Abstract
Frontotemporal dementia accounts for a significant minority of all cases of presenile dementia. Many pedigrees have been described in which frontotemporal dementia is inherited as an autosomal dominant trait. Frontotemporal dementia is genetically heterogeneous with loci identified on chromosome 17 and chromosome 3. Clinical, pathological and genetic findings are described in a large Danish family in which the disease gene lies in the pericentromeric region of chromosome 3.

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