Difference between revisions of "Creutzfeldt-Jakob disease"

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#REDIRECT[[Creutzfeldt–Jakob disease]]
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{{concept
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|wikipedia=https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
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|constitutes=disease
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|image=Practneurol-2016-001571f02.jpg
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|image_caption=Magnetic resonance image of sporadic CJD
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|description=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.
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'''Creutzfeldt–Jakob disease''' ('''CJD'''), also known as '''subacute spongiform encephalopathy''' or '''neurocognitive disorder due to prion disease''', is a fatal [[neurodegeneration|degenerative]] [[brain disorder]].<ref name=NIH2003/><ref name=CDC2015Main>https://www.cdc.gov/prions/cjd/index.html</ref> Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances.<ref name=NIH2003/> Later symptoms include [[dementia]], involuntary movements, blindness, weakness, and [[coma]].<ref name=NIH2003/> About 70% of people die within a year of diagnosis.<ref name=NIH2003/>
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CJD is caused by a type of abnormal protein known as a [[prion]].<ref name=CDC2019>https://www.cdc.gov/prions/cjd/index.html |website=www.cdc.gov</ref> Infectious prions are [[protein folding|misfolded]] proteins that can cause normally folded proteins to also become misfolded.<ref name=NIH2003/> About 85% of cases of CJD occur for unknown reasons, while about 7.5% of cases are [[heredity|inherited from a person's parents]] in an [[autosomal dominant]] manner.<ref name=NIH2003/> Exposure to brain or spinal tissue from an infected person may also result in spread.<ref name=NIH2003/> 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]].<ref>https://www.transfusionguidelines.org/transfusion-handbook/5-adverse-effects-of-transfusion/5-4-variant-creutzfeldt-jakob-disease-vcjd</ref> Diagnosis involves ruling out other potential causes.<ref name=NIH2003/> An [[electroencephalogram]], [[Lumbar puncture|spinal tap]], or [[magnetic resonance imaging]] may support the diagnosis.<ref name=NIH2003/>
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There is no specific treatment for CJD.<ref name=NIH2003>https://web.archive.org/web/20170704234755/https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Disease-Fact-Sheet</ref> [[Opioids]] may be used to help with pain, while [[clonazepam]] or [[sodium valproate]] may help with involuntary movements.<ref name=NIH2003/> CJD affects about one per million people per year.<ref name=NIH2003/> Onset is typically around 60 years of age.<ref name=NIH2003/> The condition was first described in 1920.<ref name=NIH2003/> It is classified as a type of [[transmissible spongiform encephalopathy]].<ref>https://web.archive.org/web/20170808104028/https://www.cdc.gov/prions/cjd/about.html</ref> Inherited CJD accounts for about 10% of prion disease cases.<ref name=Man2015>https://doi.org/10.3171%2F2015.8.FOCUS15328</ref> Sporadic CJD is different from [[bovine spongiform encephalopathy]] (mad cow disease) and [[variant Creutzfeldt–Jakob disease]] (vCJD).<ref name=CDC2015Ind>https://web.archive.org/web/20170718154357/https://www.cdc.gov/prions/cjd/index.html|a</ref>
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==Human growth hormones==
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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.<ref>https://www.theguardian.com/world/2008/feb/07/france.international</ref>
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==References==
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{{reflist}}
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{{PageCredit
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|site=Wikipedia
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|date=31.08.2021
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|url=https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
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Latest revision as of 01:24, 17 December 2021

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 cancer researcher at AstraZeneca dies at 61 of Creutzfeldt-Jakob disease, possibly an effect of the Covid jab.
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References

Wikipedia.png This page imported content from Wikipedia on 31.08.2021.
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