Many patients with post-mortem diagnosis of CBD are never suspected ofhaving the disease during life.1,6,8,35 Additionally, CBDpathology was found in only 50% of all clinically diagnosed patients, with othersshowing PSP, Pick's disease, FTLD- TDP43, AD, dementia with Lewy bodies, andCreutzfeldt-Jakob disease at autopsy.2,6,8,35-37 Due to this clinical-pathologicdiversity, Boeve et al. (2003) introduced the term CBS to distinguish the clinicalsyndrome from the pathologic entity, CBD.38 The gene discussed is TARDBP; the disease is frontotemporal dementia.