Creutzfeldt–Jakob disease (CJD) is a Neurodegenerative disease that is incurable and invariably fatal. CJD is at times called a human form of mad cow disease (bovine spongiform encephalopathy or BSE). However, given that BSE is believed to be the cause of Variant Creutzfeldt–Jakob disease (vCJD) in humans, the two are often confused.
CJD is caused by an infectious agent called a prion. Prions are misfolded proteins that replicate by converting their properly folded counterparts, in their host, to the same misfolded structure they possess. CJD causes the brain tissue to degenerate rapidly, and as the disease destroys the brain, the brain develops holes and the texture changes to resemble that of a kitchen sponge.
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Mehndiratta m m, bajaj b k, gupta m, anand r, tatke m, seryam s, nehru r, puri v, khwaja g a. Creutzfeldt-jakob disease : report of 10 cases from north india. Neurol india [serial online] 2001 [cited 2016 nov 29];49:338. Available from: http://www.neurologyindia.com/text.asp?2001/49/4/338/1224