You are here

CREUTZFELDT-JAKOB HASTALIĞI: OTOPSİ SUNUMU VE LİTERATÜRÜN GÖZDEN GEÇİRİLMESİ

Journal Name:

Publication Year:

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.
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.
42-48

REFERENCES

References: 

1. DeArmond SJ, Prusiner SB. Etiology and pathogenesis of prion disease. Am J Pathol 1995; 146: 785-811.
2. Brown P, Gibbs CJ, Johnson PR, et al. Human spongioform encephalopathy of experimentally transmitted disease. Ann Neurol 1994; 35: 513-529.
3. Gajdusek DC, Gibbs CJ Jr, Alpers M. Experimental transmission of a kuru-like syndrome in chimppanzees. Nature 1966; 209:794.
4. Masters CL, Gajdusek DC, Gibbs CJ Jr. Creutzfeldt-Jakob disease virus isolation from the Gerstmann-Straussler syndrome, with an analysis of the varrous forms of amyloid deposition in the virus-induced spongioform encephalopaties. Brain 1981; 104: 559.
5. Asher DM. Transmissible spongroform encephalopaties. In Gorbach SL, Bartlet JG, Black NR.Infectious Disease. Philadelphia. WB Saunders Company 1998; 2283-2312.
6. Jubelt B, Miller JR. Viral infections in Rowland LP: Merrit's Textbook of Neurology. Williams and Wilkins 1995; 142-179.
7. Zeidler M, Johnstone EC, Bamber RWK, et al. New variant Creutzfeldt-jacob disease: psychiatric features. Lancet 1997; 350: 908-910.
8. Zeidler M, Stewart GE, Barraclough CR, et al. New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet 1997; 350: 903-907.
9. Brown P, Cervenakova L, Goldfarb LG, et al. Iatrogenic Creutzfeldt-Jakob disease: An example of the interplay between ancient genes and modern medicine. Neurology. 1994; 44: 291-293.
10. Billete de villemeur T, Dslys JP, Pradel A, et al. Creutzfeldt-Jakob disease from contaminated growth hormone extracts France. Neurology 1996; 47: 690-695.
11. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: Hormones homografts, and Creutzfeldt-jakob disease. Lancet 1992; 340: 24-27.
12. Gorman DG, Benson DF, Vegel DG, et al. Creutzfeldt-Jakob disease in a pathologist. Neurology 1992; 42: 463.
13. Berger JR, David NJ. Creutzfeldt-Jakob disease in a physicran. A review of
the disorder in health care workers. Neurology 1993; 43: 205-206.
14. Miller D. Creutzfeldt-Jakob disease in histopathology technicrans. N Engl J Med 1988; 318: 853-854.
15. Brown P. The risk of bovine spongioform encephalopathy (mad cow disease) to human health. JAMA 1997, 278: 1008-1011.
16. Counsens SN, Zeidler M, Esmonde TF, et al. Sporadic creutzfeldt-Jakob disease in the United Kingdom: analysis of epidermiological surveillance data for 1970-96 BMJ 1997; 315: 389-395.
17. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1984; 216: 136.
18. Rohwer RG. Estimation of scrapie nucleic acid MW from standart curves for virus sensitivity ot ionizing radiation. Nature 1986; 320: 381.
19. Collinge J, Whittington MA, Sidle KCL, et al. Prion protein is necessary for normal synaptic function. Nature 1994; 370: 295-297.
20. Bockman JM, Kingsbury DT, McKinley MP, et al. Creutzfeldt-Jakob disease prion proteins in human brains N Eng J Med 1985; 312: 73-78.
21. Meyer RK, McKinley MP, Bowman KA, et al. Seperation and properties of cellular and scrapre proteins. Proc Natl Acad Sci USA 1986; 83: 2310-2314.
22. Kretzschmar HA, Prousiner SB, Stowring LE, et al. Scrapie prion proteins are synthesized in neurons. Am J Pathol 1986; 122: 1-5.
23. Doi S, Ito M, Shinagawa M, et al. Western blot detection of scrapie-associated fibril protein in tissues outside the central neroous system from precliclinical scrapie-infected mice. J Gen Virol 1988; 69: 955-960.
24. Collinge J, Rossor MN, Thomas D, et al. Tonsil biopsy helps diagnose new variant Creutzfeldt-Jakob disease BMJ 1998; 317: 472.
25. Masters CL, Richardson EBJ. Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spangioform change. Brain 1978; 101: 333-344.
26. Piccardo P, Safar J, Ceroni M, et al. Immunohistochemical localization of piron protein in spongioform encephalopathies and normal brain tissue. Neurology 1990; 40: 518-522.
27. Kitamoto T, Tateishi J. İmmunohistochemical conformation of creutzfeldt-Jakob disease with a long clinical course with amyloid plaque core antibodies Am J Pathol 1988; 131: 435-443.
28. Roos R, Gajdusek DC, Gibbs CJJR. The clinical characteristics of transmissible creutzfeldt-Jakob disease. Brain 1973; 96: 1-20.
29. Hsich G, Kenney K, Gibbs CJ, et al. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiofrom encephalopathies. New Eng J Med 1996; 335: 924-930.
30. Mc Conville JP, Craig JJ. Diagnosis of Creutzfeldt-Jakob disease by measurement of S-100 protein in serum. Appropriate study populations must be used. BMJ 1998; 317: 472-473.
31. Gajdusek DC, Gibbs CJ Jr, Asher DM, et al. Precautions in medical case of and in handling materials from patients with transmisible virus dementia (Creutzfeldt-Jakob disease). N Eng J Med 1977; 297: 1253- 1258.
32. Gajdusek DC, Gibbs CFJ. Survival of Creutzfeldt-Jakob disease virus in formal-fixed brain tissue. N Eng J Med 1976; 294: 553.
33. Brown P, Wolf A, Gajdusek DC. A simple and effective method for inactivating virus infectivity in formalin fixed tissue samples from patients with Creutzfeldt-Jakob disease Neurology 1990; 40: 887-890.

Thank you for copying data from http://www.arastirmax.com