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Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu

Year 2006, Volume: 37 Issue: 2, 58 - 63, 18.07.2014

Nurhak Demir Belgin Mutluay Musa Öztürk Yavuz Altunkaynak Vedat Sözmen Hayriye Küçükoğlu Ayten Dirican Sevim Baybaş

Abstract

Sporadic Creutzfeldt-Jakob Hastalığı (CJH), 50-70 yaş arasında görülen demansla ilişkili süreçlerde akla getirilmesi gereken hastalıklardan biridir. Klinik özellikleri, tipik elektroensefalografi (EEG) bulguları, laboratuar ve radyolojik görüntüleme özellikleri ile demansla giden diğer hastalıklardan ayırt edilebilir. Bu çalışmada klinik, EEG, laboratuar ve radyolojik özellikleri ile birlikte beş sporadic CJH olgumuzu sunmayı amaçladık. Sporadic CJH tanısı ile kliniğimizde takip edilmiş olan beş olgu değerlendirildi. Yaş ortalaması 63,8 olan hastaların ikisi kadın, üçü erkekti. Bütün hastalarda EEG kayıtları yapıldı. Beyin görüntüleme, bir hastada bilgisayarlı tomografi (BT), dört hastada manyetik rezonans görüntüleme (MRG) ile değerlendirildi. Üç hastada, beyin omurilik sıvısında (BOS) 14.3.3 protein, iki hastada nöron spesifik enolaz (NSE) bakıldı. Beyin biyopsisi bir hastada yapılabildi ve kesin CJH tanısı konuldu. Diğer olgularda biyopsi yapılamadığı için muhtemel sporadic CJH tanısı klinik özellikler, EEG bulguları, radyolojik ve laboratuar özellikleri ile konuldu. Demans bulguları olan hastaların dördünde EEG'de periyodik keskin ve yavaş dalga aktivitesi saptandı. İlk EEG kayıtları nonspesifik iken, tekrarlanan EEG kayıtlarında özgün bulgular saptandı. 14.3.3 proteini bakılan üç hastada da sonuç pozitif idi. Bazal gangliyonlarda hiperintensite beyin MRG tetkiki yapılan, dört hastadan birinde mevcuttu. Klinik özellikleri ile sporadic CJH düşünülen hastalarda, tekrarlayan EEG kayıtları yapmak tanıda yararlıdır.

Keywords

Sporadic Creutzfeldt-Jacob Hastalığı elektroensefalografi

References

  • 1. Johnson RT, Gibbs CJ; Creutzfeldt-Jacob disease and related transmissible spongiform encephalopathies. NEJM 1998; 339: 1994-2000.
  • 2. Brown P, Will RG, Bradley R, Asher DM, Detwiler L; Bovine spongiform encephalopathy and variant Creutzfeldt-Jacob disease: Background, evolution, and current concerns. Emerg Infect Dis 2001; 7: 6-16.
  • 3. Prusiner SB; Prion diseases and the BSE crisis. Science 1997; 278: 245-251.
  • 4. Prusiner SB; Neurodegenerative diseases and prions. NEJM 2001; 344:1516-1526.
  • 5. Brown P; Infections of the nervous system. In: Butterworth-Heinemann, Philadelphia. Bradley WG, Daroff RB, Fenichel GM, Marsden CD; Neurology in Clinical Practice 2000:1424-1429.
  • 6. Steinhoff BJ, Raecker S, Herrendorf G et al.; Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt- Jacob Disease. Arch Neurol 1996;53:162-166.
  • 7. Parchi P, Giese A, Capellari S et al.; Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46: 224-233.
  • 8. Zerr I, Pocchiari M, Collins S et al.; Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000; 55:811-815.
  • 9. Poser S, Mollenhauer B, Krauss A, Zerr I, Steinhoff BJ, Schroeter A, Finkenstaedt M, Schulz-Schaeffer WJ, Kretzschmar HA, Felgenhauer K; How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain 1999; 122: 2345-2351.
  • 10. Knopmann DS, DeKosky ST, Cummings JL, Chui H, Corey-Bloom J, Relkin N, Small GW, Miller B, Stevens JC; Practice parameter: Diagnosis of dementia (an evidence- based review ). Neurology 2001; 56: 1143-1153.
  • 11. Zerr I, Bodemer M, Racker S, Groche S, Poser S, Kretzschmar HA, Weber T; Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of CreutzfeldtJakob disease. The Lancet 1995; 345: 1609-1610.
  • 12. Dahbour SS, Ghanmi HA, Taleb AA; Creutzfeldt-Jacob Disease: Cerebrospinal fluid protein 14-3-3 in a Saudi woman. Kuwait Med 2002; 34: 308-312.
  • 13. Draayer YM; Braff SP, Nagle KJ, Pendlebury W, Penar PL, Shapiro RE; Emerging patterns of diffusion-weigted MR imaging in Creutzfeldt-Jakob disease: Case report and review of the literature. AJNR 2002; 23: 550-556.
  • 14. Schaefer PW, Ellen Grant P, Gilberto Gonzales R; Diffusion-weigted MR imaging of the brain. Radiology 2000; 217: 331-345.
  • 15. Sellars RJ, Collie DA, Will RJ; Progress in understanding Creutzfeldt-Jakob disease. AJNR 2002; 23: 1070- 1072.
  • 16. Seipelt M, Zerr I, Nau R, Mollenhauer B, Kropp S, Steinhoff BJ, Wilhelm-Gössling C, Bamberg C, Janzen RWC, Berlit P, Manz F, Felgenhauer K, Poser S; Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease. JNNP 1999; 66: 172-176.
  • 17. Samman I, Schulz-Schaeffer WJ, Wöhrle JC, Sommer A, Kretzschmar HA, Hennerici M; Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2. JNNP 1999; 67: 678-681.
  • 18. Hsich G, Kenney K, Gibbs CJ, Lee KH, Harrington MG; The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. NEJM 1996; 335: 924-930.
  • 19. Keohane C; Pulvinar sign on MRI images in variant Creutzfeldt-Jakob disease.The Lancet 2000; 355, (9213): 1384.
  • 20. Bahn MM, Parchi P; Abnormal diffusion-weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 1999;56:577-583.
  • 21. Mittal S, Farmer P, Kalina P, Kingsley PB, Halperin J; Correlation of diffusion- weighted magnetic resonance imaging with neuropathology in Creutzfeldt- Jakob disease. Arch Neurol 2002;59:128-134.
  • 22. Schröter A, Zerr I, Henkel K, Tschampa HJ, Finkenstaedt M, Poser S; Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 2000;57:1751-1757.
  • 23. Sellars RJ, Collie DA, Will RJ; Progress in understanding Creutzfeldt-Jakob disease. Am Neuroradiol 2002; 23: 1070-1072.
  • 24. Prabhakar S, Bhatia R; Diagnosis of Creutzfeldt-Jacob disease. Neurology India 2001;49:325-328.
  • 25. Brown P, Preece M, Brandel J-P, Sato T, McShane L, Zerr I, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000;55:1075-1081.
  • 26. World Health Organization. Infection control guidelines for transmissible spongiform encephalopathies: report of a WHO Consultation. Geneva: WHO, 1999.

Year 2006, Volume: 37 Issue: 2, 58 - 63, 18.07.2014

Nurhak Demir Belgin Mutluay Musa Öztürk Yavuz Altunkaynak Vedat Sözmen Hayriye Küçükoğlu Ayten Dirican Sevim Baybaş

Abstract

References

  • 1. Johnson RT, Gibbs CJ; Creutzfeldt-Jacob disease and related transmissible spongiform encephalopathies. NEJM 1998; 339: 1994-2000.
  • 2. Brown P, Will RG, Bradley R, Asher DM, Detwiler L; Bovine spongiform encephalopathy and variant Creutzfeldt-Jacob disease: Background, evolution, and current concerns. Emerg Infect Dis 2001; 7: 6-16.
  • 3. Prusiner SB; Prion diseases and the BSE crisis. Science 1997; 278: 245-251.
  • 4. Prusiner SB; Neurodegenerative diseases and prions. NEJM 2001; 344:1516-1526.
  • 5. Brown P; Infections of the nervous system. In: Butterworth-Heinemann, Philadelphia. Bradley WG, Daroff RB, Fenichel GM, Marsden CD; Neurology in Clinical Practice 2000:1424-1429.
  • 6. Steinhoff BJ, Raecker S, Herrendorf G et al.; Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt- Jacob Disease. Arch Neurol 1996;53:162-166.
  • 7. Parchi P, Giese A, Capellari S et al.; Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46: 224-233.
  • 8. Zerr I, Pocchiari M, Collins S et al.; Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000; 55:811-815.
  • 9. Poser S, Mollenhauer B, Krauss A, Zerr I, Steinhoff BJ, Schroeter A, Finkenstaedt M, Schulz-Schaeffer WJ, Kretzschmar HA, Felgenhauer K; How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain 1999; 122: 2345-2351.
  • 10. Knopmann DS, DeKosky ST, Cummings JL, Chui H, Corey-Bloom J, Relkin N, Small GW, Miller B, Stevens JC; Practice parameter: Diagnosis of dementia (an evidence- based review ). Neurology 2001; 56: 1143-1153.
  • 11. Zerr I, Bodemer M, Racker S, Groche S, Poser S, Kretzschmar HA, Weber T; Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of CreutzfeldtJakob disease. The Lancet 1995; 345: 1609-1610.
  • 12. Dahbour SS, Ghanmi HA, Taleb AA; Creutzfeldt-Jacob Disease: Cerebrospinal fluid protein 14-3-3 in a Saudi woman. Kuwait Med 2002; 34: 308-312.
  • 13. Draayer YM; Braff SP, Nagle KJ, Pendlebury W, Penar PL, Shapiro RE; Emerging patterns of diffusion-weigted MR imaging in Creutzfeldt-Jakob disease: Case report and review of the literature. AJNR 2002; 23: 550-556.
  • 14. Schaefer PW, Ellen Grant P, Gilberto Gonzales R; Diffusion-weigted MR imaging of the brain. Radiology 2000; 217: 331-345.
  • 15. Sellars RJ, Collie DA, Will RJ; Progress in understanding Creutzfeldt-Jakob disease. AJNR 2002; 23: 1070- 1072.
  • 16. Seipelt M, Zerr I, Nau R, Mollenhauer B, Kropp S, Steinhoff BJ, Wilhelm-Gössling C, Bamberg C, Janzen RWC, Berlit P, Manz F, Felgenhauer K, Poser S; Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease. JNNP 1999; 66: 172-176.
  • 17. Samman I, Schulz-Schaeffer WJ, Wöhrle JC, Sommer A, Kretzschmar HA, Hennerici M; Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2. JNNP 1999; 67: 678-681.
  • 18. Hsich G, Kenney K, Gibbs CJ, Lee KH, Harrington MG; The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. NEJM 1996; 335: 924-930.
  • 19. Keohane C; Pulvinar sign on MRI images in variant Creutzfeldt-Jakob disease.The Lancet 2000; 355, (9213): 1384.
  • 20. Bahn MM, Parchi P; Abnormal diffusion-weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 1999;56:577-583.
  • 21. Mittal S, Farmer P, Kalina P, Kingsley PB, Halperin J; Correlation of diffusion- weighted magnetic resonance imaging with neuropathology in Creutzfeldt- Jakob disease. Arch Neurol 2002;59:128-134.
  • 22. Schröter A, Zerr I, Henkel K, Tschampa HJ, Finkenstaedt M, Poser S; Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 2000;57:1751-1757.
  • 23. Sellars RJ, Collie DA, Will RJ; Progress in understanding Creutzfeldt-Jakob disease. Am Neuroradiol 2002; 23: 1070-1072.
  • 24. Prabhakar S, Bhatia R; Diagnosis of Creutzfeldt-Jacob disease. Neurology India 2001;49:325-328.
  • 25. Brown P, Preece M, Brandel J-P, Sato T, McShane L, Zerr I, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000;55:1075-1081.
  • 26. World Health Organization. Infection control guidelines for transmissible spongiform encephalopathies: report of a WHO Consultation. Geneva: WHO, 1999.
There are 26 citations in total.

Details

Primary Language Turkish
Journal Section Olgu Sunumları
Authors

Nurhak Demir This is me

Belgin Mutluay This is me

Musa Öztürk This is me

Yavuz Altunkaynak This is me

Vedat Sözmen This is me

Hayriye Küçükoğlu This is me

Ayten Dirican This is me

Sevim Baybaş This is me

Publication Date July 18, 2014
Published in Issue Year 2006 Volume: 37 Issue: 2

Cite

APA Demir, N., Mutluay, B., Öztürk, M., Altunkaynak, Y., et al. (2014). Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu. Cerrahpaşa Tıp Dergisi, 37(2), 58-63.
AMA Demir N, Mutluay B, Öztürk M, Altunkaynak Y, Sözmen V, Küçükoğlu H, Dirican A, Baybaş S. Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu. Cerrahpaşa Tıp Dergisi. July 2014;37(2):58-63.
Chicago Demir, Nurhak, Belgin Mutluay, Musa Öztürk, Yavuz Altunkaynak, Vedat Sözmen, Hayriye Küçükoğlu, Ayten Dirican, and Sevim Baybaş. “Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu”. Cerrahpaşa Tıp Dergisi 37, no. 2 (July 2014): 58-63.
EndNote Demir N, Mutluay B, Öztürk M, Altunkaynak Y, Sözmen V, Küçükoğlu H, Dirican A, Baybaş S (July 1, 2014) Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu. Cerrahpaşa Tıp Dergisi 37 2 58–63.
IEEE N. Demir, B. Mutluay, M. Öztürk, Y. Altunkaynak, V. Sözmen, H. Küçükoğlu, A. Dirican, and S. Baybaş, “Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu”, Cerrahpaşa Tıp Dergisi, vol. 37, no. 2, pp. 58–63, 2014.
ISNAD Demir, Nurhak et al. “Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu”. Cerrahpaşa Tıp Dergisi 37/2 (July 2014), 58-63.
JAMA Demir N, Mutluay B, Öztürk M, Altunkaynak Y, Sözmen V, Küçükoğlu H, Dirican A, Baybaş S. Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu. Cerrahpaşa Tıp Dergisi. 2014;37:58–63.
MLA Demir, Nurhak et al. “Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu”. Cerrahpaşa Tıp Dergisi, vol. 37, no. 2, 2014, pp. 58-63.
Vancouver Demir N, Mutluay B, Öztürk M, Altunkaynak Y, Sözmen V, Küçükoğlu H, Dirican A, Baybaş S. Sporadic Creutzfeldt-Jacob Hastalığı: 5 Olgu Sunumu. Cerrahpaşa Tıp Dergisi. 2014;37(2):58-63.