Creutzfeldt-Jakob disease

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Concept.png Creutzfeldt-Jakob disease 
(disease)Rdf-entity.pngRdf-icon.png
Practneurol-2016-001571f02.jpg
Magnetic resonance image of sporadic CJD
A fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis.

Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal degenerative brain disorder.[1][2] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances.[1] Later symptoms include dementia, involuntary movements, blindness, weakness, and coma.[1] About 70% of people die within a year of diagnosis.[1]

CJD is caused by a type of abnormal protein known as a prion.[3] Infectious prions are misfolded proteins that can cause normally folded proteins to also become misfolded.[1] About 85% of cases of CJD occur for unknown reasons, while about 7.5% of cases are inherited from a person's parents in an autosomal dominant manner.[1] Exposure to brain or spinal tissue from an infected person may also result in spread.[1] There is no evidence that sporadic CJD can spread between people via normal contact or blood transfusions, although this is possible in variant Creutzfeldt–Jakob disease.[4] Diagnosis involves ruling out other potential causes.[1] An electroencephalogram, spinal tap, or magnetic resonance imaging may support the diagnosis.[1]

There is no specific treatment for CJD.[1] Opioids may be used to help with pain, while clonazepam or sodium valproate may help with involuntary movements.[1] CJD affects about one per million people per year.[1] Onset is typically around 60 years of age.[1] The condition was first described in 1920.[1] It is classified as a type of transmissible spongiform encephalopathy.[5] Inherited CJD accounts for about 10% of prion disease cases.[6] Sporadic CJD is different from bovine spongiform encephalopathy (mad cow disease) and variant Creutzfeldt–Jakob disease (vCJD).[7]

Human growth hormones

In 2008, Seven French doctors and pharmacists went on trial over the deaths of more than 100 people from Creutzfeldt-Jakob disease which occurred years after the victims were treated, while still children, with tainted human-growth hormones. Up to the late 1980s children in France who had stunted growth were treated with hormones collected from the pituitary glands of human corpses, a practice discontinued in the United States a decade earlier over safety concerns. Of 1,698 children treated in the 1980s, 110 have succumbed to the rare disease.[8]


 

A Creutzfeldt-Jakob disease victim on Wikispooks

TitleDescription
José BaselgaChief medical scientist at Covid-19 vaccine maker AstraZeneca dies at 61.


References

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