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). The gene discussed is SQSTM1; the disease is frontotemporal dementia.