Summary
In a 53-year-old man with a progressive mental deterioration and myoclonic jerks, brain biopsy failed to show any significant light microscopical findings. Electron microscopy revealed membranebound vacuolar inclusions in many neuronal nuclei as the only prominent finding. Hamsters intracerebrally inoculated with the biopsy material demonstrated typical spongiform changes in the gray structures of the brain when sacrificed on the 309th and 332nd days post inoculation, characteristic of experimental Creutzfeldt-Jakob disease (CJD). These intranuclear vacuolar inclusions, originally reported in experimental Creutzfeldt-Jakob disease in this laboratory, may be a valuable electron microscopic feature in some CJD cases and may play an important role in supporting the diagnosis of CJD.
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References
Cervos-Navarro J, Cruz-Sanchez F, Gimeno-Alava A, Ferszt R (1986) Intranuclear vacuoles in Creutzfeldt-Jakob disease. Neuropathol Appl Neurobiol 12:477–482
David S, Nathaniel EJH (1978) Intranuclear inclusions in the developing neurons of the rat cuneate nuclei. Cell Tissue Res 193:522–532
Dickinson AG, Fraser H (1977) Scrapie. Pathogenesis in inbred mice: An assessment of host control and response involving many strains of mice. In: TerMeulen V, Katz M (eds) Slow virus injections of the central nervous system. Springer New York pp 3–15
Field EJ, Peat A (1971) Intranuclear inclusions in neurons and glia: a study in the aging mouse. Gerontology 17:129–138
Kim JH, Manuelidis EE (1983) Ultrastructural findings in experimental Creutzfeldt-Jakob disease in guinea pigs. J Neuropathol Exp Neurol 42:29–43
Kim JH, Manuelidis EE (1986) Serial ultrastructural study of experimental Creutzfeldt-Jakob disease in guinea pigs. Acta Neuropathol (Berl) 69:81–90
Lampert PW, Gajdusek DC, Gibbs CJ Jr (1972) Subacute spongiform virus encephalopathies. Am J Pathol 68:626–646
Manuelidis EE (1975) Transmission of Creutzfeldt-Jakob disease from man to guinea pig. Science 190:571–572
Manuelidis EE (1985) Presidential Address. Creutzfeldt-Jakob disease. J Neuropathol Exp Neurol 44:1–17
Manuelidis EE, Manuelidis L, Pincus JH, Collins WF (1978) Transmission from man to hamster of Creutzfeldt-Jakob disease with clinical recovery. Lancet II:40–42
Manuelidis EE, Gorgacz EJ, Manuelidis L (1978) Interspecies transmission of Creutzfeldt-Jakob disease to Syrian hamsters with reference to clinical syndromes and strains of agent. Proc Natl Acad Sci USA 75:3432–3436
Manuelidis L, Murdoch G, Manuelidis EE (1988) Potential involvement of retroviral elements in human dementias. In: CIBA Foundation symposium 35. Novel injections agents and the central nervous system. Wiley & Sons Chichester pp 117–134
Masters CL, Richardson EP Jr (1978) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain 101:333–344
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Supported in part by the NIH grants AG 03106 and NS 12674
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Kim, J.H., Lach, B. & Manuelidis, E.E. Creutzfeldt-Jakob disease with intranuclear vacuolar inclusions: a biopsy case of negative light microscopic findings and successful animal transmission. Acta Neuropathol 76, 422–426 (1988). https://doi.org/10.1007/BF00686980
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DOI: https://doi.org/10.1007/BF00686980