The Forgotten Disease, an Unusual Case of CJD

Akter F and Abosaif N

Published on: 2019-11-21

Abstract

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, invariably fatal neurodegenerative disease which is caused by deposition of an abnormal isoform of a cellular glycoprotein known as the prion protein [1]. The word prion means proteinaceous infectious particle. Misfolding of prion protein leads to formation of prion protein scarpie (PrPSc) which form insoluble aggregates in the brain tissue. Deposition of prion protein scarpie (PrPSc) leads to neuronal loss and cause small vacuoles formation and spongiform appearance of the cerebral cortex [2]. The annual incidence of human prion disease is one or two per million [3].