From the Guidelines
Lennox-Gastaut syndrome should be managed with a combination of anti-seizure medications, with valproic acid often used as first-line therapy, and consideration of [18F]FDG PET for surgical treatment planning in patients with unilateral focal hypometabolism, as it may provide valuable information for localization of epileptogenic foci and prediction of postsurgical seizure outcome 1.
Key Considerations
- Lennox-Gastaut syndrome is a severe form of epilepsy characterized by multiple types of seizures, intellectual disability, and a distinctive EEG pattern.
- Treatment typically involves a combination of anti-seizure medications, with valproic acid often used as first-line therapy at doses of 10-60 mg/kg/day divided into 2-3 doses.
- Other medications commonly used include lamotrigine (1-15 mg/kg/day), rufinamide (10-45 mg/kg/day), topiramate (1-9 mg/kg/day), clobazam (0.2-1 mg/kg/day), and cannabidiol (Epidiolex, 5-20 mg/kg/day).
- For treatment-resistant cases, additional options include corpus callosotomy surgery, vagus nerve stimulation, or ketogenic diet therapy.
Diagnostic and Treatment Planning Tools
- [18F]FDG PET may provide important information for localization of epileptogenic foci and prediction of postsurgical seizure outcome, especially in patients with unilateral focal hypometabolism 1.
- The use of [18F]FDG PET in Lennox-Gastaut syndrome may help identify patients who could benefit from surgical intervention, such as those with unilateral focal hypometabolism, and provide valuable information for surgical treatment planning 1.
Management and Support
- Long-term management of Lennox-Gastaut syndrome focuses on balancing seizure control with medication side effects while providing supportive care for developmental and behavioral issues.
- Patients often require multiple medications simultaneously, and treatment should be individualized to address the specific needs of each patient.
- A multidisciplinary approach to care, including neurology, psychology, and social work, is essential for providing comprehensive support to patients with Lennox-Gastaut syndrome.
From the Research
Definition and Characteristics of Lennox-Gastaut Syndrome
- Lennox-Gastaut syndrome (LGS) is a severe childhood-onset epileptic encephalopathy characterized by multiple seizure types, generalized slow spike-and-wave complexes in the EEG, and cognitive impairment 2.
- It is defined by a triad of multiple drug-resistant seizure types, a specific EEG pattern, and intellectual disability 3.
- The syndrome is often associated with long-term adverse effects on intellectual development, social functioning, and independent living 3.
Treatment Options for Lennox-Gastaut Syndrome
- Valproate is considered the first-line treatment for LGS, despite the absence of randomized, double-blind, placebo-controlled trials (RDBCTs) specifically in LGS 2, 4.
- Adjunctive therapy with lamotrigine, rufinamide, cannabidiol, clobazam, felbamate, fenfluramine, and topiramate may be effective in reducing seizure frequency 2, 4.
- Non-pharmacological treatment approaches, such as ketogenic diet therapies, vagus nerve stimulation, and corpus callosotomy, may be used in conjunction with antiseizure medication (ASM) therapy 4, 5, 6.
- Treatment decisions should be individualized and take into account adverse effects, comorbidities, general quality of life, and drug interactions, as well as individual efficacy 2.
Management and Treatment Algorithms
- A panel of epileptologists has provided updated guidance and treatment algorithms for LGS, incorporating recent advancements in ASMs and understanding of developmental and epileptic encephalopathies (DEEs) 4.
- The recommended treatment approach includes valproate as the first-line treatment, followed by adjunctive therapy with lamotrigine and rufinamide if necessary 4, 5.
- Patients with LGS should undergo review by a neurologist specialized in epilepsy on at least an annual basis, including a thorough reassessment of their diagnosis and treatment plan 4, 5.