SIGMAR1 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy: Moreover, as the neuromodulatory role of Sig1R on neurotransmitter release, receptor activation and regulation of numerous ionophores is well-described, Sig1R modulation must be considered in the potential mechanism of action of its anti-convulsant activity demonstrated for Dravet syndrome (Polster, 2018).