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PrP amyloid plaques in Creutzfeldt-Jakob disease of short duration: immunohistochemical studies of 5 cases from Poland.

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Abstract

We report here PrP-immunohistochemistry performed on brains from CJD cases from Poland. Only one of five definitive CJD cases exhibited typical PrP-immunoreactive kuru-like plaques and this was case of a short duration. We thus confirm the low frequency of PrP plaques in CJD of Eastern and Central European origin.

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References

  1. AdamsH., BeckE. and ShenkinA.M. (1974): Creutzfeldt-Jakob disease: further similarities with kuru. - J. Neurol. Neurosurg. Psychiatr., 37: 195–200.

    Google Scholar 

  2. AzzarelliB., MullerJ., GhettiB., DykenM. and ConneallyP.M. (1985): Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant) - Acta Neuropathol. (Berl.), 65: 235–246.

    Google Scholar 

  3. BarlowR.M. and ModdletonD.J. (1990): Dietary transmission of bovine spongiform encephalopathy to mice. - Vet. Rec., 126: 111–112.

    Google Scholar 

  4. BeckE. and DanielP.M. (1979): Kuru and Creutzfeldt-Jakob disease: neuropathological lesions and their significance In: Slow Transmissible Diseases of the Nervous System., Vol 1, Edit.: Prusiner S.B. and Hadlow W.J. - Academic Press, New York, 253–270.

    Google Scholar 

  5. BeckE. and DanielP.M. (1987): Neuropathology of transmissible spongiform encephalopathies In: Prions. Novel Infectious Pathogens causing Scrapie and Creutzfeldt-Jakob Disease, Edit.: Prusiner S.B. and McKinley M.P. - Academic Press, New York, 331–386.

    Google Scholar 

  6. BoellaardJ W. and SchloteW. (1980): Subakute spongiforme Encephalopathie mit multiformer Plaquebildung. Eigenartige familiaer-hereditarae Krankenheit des Zentralnervensystems [spinocerebellare Atrophie mit Demenz, Plaques und plaqueaehnlichen im Klein- und Grosshirn (Gerstmann, Sträussler, Scheinker)] - Acta Neuropathol. (Berl), 49: 205–212.

    Google Scholar 

  7. BoellaardJ.W. and SchloteW. (1981): Glial plaques: amyloid deposits characteristic of slow transmissible encephalopathies - Virchows Arch. (Cell Pathol.)., 37: 337–341.

    Google Scholar 

  8. BoellaardJ.W., SchloteW. and HeldtN. (1989): The development of amyloid plaques in human transmissible encephalopathy and the role of astrocytes in their formation. In: Unconventional Virus Diseases of the Central Nervous System, Edit.: Court L.A., Dormont D., Brown P. and Kingsbury D.T., Commissariat a' 1'Energie Atomique, Paris, 162–171.

    Google Scholar 

  9. BrownP. (1989): Central nervous system amyloidosis. A comparison of Alzheimer's disease and Creutzfeldt-Jakob disease - Neurology, 39: 1103–1105.

    Google Scholar 

  10. Brown P. (1989): The relevance of Creutzfeldt-Jakob disease to the study of Alzheimer's Disease. In: Alzheimer's Disease and Related Disorders. Edit.: Iqbal K., Wisniewski H.M. and Winblad B., Alan R. -Liss Inc., 535–548.

  11. BrownP., Goldfarb.L.G., BrownW.T., GolgaberD., RubensteinR., KascakR.J., GuiroyD.C., PiccardoP., BoellaardJ.W. and GajdusekD.C. (1991) Clinical and molecular genetic study of a large German kindred with Gerstmann-Sträussler-Scheinker syndrome -Neurology, 41: 375–379.

    Google Scholar 

  12. BrownP., LiberskiP.P., WolffA. and GajdusekD.C. (1990): Conservation of infectivity in purified fibrillary extracts of scrapie-infected hamster brain after sequential enzymatic digestion or polyacrylamide gel electrophoresis - Proc. Natl. Acad. Sci. USA., 87: 7240–7244.

    Google Scholar 

  13. ChouS.M. and MartinJ.D. (1971): Kuru-plaques in a case of Creutzfeldt-Jakob disease - Acta Neuropathol. (Berl.), 17: 150–155.

    Google Scholar 

  14. DeArmondS.J., McKinleyM.P., BarryR.A., BraunfeldMR, McCollochJ.R. and PrusinerS.B. (1985): Identification of prion amyloid filaments in scrapie-infected brain - Cell, 41: 221–235.

    Google Scholar 

  15. deCourten-MyersG. and MandyburT.I. (1987): Atypical Gerstmann-Straussler syndrome or familial ataxia and Alzheimer's disease? Neuroloy, 37: 269–275.

    Google Scholar 

  16. FarlowM.R., YeeR.D., DlouhyS.R., ConneallyP.M., AzzarelliB. and GhettiB. (1989): GerstmannSträussler-Scheinker disease. I. Extending the clinical spectrum - Neurology, 39: 1446–1452.

    Google Scholar 

  17. Gajdusek D.C. and Gibbs C.J. Jr. (1990): Brain amyloidoses. Precursor proteins and the amyloids of transmissible and non transmissible dementias: scrapie-kuru-cjd viruses as infectious polypeptides or amyloid enhancing factors. In: Biomedical Advances in Aging. Edit.: Goldstein A.L. - Plenum Publ Corp, 3–24.

  18. GerstmannJ., SträusslerE. and ScheinkerI. (1936): Uber eine eigenartige hereditear-familiaere Erkrankung des Zentralnervensystems. Zugleich ein Beitrag zur Frage des vorzeitigen lokalen Alterns. Z. ges. Neurol. Psychiat., 154: 736–762.

    Google Scholar 

  19. GhettiB., TagliaviniF., MastersC.L., BeyreutherK., GiacconeG., VergaL., FarlowM.R., ConneallyP.M., DlouhyS.R., AzzarelliB. and BugianiO. (1989): Gerstmann-Straussler-Scheinker disease. II. Neurofibrillary tangles and plaques coexist in an affected family - Neurology, 39: 1453–1461.

    Google Scholar 

  20. GoldfarbL., BrownP., GoldgaberD., GarrutoR.M., YanagiharaR., AsherD.M. and GajdusekD.C. (1990): Identical mutation in unrelated patients with Creutzfeldt-Jakob disease - Lancet, 336: 174–175.

    Google Scholar 

  21. GoldfarbL.G., MitrovaE., BrownP., TohB.H. and GajdusekD.C. (1990): Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia - Lancet, 336: 514

    Google Scholar 

  22. GoldfarbL.G., KorczynA.D., BrownP., ChapmanJ. and GajdusekD.C. (1990): Mutation on codon 200 of scrapie amyloid protein gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan origin and non-Libyan origin - Lancet, 336: 637.

    Google Scholar 

  23. GoldgaberD., GoldfarbL., BrownP., AsherD., BrownT., LinS., TeenerJ.W., FeinstoneS.M., RubensteinR., KascakR., BollaardJ.W. and GajdusekD.C. (1989): Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker syndrome - Exp. Neurol., 106: 204–206.

    Google Scholar 

  24. GuiroyD.C., WilliamsE., LiberskiP.P. and YanagiharaR. (1990): Amyloid plaques of chronic wasting disease in captive mule deer, hybrids of mule deer and white-tailed deer, Rocky Mountain elk, and experimental scrapie in hamsters are immunoreactive to scrapie-associated fibrils/ protease-resistant protein and contain sulfated glycosaminoglycans - Ann. Neurol., 28: 290–291.

    Google Scholar 

  25. HayekJ. and UlrichJ. (1975): Kuru-plaques in Creutzfeldt-Jakob disease - Eur. Neurol., 13: 251–257.

    Google Scholar 

  26. HiranoT., TsuchiyamaH., Kawai. and MoriK.K. (1977): An autopsy case of Creutzfeldt-Jakob disease with kuru-like neuropathological changes - Acta Pathol. Jpn., 27: 231–238.

    Google Scholar 

  27. HoroupianD.S., PowersJ.M. and SchaumburgH.H. (1972): Kuru-like neuropathological changes in a North American - Arch. Neurol., 27: 555–561.

    Google Scholar 

  28. HsiaoK., Doh-UraK., KitamotoT., TateishiJ. and PrusinerS.B. (1989): A prion protein amino acid substitution in ataxic Gerstmann-Straussler syndrome - Ann. Neurol., 26: 106.

    Google Scholar 

  29. HsiaoK., BakerH.E., CrowT.J., PoulterM., OwenF., TerwilligerJ.D., WestawayD., OttJ. and PrusinerS.B. (1989): Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome - Nature, 338: 342–345.

    Google Scholar 

  30. HsiaoK., ScottM., FosterD., GrothD.F., DeArmondS.J. and PrusinerS.B. (1990): Spontaneous neurodegeneration in transgenic mice with mutant prion protein - Science, 250: 1587–1590.

    Google Scholar 

  31. HudsonA.J., FarrellM.A., KalninsR. and KaufmannC.E. (1983): Gerstmann-Sträussler-Scheineker disease with coincidental familial onset - Ann. Neurol., 14: 670–678.

    Google Scholar 

  32. KitamotoT., TateishiJ. and SatoY. (1988): Immunohistochemical verification of senile and kuru plaques in Creutzfeldt-Jakob disease and the allied disorders - Ann. Neurol., 24: 537–542.

    Google Scholar 

  33. KlatzoI. and GajdusekD.C. (1959): Pathology of kuru- Lab. Invest., 8: 799–847.

    Google Scholar 

  34. KlatzoI., GajdusekD.C. and ZigasY. (1959): Evaluation of pathological findings in twelve cases of kuru. In: Encephalitides, Edit.: Van Bogaert L., Radermecker J., Hozay J. and Lewenthal A. -Elsevier Publ. Comp., Amsterdam, 172–190.

    Google Scholar 

  35. KruckeW., BeckE. and VitzthumH. (1973): Creutzfeldt-Jakob disease. Some unusual morphological features reminiscent of kuru - Z. Neurol., 206: 1–24.

    Google Scholar 

  36. KuzuharaS., KanazawaL., SasakiH., NakanishiT. and ShimamuraK. (1983): Gerstmann-Sträussler-Scheinker's disease - Ann. Neurol., 14: 216–225.

    Google Scholar 

  37. LiberskiP.P., PapierzW. and AlwasiakJ. (1987): Creutzfeldt-Jakob disease with plaques and paired helical filaments - Acta Neurol. Scandinav., 76: 428–432.

    Google Scholar 

  38. MastersC.L., GajdusekD.C. and GibbsCJ.Jr. (1981): Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome. With an analysis of the various forms of amyloid plaque deposition in the virus induced spongiform encephalopathies -Brain, 104: 559–588.

    Google Scholar 

  39. MerzP.A., RohwerR.G., KascakR., WisniewskiH.M., SomervilleR.A., GibbsC.J.Jr. and GajdusekD.C. (1984): Infection specific particle from the unconventional slow virus diseases - Science, 225: 437–476.

    Google Scholar 

  40. MerzP.A., SomervilleR.A., WisniewskiH.M., ManuelidisL. and ManuelidisE.E. (1983): Scrapie-associated fibrils in Creutzfeldt-Jakob disease -Nature, 306: 474–476.

    Google Scholar 

  41. MerzP.A., SomervilleR.A., WisniewskiH.M. and IqbalJ. (1981): Abnormal fibrils from scrapie-infected brain - Acta Neuropathol. (Berl.), 60: 63–74.

    Google Scholar 

  42. MizutamiT. (1985): Chronicspongiform encephalopathy with plaques characterized by antecedent and longlasting cerebellar ataxia. In: Clinicopathological Aspects of Creutzfeldt-Jakob Disease Edit.: Mizutami T. and Shiraki H. - Elsevier, Nishimura, Amsterdam, Niigata, 163–175.

    Google Scholar 

  43. NeumanM.A., GajdusekD.C. an ZigasV. (1964): Neuropathologic findings in exotic neurologic disorder among natives of the Highlands of New Guinea - J. Neuropathol. Exp. Neurol., 23: 486–507.

    Google Scholar 

  44. NochlinD., SumiS.M., BirdT.D., SnowA.D., LeventhalC.M., BeyreutherK. and MastersC.L. (1989): Familial dementia with PrP positive amyloid plaques: a variant of Gerstmann-Straussler syndrome -Neurology, 39: 910–918.

    Google Scholar 

  45. OeschB., WestawayD., WalchliiM., McKInleyM.P., KentS.B.H., AebersoldR., BarryR.A., TempstP., TeplowD.B., HoodL.E., PrusinerS.B. and WeissmannC. (1985): A cellular gene encodes scrapie PrP 27–30 protein - Cell, 40: 735–746.

    Google Scholar 

  46. PearlmanR.L., TowfighiJ., PezeshkpourG.H., TenserR.B. and TurelA.P. (1988): Clinical significance of types of cerebellar amyloid plaques in human spongiform encephalopathies - Neurology, 38: 1249–1254.

    Google Scholar 

  47. PiccardoP., SafarJ., CeroniM., GajdusekD.C. and GibbsC.J.Jr. (1990): Immunohistochemical localization of prion protein in spongiform encephalopathies and normal brain tissue -Neurology, 40: 518–522.

    Google Scholar 

  48. PrusinerS.B., McKinleyM.P., BowmanK.A., BoltonD.C, BendheimP.E., GrothD.C. and GlennerG.G. (1983): Scrapie prions aggregate to form amyloid-like birefringent rods - Cell, 35: 349–358.

    Google Scholar 

  49. SchloteW., BoellaardJ.W., SchummF. and StohrM. (1980): Gerstmann-Sträussler-Scheinker's disease. Electron microscopic observations on a brain biopsy- Acta Neuropathol. (Berl.), 52: 203–211.

    Google Scholar 

  50. SeitelbergerF. (1962): Eigenartige familair-hereditare Krankheit des Zetralnervensystems in einer niederosstereichischen Sippe - Wien. Klin. Wochen., 74: 687–691.

    Google Scholar 

  51. TateishiJ., KitamotoT., HashigushiH. and ShiiH. (1988): Gerstmann-Sträussler-Scheinker disease: immunological and experimental studies - Ann. Neurol., 24: 35–40.

    Google Scholar 

  52. VintersH.Y., HudsonA.J. and KaufmannJ.C.E. (1986): Gerstmann-Sträussler-Scheinker disease: autopsy study of a familial case - Ann. Neurol., 20: 540–543.

    Google Scholar 

  53. YagishitaS., IwabuchiK., AmanoN. and YokoiS. (1989): Further observation on Japanese Creutzfeldt-Jakob disease with widespread amyloid plaques - J. Neurol., 236: 145–148

    Google Scholar 

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Liberski, P.P., Kwiecinski, H., Barcikowska, M. et al. PrP amyloid plaques in Creutzfeldt-Jakob disease of short duration: immunohistochemical studies of 5 cases from Poland.. Eur J Epidemiol 7, 505–510 (1991). https://doi.org/10.1007/BF00143130

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