Creutzfeldt-Jakob disease

Creutzfeldt-Jakob Disease (CJD) is a very rare and incurable brain disease that is ultimately fatal. It is the most common of the transmissible spongiform encephalopathies (TSEs). TSEs (also known as prion diseases) are caused by a unique type of infectious agent called prions, an abnormally-structured form of a protein found in the brain. Other prion diseases include Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru in humans, as well as BSE and scrapie in animals.

Clinical features of CJD

Although CJD is the most common human prion disease, it is still extremely rare and only occurs in about one out of every one million people. It usually affects people aged 45-75, most commonly appearing in people between the ages of 60-65. The exception to this is the more recently-recognised 'variant' CJD (vCJD), which occurs in younger people. 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).

Related Topics:
Prion - Symptom - Dementia - Memory - Personality - Hallucinations - Speech - Myoclonus - Ataxia - Gait - Posture - Seizures

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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. 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 'spongy' appearance of the brain tissue. In the United States the first case of Mad Cow disease was found in December 2003 in a Canadian born cow in Washington state and in June 2005 in a Texas born cow.http://seattletimes.nwsource.com/html/nationworld/2002479654_madcow08.html

Related Topics:
Nerve cells - Proteins - Microscope

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