Under this blurred clinical-genetic scenario, we foresee three forms of MYH7-myopathies: 1) an early onset distal myopathy with cores, 2) a late onset form of distal myopathy without histological evidence of cores and with variable association of cardiomyopathy and/or FTD and 3) the least frequent form of limb girdle involvement with myofibrillar damage resembling the myosin storage myopathy. This evidence concerns the gene MYH7 and frontotemporal dementia.