To our knowledge, one Japanese patient with V180I CJD showed parkinsonism and a neurocognitive disorder resembling a DLB clinical presentation with dopamine transporter single photon emission computed tomography (DAT-SPECT) showing reductions in tracer binding in the striatum 17, and previous reports have shown V180I cases to exhibit a senile plaque pathology in addition to CJD conventional pathological finding (spongiform changes and coexistence of pathological features of CJD and AD) [9,18–20]. The gene discussed is SLC6A3; the disease is Lewy body dementia.