Difference between revisions of "Creutzfeldt–Jakob disease"

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#REDIRECT[[Creutzfeldt-Jakob disease]]
|wikipedia=https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
 
|constitutes=disease
 
|image=Practneurol-2016-001571f02.jpg
 
|image_caption=Magnetic resonance image of sporadic CJD
 
|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.
 
}}
 
'''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/> The name Creutzfeldt–Jakob disease was introduced by [[Walther Spielmeyer]] in 1922, after the German neurologists [[Hans Gerhard Creutzfeldt]] and [[Alfons Maria Jakob]].<ref>http://www.whonamedit.com/synd.cfm/696.html Creutzfeldt–Jakob disease</ref>
 
 
 
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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/>
 
 
 
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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==References==
 
{{reflist}}
 
 
 
{{PageCredit
 
|site=Wikipedia
 
|date=31.08.2021
 
|url=https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
 
}}
 

Latest revision as of 13:33, 2 September 2021