The NaV1.8 p.V1287I variant renders DRG neurons hyperexcitable by compromising channel inactivation as it was demonstrated previously for another NaV1.8 variant associated with painful small fiber neuropathy [28] and several NaV1.7 variants linked to PEPD [13, 20, 37, 56]. Here, SCN10A is linked to paroxysmal extreme pain disorder.