Lennox-Gastaut Syndrome

Excerpt

Lennox-Gastaut syndrome (LGS) is a lifelong, severe developmental epileptic encephalopathy characterized by treatment-resistant epilepsy, intellectual disability, and multiple seizure types—most notably tonic, atypical absence, and atonic seizures. A hallmark electroencephalogram (EEG) feature includes interictal slow spike-wave patterns, often accompanied by generalized paroxysmal fast activity during sleep. LGS can arise from a variety of underlying causes, such as genetic mutations, cortical malformations, brain tumors, neurocutaneous disorders such as tuberous sclerosis complex, hypoxic-ischemic injury, meningitis, or head trauma. However, in approximately 40% of cases, no identifiable cause is found.

LGS is diagnosed by the presence of tonic seizures, along with at least one additional type of seizure, with onset before the age of 18. The diagnosis also requires the characteristic EEG findings, coupled with a long-term history of treatment-resistant epilepsy and severe intellectual disability. Sleep EEG is particularly useful in identifying key patterns of seizure activity. Magnetic resonance imaging (MRI) of the brain and genetic testing are crucial for determining the underlying cause. Seizure management is often challenging in patients with LGS and typically involves a combination of antiseizure medications. Despite treatment, most patients continue to experience seizures and require ongoing multidisciplinary care.