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Q&A with Australian Health Practitioners
What causes Creutzfeldt-Jakob disease?
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This answers depends on which form of Creutzfeldt-Jakob disease the question is referring to.
Sporadic CJD - It is believed to be the result of a spontaneous conformational change in the native prion protein that occurs at random in about 1 to 2 people per million of the population per year.
Why my loved one? This is the question that all family members ask!
Genetic CJD - Is an inherited forms of prion diease The form of CJD is caused by a mutation in the prion protein gene that is typically inherited in families in an autosomal dominant fashion, meaning that from generation to generation aprroximately 50% of family members will carry the mutation and develop CJD during their life time if they life long enough.
Acquired forms of CJD are caused by a tranmission incident that has exposed the patient to contaminated prions.- Iatrogenic or medically acquired CJD - exposure to contaminated surgical instruments through an invasive medical procedure or contaminated medical products ie human pituitary hormones of dura mater grafts.
- Kuru - transmitted through cannibalism.
- Variant CJD, commonly known as Mad Cow disease is recognised to be caused by the consumption of contaminated BSE beef (No identified cases in Australia).
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- Iatrogenic or medically acquired CJD - exposure to contaminated surgical instruments through an invasive medical procedure or contaminated medical products ie human pituitary hormones of dura mater grafts.
