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Lennox-Gastaut syndrome is a severe form of childhood-onset epilepsy. This page discusses the causes, diagnosis, and treatments for Lennox-Gastaut syndrome.
What is Lennox-Gastaut syndrome?
Lennox-Gastaut (LEN-icks gah-STOE) syndrome is a severe form of epilepsy that usually begins in early childhood. LGS typically involves several features, including:
- Multiple seizure types that can be difficult to control with medicines
- Issues with development or behavior (either before or after seizures have started)
- Certain abnormal findings on electroencephalogram (EEG).
What causes Lennox-Gastaut?
Lennox-Gastaut syndrome may be caused by:
- Abnormal brain development
- Certain genetic conditions
- Rare metabolic disorders
- Central nervous system infections, such as meningitis
- Brain damage from lack of oxygen around the time of birth
- Traumatic brain injury
Lennox-Gastaut syndrome may also develop because of earlier seizure disorders such as infantile spasms (known as West syndrome). In some cases, LGS has no known cause.
Is Lennox-Gastaut syndrome inherited or genetic?
While there are many genes that have been identified as being related to LGS, the change (mutation) that results in LGS is usually spontaneous—that is, it is not inherited from a parent.
How is Lennox-Gastaut syndrome diagnosed?
Lennox-Gastaut syndrome is diagnosed based on a thorough physical exam and health history, magnetic resonance imaging (MRI), genetic testing, and electroencephalogram (EEG). A pediatric neurologist looks at the results of a patient’s EEG to find the specific brainwave patterns that indicate LGS.
Children who have Lennox-Gastaut syndrome typically experience multiple types of treatment-resistant seizures that begin in early childhood.
How is Lennox-Gastaut syndrome treated?
Lennox-Gastaut can be treated in a variety of ways. Because seizures caused by LGS tend to be resistant to anti-seizure medication, it is often treated using a combination of different medicines.
Other treatment options for Lennox-Gastaut include:
A vagus nerve stimulator, or VNS, is a small medical device that is implanted through surgery in the left side of the chest, with a lead going up to the vagus nerve in the neck. A VNS sends electrical current to the vagus nerve, which sends the current to the brain. Sending electrical current to the brain via VNS can help reduce the length and number of seizures.
A deep brain stimulator (DBS) is a medical device that is implanted under the skin of the abdomen or chest, with a lead implanted in the brain. Similar to VNS, deep brain stimulation delivers electrical impulses to targeted areas of the brain to reduce seizure activity.
A medical ketogenic diet is a high-fat, low-carbohydrate diet that forces the body to use fat as fuel—a state known as “ketosis.” It is different from the fad keto diet and needs to be medically supervised and strictly followed to maintain a state of ketosis. Being in ketosis has been shown to reduce seizures in some children who have complex epilepsy like LGS.
Children who are candidates for the diet can eat by mouth, use feeding tubes, or use a combination of both methods to take in nutrition.
Some children may benefit from surgery to help control their seizures that have not responded to other treatments. Surgeries that can help control epilepsy like Lennox-Gastaut syndrome include:
- Resective epilepsy surgery, where the part of the brain affected by seizures is partially or fully removed
- Corpus callosotomy (also known as callosal sectioning or split-brain surgery), where the band of nerve fibers that connects the two halves (hemispheres) of the brain is cut. This stops seizure signals from going back and forth between the brain's two hemispheres.
- Hemispherectomy, a rare procedure which disconnects or removes (partially or fully) half of a child's brain to bring seizures under control.
Why Choose Gillette Children's for Lennox-Gastaut Care
When you come to Gillette Children’s, you have access to our board-certified pediatric neurologists and advanced practice providers who will work with your family on designing an individualized care plan for your child’s unique needs.
Gillette Children's is proud to be a Level 3 Epilepsy Center, as designated by the National Association of Epilepsy Centers (NAEC). This designation reflects our commitment to excellence in the care and treatment of patients who have epilepsy.
Our status as a Level 3 Epilepsy Center means that children who come to Gillette for epilepsy care benefit not just from our expert neurology care, but also the neurodiagnostic, neuropsychology, and psychosocial services necessary for successful long-term management of epilepsy.
Gillette's EEG lab is accredited by the American Board of Registration of Electroencephalographic Technologists (ABRET). This accreditation highlights our neurodiagnostic lab's independent review against national standards for equipment, staffing qualifications, safety protocols, and quality of recordings/interpretations, and has been verified to meet those standards.
The advanced capabilities of our imaging services include 3 Tesla MRI, a scanner that is twice as powerful as standard MRI scanners (which operate at 1.5 Teslas).
3T MRI offers much greater detail in imaging results, which allows providers to get a more thorough look at the brain, blood vessels, and tissue of children who have complex epilepsy.
Our Keto Diet Clinic—staffed by our experts in neurology, complex care, nursing, and pharmacy services—assists with the evaluation, implementation, and ongoing monitoring support for pediatric patients recommended for the ketogenic diet to manage and reduce their seizures.
From initial hospitalization to begin the diet, to family education about keto-compliant nutrition and medicine, to continuous medical monitoring and adjustment, the Keto Diet Clinic at Gillette is here to support patients and their families as they adapt to a new way of eating for epilepsy management.
The information on this page is reviewed by Gillette Children's medical editors for general educational purposes.
