Currently, ~30 genes of distal weakness (MYH7, HSPB8, FLNC, BAG3, VCP, DESMIN, DNAJB6, HNRNPA1, ACTN2, ACTA1, NOTCH2NLC, LRP12, GIPC1, CRYAB, MYOT, ZASP, TIA1, TTN, MATR3, SQSTM1, SQSTM1/TIA1, and PLIN4) and proximal weakness (COL6A2, CACNA1S, TNPO3, D4Z4, SMCHD1, HNRNPDL, PABPN1, and CAV3) are associated with the autosomal dominant (AD) form of RVM. Here, DES is linked to Alzheimer disease.