Mutations in the VCP gene (encoding valosin containing protein), at the 9p21 locus, were identified as the cause of the autosomal dominant syndrome “Inclusion Body Myopathy, Paget’s disease of bone, Fronto-temporal Dementia” (IBMPFD), characterized by skeletal manifestations similar to classical PDB, associated with myopathy, amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (Kovach et al., 2001; Watts et al., 2004). Here, VCP is linked to frontotemporal dementia.