You have done important research on prion diseases. It is CJD, Creutzfeldt-Jakob disease. What are your findings? Dr. Anton Titov, MD. What are the implications for the diagnostics, prevention and treatment of prion disease? First of all, we need to clarify a few issues around CJD, Creutzfeldt-Jakob disease. CJD in its usual form is spontaneous. It is a sporadically occurring neurodegenerative disease. Dr. Sebastian Brandner, MD. It is like Alzheimer's disease, or like Parkinson's disease. Like in Alzheimer's disease, like in Parkinson's disease, in CJD there is an incorrect folding of the brain protein. It turns into something that clamps together. It essentially starts killing the brain cells. That's what happens in CJD. This is a reason why CJD (Creutzfeldt-Jakob disease) has become such a hot topic. A long time ago it was discovered that CJD is actually transmissible between humans. Later it was also shown that Creutzfeldt-Jakob disease is transmissible between different other species. That included infection transmission from cattle into humans. That's BSE [Bovine Spongiform Encephalopathy] epidemics. CJD could be transmitted from sheep into other species, usually experimentally. Dr. Sebastian Brandner, MD. The first convincing demonstration that CJD is a human transmissible neurodegenerative disease comes from indigenous population in Papua New Guinea. They had a ritual to eat body parts of relatives. This was part of the festivities when someone died. Body parts were dissected and distributed to the community. That habit probably survived for many hundreds of years in this population. Similarly to other cases of Creutzfeldt-Jakob disease [CJD] this happened. One of these patients may have just accidentally have developed CJD. Then the person died. These body parts were then distributed in the community. It takes many years to develop CJD. These patients received and ate these body parts. They developed CJD themselves. After that the incubation time of Creutzfeldt-Jakob disease was shorter and shorter. In the 1950s and 1960s the Australian government came in. It looked at the situation. Dr. Sebastian Brandner, MD. Because it was thought to be a form of slow virus disease. Because it took a long time for brain degeneration to develop. CJD, Creutzfeldt-Jakob disease caused all this shaking and ataxia. It is a gait disorder. Dr. Daniel Gajdusek discovered that a brain protein itself can be transmitted. Abnormal protein can cause the disease in other humans. When this was recognized, then obviously all this habit or eating dead relatives was banned. Very soon the big epidemics of CJD stopped. But patients still were developing new CJD cases many many years later. The longest incubation time for Creutzfeldt-Jakob disease we recently observed was 40 years. Someone who had in the 1960s participated in one of human body parts feasts. That patient died in 2003. We did an autopsy. Dr. Anton Titov, MD. So that was the first really good evidence for CJD transmissibility. CJD, Creutzfeldt-Jakob disease, is usually spontaneous neurodegenerative disease. It is like Alzheimer’s disease, or Parkinson’s disease. In CJD a brain protein folds incorrectly into the form that kills the brain cells. Leading neurodegenerative disease expert explains links between different dementias.
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