Incidence of Creutzfeldt-Jakob disease in Taiwan: a prospective 10-year surveillance

Abstract

This study was performed to estimate the incidence of Creutzfeldt-Jakob Disease (CJD) in Taiwan from 1998 to 2007. Suspected cases of CJD were reported to the Taiwan Creutzfeldt-Jakob Disease Surveillance Unit, a nationwide, hospital-based case report system initiated since 1996 to prospectively conduct a CJD epidemiological study. Consecutive patients who met the diagnostic criteria recommended by the World Health Organization were enrolled. The clinical information of each suspected case was collected and case ascertainment was performed by an expert committee. A total of 123 sporadic CJD were identified without any iatrogenic or new variant CJD cases. The overall annual incidence rate (95% CI) was 0.55 (0.46-0.65) cases per million person-year. There was no statistically significant difference between the calendar year of disease onset (P = 0.97). The incidence rates were not significantly different between women and men (P = 0.63). Age was the main factor for the risk of CJD (P < 0.0001). Age-specific incidence rate increased after the age of 40 years with the peak being in the 70-79 years age group. Our data showed low annual incidence rate and high frequency of methionine homozygous prion protein genotype of sCJD in Taiwan. This report provided important epidemiological data on ethnic Chinese.

Probable variant Creutzfeldt–Jakob disease in Asia: A case report from Taiwan and review of two prior cases

“…According to CJD surveillance data in Taiwan, 123 patients of sCJD were identified from 1998 to 2007, including 54 possible cases, 68 probable cases and one definite case without any iatrogenic or vCJD cases. 9 The annual incidence of sCJD (probable and definite cases included) was 0.31 (95% confidence interval 0.12-0.50) cases/million person-years, which was slightly lower than the incidence of 0.5-1.0 cases/million person-years worldwide. 8 The clinical features of this patient are consistent with the cases of vCJD identified in the UK and France, 10 including psychiatric manifestations at onset, painful sensory symptoms, delayed occurrence of neurological signs, ataxia and dementia (Table 1).…”

“…According to CJD surveillance data in Taiwan, 123 patients of sCJD were identified from 1998 to 2007, including 54 possible cases, 68 probable cases and one definite case without any iatrogenic or vCJD cases 9 . The annual incidence of sCJD (probable and definite cases included) was 0.31 (95% confidence interval 0.12–0.50) cases/million person‐years, which was slightly lower than the incidence of 0.5–1.0 cases/million person‐years worldwide 8…”

Characteristics of Korean patients with suspected Creutzfeldt-Jakob disease with 14-3-3 protein in cerebrospinal fluid: Preliminary study of the Korean Creutzfeldt-Jakob disease active surveillance program

“…15 In Taiwan, the overall annual incidence rate was 0.55 cases per million. 16 Our estimated annual occurrence of 0.33 cases per million is still low, however there was a tendency that the annual incidence rates of Asian countries were lower than those of European countries or worldwide incidences. Although, our surveillance program might not be perfectly recognized all the corresponding clinical cases, we suggest that our results are still reportable and comparable to Asian epidemiological data.…”

Neuroepidemiology and the epidemiology of viral infections of the nervous system

“…sCJD is uncommon, and occurs worldwide, with an incidence of about 1 per million population per year in North America and Europe ( Brown and Mastrianni, 2010 ). The incidence may be slightly less in Asia ( Shi et al, 2008 , Lu et al, 2010 , Nozaki et al, 2010 ), and little is known about the epidemiology of sCJD in sub-Saharan Africa. sCJD is an illness of advanced age, with a mean age at onset of approximately 62 years; the age-adjusted incidence of sCJD in persons 65 years and older is approximately 5 per million per year.…”