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04.03.2014

Italian sleepless disease, cure tinnitus with sound - Try Out

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Prion diseases are rare infectious degenerative diseases of the brain characterised by massive neuronal death leaving open areas in the brain resembling a sponge – hence they are often known as transmissible spongiform encephalopathies. These diseases are thought to be caused by a protein called PrPc that normally functions in cell signalling. Mutations in different regions of the gene for PrPc can increase the likelihood of PrPc proteins converting to prions and causing a number of different autosomal dominant inherited prion diseases with overlapping symptoms such as familial CJD, Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia that is characterised by the degeneration of an area which influences sleep. Around one in every million people each year develops CJD, although this may be a low estimate; the disease is very difficult to diagnose as there is no definitive diagnosis without examining the brain. Only a small percentage of prion disease cases run in families with most prion diseases developing sporadically in the absence of any mutation or as a result of infection with abnormally formed prion protein. Prion diseases occur in many other animals such as cows, where it is known as mad cow disease (bovine spongiform encephalopathy), sheep (scrapie), cats, and even ostriches. Prion diseases are a category of rare fatal brain diseases that can strike both humans and animals.


In humans, there are five known prion diseases: Creutzfeldt-Jakob Disease, Variant Creutzfeldt-Jakob Disease, Gerstmann-Straussler-Scheinker Syndrome, Kuru, and Fatal Familial Insomnia. The first prion disease to be identified was in the 1730s when sheep and goats in Great Britain started to come down with a mysterious illness that caused them to be irritable, lose weight, and become uncoordinated. Like all prion diseases, Fatal Familial Insomnia (FFI) is caused by a change or mutation of the PRNP gene, which allows it to clump together in the thalamus region of the brain, eventually destroying the cells there. His wife's aunts both died after developing confusion, paranoia and remaining sleepless, vegetating in a dream-like state for the last months of their lives.
It is conceivable that he might have even had an understanding of the transmission of the disease by consumption of infected neuronal tissue: “…when the brains were out, the man would die. The disease hits the nervous system and impairs essential brain functions, which can cause memory loss, decrease in intelligence, personality and behavioral changes, and insomnia. During funeral rituals members of the tribe would eat the brains of dead relatives leading to the spread of a prion disease known as kuru, which means "trembling" in the language of the Fore.


This ‘specificity’ phenomenon is thought to explain why transmission of prion diseases from one species to another (such as from sheep to cows or from cows to humans) is likely to be a rare, if not impossible, event. Scrapie was only found in Western Europe for the first two hundred years of its known existence, but in the mid-20th century, a Michigan farmer imported sheep from England and his entire flock was killed off by the disease. Despite over 11,000 sleepless nights, he claimed that he suffered very little ill physical or mental effects. These diseases can either be inherited, such as familial Creutzfeldt-Jakob disease (CJD), or acquired as in the variant CJD and kuru.



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