Abstract
Familial occurrence of three definitive and two possible cases of Creutzfeldt-Jakob disease (CJD) with temporal and spatial separation in the area of focal CJD accumulation in Slovakia is reported. Incubation period is 51 and 53 years respectively, if spatial and temporal separation of affected siblings is considered, and 51 years when the time interval between the death of the affected mother and the clinical onset in the first affected child is determined.
Affected children tend to die at the same time (mean difference 3.3 years) and not at the same age (mean difference 6 years). Due to separation of the affected children, a possible common exposure to CID infection was limited to approximately seven years during their childhood. Potential endo- and exogenous risk factors and a possible mode of CJD transmission in the described family, as well as in the CJD focus, is discussed.
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AkaiJ., Ishihara0. and HiguchiS. (1989): Creutzfeldt-Jakob disease in Japan: An epidemiological study done in a select prefecture between 1976 and 1976. - Neuroepidem. 8: 32–37.
Barnoulli C., Seigfried J., Baumgartner G. et al. (1977): Danger of accidental person-to-person transmission of CID by surgery. - Lancet i 478–479.
BrownP. (1980): An epidemiological critique of Creutzfekdt - Jakob disease. - Reviews 2: 113–145.
BrownP., GajdusekD.C. and GibbsJ. (1985): Potential epidemic of CJD from huma growth hormone therapy - New Engl. J. Med. 313: 728–731.
Brown P., Cathala F., Raubertas R.F.et al. (1987): The epidemiology of CID: conclusion of a 15-year investigation in France and a review of the world literature. - Neurology, 37: 895–904.
GajdusekD.C. (1977): Unconventional viruses and the origin and disappearance of kuru. - Science 197: 943–960.
Gajdusek D.C. and Gibbs J. (1968): Slow, latent and temperate virus infections of the CNS In: Infections of the nervous system. - Edit. H. Zimmerman, pp. 254–280.
Galvez S. and Cartier L. (1979): A new familial clustering of CJD in Chile. - Neurochirurg. Vol. XXXVIII.
Guidon G. (1978): Formes familiales de la maladie de Creutzfeldt-Jacob. Thése pour la Doctorat en Médicine. - Université de Rennes.
Haltia K. Kovanen J., Krevelvan H.et al. (1979): Familial CJD. - J. Neurol. Sci., 42: 381–389.
HoltT. and PhillipsJ. (1988): Bovine spongiform encephalopathy. - Brit. M.J. 296: 1581–1582.
Mejt'enyi K. (1975): The familial occurrence of CJD. In: Proc. of the Seventh Congr. Neuropathol., Ed. Környex. - Amsterdam, Excepta Med. 362: 75–78.
Masters C., Harris J., Gajdusek CD. et al. (1979): CJD: patterns of worldwide occurrence. In: Slow transmissible diseases of the nervous system. - Ed. Prusiner S. and Hadlow W. - Acad. Press. NY, 113–142.
MastersC., GajdusekC.D. and GibbsJ. (1981): The familial occurrence of CJD and Alzheimer's disease. -Brain 104: 535–558.
MayerV., OrolinD. and MitrováE. (1977): Cluster of CJD and other presenile dementias - Lancet 2: 256.
MayerV., MitrováE. and OrolinD. (1979): CJD in Czechoslovakia and working concept of its surveillance. In: Slow transmissible diseases of the nervous system, Ed. Prusiner S. and Hadlow W. -Acad. Press. NY, 287–303.
Mitrová E. (1980): Focal accumulation of CJD in Slovakia. In: Search for the cause of MS and other chronic CNS diseases. - Ed. Boese J., Frankfurt, 356–366.
Mitrová E. (1986): Epidemiological analysis of CJD in Slovakia (1972–1985). In: Unconventional virus diseases of the CNS. Edit. Court L., Dormont D., Brown P., 19–29.
MitrováE. and MayerV. (1985): Descriptive epidemiology of CJD in Slovakia, J. Neurol. suppl. 232: 71.
Mitrová E., Czuhajová L., Gajdoš E. et al. (1986): Analysis of familial CJD in Slovakia. - Bratisl. lek. listy 86: 202–210.
Roberts D.F. (1985): A definition of genetic epidemiology. In: Diseases of Complex Etiology in Small Populations: Ethnic Differences and Research Approaches. - Edit. Alan R. Liss, 9–20.
Sládkovič A. (1853): Detvan, Almanach “NITRAN”, 1–35.
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Mitrova, E., Lowenthal, A. & Appeal, B. Familial Creutzfeldt-Jakob disease with temporal and spatial separation of affected members. Eur J Epidemiol 6, 233–238 (1990). https://doi.org/10.1007/BF00150424
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DOI: https://doi.org/10.1007/BF00150424