Human neurodegenerative prion disease: what we know and their epidemiology in Brazil
DOI:
https://doi.org/10.33448/rsd-v11i9.31533Keywords:
Prion proteins; Creutzfeldt-Jakob disease; Neurodegenerative diseases.Abstract
Human prion diseases (PRD) are a group of progressive, incurable and fatal neurodegenerative diseases caused by a protein infectious agent (PrP), capable of propagating the disease by altering the conformational structure of proteins, which undergo aggregation and deposit in neuronal tissue. Due to the neurodegenerative potential of this disease, as well as a global increase in cases, the objective of our article is to review the current knowledge about human PrD and analyze the epidemiological situation of these diseases in Brazil in the last 17 years. We know that there are different types of DPr with differences related to their transmission/manifestation; neuropathology and clinical manifestations and those prions consist of PrPSc, the aggregated pathological form of the cellular prion protein PrPc. Although the mechanisms involved in neurodegeneration are not fully described, we know that they involve multiple processes operating simultaneously and synergistically in the brain, including spongiform degeneration, synaptic changes, brain inflammation, neuronal death and accumulation of protein aggregates. In Brazil, PrD became diseases of compulsory notification to the Notifiable Diseases Information System from 2005, with more than 400 cases of PrD confirmed by 2020, with a significant increase in cases from the year 2012, mainly in the states of Sao Paulo, Minas Gerais and Parana, probably due to the greater diagnostic capacity of these states. In this way, a more comprehensive understanding of these diseases and their epidemiology can help in the early diagnosis and development of much-needed therapies for these devastating diseases.
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