Family members can have the typical centrotemporal discharges seen in this syndrome in an age-dependent manner without clinical seizures, however families with this clinical syndrome are very rare. Inheritance is likely complex.
No pathogenic variants that cause this syndrome have been identified, rare cases have been reported with copy number variants. Pathogenic variants in GRIN2A have been found in those who evolve to developmental and/or epileptic encephalopathy with spike-wave activation in sleep.
Although cases have been reported with a first-degree relative who also has epilepsy, it is rarely self-limited epilepsy with centrotemporal spikes.