PEO is present in ~ 26% of Spanish patients, although frequency doubles in patients not carrying the common p.Ala510Val mutation in homozygosis.22, 24 Given that SPG7 is one of the most frequent causes of HSP, and also a relatively frequent cause underlying hard‐to‐diagnose ataxias,25 it would be prudent to search for intronic SPG7 variants in patients with ataxia or spastic paraplegia, especially if they have associated PEO, or are already known to harbor one suspicious heterozygous variant in this gene. This evidence concerns the gene SPG7 and hereditary spastic paraplegia.