Considering comorbid cases of CJD and frontotemporal lobar degeneration (CJD/FTLD) with characteristic frontotemporal clinical symptomatology, seven cases (3.26%) were found, of which six cases (2.80%) had FTLD/tau, and one (0.47%) had frontotemporal lobar degeneration with positive inclusions for ubiquitin-proteasome system markers (FTLD/UPS). The gene discussed is MAPT; the disease is frontotemporal dementia.