PRION DISEASES: PATHOPHYSIOLOGY AND INTERDISCIPLINARY TREATMENT

Abstract

Prion diseases are rare, progressive, and invariably fatal neurodegenerative conditions that are classified into three main categories: sporadic, genetic, and acquired. This article presents a systematic review of the literature, based on data obtained from renowned platforms such as PubMed, Nature, and Scielo. Based on the established criteria, it was possible to develop a comprehensive analysis that discusses prion diseases from initial transmission to therapeutic perspectives and clinical outcomes. The pathophysiology involves the conversion of the normal cellular prion protein (PrPC) into its pathological form (PrPSc), resulting in protein accumulation and neurotoxicity. The diagnosis is based on a detailed clinical evaluation, complemented by tests such as electroencephalogram and magnetic resonance imaging. Recent advances in potential therapies, such as the use of heterologous prion proteins and compounds such as AP1, have shown promising results. Despite these advances, it is imperative that translational studies continue to explore existing gaps to develop effective therapies. Prion diseases remain a challenging field that requires research investigation for future scientific and clinical breakthroughs.