The known pathogenic variants in ALPK1, particularly those that lead to a gain of function, result in persistent activation of the NF-κB pathway, contributing to the broad spectrum of inflammatory symptoms seen in ROSAH syndrome [1, 6, 7]. This evidence concerns the gene NFKB1 and retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache syndrome.