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Abstract

The purpose of the study is to investigate the epidemiology of Creutzfeldt-Jakob disease (CJD) and multiple sclerosis (MS) in Taiwan. Cases of CJD and MS were identified from the National Health Insurance Research Database from January 2000 to December 2005. Age- and sex-specific incidences of these diseases were estimated by dividing the incidence number by population data obtained from the Department of Statistics, Ministry of the Interior. During the study period, 79 cases of CJD, 41 women and 38 men, were identified. The average annual incidence rate was 0.63/million. Most cases were older than 60 years. There were 1,262 cases of MS. The male-to-female ratio was 0.4. The average annual incidence rate was 0.79/100,000. The incidence reached a peak at the age group of 40-60 years for females and at the age group of over 55 years for males. The prevalence of MS was 2.96/100,000 in 2005, which is higher than those of previous studies in Taiwan. The annual incidence rate of CJD in Taiwan is lower than in western countries. The annual incidence rate and prevalence of MS in Taiwan are low. The prevalence of MS in Taiwan increased in recent decades. Copyright 2009 S. Karger AG, Basel.

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Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada.

A Ladogana, M Puopolo, E Croes … (2005)

An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes. Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK. This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.

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Variant Creutzfeldt-Jakob disease.

John Collinge (1999)

It is clear that the prion strain causing bovine spongiform encephalopathy (BSE) in cattle has infected human beings, manifesting itself as a novel human prion disease, variant Creutzfeldt-Jakob disease (CjD). Studies of the incubation periods seen in previous epidemics of human prion disease and of the effect of transmission barriers limiting spread of these diseases between species, suggest that the early variant CJD cases may have been exposed during the preclinical phase of the BSE epidemic. It must therefore be considered that many cases may follow from later exposure in an epidemic that would be expected to evolve over decades. Since the number of people currently incubating this disease is unknown, there are concerns that prions might be transmitted iatrogenically via blood transfusion, tissue donation, and, since prions resist routine sterilisation, contamination of surgical instruments. Such risks remain unquantified. Although variant CJD can be diagnosed during life by tonsil biopsy, a prion-specific blood test is needed to assess and manage this potential threat to public health. The theoretical possibility that BSE prions might have transferred to other species and continue to present a risk to human health cannot be excluded at present.