Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare transmissible disease. According to molecular classification, six clinical phenotypes of sCJD have been described: MM1, MM2, MV1, MV2, VV1 and VV2. MV2 subtype comprises 9% of sCJD cases. Atypical clinical course has been reported to be the main caveat for the diagnosis of the MV2 subtype. We hereby present a rare case of MV2 subtype of sCJD, highlighting the evolution of clinical, EEG and imaging attributes over a two-year period, thus underlining the atypical course of the disease.
Keywords: MV2 subtype; PRNP gene sequence; cortical ribbon; periodic sharp wave complexes; sporadic CJD.