Recessive mutations in the ALS2 gene are responsible for distinct MND conditions, namely Infantile-onset ascending hereditary spastic paralysis (IAHSP, OMIM:607225), Juvenile Primary Lateral Sclerosis (JPLS, OMIM:606353), and Juvenile Amyotrophic Lateral Sclerosis (JALS OMIM:205100) [3,14]. Here, ALS2 is linked to juvenile primary lateral sclerosis.