Lennox-Gastaut syndrome or LGS is a severe and rare form of epilepsy which starts in childhood. Children suffering from Lennox-Gastaut syndrome or LGS have frequent and various types of seizures. The seizures commonly start between the ages of 2 and 6. Patients suffering from Lennox-Gastaut syndrome or LGS will also have moderate to severe developmental delays and learning difficulties. They also may have behavioral problems.
It is not possible to predict the course of this condition in a child with Lennox-Gastaut syndrome, as each child develops differently. Many children have constant seizures and some type of learning disability, whereas there are some children who respond well to treatment and have decreased number of seizures. Also, there are some children who continue to have frequent seizures along with problems with development, thinking, and behavior, and need constant help with daily activities of living.
There is no cure for Lennox-Gastaut syndrome or LGS and this condition is difficult to treat, but new therapies are being constantly developed. The aim of treatment for Lennox-Gastaut syndrome is giving the child a good quality of life with emotional and practical support along with facing the stress and challenges of this condition. Ketogenic diet is a special diet which helps in some patients with this type of epilepsy.
Causes of Lennox-Gastaut Syndrome
It is difficult to find out the exact cause of Lennox-Gastaut syndrome or LGS. Some of the causes behind this condition include:
- Lack of oxygen or insufficient oxygen during birth can cause Lennox-Gastaut syndrome in child.
- Brain infections, such as meningitis, encephalitis and rubella can also cause the child to have Lennox-Gastaut syndrome.
- Acute injuries to the brain in association with pregnancy or birth, such as premature birth or low birth weight.
- West's syndrome or infantile spasms are the seizures, which start in infancy and can lead to Lennox-Gastaut syndrome or LGS.
- Tuberous sclerosis is a condition where multiple non-cancerous tumors are formed in different places in the body, including the brain.
- Cortical dysplasia is a brain problem in which some of the brain nerve fibers fail to develop when the child is in the womb.
Signs & Symptoms of Lennox-Gastaut Syndrome
- First sign of Lennox-Gastaut syndrome or LGS in some children is a constant seizure which lasts for half an hour or continuous seizures without complete recovery between them. This condition is known as status epilepticus and is a medical emergency.
- Children having Lennox-Gastaut syndrome or LGS often have a slower reaction time and some of them will have learning problems and also difficulty in processing information. Behavioral problems are also commonly seen in such patients.
Children suffering from Lennox-Gastaut syndrome or LGS have severe, frequent and different types of seizures including:
- Atonic Seizures: These are also known as "drop attacks," as there is loss of muscle tone which results in the patient falling to the ground. There is also jerking of the muscles. These seizures are brief in nature and often last a few seconds only.
- Tonic Seizures: These seizures are also called "drop attacks" and last from a few seconds to a minute and cause stiffening of the person's body. These seizures often occur when the patient is asleep. If they occur when the person is awake, then it causes falls in the patient.
- Absence Seizures: In these seizures, the patient has a blank stare or blinks quickly or nods their head.
Investigations to Diagnose Lennox-Gastaut Syndrome
- Medical history of the patient is taken where the doctor asks details regarding previous seizures and any family history of seizures.
- Urine tests, blood tests and lumbar puncture to test the cerebrospinal fluid are done to find out any underlying metabolic or genetic cause.
- Other than this, neurological examination is also carried out.
Three important signs which help in diagnosis of Lennox-Gastaut syndrome or LGS are:
- Multiple types of seizures which are difficult to control.
- Intellectual disability or developmental delays in the child.
- Electroencephalogram (EEG) when done, displays a specific kind of pattern known as the slow spike-wave pattern, in between the seizures.
Common Questions which the Doctor will ask are:
- When the problem was first observed?
- Is the child having seizures? If yes, then how many and how frequent?
- How long does the seizures last? And what does the patient experience during a seizure?
- Does the child have any medical conditions?
- Does the child take any medications?
- Does the child have any brain injuries?
- Were there any complications during birth?
- Does the child have any learning or behavioral problems?
Questions you need to ask your Doctor:
- Has the doctor treated other children suffering from this condition?
- Does the patient need any additional tests?
- What is the best treatment for the child?
- How will my child feel during and after the treatment?
- What can be done to keep my child safe during the seizures?
- Are there any clinical trials in which my child can enroll?
Treatment for Lennox-Gastaut Syndrome
- Lennox-Gastaut syndrome or LGS has no cure. Lot of research is being carried out to find new treatments which will work better.
- There are different types of medications, which are prescribed to treat seizures in Lennox-Gastaut Syndrome. The aim of these medicines is to decrease the number of seizures using that medication which causes the least side effects. It takes time to find out the right treatment for the child. Medicines used in treating seizures are: Clobazam (Onfi), rufinamide (Banzel), divalproex sodium (Depakote), lamotrigine (Lamictal ) and topiramate (Topamax). There's no single drug which completely controls the seizures. Close monitoring of the child's medication is required, particularly if the child is taking more than one medicine at a time.
- Diet for Lennox-Gastaut Syndrome: Ketogenic diet is a special diet comprising of low carbohydrate and high fat. This diet helps in seizure management. This diet increases the ketones in the body and helps with the seizures.
- Surgical procedure comprising of corpus callosotomy and vagus nerve stimulation can also be done.
- If the child is having frequent seizures, then he/she needs to wear a helmet to protect from any head injury in case of falls. Parents also have to deal with different behavioral problems of the children, such as the child will act out, and also have to manage their child from different side effects of anti-seizure drugs.
- Each child suffering from Lennox-Gastaut syndrome or LGS has different needs. Many of them continue to have intellectual disabilities and seizures even after they have grown up. Some patients are able to live independently, but majority of them need help with their daily activities of living. Many patients suffering from Lennox-Gastaut syndrome need to live in an assisted living home or a group.
- Parenting a child suffering from Lennox-Gastaut syndrome or LGS can be tough. For this reason, it is important for parents and the rest of the family members to get outside support. Talking with other families who are facing the same challenges helps in reducing the feelings of isolation. It also makes life easier to share tips and other information with different family members who are suffering similar situation in their lives.
Prognosis & Life Expectancy of Lennox-Gastaut Syndrome
- The prognosis for Lennox Gastaut Syndrome is different from patient to patient. The long term prognosis of this syndrome, as far as intellectual development and seizure control goes, is poor.
- There is no cure or any particular treatment for Lennox-Gastaut syndrome or LGS and the patient will continue to have seizures throughout his/her life.
- The IQ of the patient suffering from Lennox-Gastaut syndrome will also deteriorate as time goes on and as the patient grows older, he/she will require assistance from other people.
- Patients suffering from Lennox-Gastaut syndrome will also have behavioral problems, which occur as a side effect of antiepileptic medicines, brain dysfunction and seizures.
- There are some Lennox-Gastaut syndrome patients who will outgrow seizures as they reach adolescence, whereas some patients continue to have seizure episodes till they reach adulthood in which case they will always be dependent on other people for assistance.
- Some Lennox-Gastaut syndrome patients can develop infections, which will result in decreased life expectancy and even poorer prognosis.