Prion Disease Essay

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Introduction
TSEs or more commonly prion diseases are a group of invariably fatal neurodegenerative diseases that occur in humans and animals . This disease is caused by a protease –resistant protein (PrPsc) after misfolding of a host-encoded prion protein (PrP). TSEs can exist as genetic, infectious or sporadic forms. The diseases are characterized by dementia, ataxia and neuropathlogically due to loss of specific neurons in the brain. Other clinical features include persistent painful stimuli, dystonia, visual or cerebellar problems and gliosis (1).
PrP can occur in two forms- a normal cellular prion protein known as PrPc and a pathogenic misfolded conformer known as PrPsc. The abnormal PrPsc differs from the normal prion protein PrPc in both secondary and tertiary structure. PrPsc is principally rich in Beta sheet contents but PrPc is principally rich in alpha helical contents. Due to this difference of between the isoforms, prions are extremely resistant to certain decontamination systems. The Two tables below outline both human and animal diseases (2).

Table 1.1 The Human Prion Diseases (3)
Disease Abbreviation Mechanism of Transmission
Kuru Infection through ritualistic cannibalism
Creutzfeldt-Jakob Disease CJD Unidentified mechanism
Sporadic CJD sCJD Somatic Mutations or spontaneous conversion of PrPc to PrPSc
Variant CJD vCJD Infection from bovine prions
Familial CJD fCJD Germline mutations in the PrP gene
Latrogenic CJD lCJD Infection from contaminated corneal and dural grafts, pituitary hormone, neurosurgical apparatus
Gerstmann-Straussler-Scheinker syndrome GSS Germline mutations in the
PrP gene
Fatal Familial Insomnia FFI Germline mutations in the
PrP gene...

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Following the proper guidelines for reducing the risk of transmission of TSEs in a clinical setting, the risk has been greatly reduced. As mentioned before no iatrogenic transmission from a medical instrument has been recorded since 1976 since following the standard guidelines. In an experiment by Amin et al., they examined the risk of transmission of vCJD via contact tonometry. They outlined that transmission could be further reduced by using larger volumes of washes for extended periods of time and also adding wiping between washes (16). This could help in the future to completely eradicate TSE transmission completely by increasing sterilisation methods. Proper training on all aspects of transmission of TSEs through medical equipment and proper sterilisation methods should be updated just to keep staff up to date to prevent the risks of TSEs transmission.

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