The first EAATopathy was a genetic condition combining ataxia, hemiplegia, and seizure, called episodic ataxia type 6 (EA6), and shown to be caused by mutations in SLC1A3, the gene encoding the glial glutamate transporter EAAT1 (Jen et al., 2005; de Vries et al., 2009; Pyle et al., 2015; Choi et al., 2017a; Choi et al., 2017b; Iwama et al., 2017; Chivukula et al., 2020). The gene discussed is SLC1A3; the disease is cerebellar ataxia.