal. It is one of the transmissible spongiform encephalopathies caused by prions. Multiple vacuoles in neuropil and neuronal loss in the gray matter gives the classical sponge–like appearance of brain and are responsible for the typical clinical symptoms.

In this report, we present 4 cases referred to the neurology department of Uludağ University with neurological symptoms. Patients were evaluated with electroencephalogram and magnetic resonance imaging, and performed brain biopsies for further investigation. For definitive diagnosis of Creutzfeldt-Jakob disease, accumulation of prion protein in brain was detected immunohistochemically. Patients died within weeks in consequence of rapid progression of the disease.

Although Creutzfeldt-Jakob disease is an infrequent disorder, when a patient presents with characteristic clinical symptoms such as rapidly progressive dementia with myoclonus, the diagnosis of Creutzfeldt-Jakob disease should be taken into consideration.

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