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CJD
A guide for GPs
What are the different types of CJD?
The four forms of CJD involve the same basic disease process: the accumulation of abnormal prion protein in brain tissue. However, they have different causes with somewhat different age of onset, symptoms and
varied pathological changes in the brain.
varied pathological changes in the brain.
Sporadic CJD (sCJD)
This is the commonest form, but still very rare, affecting about one person per million each year (approximately 50-60 UK cases annually). It occurs worldwide and mostly affects middle-aged and elderly people, although younger people can be affected.
Genetic or familial CJD (gCJD or fCJD)
This is very rare, with only a few cases each year in the UK, and is due to an autosomal dominant inherited genetic defect.
Iatrogenic CJD (iCJD)
These cases result from accidental transmission by medical or surgical treatment. Only a few such cases occur each year in the UK, and to date only about 250 have been identified worldwide.
Variant CJD (vCJD)
This form of CJD was identified in 1996 and was originally called ‘new variant CJD’ or nvCJD.This is the only form of CJD which is believed to have resultedfrom BSE in cattle, and no case has been identified with symptoms beginning before 1994. By September 2002, 127 cases had been confirmed in the UK, six in France, one in the Republic of Ireland, one in Italy, one in Canada and one in the USA. In contrast to sporadic CJD, it predominantly affects young people, often in their twenties.
This is the commonest form, but still very rare, affecting about one person per million each year (approximately 50-60 UK cases annually). It occurs worldwide and mostly affects middle-aged and elderly people, although younger people can be affected.
Genetic or familial CJD (gCJD or fCJD)
This is very rare, with only a few cases each year in the UK, and is due to an autosomal dominant inherited genetic defect.
Iatrogenic CJD (iCJD)
These cases result from accidental transmission by medical or surgical treatment. Only a few such cases occur each year in the UK, and to date only about 250 have been identified worldwide.
Variant CJD (vCJD)
This form of CJD was identified in 1996 and was originally called ‘new variant CJD’ or nvCJD.This is the only form of CJD which is believed to have resultedfrom BSE in cattle, and no case has been identified with symptoms beginning before 1994. By September 2002, 127 cases had been confirmed in the UK, six in France, one in the Republic of Ireland, one in Italy, one in Canada and one in the USA. In contrast to sporadic CJD, it predominantly affects young people, often in their twenties.
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Page 3 of 5
Contents
- Introduction
- What is CJD?
- What are the different types of CJD?
- What do I do if I suspect a patient has CJD?
- Further advice and information
