The group of Autoimmune Encephalitis (AE) manifesting with SE includes several disorders mediated by antibodies as those acting against the gamma-aminobutyric acid receptor (GABA R) A [2, 3], and (GABA R) B [4], leucine-rich glioma inactivated 1 (LGI1) [5], and with less frequency disorders of antibodies mediated against anti-N-methyl-D-aspartate receptor (NMDAR) [6], alfa-amino-3-hydroxy-5-methyl-4-isoxazoleproprionic acid receptor (AMPAR), [7] contactin-associated protein-2 receptor (CASPR 2), [8] and several other antibodies mediated disorders. The gene discussed is LGI1; the disease is autoimmune encephalitis.