Creutzfeldt–Jakob disease
 Creutzfeldt–Jakob disease  (disease)   | |
|---|---|
 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] The name Creutzfeldt–Jakob disease was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob.[3]
CJD is caused by a type of abnormal protein known as a prion.[4] 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.[5] 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.[6] Inherited CJD accounts for about 10% of prion disease cases.[7] Sporadic CJD is different from bovine spongiform encephalopathy (mad cow disease) and variant Creutzfeldt–Jakob disease (vCJD).[8]
References
- ↑ a b c d e f g h i j k l m n https://web.archive.org/web/20170704234755/https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Disease-Fact-Sheet
- ↑ https://www.cdc.gov/prions/cjd/index.html
- ↑ http://www.whonamedit.com/synd.cfm/696.html Creutzfeldt–Jakob disease
- ↑ https://www.cdc.gov/prions/cjd/index.html |website=www.cdc.gov
- ↑ https://www.transfusionguidelines.org/transfusion-handbook/5-adverse-effects-of-transfusion/5-4-variant-creutzfeldt-jakob-disease-vcjd
- ↑ https://web.archive.org/web/20170808104028/https://www.cdc.gov/prions/cjd/about.html
- ↑ https://doi.org/10.3171%2F2015.8.FOCUS15328
- ↑ https://web.archive.org/web/20170718154357/https://www.cdc.gov/prions/cjd/index.html%7Ca
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