Seven patients (5 VCP, 1 SQSTM1 + TIA1, 1 TFG) without symptoms or signs of cognitive impairment underwent brain MRI 8 years (median, range 2–20) after the onset of the weakness: 1 (VCP) demonstrated mild atrophy of the medial anterior temporal lobes, 2 (VCP) showed mild generalized atrophy, and the remaining 4 had normal findings. Here, SQSTM1 is linked to Cognitive impairment.