Definition:

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a group of chronic disorders characterized by prominent neuronal loss and gliosis in the hippocampus and amygdala. MTLE-HS is restricted to patients in whom hippocampal atrophy and/or abnormal signal intensity on MRI, anterior temporal interictal epileptiform discharges, and additional evidence of temporal dysfunction on functional images and neuro-psychological assessment are demonstrated. Patients with MTLE-HS often have a history of early risk factors such as febrile seizures, status epilepticus (SE) or infection. A seizure-free period may ensue before uncontrolled partial seizures begin. Epileptic focus is located deeply in the mesial temporal region including mainly hippocampus, parahippocampal gyrus and amygdala. The anatomopathological hallmark is sclerosis of the hippocampus. Since most of the cases are drug resistant, epilepsy surgery is the current gold standard therapy option for cessation of seizures. Familial forms of MTLE-HS have been recognized, but no causal gene has been identified so far.