SOD1 and amyotrophic lateral sclerosis: Structural and functional impairment of BBB and BSCB have been demonstrated in a SOD1 mutated (G93A) mouse model of ALS through Evans blue leakage and the ultrastructure observation of the BBB components by TEM showed degenerated endothelial cells and astrocytes, extracellular edema or erythrocytes infiltration, which seem to worsen with disease evolution [17,18].