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Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. [ 4 ] [ 1 ] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. [ 4 ]
Human infectious diseases may be characterized by their case fatality rate (CFR), the proportion of people diagnosed with a disease who die from it (cf. mortality rate).It should not be confused with the infection fatality rate (IFR), the estimated proportion of people infected by a disease-causing agent, including asymptomatic and undiagnosed infections, who die from the disease.
Variant Creutzfeldt–Jakob disease (vCJD), formerly known as New variant Creutzfeldt–Jakob disease (nvCJD) and referred to colloquially as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family. [7]
Fatal insomnia is an extremely rare neurodegenerative prion disease that results in trouble sleeping as its hallmark symptom. [2] The majority of cases are familial (fatal familial insomnia [FFI]), stemming from a mutation in the PRNP gene, with the remainder of cases occurring sporadically (sporadic fatal insomnia [sFI]).
For example, Creutzfeldt–Jakob disease has been transmitted to patients taking injections of growth hormone harvested from human pituitary glands, from cadaver dura allografts and from instruments used for brain surgery (Brown, 2000) (prions can survive the "autoclave" sterilization process used for most surgical instruments).
Jonathan Simms (1 June 1984 – 5 March 2011) was a man from Belfast, Northern Ireland, who contracted variant Creutzfeldt–Jakob disease (vCJD) in his late teenage years. He was given a post-diagnosis life expectancy of one year, similar to that of other young people who were diagnosed in the same age bracket.
Electroencephalogram (EEG) is used to distinguish kuru from Creutzfeldt–Jakob disease, a similar encephalopathy (any disease that affects the structure of the brain). [28] EEGs search for electrical activity in the person's brain and measure the frequency of each wave to determine if there is an issue with the brain's activity. [29]
There is conflicting information about the time between infection and onset of symptoms. In 2002, the World Health Organization suggested it to be approximately four to five years. [2] Time from onset of symptoms to death is generally weeks to months. [2] Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). [3]