Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering.

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 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.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Masters CL,Harris JO,Gajdusek DC,Gibbs CJ Jr,Bernoulli C,Asher DM

doi

10.1002/ana.410050212

subject

Has Abstract

pub_date

1979-02-01 00:00:00

pages

177-88

issue

2

eissn

0364-5134

issn

1531-8249

journal_volume

5

pub_type

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