Several recent evidence pointed in fact towards an immune dysregulation in SMA, as SMN seem to have a central role for the healthy development of the lymphoid system (Deguise et al. 2017), as well as specific drugs like nusinersen have been reported to improve some features more likely related to neuroinflammation (Bonanno et al. 2022; Nuzzo et al. 2023b). This evidence concerns the gene SMN2 and proximal spinal muscular atrophy.