A systemic analysis of Creutzfeldt Jakob disease cases in Asia

Abstract

Creutzfeldt Jakob Disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder, also known as a subacute spongiform encephalopathy. There are three major subtypes of CJD i.e. Sporadic CJD, which occurs for reasons unbeknown to science (85% of known cases), Genetic or Familial CJD which is characterized by the presence of mutations in the human prion protein (PRNP) gene (10-15% cases) and Iatrogenic CJD that occurs via accidental transmission through medical and surgical procedures (1-2% cases). CJD cases occur globally with 1 case per one million population/year. Considerable data is available related to the incidence and prevalence of CJD in Europe and America. However, the global surveillance database is yet to include Asia even though several Asian countries have their own CJD monitoring units. sCJD is the highest among all CJD cases in Asia. China (1957) and Japan (1705) have reported more cases of sCJD than any Asian country and Hong Kong (1) has reported the least. On the other hand, gCJD is highest in Japan (370) and least in India (2). Our analysis establishes the presence of all variants of CJD across Asia. However, in most Asian countries in general and Southeast Asian countries in particular, CJD cases are misdiagnosed and often underreported. Since Asia is the most populated continent in the world, the actual global prevalence of CJD cannot be estimated until and unless these countries are accounted for. Concrete and reliable surveillance networks are needed across Asia to evaluate the prevalence and incidence of CJD in the region. [Figure: see text]The graphical abstract demonstrates the prevalence of CJD cases in the world and systematically analyses the incidence of CJD in Asian countries between the year 1986-2022. Highest number of cases were reported in Japan followed by China. The study emphasizes the need for assimilation of Asian data in global prevalence.

Keywords: Asia; Creutzfeldt Jakob disease; genetic CJD; genetic prion diseases; latrogenic CJD; sporadic CJD.

Figure 1.

Variants of CJD and symptoms: the figure briefly describes the variants of CJD – sporadic, genetic, and iatrogenic along with their generalized symptoms.

Figure 2.

Total cases of CJD reported in Asia from 1986 to 2023. this graph indicates the number of CJD cases reported from the year 1986 to 2023 in Asia. sCJD were the highest among all CJD cases. China (1957) and Japan (1705) reported more cases of sCJD than any Asian country and Hong Kong (1) reported the least. On the other hand, gCJD was highest in Japan (370) and least in India (2). Iatrogenic cases were found to be the least among all CJD types and showed 85 cases overall in Asia, 82 being in Japan and 3 in India.