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Latest revision as of 16:13, 20 November 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

Creutzfeldt-Jakob disease is a rare disorder with an incidence of approximately 0.1 to 0.3 cases per 100,000 individuals. In the USA, less than 300 cases of Creutzfeldt-Jakob disease have been reported. Individuals > 50 years of age are at higher risk of developing Creutzfeldt-Jakob disease than younger individuals. The most common age at diagnosis is approximately 60-65 years (range 45-90). On the other hand, variant (non-classic) Creutzfeldt-Jakob disease is more common among younger individuals. There is no gender or racial predilection for the development of Creutzfeldt-Jakob disease. Creutzfeldt-Jakob disease is more commonly described in Africa, but this is thought to be attributed to cannibalism practices in certain African tribes in the 1950s and ingestion of infected human brains, rather than true racial variation.

Epidemiology and Demographics

Incidence

Creutzfeldt Jakob disease is a very rare disorder
The incidence of Creutzfeldt Jakob disease is approximately 0.1 to 0.3 cases per 100,000 individuals.
In the USA, less than 300 cases of Creutzfeldt Jakob disease have been reported.

Age

Individuals > 50 years of age are at higher risk of developing Creutzfeldt Jakob disease than younger individuals.
The most common age at diagnosis is approximately 60-65 years (range 45-90).
On the other hand, variant (non-classic) Creutzfeldt-Jakob disease is more common among younger individuals.

Gender

There is no gender predilection for the development of Creutzfeldt Jakob disease.

Race

There is no racial predilection for the development of Creutzfeldt Jakob disease.
Creutzfeldt Jakob disease is more commonly described in Africa, but this is thought to be attributed to cannibalism practices in certain African tribes in the 1950s and ingestion of infected human brains, rather than true racial variation.

References

Template:WikiDoc Sources

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 __NOTOC__
 {{‪Creutzfeldt-Jakob disease‬}}
-{{CMG}}
+{{CMG}}{{AE}}{{MMJ}}
 ==Overview==
-Creutzfeldt Jakob disease is a very rare disorder with an incidence of 1 case per 1 million per year.
+Creutzfeldt-Jakob disease is a rare disorder with an incidence of approximately 0.1 to 0.3 cases per 100,000 individuals. In the USA, less than 300 cases of Creutzfeldt-Jakob disease have been reported. Individuals > 50 years of age are at higher risk of developing Creutzfeldt-Jakob disease than younger individuals. The most common age at diagnosis is approximately 60-65 years (range 45-90). On the other hand, [[Variant Creutzfeldt-Jakob disease|variant (non-classic) Creutzfeldt-Jakob disease]] is more common among younger individuals. There is no gender or racial predilection for the development of Creutzfeldt-Jakob disease. Creutzfeldt-Jakob disease is more commonly described in Africa, but this is thought to be attributed to cannibalism practices in certain African tribes in the 1950s and ingestion of infected [[human]] brains, rather than true racial variation.
 ==Epidemiology and Demographics==
-Classic [[CJD]] has been recognized since the early 1920s.  The most common form of classic [[CJD]] is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions.
+===Incidence===
+*Creutzfeldt Jakob disease is a very rare disorder
+*The incidence of Creutzfeldt Jakob disease is approximately 0.1 to 0.3 cases per 100,000 individuals.
+*In the USA, less than 300 cases of Creutzfeldt Jakob disease have been reported.
-*This sporadic disease occurs worldwide, including the United States, at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual.
+[[Image:Figure annual deaths 2004.png|600px|CJD deaths and age-adjusted death rate, USA, 1979-2004]]
-*The risk of [[CJD]] increases with age, and in persons aged over 50 years of age, the annual rate is approximately 3.4 cases per million.
-*In recent years, the United States has reported fewer than 300 cases of [[CJD]] a year.
-Whereas the majority of cases of [[CJD]] (about 85%) occur as sporadic disease, a smaller proportion of patients (5-15%) develop CJD because of inherited mutations of the prion [[protein]] gene.  These inherited forms include [[Gerstmann-Straussler-Scheinker syndrome]] and [[fatal familial insomnia]].
+===Age===
+*Individuals > 50 years of age are at higher risk of developing Creutzfeldt Jakob disease than younger individuals.
-[[Image:Figure annual deaths 2004.png|center|600px|CJD deaths and age-adjusted death rate, USA, 1979-2004]]
+*The most common age at diagnosis is approximately 60-65 years (range 45-90).
+*On the other hand, [[Variant Creutzfeldt-Jakob disease|variant (non-classic) Creutzfeldt-Jakob disease]] is more common among younger individuals.
-===Incidence and Prevalence===
+===Gender===
-Although CJD is the most common human [[prion]] disease, it is still rare and only occurs in about one out of every one million people.  It usually affects people aged 45&ndash;75, most commonly appearing in people between the ages of 60&ndash;65.  The exception to this is the more recently-recognised 'variant' CJD (vCJD), which occurs in younger people.
+*There is no gender predilection for the development of Creutzfeldt Jakob disease.
-[[Centers for Disease Control and Prevention|CDC]] monitors the occurrence of CJD in the United States through periodic reviews of national mortality data. According to the CDC:
-* CJD occurs worldwide at a rate of about 1 case per million population per year.
-* On the basis of mortality surveillance from 1979 to 1994, the annual [[incidence (epidemiology)|incidence]] of CJD remained stable at approximately 1 case per million persons in the United States.
-* In the United States, CJD deaths among persons younger than 30 years of age are extremely rare (fewer than 5 deaths per billion per year).
-* In more than 85% of cases, the duration of CJD is less than 1 year (median: 4 months) after onset of symptoms.<ref name="titleHomepage | CDC Classic CJD">{{cite web |url=http://www.cdc.gov/ncidod/dvrd/cjd/index.htm |title=Homepage | CDC Classic CJD |accessdate=2007-12-02 |format= |work=}}</ref><ref name="titleVariant CJD, Fact Sheet | CDC vCJD">{{cite web |url=http://www.cdc.gov/ncidod/dvrd/vcjd/factsheet_nvcjd.htm |title=Variant CJD, Fact Sheet | CDC vCJD|accessdate=2007-12-02 |format= |work=}}</ref>
-====New Concerns on Incidence and Prevalence====
-In [[The Lancet]] (June 2006), a University College London team suggested that it may take more than 50 years for vCJD to develop, from their studies of [[kuru (disease)|kuru]], a similar disease in Papua New Guinea.  The reasoning behind the claim is that kuru was transmitted through [[cannibalism]] in Papua New Guinea when relatives would eat their dead relative's bodies as a sign of mourning.  In the 1950s, the practice was banned, thereby preventing any further possible transmission. In the late 20th century, however, kuru reached epidemic proportions in certain Papua New Guinean communities, therefore suggesting that vCJD may also have a similar [[incubation period]] of 30 to 50 years.  A critique to this theory is that while mortuary cannibalism was banned in Papua New Guinea in the 1950s, that does not necessarily mean that the practice ended. Fifteen years later [[Jared Diamond]] was informed by Papuans that the practice continued.<ref>{{cite web|url=http://www.nature.com/nature/journal/v407/n6800/full/407025a0.html|title=Diamond, J.M. (2000)"Archaeology: Talk of cannibalism" ''Nature'' 407, 25-26<!--INSERT TITLE-->}}</ref>
-These researchers noticed a genetic variation in some kuru patients that has been known to promote long [[incubation period]]s.  They have also proposed that individuals who contracted CJD in the early 1990s represent a distinct genetic subpopulation, with unusually short incubation periods for BSE.  This means that there may be many more vCJD patients who have longer incubation periods, which may surface many years later.
-===Age===
-* The risk of [[CJD]] increases with age.
-* The disease is found most frequently in patients 55 to 65 years of age, but cases can occur in persons older than 90 years and younger than 55 years of age.
+===Race===
+*There is no racial predilection for the development of Creutzfeldt Jakob disease.
+*Creutzfeldt Jakob disease is more commonly described in Africa, but this is thought to be attributed to cannibalism practices in certain African tribes in the 1950s and ingestion of infected human brains, rather than true racial variation.
 ==References==
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 {{WikiDoc Sources}}
-[[Category:Infectious disease]]
 [[Category:Neurology]]
 [[Category:Disease]]
 [[Category:Transmissible spongiform encephalopathies]]
-[[Category:Needs overview]]