43 people, including 11 children, were told in Auckland that they were operated on with the same surgical instruments as a woman diagnosed as having symptoms consistent with sporadic CJD.
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive disease causing damage to the brain. It is one of a group of rare diseases that affects humans and animals, known as transmissible spongiform encephalopathies (TSE) and is believed to be caused by a prion, a newly identified type of disease-causing agent.
Creutzfeldt-Jakob disease is characterized by dementia and walking difficulties. Death can occur up to two years after the first symptoms; however, most people die within seven months. There is no treatment or cure.
It is doubted that hundreds more people may have been infected with the same brain-wasting disease that has caused a scare at Auckland City Hospital.
Neurosurgeon Dr Barry Snow said sporadic CJD should not be confused with variant CJD, a related disease thought to be contracted by eating cattle meat infected with mad cow disease, or bovine spongiform encephalopathy (BSE).
There are three main forms of CJD, each one with its distinctive basic features. The sporadic CJD, which accounts for approximately 85% of all cases worldwide and occurs by chance, is associated with the presence of a misshapen protein in the brain, known as a prion (“proteinaceous infectious particle”). Sporadic CJD cannot be caught from another person or animal, is not related to diet, nor can it be inherited. On the contrary, inherited (or familial) CJD accounts for 5?10% of all cases of CJD and is caused by a faulty gene called prion-related protein (PRPN) that is passed down from parents to their children in a dominant inheritance, which means patients will develop the disease if they inherit a defective gene from just one parent. Symptoms are similar to sporadic CJD, but they appear earlier and have a longer time course.
Unlike the previous two CJD forms, acquired CJD affects those people who have not inherited the condition by two other ways. The iatrogenic CJD occurs due to accidental infection after medical procedures such as human pituitary hormone injection or dura mater transplantation. The variant CJD (vCJD), a type of CJD that was first identified in 1996, is passed from cows with bovine spongiform encephalopathy (BSE, or “mad cow disease”) to humans. The variant form affects mostly younger adults and has different clinical and pathological characteristics.