Abstract

VILLEGAS LANAU, Carlos Andrés. Prion diseases: from molecular biology to clinical practice. Acta Neurol Colomb. [online]. 2010, vol.26, n.2, pp.87-111. ISSN 0120-8748.

SUMMARY Illnesses caused by prions are also known as Transmissible Spongiform Encephalopathies or infectious dementias. The most recognized clinic presentations in humans are the Creutzfeldt-Jakob disease, the Gerstmann-Sträussler-Scheinker syndrome and the Fatal Familial Insomnia. They are considered uncommon pathologies, but discoveries in molecular biology in recent years, reveal that pathological mechanisms that allow its development could be common to several neurodegenerative diseases in which the pathologic spectrum can be amplified, becoming not infrequent neurological diseases. This review gives tools to the clinical attendant in order to recognize these disorders, discussing the clinical presentations: sporadic, infectious and familiar, diagnostic work up, diagnostic criteria and therapeutic approach.

Keywords : Prions; molecular biology; encephalitis.

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