Prion Diseases: Clinical Manifestations and Diagnosis
Keywords:
Creutzfeldt-Jakob, prion diseases, transmissible spongiform encephalopathies, prionsAbstract
Introduction: Prion diseases or transmissible spongiform encephalopathies are a group of neurodegenerative diseases that affect humans and animals. Globally, there is an incidence of one to two cases per million inhabitants. They are rare and have a fatal outcome. A better understanding of these entities will allow, in the future, the identification of potential therapeutic targets and the development of treatments that can prevent or slow their progression.
Objective: To characterize prion diseases, such as advances in diagnosis and treatment.
Methods: A search was carried out in the PubMed, SCOPUS and SciELO databases. 28 sources were included in this review, of which 100% corresponded to the last five years.
Development: Prion diseases are characterized by the accumulation of prion protein in the brain, which causes neuropathological changes that progress to neuronal death. Although protein markers have been found in cerebrospinal fluid that have a high sensitivity and specificity for diagnosis and imaging findings have been correlated, a specific treatment for them is still being sought.
Conclusions: Early detection of prion-induced diseases is essential to positively impact the quality of life of patients who suffer from them. The only treatment available is symptom management and control, until death.
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