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

Yang, Chih Wen; Fuh, Jong Ling; Wang, Shuu Jiun; Lirng, Jiing Feng; Yang, Wen-Chi; Cheng, Shih‐Jung

doi:10.1111/j.1440-1819.2010.02151.x

Cited by 5 publications

(7 citation statements)

References 19 publications

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“…The patient developed akinetic mutism at 16 months and died 28 months after onset. 19 A positive CSF 14-3-3 protein test was found in 66.7% (2/3) of definite cases, 67.0% (183/275) of probable cases, and 20% (5/25) of possible cases. The CSF 14-3-3 protein test's sensitivity was 63%, and the specificity was 79.6%, which were calculated from the data after 2011 because the laboratory records of the excluded cases before then were incomplete.…”

Section: Diagnostic Classification and Genetic And Laboratory Studiesmentioning

confidence: 94%

<p>Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017</p>

Sun¹,

Liu²,

Fan³

et al. 2020

CLEP

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Introduction: Epidemiologic studies of Creutzfeldt-Jakob disease (CJD) have been undertaken worldwide since the new variant CJD outbreak in 1996 in the United Kingdom. A nationwide report system, the Creutzfeldt-Jakob Disease Surveillance Unit (CJDSU), directed by the Centers for Disease Control of Taiwan, was established in 1997 to identify human prion diseases. Methods: From 1998 to 2017, 647 cases were referred to the committee for confirmation. The report to CJDSU included a structured questionnaire recording the clinical, demographic data, and potential iatrogenic exposure, and the results of the clinical and laboratory examination, including tests of blood and cerebrospinal fluid, electroencephalography, and brain magnetic resonance imaging. Results: In total, 356 cases (women, n=178) were ascertained to be human prion diseases, and 97.4% (n=347) were sporadic CJD, including three definite, 314 probable, and 30 possible cases; one probable variant CJD and 8 cases of the genetic form human prion diseases. The age-and gender-specific average annual incidence were also significantly higher in the second decade (0.95/1,000,000) than in the first decade (0.63/1,000,000), with an incidence rate ratio of 1.51. The incidences increased with increasing age, reaching a peak at the age of 70-79 years. The 10-year survival curve for sCJD patients showed that the 1-, 5-, and 10-year cumulative survival rate were 52%, 5%, and 1%, respectively. PRNP polymorphisms in 170 patients showed that 98.8% were M129M and 97.6% E219E. Discussion: The significant increase in incidence after 2008 suggests the increase in the awareness of this rare disease among physicians. The longer disease duration in patients with sCJD in Taiwan than in other countries indicates that the comprehensive support of the health care system, as well as the end-of-life care culture in Taiwan, may prolong survival time in patients with such a progressive and fatal disease.

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Section: Diagnostic Classification and Genetic And Laboratory Studiesmentioning

confidence: 94%

<p>Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017</p>

Sun¹,

Liu²,

Fan³

et al. 2020

CLEP

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“…This potential relationship is shown by the peak of the number of deaths from vCJD in France in 2005, five years after a similar peak in the number of deaths occurred in the UK [ 78 , 79 ]. Additionally, three East Asian vCJD cases has been identified in Hong Kong SAR [ 80 ], Japan [ 81 , 82 ], and China-Taiwan [ 83 ]. All three are assumed to be imported cases from the UK due to patients who either previously resided in or travelled to the UK bringing them in.…”

Section: Epidemiological Characteristics Of Human Prion Diseasesmentioning

confidence: 99%

“…In France, despite a median age of onset is 35 years (range: 18–57), which is higher than in the UK, all other data are similar [ 84 ]. The disease duration in Asians (mean 28.3, range 14–43 months) seems to be longer than in patients in the UK and France [ 83 ]. A further study supports the hypothesis that a single strain of infectious agent is responsible for all vCJD infections [ 85 ].…”

Section: Epidemiological Characteristics Of Human Prion Diseasesmentioning

confidence: 99%

Epidemiological characteristics of human prion diseases

2016

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Human prion diseases are a group of transmissible, progressive, and invariably fatal neurodegenerative disorders, which include Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia. Human prion diseases affect approximately 1–2 persons per million worldwide annually, occurring in sporadic, inherited, and acquired forms. These diseases have attracted both scientific and public attention not only because of their mysterious pathogen, but also due to their considerable threat to public health since the emergence of the variant CJD.There are still no specific therapeutic and prophylactic interventions available for prion diseases, thus active surveillance of human prion diseases is critical for disease control and prevention. Since 1993, CJD surveillance systems have been established in many countries and regions, and several long-term multinational cooperative projects have been conducted.In this paper, the epidemiological characteristics of various human prion diseases and the active surveillance systems pertaining to them in different countries and regions are summarized and reviewed.Electronic supplementary materialThe online version of this article (doi:10.1186/s40249-016-0143-8) contains supplementary material, which is available to authorized users.

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“…For the same reasons, it is not surprising that four of the nine patients with vCJD who resided in countries outside the UK and Western Europe at time of onset—including three of the seven who became ill and/or were diagnosed in Canada and the USA—had resided in the UK for extended periods between 1980 and 1996 (Jansen et al , 2003; Holman et al , 2010; Yang et al , 2010). A fifth case, the only one reported to date in a Japanese resident, was attributed to exposure during a 24-day visit to the UK in early 1990, close to the peak of the human BSE exposure risk there (Yamada and Variant CJD Working Group, Creutzfeldt-Jakob Disease Surveillance Committee, Japan, 2006).…”

Section: Discussionmentioning

confidence: 99%

“…Among the other nine cases, four were linked to residence in the UK between 1980 and 1996 when BSE exposure risk was highest there and globally. One additional case was attributed to a brief UK visit in 1990, and another most likely to residence for more than 6 years each in Kuwait or Russia between 1980 and 1996 (Jansen et al , 2003; Yamada and Variant CJD Working Group, Creutzfeldt-Jakob Disease Surveillance Committee, Japan, 2006; Holman et al , 2010; Yang et al , 2010; Maheshwari et al , 2015). …”

Section: Introductionmentioning

confidence: 99%

A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia

Coulthart

Geschwind

Qureshi

et al. 2016

Brain

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As of mid-2016, 231 cases of variant Creutzfeldt-Jakob disease—the human form of a prion disease of cattle, bovine spongiform encephalopathy—have been reported from 12 countries. With few exceptions, the affected individuals had histories of extended residence in the UK or other Western European countries during the period (1980–96) of maximum global risk for human exposure to bovine spongiform encephalopathy. However, the possibility remains that other geographic foci of human infection exist, identification of which may help to foreshadow the future of the epidemic. We report results of a quantitative analysis of country-specific relative risks of infection for three individuals diagnosed with variant Creutzfeldt-Jakob disease in the USA and Canada. All were born and raised in Saudi Arabia, but had histories of residence and travel in other countries. To calculate country-specific relative probabilities of infection, we aligned each patient’s life history with published estimates of probability distributions of incubation period and age at infection parameters from a UK cohort of 171 variant Creutzfeldt-Jakob disease cases. The distributions were then partitioned into probability density fractions according to time intervals of the patient’s residence and travel history, and the density fractions were combined by country. This calculation was performed for incubation period alone, age at infection alone, and jointly for incubation and age at infection. Country-specific fractions were normalized either to the total density between the individual’s dates of birth and symptom onset (‘lifetime’), or to that between 1980 and 1996, for a total of six combinations of parameter and interval. The country-specific relative probability of infection for Saudi Arabia clearly ranked highest under each of the six combinations of parameter × interval for Patients 1 and 2, with values ranging from 0.572 to 0.998, respectively, for Patient 2 (age at infection × lifetime) and Patient 1 (joint incubation and age at infection × 1980–96). For Patient 3, relative probabilities for Saudi Arabia were not as distinct from those for other countries using the lifetime interval: 0.394, 0.360 and 0.378, respectively, for incubation period, age at infection and jointly for incubation and age at infection. However, for this patient Saudi Arabia clearly ranked highest within the 1980–96 period: 0.859, 0.871 and 0.865, respectively, for incubation period, age at infection and jointly for incubation and age at infection. These findings support the hypothesis that human infection with bovine spongiform encephalopathy occurred in Saudi Arabia.

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

Cited by 5 publications

References 19 publications

<p>Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017</p>

<p>Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017</p>

Epidemiological characteristics of human prion diseases

A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia

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