Frontotemporal dementia accounts for a significant minority of all cas
es of presenile dementia. Many pedigrees have been described in which
frontotemporal dementia is inherited as an autosomal dominant trait. F
rontotemporal dementia is genetically heterogeneous with loci identifi
ed on chromosome 17 and chromosome 3. Clinical, pathological and genet
ic findings are described in a large Danish family in which the diseas
e gene lies in the pericentromeric region of chromosome 3.