How is LGS Treated?

Treatment options vary for individuals living with Lennox-Gastaut Syndrome. The goal of all treatments is seizure freedom but this is often not possible. The goal then becomes; the best quality of life for the LGS patient and his/her loved ones.

This can occur by trying to minimize the seizures, treatment side effects, and number of medications. Although there is no cure for Lennox-Gastaut Syndrome, numerous treatments for seizures are available.

It is the LGS Foundation’s mission to fund research in new therapies for LGS. Learn about our research initiatives here. Below are the treatment options available.

Watch: The Latest Treatment Options for LGS

Scott Perry, MD, Medical Director of Neurology & Genetics Epilepsy Center at Cook Children’s Hospital updates us on the latest treatments available for those living with Lennox-Gastaut Syndrome. Dr. Perry also offers insight on how your medical team can determine the best options for your loved one with LGS.

Treatments

There are several ways to treat LGS.

Anti-seizure Medication

Medications can treat LGS. By significantly reducing the seizure frequency, but they do not eliminate seizures.

Valproic acid is a first-line medication. Many other medications have been shown to be effective, including several antiseizure medications such as felbamate, rufinamide, lamotrigine, topiramate, clobazam, cannabidiol, and others.

Side Effects

All seizure medications can be associated with fatigue and sedation. Other side effects are specific to certain medications and treatments. Speak to your doctor about side effects before deciding on treatment.

Speak to your doctor about medication options and their side effects.

Dietary Therapy

The ketogenic diet can be effective in LGS. It can reduce seizures by 50% in more than half of children. The diet is:

  • High fat
  • Adequate protein
  • Low carbohydrate

There are various types of the ketogenic diets. The classical diet is the strictest. It is used mostly in infants and toddlers. Milder versions include the modified Atkins or low-glycemic-index diets. These are better options for school-aged children, teens, and adults. Dietary therapy should be prescribed and monitored by an experienced dietician.

Surgery

Corpus callosotomy surgery can help with atonic seizures in most LGS patients. This involves separating the brain tissue on the two sides of the brain. It may be used if atonic seizures persist despite medicine and diet therapies.

In rare cases, LGS is caused by a localized brain abnormality, and sometimes surgery on that tissue can lead to fewer or no seizures.

Learn more about Epilepsy Surgery >

Neuromodulation

Vagus nerve stimulation (VNS) involves placing a device in the chest. The device sends mild pulses of electricity to the brain via the vagus nerve. Several studies show VNS can reduce seizures. It can reduce them by 50% in more than half of patients.

Other management issues

Children with LGS can often benefit from ongoing physical, occupational, and speech therapy. In school, they usually require an Individualized Education Plan (IEP), which allows the school curriculum to be targeted to their specific needs.

Given the high medical needs of persons with LGS, families often require some type of respite care. Working with a knowledgeable social worker is important to identify resources to support families.

Costs

A 2015 report showed that average yearly medical costs are about $64,000 for LGS. This number depends on insurance status. Hospitalizations and home health services are a big expense. Many medication options are generic and are relatively inexpensive. Newer medications, such as cannabidiol, are more expensive but are generally covered by insurance.

Sometimes, a therapy is denied insurance coverage initially. However, working with a healthcare provider to appeal the decision can help.

Outlook

LGS always persists into adulthood. Certain types of seizures are more common in adults than others with LGS. Over time, those with LGS experience developmental slowing, plateauing or regression. This culminates in moderate to severe intellectual disability in more than 90% of patients. Behavior disorders are common in childhood and adolescence.

Untreated seizures can lead to head injury and a higher risk of sudden death. LGS requires lifelong antiseizure treatment.

Related Disorders

These disorders can sometimes be mistaken for LGS:

  • Infantile spasms syndrome. This may progress to LGS. It can be hard to tell them apart. LGS seizures look different from spasms, however.
  • Myoclonic-atonic epilepsy (or Doose syndrome). In this disorder, seizures are preceded by normal development. Persons with Doose syndrome typically have a unique seizure type called a myoclonic-atonic seizure, with a brief jerk or vocalization that precedes the fall. It is often outgrown in early childhood.
  • Dravet syndrome. Dravet syndrome typically presents with recurrent prolonged seizures in the first year of life. It has its own brain and seizure pattern.
  • Other early-onset DEEs. Like LGS, other DEEs include multiple types of seizures.
  • Frontal lobe epilepsy. This has its own brain and seizure pattern. The seizures often have asymmetrical features.

Research

ClincalTrials.gov for Lennox-Gastaut Syndrome lists clinical trials that are recruiting or will be recruiting. Updates are made daily, so you are encouraged to check back frequently.

ClinicalTrials.gov is a database of privately and publicly funded clinical studies conducted around the world. This is a resource provided by the U.S. National Library of Medicine (NLM), which is an entity within the National Institutes of Health (NIH).  The listing of a study does not mean it has been evaluated by the U.S. Federal Government. Please read the NLM disclaimer for details.

Before participating in a study, you are encouraged to talk to your health care provider and learn about the risks and potential benefits.

Find Clinical Trials For Lennox-Gastaut syndrome →

Thank you to the Child Neurology Foundation for allowing us to adapt this article for this site.

Authors: Shaun Ajinkya, MD; Elaine Wirrell, MD,  Mayo Clinic – Rochester, Minnesota Reviewed: April 2021

The information here is not intended to provide diagnosis, treatment, or medical advice and should not be considered a substitute for advice from a healthcare professional. Content provided is for informational purposes only. LGSF is not responsible for actions taken based on the information included on this webpage. Please consult with a physician or other healthcare professional regarding any medical or health-related diagnosis or treatment options.