Proband 8 has a variant in SCN1A, p.R187Q; however, this variant was inherited from an unaffected father, is present in gnomAD in one heterozygote, and, according to the referring clinician, the phenotype observed in the proband is not consistent with Dravet syndrome. Here, SCN1A is linked to encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.