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

Abstract

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.

Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues

“…Survival heterogeneity in sCJD and gTSE warrants particular attention. Our median figures of 5.2 months overall and 4.9 months for sCJD are similar to those described in Europe (Pocchiari et al, 2004) and China (Yang et al, 2020), and lower than those described in Taiwan, 13.5 months (Sun et al, 2020), and in greater detail in Japan, where the mean reached 17.4 months for all TSEs and 15.7 months for sCJD (Nagoshi et al, 2011;Iwasaki et al, 2015). Factors underlying the long survival of Japanese CJD patients are not well known, although some aspects of clinical management of akinetic mutism, such as tube feeding, have been suggested (Iwasaki et al, 2015).…”

“…In the case of TSEs, such a trait would imply that gTSE PrP might act as a transmission agent, a phenomenon suggested by the spatial clustering of high regional sTSE and gTSE incidences close to the Basque Country in Spain ( De Pedro-Cuesta et al, 2021 ). The higher sCJD survival in Taiwan, where only eight genetic cases have been described ( Sun et al, 2020 ) and particular genetic traits have been seen ( Wang et al, 2007 ), merits further study. The fact that survival of gTSE in Spain is equal to that of sCJD, when age at onset, 129 codon polymorphism, and other variables are introduced into models ( Brown and Mastrianni, 2010 ), would support similar survival in sTSE and gTSE forms.…”

“…Knowledge of factors relating to survival might prove helpful in predicting the course of the disease, guiding clinical management and decision-making, and assessing the effectiveness of possible future treatments. To our knowledge, there are few studies on TSE survival ( Puopolo et al, 2003 ; Pocchiari et al, 2004 ; Sanchez-Valle et al, 2004 ; Heinemann et al, 2007 ; Nagoshi et al, 2011 ; Iwasaki et al, 2015 ; Sun et al, 2020 ; Yang et al, 2020 ).…”

Human prion disease surveillance in Spain, 1993-2018: an overview

“…The clinical profile of sCJD in Spain fits well with that of sCJD in EU countries, in terms of both clinical presentation and median survival, 5.0 months [21]. However, sCJD survival in Spain differed from that described for sCJD in Japan [22] and Taiwan [23], where patients with sCJD had around a threefold survival time. In Spain, an extremely atypical definite case with onset at 11 years of age remains aetiologically unexplained.…”

Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients

“…The sCJD prevalence in industrialized countries is estimated to be approximatively 1.5 cases per million people and per year [ 35 ]. In people aged over 50, sCJD frequency significantly increases to reach 5.5 to 7.5 case per million people per year in individuals aged between 65 and 79 [ 36 , 37 ]. However, in France and in the UK (each with a similar population size), between 600,000 and 700,000 deaths are recorded each year, with 80–130 sCJD cases diagnosed annually.…”