Connection of LGMD1D to DNAJB6 raised interest in whether LGMD1D patients develop central nervous system (CNS) disease in addition to their myopathy, and in fact one patient (with a known DNAJB6 mutation) has since been reported with pathologically confirmed frontotemporal dementia (FTD) and no mutation in any of the known FTD genes [10]. This evidence concerns the gene DNAJB6 and myopathy.