Autoimmune encephalitis (AE) caused by antibodies (abs) against the leucine-rich glioma-inactivated 1 (LGI1) protein has a common clinical phenotype and disease progression most of the time, starting from faciobrachial dystonic seizures and/or epileptic seizures, leading to full-blown limbic encephalitis (LE) with permanent cognitive impairment and hippocampal atrophy without instant immunosuppressive treatment [1,2,3]. This evidence concerns the gene LGI1 and acrodermatitis enteropathica.