Frontotemporal Dementia

The discovery of genetic linkage to chromosome 17 q21-22 of several large families with significant resemblance to Pick complex, supports the concept of syndrome (Wilhelmsen). The chromosome region common to all these families, called FTDP-17, contains the gene for the microtubule associated protein tau. At this point, several mutations in the tau gene have been identified in FTDP-17 families (Hutton et al.). Not all families with FTD have tau mutations, but some have ubiquitin-positive, tau-negative inclusions (Kertesz et al., 2000). Genetic biochemical and histochemical distinctions provide further understanding of the syndrome but we must be careful not to lose sight of the clinical, pathological, and genetic cohesiveness, and exercise caution in interpreting the differences.