|
|
|
|
||||
Creutzfeldt-Jakob disease
Background
Creutzfeldt-Jakob disease: CJD
Creutzfeldt-Jakob disease is a rare, untreatable, fatal illness affecting the brain resulting in progressive neurological symptoms including dementia, involuntary movements, ataxia (incoordination and unsteadiness) and speech disturbances. It is a member of the group of Transmissible Spongiform Encephalopathies (TSEs) that are caused by an incompletely understood change in a normal cellular protein (designated PrPC) to an abnormal form (designated PrPSc). The manufacture of the normal protein (PrPC) is governed by a gene designated the PRNP gene (on human chromosome 20). The accumulation of the abnormal PrPSc in brain tissue is an essential underlying feature of CJD. Under certain, very specific, circumstances, CJD can be transmitted from one individual to another. The nature of this infectivity is not fully understood.
CJD is divided into four different forms (sporadic, genetic, iatrogenic, variant), partly on differences in symptoms and pathological changes in the brain, but essentially on differences in causation.
Sporadic CJD is the commonest form and is of unknown cause, apparently occurring randomly, mainly in the middle-aged and elderly. Most countries (including the UK) report incidences around 1 to 2 in 1,000,000 persons per year. A small number of cases are genetic, due to inherited defects in the PRNP gene. A minority of cases (iatrogenic CJD) are due to transmission of disease via medical or surgical treatments.
In 1996, a new variant of CJD, affecting relatively young people, was reported. Now called 'variant CJD', it is believed to be due to dietary contamination of food with Bovine Spongiform Encephalopathy (BSE). The number of cases of this form of CJD are in decline in the UK. Other countries, especially France, have also identified cases. In the UK, accidental transmissions of variant CJD by blood has been reported; this has not been reported for other forms of CJD.
Donate Now
makingcontact.org
Pounds for parents
Subscribe to the 
