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A retrospective study of Creutzfeldt-Jakob disease in Italy (1972–1986)

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Abstract

In a retrospective study of Creutzfeldt-Jakob disease (CJD) in Italy from 1972 to 1986, we found 79 cases which fulfilled the diagnostic criteria for CJD. The annual mortality rate was 0.09 cases per million inhabitants. In this series the female to male ratio was 2.59, a value significantly higher than that found in Italian population (1.05). The mean age at death was 62.1 ± 9.4 years and the mean duration of the disease was 5.3 ± 3.0 months. No familial cases of CJD were found in our series. Mental deterioration was present in all of our cases, myoclonus in 85% and the other clinical signs were present at a lower rate. Periodic EEG activity was found in 92% of the cases. Two patients had had neurological or ophthalmic surgery and 17% of our cases had undergone general surgery within 5 years prior to the clinical onset of CJD.

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References

  1. Alemà G. (1971): Aspectos epidemiologicos de la enfermedad de Jakob-Creutzfeldt (consideraciones sobre los casos italianos). Proceedings of the 5th World Congress of Psychiatry, R. de la Fuente and M.N. Weisman. - Eds., pp. 1221–1227.

  2. Belloni G.B. and Cortesi M.C. (1970): Contributo alla conoscenza del quadro istopatologico della malattia di Creutzfeldt-Jakob. - Riv. Pat. Nerv. Ment., 91: 69–84.

    Google Scholar 

  3. Bernoulli C., Siegfried J., Baumgartner G., Regli F., Rabinowicz T., Gajdusek D.C. and Gibbs C.J. Jr. (1977): Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery.- Lancet, ii 478–479.

    Google Scholar 

  4. Brown P., Cathala E., Castaigne P. and Gajdusek D.C. (1986): Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. - Ann. Neurol., 20: 597–602.

    Google Scholar 

  5. Brown P., Cathala F. and Gajdusek D.C. (1979): Creutzfeldt-Jakob disease in France: III Epidemiological study of 170 patients dying during the decade 1968–1977. - Ann. Neurol., 6: 438–446.

    Google Scholar 

  6. Brown P., Cathala F., Raubertas R.F., Gajdusek D.C. and Castaigne P. (1987): The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. - Neurology, 37: 895–904.

    Google Scholar 

  7. Brown P., Gajdusek D.C., Gibbs CJ. Jr. and Asher D.M. (1985): Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy. - N. Engl. J. Med., 313: 728–731.

    Google Scholar 

  8. Cathala E., Brown P., LeCanuet P. and Gajdusek D.C. (1985): High incidence of Creutzfeldt-Jakob disease in North African immigrants to France. - Neurology, 35: 894–895.

    Google Scholar 

  9. Duffy P., Wolf J., Collins G., DeVoe A.G., Streeten B. and Cowen D. (1974): Possible person-to-person transmission of Creutzfeldt-Jakob disease. - N. Engl. J. Med., 290: 692–693.

    Google Scholar 

  10. Fattovich G. (1952): La malattia di Jakob-Creutzfeldt (osservazione clinica ed anatomica di un caso). -Cervello, 28: 81–122.

    Google Scholar 

  11. Fieschi C., Orzi F., Pocchiari M., Nardini M., Rocchi F., Asher D.M., Gibbs C.J.Jr. and Gajdusek D.C. (1983): Creutzfeldt-Jakob disease in the province of Siena: two cases transmitted to monkeys. - Ital. J. Neurol. Sci., 1: 61–64.

    Google Scholar 

  12. Gajdusek D.C. (1977): Unconventional viruses and the origin and disappearance of kuru. - Science, 197: 943–960.

    Google Scholar 

  13. Galvez S., Masters C. and Gajdusek D.C. (1980): Descriptive epidemiology of Creutzfeldt-Jakob disease in Chile. - Arch., Neurol., 37: 11–14.

    Google Scholar 

  14. Gibbs C.J. Jr. and Gajdusek D.C. (1973): Experimental Subacute Spongiform Virus Encephalopathies in primates and other laboratory animals. -Science, 182: 67–68.

    Google Scholar 

  15. Kondo K. and Kuroiwa Y. (1982): A case control study of Creutzfeldt-Jakob disease: association with physical injuries. - Ann. Neurol., 11: 377–381.

    Google Scholar 

  16. Lechi A., Tedeschi F., Mancia D., Pietrini Y., Tagliavini F., Terzano M.G. and Trabattoni G. (1983): Creutzfeldt-Jakob disease: clinical, EEG, and neuropathological findings in a cluster of 11 patients.- Ital. J. Neurol. Sci., 1: 47–59.

    Google Scholar 

  17. Macchi G., Abbamondi A.L., DiTrapani G. and Sbriccoli A. (1984): On the white matter lesions of the Creutzfeldt-Jakob disease. - J. Neurol. Sci., 63: 197–206.

    Google Scholar 

  18. Macchi G., Masullo C. and Pocchiari M. (1985): Le demenze trasmissibili. - Federazione Medica, 38: 1156–1160.

    Google Scholar 

  19. Majtenyi C. (1986): Study on the cases of Creutzfeldt-Jakob's disease in Hungary. II Symposium International sur les virus non conventionnels du Systeme Nerveux Central, Paris, December 2–6 (Abstract).

  20. Manuelidis E.E. (1985): Creutzfeldt-Jakob disease. -J. Neuropathol. Exp Neurol., 44: 1–17.

    Google Scholar 

  21. Masters C.L., Harris J.O., Gajdusek D.C., Gibbs C.J. Jr., Bernoulli C. and Asher D.M. (1979): Creutzfeldt-Jakob disease: patterns of world-wide occurrence and the significance of familial and sporadic clustering. -Ann. Neurol., 5: 177–188.

    Google Scholar 

  22. Matthews W.B. (1986): A case control study of Creutzfeldt-Jakob disease. II Symposium International sur les virus non conventionnels du Systeme Nerveux Central, Paris, December 2–6 (Abstract).

  23. Mizutani T., Okumara A., Oda M. and Shiraki H. (1981): Panencephalopathic type of Creutzfeldt-Jakob disease: primary involvement of the cerebral white matter. - J. Neurol. Neurosurg. Psychiatry, 44: 103–115.

    Google Scholar 

  24. Scuccato R., Iannucci A.M., Vio M., Nardelli E., Toffolo G.G., Toso Y. and Rizzuto N. (1978): La malattia di Creutzfeldt-Jakob. Considerazioni su quattro casi. - Riv. Pat. Nerv. Ment., 99: 349–365.

    Google Scholar 

  25. Tabaton M., Bugiani O., Mancardi G.L., Leonardi A., Demartini L. and Primavera A. (1981): Creutzfeldt-Jakob disease in the city and district of Genoa: estimated mortality rate in the six year period 1974–1979.- Ital. J. Neurol. Sci., 2: 189–192.

    Google Scholar 

  26. Tsuji S. and Kuroiwa Y. (1983): Creutzfeldt-Jakob disease in Japan. - Neurology, 33: 1503–1506.

    Google Scholar 

  27. Will R.G. and Matthews W.B. (1984): A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970–1979 1: Clinical features. - J. Neurol. Neurosurg. Psychiatry, 47: 134–140.

    Google Scholar 

  28. Will R.G., Matthews W.B., Smith P.G. and Hudson C. (1986): A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970–1979 II: Epidemiology. - J. Neurol. Neurosurg. Psychiatry, 49: 749–755.

    Google Scholar 

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Authors and Affiliations

  1. Istituto di Neurologia, Universittà Cattolica S. Cuore, Largo F. Vito 1, 00168, Rome, Italy

    C. Masullo, P. Casaccia, A. Iavarone, A. Ladogana & G. Macchi

  2. Dipartimento di Biologia, Università di Lecce, 73100, Lecce, Italy

    M. Pocchiari

  3. Divisione di Neurologia, S. Filippo Neri Hospital, 00135, Rome, Italy

    G. Neri

Authors
  1. C. Masullo
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  2. M. Pocchiari
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  3. G. Neri
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  4. P. Casaccia
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  5. A. Iavarone
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  6. A. Ladogana
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  7. G. Macchi
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Masullo, C., Pocchiari, M., Neri, G. et al. A retrospective study of Creutzfeldt-Jakob disease in Italy (1972–1986). Eur J Epidemiol 4, 482–487 (1988). https://doi.org/10.1007/BF00146403

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