Q1.  Our 4 year old with Lennox Gastaut appears to be losing information he has learned. He is developmentally delayed  and essentially non-verbal but had been using simple words to communicate. He recently started losing words he had been using for nearly a year. We noticed it after a cluster of seizures one day. Is it possible that the seizures (sometimes tonic, sometimes partial complex) can cause this loss?

The seizure course with Lennox Gastaut Syndrome can widely vary. Very rarely will patients have complete seizure control. Cognitive abilities, behavior, and language often parallel seizure control. The more seizures are controlled, the better the development. Although cognitive regression is not always associated w/ LGS, it is often part of the progressive nature of this syndrome.

Q2.  My son, who is now 13, has had complex and partial seizures from the age of 5. About a month ago, he went in the hospital for a video/EEG monitor series for 4 days. The doctors changed his meds and all seizure activity stopped. The following 3 weeks were spent going to the emergency room with the final trip flying him to a children’s hospital with a severe allergic reaction to what seems to be all his meds--including that which he had been taking for quite some time.

Doctors don't seem to know what to do other than dope him up and and try 1 med at a time. Is this common? Should I find another center to take him to (specialist)? I'm worried about the possible long term effect on his body and mind. They have done the VNS surgery this past week and it has slowed the seizures down, but not stopped them. Any suggestions would be greatly appreciated. Thanks

Epilepsy is a dynamic condition where multiple factors contribute to the success of the medication regimen.  The most common limiting factors are: efficacy, tolerability and safety.  It is very unpredictable how your son will respond to each individual drug, and the systemic administration of medications will allow you to arrive what it is the best combination for him.  The care at a comprehensive epilepsy center may provide therapeutic modalities not be available to your son at the local neurology office. The benefit of VNS is often seen over time and in combination with medications.

Q3.  My daughter’s neurologist brought this up as an option for her at our last appointment. We are trying to get all the  information possible. In your experience what is the success rate in older children (my daughter is 10) who are at a higher functional level. Her IQ is 60, she is at a preschool level in all areas except speech/language. Could this surgery affect her speech/cognitive functions in a negative way or would it even out her skills? I do understand that this surgery is only palliative and may not stop all of the seizures.

Epilepsy surgery has been successful in improving the quality of life for those patients affected with the most medically refractory seizures. An extensive work up is performed prior to surgery in order to establish what type of surgery would be beneficial for your child.  As part of the pre surgical workup speech, language, and other important brain functions will be mapped to prevent the risk of losing skills already acquired.  Expertise of the team involved in evaluating your child for surgery will play a big role in the success of such a complex procedure.

Juvenile Myoclonic Epilepsy is one of the common epilepsy syndromes with a good chance of seizure control. This type of epilepsy usually begins in late childhood or early adulthood. Patients with JME have myoclonic jerks that often occur upon wakening or early morning hours. Others seizure types associated with this syndrome include absence, and generalized tonic/clonic seizures.  More than 80% of those diagnosed with JME are well controlled with medication. There are several treatment options for this syndrome.

Q5.  My son is 5 years old and extremely developmentally delayed (skill sets of a 3 month old). At times I have noticed that  he will show signs of being "out of it" and then have a seizure. Immediately after the seizure he will be more alert then before it. Does this make sense? It kind of seems to me that he needed that seizure to clear his head. Does that make sense?

It is possible that the behavioral changes that you observe prior to a seizure (”being out of it”), represents a manifestation of abnormal electrical activity. One way to evaluate this situation is to use a prolonged ambulatory EEG monitor.

Q6. Can a person that has epilepsy not be hired as alicensed nurse? I would like to know the answer to this question so that I am able to plan my future. I do not want to go to school to be a LPN or an RN and not get hired due to having epilepsy. Also do I need to tell anyone—the school? My employer? or state board of nursing? My seizures are under control. I apprecate in advance you answering my questions, if you are able to. If not, I understand.

The patient that is fully controlled is able to practice and perform in any profession. There are no specific laws for nursing.

Q7. My granddaughter is 23 months and has been diagnosed with LGS based primarily on the slow peak and wave form EEG, and has not responded to Topomax and Kepra. If anything her seizures are increasing. She only has one type,  she raises her eyebrows in rapid succession for 3-10 seconds, sometimes her eyes blink and roll a little bit, sometimes not. She does not lose consciousness and goes back to whatever she was doing before the seizure. We are on a waiting list for the Ketogenic diet. Is what I've told you enough to make a dx of LGS? I just want to be sure were fighting the right demon, and that we are using meds appropriate to her particular syndrome. I would hate to be going in the    wrong direction all this time. It's so very hard to not to get depressed sometimes.

I suggest that you contact the Lennox Gastaut Syndrome Foundation at lgsfoundation.org.  This will be a good resource for you and your family.  Although regression can be seen in LGS, with aggressive management of seizures and implementing therapies, i.e., occupational therapy, physical therapy, and speech early on are known to improve the quality of life for patients and families living with LGS.

Q9. Are you aware of any new research for LGS that give you hope there will be better treatments available for children with LGS in the next few years?

There is a great interest in research for catastrophic epilepsies.  Lennox Gastaut Syndrome includes many neurological conditions with similar clinical presentation. This year the FDA approved a new medication Banzel for the seizures associated with LGS.  The field of genetics is rapidly advancing and other treatment modalities like devices and surgery are in clinical trials.

Q10. I have two children with seizures, both are special needs. The youngest (9 yrs) has LGS since age 3. We have never had much success with medications. We have been on everything and are now going to try Banzel. I am trying to be hopeful but really don't expect improvement. Can you give us any encouragement?.

The results of clinical trials for Banzel and seizures associated with Lennox Gastaut Syndrome were impressive.  Efficacy was found in more than 40% of those treated for drop seizures and with good tolerability. Combination therapy with other medications may provide a desired seizure control for your children. 

Q11.  Please help us. We are thinking of starting our daughter on Felbamate. We have heard about the aplastic anemia side effects, but we are getting to the end of medicine choices. She has LGS and currently takes Depakote, Keppra & Zarontin. She has previously been on Topomax, Lamicatal, Zonegran, & Klonopin. She also has a VNS (didn't really help), tried the keto diet (lasted 1 week - couldn't tolerate it) and is now doing the modified Atkins diet.

We want to get her off the Keppra - even though she has been on it for 6 years—because the behavioral side effects are pretty bad. She does take Vitamin B6 (200 mg) & Risperdal to help with behavior, but it can still get pretty bad. And now she is starting to get more violent. Have you had good experience with Felbamate?

With the right blood work monitoring, felbamate could prove to be a great treatment option for the seizures associated with Lennox Gastaut Syndrome. Your commitment to be diligent about the monitoring will help your doctor with this.  The serious cases described in the literature were older patients with other more serious systemic illnesses, none in the pediatric population. There are patients on felbamate for more than a decade doing well.

Q12.  My 35 year old son has LGS (we're told). He has been taking Banzel for 7 weeks. He is now at 600mg T.I.D. So far, no appreciable positive effect. According to the literature, the target dosage is 3200mg (1800 mg b.i.d.). My son's neurologist said he was not too impressed with Banzel but agreed, at our request, to give it a shot. He seems to  think Vimpat (Lacosamide) might be a better choice. What is your experience with Vimpat?

Vimpat is approved for partial onset seizures in patients 17 years or older.  In clinical trials the safety and efficacy of Vimpat was impressive. In epilepsy there is no universal drug, patients may respond to a new agent when others didn’t work.  It is often in Lennox Gaustat Syndrome we have to combine medications to achieve seizure control.

Q13.  How common are psychological issues in individuals with LGS? There is a bit of general information available regarding adults with LGS who have schizophrenia or psychosis, but there is virtually no information regarding children. Are there measures that should be considered when the individual with LGS is a child, in an effort to help prevent these issues   from worsening as they get older?

The behavior aspect of Lennox Gastaut Syndrome is multi-factorial and related to the seizure frequency, developmental disability, impulse control, and medication side effects.  Not all patients with LGS will have behavior problems.  There is no way to predict or prevent this aspect of the syndrome.  Behavior modification early on will help your child develop better social skills and impulse control.