Management of Lennox-Gastaut syndrome beyond childhood: A comprehensive review

Abstract

Lennox-Gastaut syndrome (LGS) is a childhood-onset epileptic encephalopathy characterized by multiple types of medically intractable seizures, cognitive impairment, and generalized slow spike-wave discharges in electroencephalography (EEG). Although the onset of this epileptic syndrome occurs typically before eight years of age with a peak age between 3 and 5 years, lifelong persistence of the syndrome is usual. The evolution of clinical features, EEG findings, and paucity of knowledge about LGS among adult health care providers can make LGS significantly underdiagnosed in the adult population. Management of LGS remains problematic beyond childhood due to intractable seizures, the difficult transition from pediatric to adult neurologists, challenging behaviors, impaired cognition, poor quality of life, and disabled social life. In focusing on the management of LGS beyond childhood, this narrative review describes medical and surgical management of epilepsy, the transition from pediatric to adult care, and management of other common comorbidities associated with LGS. Several antiepileptic drugs (AEDs) such as lamotrigine, topiramate, felbamate, rufinamide, clobazam, and Epidiolex (pure pharmaceutical grade cannabidiol (CBD) oil) have been noted to be effective in well-designed, randomized controlled trials. Other non-pharmacological therapies, such as vagus nerve stimulation, ketogenic diet, and epilepsy surgery, have been frequently utilized in the management of intractable seizures associated with LGS. However, effective management of LGS requires a broader perspective to not only control seizures but improve the quality of life by addressing cognitive and behavioral problems, sleep disturbances, physical disability, social disability, and educational and employment challenges.