BBC London Report on Variant CJD (Human BSE) from 2010

www.foodsafetypolicy.com Creutzfeldt–Jakob disease or CJD is a degenerative neurological disorder (brain disease) that is incurable and invariably fatal.CJD is at times called a human form of mad cow disease, given that bovine spongiform encephalopathy is believed to be the cause of variant Creutzfeldt–Jakob disease in humans. CJD is the most common among the types of transmissible spongiform encephalopathy found in humans. In CJD, the brain tissue develops holes and takes on a sponge-like texture. This is due to a type of infectious protein called a prion. Prions are misfolded proteins which replicate by converting their properly folded counterparts The first symptom of CJD is rapidly progressive dementia, leading to memory loss, personality changes and hallucinations. This is accompanied by physical problems such as speech impairment, jerky movements (myoclonus), balance and coordination dysfunction (ataxia), changes in gait, rigid posture, and seizures. The duration of the disease varies greatly, but sporadic (non-inherited) CJD can be fatal within months or even weeks (Johnson, 1998). In some people, the symptoms can continue for years. In most patients, these symptoms are followed by involuntary movements and the appearance of an atypical diagnostic electroencephalogram tracing. Most victims die 6 months after initial symptoms appear, often of pneumonia due to impaired coughing reflexes. About 15% of patients survive 2 or more years. The symptoms of CJD are caused <b>…</b>