Lennox–Gastaut Syndrome: A Narrative Review of Etiology, Pathophysiology, Clinical Features, Diagnosis, and Management

Abstract

Lennox-Gastaut syndrome (LGS) represents a serious form of developmental and epileptic encephalopathy, which is defined by drug-resistant seizures of various types, cognitive problems and typical EEG slow spike wave discharges. LGS starts in childhood and is usually associated with a high neurologic, developmental, and psychosocial burden. The cause of LGS is different and can be represented by structural, genetic, metabolic problems, infections and idiopathic forms of LGS. Even with all progress in diagnostic and therapeutic approaches, a complete cure of seizures is difficult to attain for most of the LGS patients; thus, many individuals remain affected with seizures throughout their lifetime. The diagnosis is made based on clinical history, semiology of seizures, specific EEG pattern, neuroimaging and genetic testing. Treatment is complex and involves the use of anti-seizure medications, dietary management, neuroimaging, surgery, rehabilitation, and psychosocial support. New therapeutic approaches approved in recent years and precision medicine have changed the treatment and improved prognosis in some LGS cases.