CJD Support Network Group | Australian Creutzfeldt-Jakob Disease Support Group

CLASSICAL CJD (cCJD)

For simplicity, the term "classical CJD" is used to describe all forms of human CJD except vCJD.

SPORADIC CJD (sCJD) is a rapidly progressive disease that occurs at random in approximately one in a million per year. Sporadic CJD accounts for 85% of all cases and mainly affects people in the 50-70 year age group.

Sporadic CJD is not inherited and does not affect or put family members at risk of CJD.

GENETIC CJD is very rare and account for only about 13% of cases. These include:

Familial CJD (fCJD)
Gerrstmann-Straussler Scheinker Syndrome (GSS)
Fatal Familial Insomnia (FFI)

Inherited prion disease is usually recognised from a family history of the illness in two or more blood relatives or positive prion protein gene (PRNP) testing.

A child of a parent who carries a genetic CJD mutation has a 50% chance of inheriting the disease.

IATROGENIC CJD (iCJD) although rare, has occurred worldwide as a result of a number of medical treatments including the use of human pituitary hormones for infertility and short stature, dura mater grafts and corneal transplants. There is also a recognised risk of transmission from the use of contaminated neuro-surgical equipment.