Serial Magnetic Resonance Imaging Changes in Sporadic Creutzfeldt-Jakob Disease With Valine Homozygosity at Codon 129 of the Prion Protein Gene

Sporadic Creutzfeldt-Jakob disease (sCJD) accompanied by type 2 PrPSc and valine homozygosity at codon 129 (VV2) of the prion protein gene (PRNP) is a representative sCJD having the 129VVgenotype, which does not show prominent myoclonus or periodic sharp wave complexes. Magnetic resonance imaging (MRI) findings of patients with sCJD having the 129VV genotype have rarely been reported.1

JAMA Neurology 2014