A case of anti-voltage-gated potassium channel complex (LGI-1) associated limbic encephalitis manifesting faciobrachial dystonic seizures

Abstract

A patient with elderly onset temporal lobe epilepsy also manifested amnesia, mood disorder, sleep disorder, urination disorder, and salivary hypersecretion. Anti-voltage-gated potassium channel (VGKC)-complex (leucine-rich glioma inactivated 1 protein : LGI-1) antibody was positive, leading to a diagnosis of anti-VGKC complex (LGI-1) antibody-associated limbic encephalitis (VGKC-LE). This patient had seizures of choking and grimacing face lasting several seconds more than 100 times a day, sometimes with left dystonic upper limb. Irani et al. reported that among VGKC-LE patients, those with anti LGI-1 antibody manifest faciobrachial seizures (FBDS) with grimacing face and dystonic limbs of 3-5 second durations. The present case is the first reported in Japan indicating a relationship between anti-LGI-1 antibody and FBDS.