SQSTM1 variations are associated with multisystem proteinopathies (MSPs), including Paget disease of the bone (PDB) (4), amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) (5), and myopathy with rimmed vacuoles (MRV) (6, 7). This evidence concerns the gene SQSTM1 and myopathy.