Historically, frontotemporal dementia (FTD) were often classified as a form of Pick's disease, even when Pick cells or Pick bodies were not found. However, this denomination may involve different subgroups of pathologies, and the Lund and Manchester groups published in 1994 a consensus on Clinical and Neuropathological Criteria for Frontotem- poral Dementia (3). This publication clarified the position of Pick's disease within FTD, and several of the reported cases of familial Pick's disease were probably cases of familial FTD. Indeed it is difficult to ascertain families which have the classic pathological features of Pick's disease from the literature, because they often have unusual clinical features. Also, in 1996, Brun and Passant characterized "Frontal lobe degeneration" as a subgroup of frontotemporal dementia distinct from the others because of the lack of tau pathology