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Descriptive Epidemiology of Creutzfeldt-Jakob Disease in Chile

Descriptive Epidemiology of Creutzfeldt-Jakob Disease in Chile Abstract • Descriptive epidemiological data are presented from a survey of 35 cases of Creutzfeldt-Jakob disease (CJD) that occurred in Chile in the period 1955 to 1977. The average annual mortality in Chile (0.31 deaths per 1 million) and in urban Santiago (0.73 deaths per 1 million) is compared with data reported from other countries. An increasing incidence in recent years is probably related to a greater awareness of the disease. The familial occurrence of CJD in nine patients from five affected families is described, and its analysis suggests a genetically determined susceptibility with incubation periods of more than 30 years. Horizontal transmission of the disease may have occurred in one patient in whom the disease developed 13 years after the patient married into a family with seven other affected members. References 1. Masters CL, Harris JO, Gajdusek DC, et al: Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering . Ann Neurol 5:177-188, 1979.Crossref 2. Rojas G, Kase JC: Heidenhain's syndrome: Report of one case . Neurocirugía 27:110-116, 1969. 3. Lagos N, Galvez S, Rojas G: Degeneración cortico-estrio-espinal: Síndrome de Creutzfeldt-Jakob . Neurocirugía 30:5-34, 1972. 4. Cartier L, Galvez S: Intranuclear corpuscles in the neurons of the substantia nigra: Anatomoclinical report of 11 cases of Jakob-Creutzfeldt disease . Neurocirugía 35:5-24, 1977. 5. Masters CL, Richardson EP Jr: Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): The nature and progression of spongiform change . Brain 101:333-344, 1978.Crossref 6. Bobowick AR, Brody JA, Matthews MR, et al: Creutzfeldt-Jakob disease: A case control study . Am J Epidemiol 98:381-394, 1973. 7. Meyendorf R, Förster C: Das klinische Bild idiopathischer psycho-organischer Hirnsyndrome im Präesenium ("Jakob-Creutzfeldt-Syndrom," "spongiöse Encephalopathie") Arch Psychiatr Nervenkr 218:1-40, 1973. 8. Vernon ML, Horta-Barbosa L, Fuccillo DA, et al: Virus-like particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease . Lancet 1:964-967, 1970.Crossref 9. Brown P, Cathala F: Creutzfeldt-Jakob disease in France: I. Retrospective study of the Parisian area during the ten-year period 1967-1977 . Ann Neurol 5:189-192, 1979.Crossref 10. Cathala F, Brown P, Buge A, et al: Épidémiologie de la maladie de Creutzfeldt-Jakob dans la région Parisienne . Rev Neurol 134:741-750, 1978. 11. Traub R, Gajdusek DC, Gibbs CJ Jr: Transmissible virus dementia. The relation of transmissible spongiform encephalopathy to Creutzfeldt-Jakob disease , in Kinsbourne M, Smith M (eds): Aging and Dementia . Flushing, NY, Spectrum Publications, 1977, pp 91-172. 12. Chiofalo N, Fuentes A, Galvez S: Serial electroencephalographic findings in 27 cases of Creutzfeldt-Jakob disease . Arch Neurol to be published. 13. Masters CL, Gajdusek DC, Gibbs CJ Jr, et al: Familial Creutzfeldt-Jakob disease and other familial dementias: An inquiry into possible modes of transmission of virus-induced disease , in Prusiner SB, Hadlow WJ (eds): Slow Transmissible Diseases of the Nervous System . New York, Academic Press, to be published. 14. Kahana E, Alter M, Braham J, et al: Creutzfeldt-Jakob disease: A focus among Libyan Jews in Israel . Science 183:90-91, 1974.Crossref 15. Bernoulli C, Siegfried J, Baumgartner G, et al: Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery . Lancet 1:478-479, 1977.Crossref 16. Duffy P, Wolf J, Collins G, et al: Possible person-to-person transmission of Creutzfeldt-Jakob disease . N Engl J Med 290:692-693, 1974. 17. Gibbs CJ Jr, Gajdusek DC: Atypical viruses as the cause of sporadic, epidemic and familial chronic diseases in man: Slow viruses and human diseases , in Pollard M (ed): Perspectives in Virology . New York, Raven Press, 1978, vol 10, pp 161-198. 18. Gajdusek DC: Unconventional viruses and the origin and disappearance of kuru . Science 197:943-960, 1977.Crossref 19. Nogueira G, Sundblad A, Gomez-Molina O: Creutzfeldt-Jakob disease in Argentina . Arch Neurol 36:181, 1979.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Descriptive Epidemiology of Creutzfeldt-Jakob Disease in Chile

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Publisher
American Medical Association
Copyright
Copyright © 1980 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1980.00500500041004
Publisher site
See Article on Publisher Site

Abstract

Abstract • Descriptive epidemiological data are presented from a survey of 35 cases of Creutzfeldt-Jakob disease (CJD) that occurred in Chile in the period 1955 to 1977. The average annual mortality in Chile (0.31 deaths per 1 million) and in urban Santiago (0.73 deaths per 1 million) is compared with data reported from other countries. An increasing incidence in recent years is probably related to a greater awareness of the disease. The familial occurrence of CJD in nine patients from five affected families is described, and its analysis suggests a genetically determined susceptibility with incubation periods of more than 30 years. Horizontal transmission of the disease may have occurred in one patient in whom the disease developed 13 years after the patient married into a family with seven other affected members. References 1. Masters CL, Harris JO, Gajdusek DC, et al: Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering . Ann Neurol 5:177-188, 1979.Crossref 2. Rojas G, Kase JC: Heidenhain's syndrome: Report of one case . Neurocirugía 27:110-116, 1969. 3. Lagos N, Galvez S, Rojas G: Degeneración cortico-estrio-espinal: Síndrome de Creutzfeldt-Jakob . Neurocirugía 30:5-34, 1972. 4. Cartier L, Galvez S: Intranuclear corpuscles in the neurons of the substantia nigra: Anatomoclinical report of 11 cases of Jakob-Creutzfeldt disease . Neurocirugía 35:5-24, 1977. 5. Masters CL, Richardson EP Jr: Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): The nature and progression of spongiform change . Brain 101:333-344, 1978.Crossref 6. Bobowick AR, Brody JA, Matthews MR, et al: Creutzfeldt-Jakob disease: A case control study . Am J Epidemiol 98:381-394, 1973. 7. Meyendorf R, Förster C: Das klinische Bild idiopathischer psycho-organischer Hirnsyndrome im Präesenium ("Jakob-Creutzfeldt-Syndrom," "spongiöse Encephalopathie") Arch Psychiatr Nervenkr 218:1-40, 1973. 8. Vernon ML, Horta-Barbosa L, Fuccillo DA, et al: Virus-like particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease . Lancet 1:964-967, 1970.Crossref 9. Brown P, Cathala F: Creutzfeldt-Jakob disease in France: I. Retrospective study of the Parisian area during the ten-year period 1967-1977 . Ann Neurol 5:189-192, 1979.Crossref 10. Cathala F, Brown P, Buge A, et al: Épidémiologie de la maladie de Creutzfeldt-Jakob dans la région Parisienne . Rev Neurol 134:741-750, 1978. 11. Traub R, Gajdusek DC, Gibbs CJ Jr: Transmissible virus dementia. The relation of transmissible spongiform encephalopathy to Creutzfeldt-Jakob disease , in Kinsbourne M, Smith M (eds): Aging and Dementia . Flushing, NY, Spectrum Publications, 1977, pp 91-172. 12. Chiofalo N, Fuentes A, Galvez S: Serial electroencephalographic findings in 27 cases of Creutzfeldt-Jakob disease . Arch Neurol to be published. 13. Masters CL, Gajdusek DC, Gibbs CJ Jr, et al: Familial Creutzfeldt-Jakob disease and other familial dementias: An inquiry into possible modes of transmission of virus-induced disease , in Prusiner SB, Hadlow WJ (eds): Slow Transmissible Diseases of the Nervous System . New York, Academic Press, to be published. 14. Kahana E, Alter M, Braham J, et al: Creutzfeldt-Jakob disease: A focus among Libyan Jews in Israel . Science 183:90-91, 1974.Crossref 15. Bernoulli C, Siegfried J, Baumgartner G, et al: Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery . Lancet 1:478-479, 1977.Crossref 16. Duffy P, Wolf J, Collins G, et al: Possible person-to-person transmission of Creutzfeldt-Jakob disease . N Engl J Med 290:692-693, 1974. 17. Gibbs CJ Jr, Gajdusek DC: Atypical viruses as the cause of sporadic, epidemic and familial chronic diseases in man: Slow viruses and human diseases , in Pollard M (ed): Perspectives in Virology . New York, Raven Press, 1978, vol 10, pp 161-198. 18. Gajdusek DC: Unconventional viruses and the origin and disappearance of kuru . Science 197:943-960, 1977.Crossref 19. Nogueira G, Sundblad A, Gomez-Molina O: Creutzfeldt-Jakob disease in Argentina . Arch Neurol 36:181, 1979.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Jan 1, 1980

References