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CREUTZFELDT-JAKOB HASTALIĞI: OTOPSİ SUNUMU VE LİTERATÜRÜN GÖZDEN GEÇİRİLMESİ

Journal Name:

  • Cerrahpaşa Tıp Dergisi

Publication Year:

  • 2000

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Creutzfeldt-Jakob disease (CJD) is a very rare untreatable progressive neurodegenerative disorder. Its incidence is one in 1 million per year in European countries, which is rarer in Turkey. It is associated with the presence of a protein in the brain, incompletely defined transmissible agent, called as prion. Observation.- An autopsy case of 64 years old man was presented. Grossly there was a severe cortical atrophy in the brain especially in the frontal and temporal lobes. Histopathological sections showed spongiform changes and neuronal loss in the gray matter. Immunohistochemical examination revealed synaptic and plaque like prion protein (PrP) accumulation in the cortex and severe reactive astrogliosis which was positive for GFAP.
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Abstract (Original Language): 
Creutzfeldt-Jakob Hastalığı oldukça nadir görülen, ilerleyici, tedavisi mümkün olmayan nörodegeneratif hastalıklar arasındadır. Avrupa ülkelerinde insidansı yılda bir milyonda bir olarak verilmekte iken, Türkiyede oldukça nadir sporadik vakalar bildirilmektedir. Cerrahpaşa Tıp Fakültesinde tanı alan, ilk CJD olgusu olduğundan bu otopsi olgusu literatür bilgisi ışığında sunulmaya uygun görülmüştür. 64 yaşında bir erkek hastaya ait otopsi değerlendirilmesinde frontal ve temporal loblarda daha belirgin kortikal atrofi saptandı. Işık mikroskobik değerlendirmede ise gri cevherde yaygın spongioform degenerasyon, nöronal hücrelerde kayıp ve ağır reaktif gliozis izlendi. İmmunhistokimyasal olarak korteksde plak tarzında veya sinaptik prion-protein (PrP) birikimi belirlendi.
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  • 1
42-48
  • Turkish

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