Treatment Overview
Living with Lennox Gastaut Syndrome (LGS) is a profound challenge that affects the entire family unit. The unpredictable nature of seizures, particularly “drop attacks” that cause sudden falls, often requires constant vigilance and protective measures like helmets to ensure safety. Beyond the seizures, families must navigate cognitive delays and behavioral issues that complicate daily communication and care. Treatment is vital not only to reduce the frequency of these dangerous seizures but also to improve alertness and cognitive function, allowing the child or adult to interact more fully with their world.
Because LGS is considered a drug-resistant epilepsy, finding an effective treatment plan is often a journey of trial and error. What works for one patient may not work for another, and efficacy can change over time. Treatment plans are highly customized, often requiring a combination of therapies to manage the multiple seizure types associated with the syndrome (National Institute of Neurological Disorders and Stroke, 2023).
Overview of treatment options for Lennox Gastaut Syndrome
The primary goal of treating LGS is to reduce the frequency and severity of seizures, with a special focus on preventing the falls that cause physical injury. Complete seizure freedom is rarely achieved, so the focus shifts to quality of life and minimizing the side effects of heavy medication burdens.
Medications, known as antiseizure medications (ASMs), are the cornerstone of therapy. Because LGS involves multiple types of seizures, a single drug is rarely enough; most patients are prescribed a combination of two or more medications. When medications alone are insufficient, doctors may incorporate dietary therapies like the ketogenic diet or recommend surgical options such as Vagus Nerve Stimulation (VNS) or corpus callosotomy to disrupt seizure pathways.
Medications used for Lennox Gastaut Syndrome
Doctors employ a broad range of antiseizure medications, often mixing different classes to target the brain’s electrical activity from multiple angles.
Broad-spectrum antiseizure medications: Valproate is frequently the first medication prescribed because it treats multiple seizure types. It is often used as a “backbone” therapy to which other drugs are added. Another common broad-spectrum option is lamotrigine, which can help control generalized seizures.
Benzodiazepines: Clobazam is widely used specifically for LGS. It helps calm the brain’s electrical activity and is often added to other medications when initial treatments do not provide enough control. Clonazepam is another drug in this class that may be used for rescue or maintenance.
Newer targeted therapies: Rufinamide was developed specifically to treat the drop attacks associated with LGS. Additionally, cannabidiol (CBD), a purified form of the cannabis plant derivative that does not cause a “high,” has been FDA-approved for treating seizures associated with LGS. Clinical experience suggests that CBD can be effective in reducing drop seizure frequency when added to existing regimens.
Other agents: Topiramate and felbamate are also used, though felbamate is typically reserved for severe cases due to higher safety risks.
Patients should expect a gradual adjustment period. Doctors usually start with low doses and increase them slowly to find the balance between seizure control and alertness.
How these medications work
The brain uses electrical signals between nerve cells. In LGS, these signals are excessive and chaotic, causing seizures. Antiseizure medications stabilize these electrical pathways.
Some, like valproate and lamotrigine, block sodium or calcium channels on nerve cells to prevent rapid firing.
Benzodiazepines and other drugs boost GABA, a chemical messenger that inhibits nerve activity, calming the electrical storm.
Newer drugs like cannabidiol are thought to modulate calcium levels inside cells, dampening nervous system excitability.
Side effects and safety considerations
Because patients with LGS often take multiple potent medications, side effects are common and require careful monitoring.
Common side effects include drowsiness, fatigue, dizziness, unsteadiness, and changes in appetite or weight. Behavioral changes like irritability or hyperactivity can occur, especially with clobazam or levetiracetam.
Serious risks include severe skin rashes (especially with lamotrigine). Valproate poses risks to liver function and blood clotting. Felbamate requires strict monitoring for anemia or liver failure.
Abruptly stopping these CNS-acting medications can trigger life-threatening status epilepticus. Seek immediate medical care for a prolonged seizure, breathing difficulty, or severe allergic reactions (e.g., spreading rash, facial swelling).
Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.
References
- National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov
- Epilepsy Foundation. https://www.epilepsy.com
- Lennox-Gastaut Syndrome Foundation. https://www.lgsfoundation.org
- MedlinePlus. https://medlineplus.gov
Medications for Lennox-Gastaut Syndrome (LGS)
These are drugs that have been approved by the US Food and Drug Administration (FDA), meaning they have been determined to be safe and effective for use in Lennox-Gastaut Syndrome (LGS).