LGI1 dysfunction in inherited and acquired epileptic disorders

LGI1 is a multifunctional brain protein whose dysfunction is related to several neurologic disorders as diverse as autosomal dominant lateral temporal epilepsy (ADLTE), autoimmune limbic encephalitis (LE), and glioma tumor progression. ADLTE is a genetic focal epilepsy characterized by auditory or aphasic aura and onset in infancy/adolescence, whereas autoimmune LE occurs in adult life and is characterized by amnesia, confusion, and seizures. The complex molecular mechanisms underlying these epileptic conditions are largely unknown. In this chapter, I outline the clinical features, the genetic or autoimmune causes, and a molecular mechanism possibly underlying both ADLTE and autoimmune LE.