Creutzfeldt-Jakob disease associated with a deletion of two repeats in the prion protein gene.

Capellari, S; Parchi, P; Wolff, B D; Campbell, J; Atkinson, R; Posey, D M; Petersen, R B; Gambetti, P · Neurology · 2002

case_report · Level V

Where this comes from

Abstract

A two-octapeptide repeat deletion of the prion protein gene has been recently observed in a patient with a 2-year history of dementia and a clinical diagnosis of possible Creutzfeldt-Jakob disease (CJD). The authors report a similar deletion in a patient with a definitive diagnosis of CJD. Since the two-repeat deletion has not been observed in large, population-based studies, the two cases suggest that this deletion is a new pathogenic mutation associated with CJD.

Medical subject headings