Spinal onset disease (p = 0.044, OR 8.25, 95% CI 1.06–64.49) and family history of MND (p < 5 × 10−7, OR 16.21, 95% CI 6.21–42.33) were independently associated with carrying a SOD1 pathogenic variant. This evidence concerns the gene SOD1 and mild neurocognitive disorder.