Creutzfeldt‐Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering
- 1 February 1979
- journal article
- research article
- Published by Wiley in Annals of Neurology
- Vol. 5 (2), 177-188
- https://doi.org/10.1002/ana.410050212
Abstract
The worldwide epidemiology of Creutzfeldt‐Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal‐spatial clustering of cases was not found in the United States, but reports from other countries indicate that such clustering does occur. Fifteen percent of the cases were of the familial type, suggesting a genetic susceptibility to infection. Iatrogenic transmission by corneal transplantation and neurosurgical operations has occurred, and the possibility is raised that previous surgery or preexisting neurological disease may be associated with an increased risk of developing CJD. It remains to be determined whether the virus of CJD is maintained only by patient‐to‐patient transmission, has a zoonotic reservoir such as scrapie, or causes widespread latent infection of man that is occasionally activated.This publication has 21 references indexed in Scilit:
- Creutzfeldt‐Jakob disease in France: I. Retrospective study of the Paris area during the ten‐year period 1968–1977Annals of Neurology, 1979
- Incidence of Creutzfeldt-Jakob Disease in Brooklyn and Staten IslandThe New England Journal of Medicine, 1978
- Precautions in Medical Care of, and in Handling Materials from, Patients with Transmissible Virus Dementia (Creutzfeldt-Jakob Disease)The New England Journal of Medicine, 1977
- CLUSTER OF CREUTZFELDT-JAKOB DISEASE AND PRESENILE DEMENTIAThe Lancet, 1977
- DANGER OF ACCIDENTAL PERSON-TO-PERSON TRANSMISSION OF CREUTZFELDT-JAKOB DISEASE BY SURGERYThe Lancet, 1977
- A TRANSMISSIBLE SUBACUTE SPONGIFORM ENCEPHALOPATHY IN A VISITOR TO THE EASTERN HIGHLANDS OF NEW GUINEABrain, 1976
- Creutzfeldt-Jakob Disease: Focus among Libyan Jews in IsraelScience, 1974
- Unusual Electroencephalographic and Clinical Evolution of a Case of Meningioma of the Left Temporo-Occipital ConvexityEuropean Neurology, 1971
- SUBACUTE SPONGIFORM ENCEPHALOPATHY—A SUBACUTE FORM OF ENCEPHALOPATHY ATTRIBUTABLE TO VASCULAR DYSFUNCTION (SPONGIFORM CEREBRAL ATROPHY)Brain, 1960
- Klinische und anatomische Untersuchungen über eine eigenartige organische Erkrankung des Zentralnervensystems im PraeseniumZeitschrift für die gesamte Neurologie und Psychiatrie, 1929