Summary
It was difficult to make a definite pathological diagnosis in a 73-year-old man with Creutzfeldt-Jakob disease (CJD) due to extensive amyloid angiopathy which lacked any severe spongiform changes. Immunostaining using anti-prion protein (PrP) antibody revealed fine granular deposits in the gray matter, after hydrolytic autoclaving pretreatment on tissue sections. Western blotting also revealed an abnormal isoform of PrP, but PrP gene analysis did not show any abnormalities. The primary transmission experiments were repeated three times and induced spongiform encephalopathy in a few mice after a long incubation period.
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Adam J, Crow TJ, Duchen LW, Scaravilli F, Spokes E (1982) Familial cerebral amyloidosis and spongiform encephalopathy. J Neurol Neurosurg Psychiatry 45:37–45
Allsop D, Ikeda S, Bruce ME, Glenner GG (1988) Cerebrovascular amyloid in scrapie-affected sheep reacts with antibodies to prion protein. Neurosci Lett 92:234–239
Bruce ME, McBride PA, Farquhar CF (1989) Precise targeting of the pathology of the sialoglycoprotein, PrP and vacuolar degeneration in mouse scrapie. Neurosci Lett 102:1–6
DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB (1987) Changes in the localization of brain prion proteins during scrapie infection. Neurology 37:1271–1280
Doh-ura K, Tateishi J, Sasaki Y, Kitamoto T, Sakaki H (1989) Pro → Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome. Biochem Biophys Res Commun 163:974–979
Doh-ura K, Tateishi J, Kitamoto T, Sasaki H, Sakaki Y (1990) Creutzfeldt-Jakob disease patients with Congophilic kuru plaques have the missense variant prion protein common to Gerstmann-Sträussler syndrome. Ann Neurol 27:121–126
Gilmour JS, Bruce ME, McKellar A (1985) Cerebrovascular amyloidosis in scrapie-affected sheep. Neuropathol Appl Neurobiol 11:173–183
Hsiao K, Prusiner SB (1990) Inherited human prion disease. Neurology 40:1820–1827
Hsiao K, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250:1587–1590
Keohane C, Peatfield R, Duchen LW (1985) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease) with amyloid angiopathy. J Neurol Neurosurg Psychiatry 48:1175–1178
Kitamoto T, Tateishi J (1988) Immunohistochemical confirmation of Creutzfeldt-Jakob disease with a long clinical course with amyloid plaque core antibodies. Am J Pathol 131:435–443
Kitamoto T, Ogomori K, Tateishi J, Prusiner SB (1987) Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids. Lab Invest 57:230–236
Kitamoto T, Tateishi J, Sawa H, Doh-ura K (1989) Positive transmission of Creutzfeldt-Jakob disease verified by murine kuru plaques. Lab Invest 60:507–512
Kitamoto T, Muramoto T, Mohri S, Doh-ura K, Tateishi J (1991) Abnormal isoform of prion protein accumulated in follicular dendritic cells in mice with Creutzfeldt-Jakob disease. J Virol 65:6292–6295
Klatzo J, Gajdusek DC, Zigas V (1959) Pathology of kuru. Lab Invest 8:799–847
Laemmli UK (1970) Cleavage of structural proteins during assembly of the head bacteriophage T4. Nature 235:383–386
Nieto A, Goldfarb LG, Brown P, McCombie WR, Trapp S, Asher DM, Gajdusek DC (1991) Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families. Lancet 337:622–623
Piccardo P, Safar H, Ceroni M, Gajdusek DC, Gibbs CJ Jr (1990) Immunohistochemical localization of prion protein in spongiform encephalopathies and normal brain tissue. Neurology 40:518–522
Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216:136–144
Roberts GW, Lofthouse R, Allsop D, Landon M, Kidd M, Prusiner SB, Crow TO (1988) CNS amyloid proteins in neurodegenerative diseases. Neurology 38:1534–1540
Schlote W (1965) Die Amyloidnatur der kongophilen drusigen Entartung der Hirnarterien (Scholz) im Senium. Acta Neuropathol (Berl) 4:449–468
Takeda T, Hosokawa M, Takeshita S, et al (1981) A new murine model of accelerated senescence. Mech Ageing Dev 17:183–194
Tateishi J, Sato Y, Koga M, Doi H, Ohta M (1980) Experimental transmission of human subacute spongiform encephalopathy to small rodents. I. clinical and histological observations. Acta Neuropathol (Berl) 51:127–134
Tateishi J, Nagara H, Hikita K, Sato Y (1984) Amyloid plaques in the brains of mice with Creutzfeldt-Jakob disease. Ann Neurol 15:278–280
Towbin H, Staehelin T, Gordon J (1979) Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications. Proc Natl Acad Sci USA 76:4350–4354
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Supported by grants from the Ministry of Education, Science and Culture, and the Science and Technology Agency, Japan
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Tateishi, J., Kitamoto, T., Doh-ura, K. et al. Creutzfeldt-Jakob disease with amyloid angiopathy: diagnosis by immunological analyses and transmission experiments. Acta Neuropathol 83, 559–563 (1992). https://doi.org/10.1007/BF00310037
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DOI: https://doi.org/10.1007/BF00310037