A pathogenic variant in the Cu/Zn superoxide dismutase (SOD1) gene was first described in 1993 [1] as a cause of familial Amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by progressive muscle weakness and a severely reduced life expectancy of about 2–5 years after onset by irreversible affection of the upper and lower motor neurons [2]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.