Sporadic Creutzfeldt-Jakob disease: clinical and diagnostic characteristics of the rare VV1 type

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a rare and fatal disease caused by prions. Recently, 6 molecular subtypes of sporadic CJD (sCJD) have been identified showing differences regarding the disease course, neuropathological lesion patterns and sensitivity to diagnostic tools. In the literature, only isolated cases of the rare VV1 subtype have been reported so far. We describe the clinical characteristics and neuropathological lesion profiles in 9 of our cases and review the literature on former VV1 case reports.