Creutzfeldt-Jakob Disease Report from 2010 and link to 2011

Report from 2011 http://www.itv.com/tynetees/cjd-research-campaign55359/

CJD Research Campaign from October 2011

Two decades on since BSE or mad cow disease hit the headlines, a father of a victim of its form in humans, new variant CJD, wants the government to maintain research into the degenerative condition. Recent research suggests up to 1 in 1000 people could still be affected.

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http://www.foodsafetypolicy.com/

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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 by the progressive death of the brain's nerve cells, which is associated with the build-up of abnormal prion proteins forming amyloids. When brain tissue from a CJD patient is examined under a microscope, many tiny holes can be seen where whole areas of nerve cells have died. The word "spongiform" in "transmissible spongiform encephalopathies" refers to the sponge-like appearance of the brain tissue.

Cause

Transmissible spongiform encephalopathy diseases are caused by prions. The diseases are thus sometimes called prion diseases. Other prion diseases include Gerstmann--Sträussler--Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru in humans, as well as bovine spongiform encephalopathy (BSE, commonly known as mad cow disease) in cattle, chronic wasting disease (CWD) in elk and deer, and scrapie in sheep. Alpers' syndrome in infants is also thought to be a transmissible spongiform encephalopathy caused by a prion.

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