WHAT IS CJD?
Creutzfeldt-Jakob Disease, or CJD is a rare and fatal degenerative brain disease of the central nervous system in humans.
It is one of a group of diseases that affects humans and animals, known as the transmissible spongiform encephalopathies (TSEs) or prion diseases. In animals, the best known TSE is BSE (bovine spongiform encephalopathy) sometimes referred to in the news as mad-cow disease. CJD is not transmitted through social contact.
There is currently no cure or treatment for CJD but research continues on many drugs. CJD is still only confirmed by autopsy although a test performed on cerebrospinal fluid to detect a protein marker can help diagnose CJD in people who already show clinical symptoms of the disease.
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THE DIFFERENT FORMS OF CJD
- CLASSICAL CJD (cCJD) which includes:
- Sporadic CJD (sCJD )a rapidly progressive disease that occurs at random approximately one in a million per year of the general population. (About 20 cases each year in Australia). Sporadic CJD accounts for 85-90% of all cases and affects mainly people in the 50 to 70 age group. Sporadic (sCJD) is not inherited and does not affect or put family members at risk of CJD.
- Inherited Prion Diseases are very rare and only accounts for about 10% of all cases. Included in this group are familial CJD (fCJD), Gerstmann-Straussler-Scheinker Syndrome (GSS) and Fatal Familial Insomnia (FFI). Genetic prion disease is usually recognised from a family history of the illness in siblings and parents and/or positive prion protein gene (PRNP) testing. It is inherited. A child born of a parent carrying genetic CJD has a 50% chance of inheriting the disease causing mutation. Not all mutations prove to be transmissible during a lifetime.
- Health care associated/Iatrogenic although rare, has occurred worldwide as a result of a number of medical treatments including:
*Pituitary Hormones- The use of human pituitary hormones has resulted in five deaths in Australia – Three definite, one probable and one possible. No recorded cases since 1991.
To 2005 worldwide there were approximately 178 deaths – this includes four cases related to human pituitary gonadotrophin treatment hPG (all in Australia), the remainder all treated with human growth hormone (hGH) [1 possible in Australia].
*Dura Mater grafts- Dura mater is a tissue that covers the brain and used in brain surgery and some other operations, to repair damaged membrane. Five cases recorded in Australia. Worldwide the figures stand at approximately 170 cases to March 2006, with Japan’s death toll currently at 122. Dura mater continued to be used in Japan until 1997, long after it ceased to be used in most other countries after a worldwide alert. It is for that reason that the Japanese regrettably expect that the deaths relating to dura mater grafts will continue for some years.
*Cornea grafts– Three cases worldwide from grafts of CJD-contaminated corneas. (No recorded cases in Australia.)
*Neurosurgical instruments- Contaminated neurosurgical equipments have resulted in CJD deaths in England and France in the 1950s and 1960s. Only five cases have been retrospectively verified. (No recorded cases in Australia.)
- CJD (iCJD)
- VARIANT CJD (vCJD)
THE SYMPTOMS OF CJD
- Personality and behavioral changes
- Visual symptoms, double vision and blindness
- Confusion, which may advance to dementia
- Weakness, loss of balance and muscle coordination
- Staggering and difficulty in walking
- Muscle spasms
Some or all of these symptoms may be indicative of many other diseases.
In actual cases of CJD the symptoms are severe and progress rapidly.
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