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What is the prevalence of Creutzfeldt Jakob Disease?

How many people does Creutzfeldt Jakob Disease affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Creutzfeldt Jakob Disease

The prevalence of Creutzfeldt Jakob Disease (CJD) is relatively low, affecting approximately 1 to 1.5 people per million worldwide. This rare and degenerative neurological disorder occurs spontaneously in most cases, but can also be inherited or acquired through exposure to infected tissues. CJD primarily impacts individuals in their later years, with an average age of onset around 60. While it is a rare condition, CJD is highly fatal, leading to rapid cognitive decline, movement disorders, and ultimately death within months or years of diagnosis.



Creutzfeldt-Jakob Disease (CJD) is a rare and fatal neurodegenerative disorder that affects the brain. It belongs to a group of diseases known as prion diseases, which are caused by abnormal proteins called prions.


The prevalence of CJD is relatively low, with an estimated incidence of about 1 to 1.5 cases per million people worldwide. However, it is important to note that CJD can occur in different forms, including sporadic, familial, and acquired variants, each with varying prevalence rates.


The most common form of CJD is sporadic CJD, accounting for approximately 85-90% of cases. Sporadic CJD occurs spontaneously without any known cause and typically affects individuals in their 60s or older. Familial CJD, on the other hand, is a rare inherited form that accounts for about 5-10% of cases and is caused by mutations in the PRNP gene.


Acquired CJD is extremely rare and can be transmitted through medical procedures, such as contaminated surgical instruments or corneal transplantation. The prevalence of acquired CJD is difficult to determine due to its rarity and the stringent safety measures in place to prevent transmission.


In conclusion, while Creutzfeldt-Jakob Disease is a rare condition, it is a devastating and incurable disorder. The sporadic form is the most prevalent, followed by the familial and acquired variants, each with varying rates of occurrence.


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2 answers
They say it's pretty rare. 1 in a million per population but I think it's more common than this as I know of several people that have had this in my area over the past couple of years and I live in a small area.

Posted Sep 16, 2017 by Gena 2500

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Social reality of prion diseases are very unknown by the population. This study aims, as last objective, to cover this gap and help in a better knowledge of the social reality of families who live and suffer the effects of prion pathologies.  T...

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