Although isolated MND has been reported in patients with mutations in various MSP‐ and MSP‐like genes (VCP, HNRNPA1, HNRNPA2B1 SQSTM1, and MATR3)3, 10, 29, 39, 40, 41 MND often coexisted with myopathy in our cohort. The gene discussed is SQSTM1; the disease is myopathy.