Lennox-Gastaut Syndrome: A Severe Form of Epilepsy
Live Chat with Expert, Jack Pellock, M.D.―October 29, 2009
Dr. Pellock is Chairman of the Division of Child Neurology, Vice Chairman of the Department of Neurology, and Professor of Neurology, Pediatrics, Pharmacy and Pharmaceutics at the Medical College of Virginia, Virginia Commonwealth University Health System, in Richmond, Virginia.
| I’ve heard some parents say it took years to get a diagnosis. What makes LGS so difficult to diagnose?— Carol |
| Answering this for most cases, I would say that there is usually a progression in that seizures do not get completely controlled and the intellectual impairment continues. However, with new treatments, we may see better prognosis if better seizure control is possible. |
| I know that this is a very sensitive topic but I was wondering if there is an increase in SUDEP among LGS patients, due to the uncontrolled nature of the seizures?—Phyllis |
| SUDEP occurs more frequetnly in those with intractable seizures. Thus, kids and adults with LGS are more at risk. |
| What are the most effective treatments that you have seen?—Stacie Brass |
| Effective treatments include valproate, lamotrigine, topiramate rufinamide and felbamate. Other anticonvulsants have also been used but not as well studied.Also the ketogenic diet should be considered and VNS |
| What about frizium? How does that rank?—Julie |
| Clobasam is not FDA approved, but certainly has a place in therapy, It appears to be better than the other benzodiazepines for long term use, but can also be used for seizure emergencies. |
| I have heard such promising outcomes for the kids who were fortunate to have IVIG who have a devastating seizure disorder. How can I get this treatment as anoption for my child?—Julie |
| Results of IVIG are generally disapointing from larger studies, but a few children have had remarkable benefits. |
| Are the EEG's different for LGS verses MAE (Doose syndrome)? I was told my child could have one of these, but since he has not fully declined mentally, unless he is in non-convulsive status, they are being conservative and say presumed MAE. I have heard such promising outcomes for the kids who were fortunate to have IVIG with a devastating seizure disorder. How can I get this treatment as an option for my child?—Julie |
| Yes EEGs differentiate the 2. |
| In your experience is there a relationship between food and seizures with LGS? or would it be additives? or perhaps gluten? or is there really no relationship?—Phyllis |
| The effects of food additives and the like are difficult to ascertain. I feel that if a certain substance increases seizures it should be avoided and only added back in limited amounts to see if seizures really change. The only proven effect of food is with ketogenic and similar diets. |
| I have heard conflicting information about VNS. How safe is it for children? And what is the success rate?—Michelle |
| VNS has been used in many children and is as safe in kids as adults. The success is variable and overall is about as effective as using a new medicine. |
| Does LGS affect life expectancy?—Lauren Drake |
| Persons with multiple handicaps are more at risk to have serious complications from infections, accidents and even sudden death. So, yes, LGS and the associated handicaps may be expected to shorten one's lifespan, but not always. |
| In your experience, do the seizures calm down a bit when the child reaches his late teens or twenties?—Phyllis Reed |
| Persons with LGS undergo changes in their seizures throughout their lifetime but usually do not become completely seizure free. |
| Is 4.5 years a good enough run for the ketogenic diet without seizure control?—Julie |
| In my opinion yes, but this needs to be discussed with your physician. |
| If meds aren't working, do you ever advise just going med-free?—Lauren Drake |
| Going med free is a risky situation. If tried, it should be done with a plan for emergencies, including possible prolonged seizures |
| Have you seen a special diet effect for treating LGS?—Tiffany |
| The ketogenoic diet and modified Atkins |
| Have you seen any particular techniques that have proven effective for language development in young children? Do children with LGS generally learn to talk?—Stacie Brass |
| Some children with LGS can speak whereas others have mutiple handicaps and development levels that make this less likely. |
| We have tried every available drug for my son, and the keto diet. His daily seizures are so awful, they make him cry, and he often bites his thumb through the skin. Do we just have to accept this?—Lauren Drake |
| Sometimes seizures are very difficuot to control. The behaviors of biting must aso be worked on and that might be through a behavior program. |
| Are there very many LGS experts in the US? How do you find them?—Tiffany |
| Most child neurologists who specialize in epilepsy have a special interest and very much keep up to date with treating LGS |
| So are you saying that even if meds don't appear to be controlling seizures, they may be providing some protections against more severe, more damaging seizures?—Lauren Drake |
| That is correct. |
| In light of the recent H1N1 concerns, are there any reasons why the LGS population should not get the vaccine?—Phyllis Reed |
| The CDC recommendation for immunization of persons with epilepsy for H1N1 is most appropriate for those with LGS. The AES and Pediatric societies have endorsed this recommendation. |
| The basic descrption of LGS, does that always include a severe mental decline?—Julie |
| Mental decline in children with LGS is directly related to seizure control , underlying cause and original developmental level. So it is difficult to give an absolute answer |
| If we are seeing good control at 5 yrs old and he is walking/talking with some developmental delay...can we be hopeful for a "better" outcome?—Tiffany |
| Children and adults who respond better to treatments have an overall better outlook. That is why we keep attempting to improve treatments and re-evaluate for the potential for other new treatments or medicines. |
| What are some of the common underlying causes of LGS?—Stacie Brass |
| LGS can be associated with many causes, just as epilepsy is. The difference is that some epilepsy is time limited and seemingly runs in families without causing any neurological handicap. |
| Does that change if our child is getting IVIG and a every other week high dose steriod injection? Should she still get the H1N1 vaccine?—Stacie Brass |
| Infantile spasms and other brain injuries in youg children frequently lead to LGS so there is a large list of disorders. |
| When a child transitions from infantile spasms to LGS, do you see any additional mental decline, where the child is already severely delayed? Or is LGS really just "more of the same"?—Lauren Drake |
| Difficult to say, but depends upon the ability to control seizures and allow some development to occur. |
| Why are many children with poor seizure control so delayed? Do the constant seizures cause actual brain damage?—Lauren Drake |
| The answer to this question is difficult and is like the chicken and egg problem. Seizures occur when the brain is not functioning correctly, but more and more seizures do not allow normal development so both are responsible for outcomes. |
| Have you seen the postictal bradycardia in children/adults with LGS? Is this typical after seizure clusters?—Denae |
| Heart rhythm changes have been observed with mutiple seizure types.Depending on the extent of these changes they should be discussed and perhaps further evaluated by a physician. |
| Is kindling a problem with LGS patients? or are the seizures too varied to cause kindling? could you explain kindling?—Phyllis Reed |
| Kindling refers to making normal areas of the brain epileptogenic--that is able to produce seizures from a new area by itself. It refers to focal seizures. LGS is a generalized epilepsy and the underlying brain is totally at risk and frequently involved with seizures already. So kindling is really not discussed in this situation. |
| What is your opinion on the use of felbatol....if all other drugs have not controlled the seizures?—Joan Sgarlata |
| For some patients with LGS felbatol has played an important part in helping to control some ofther seizures. It must be usee under proper situations with appropriate clinical monitoring |
| For children that are on multi drug cocktails, will the interactions and side effects change over time or will any negative side effects and interactions occur in the first month of the addition of another drug?—Phyllis Reed |
| All of your suggestions may be true depending on the individual medications and changes made. |
| What is the most AED's you would use at one time to try and control seizures?—Denae |
| We typically try not to use more than 3 medications chronically. But that said with additions and subtraction numbers do grow and we must then work to rmove some therapies |
| Does aplastic anemia occur without warning during use of felbatol ? what types of monitoring are you referring to?—Joan Sgarlata |
| The aplastic anemia that occurs with FBM is most common in the first 6 to 12 months of therapy, rarely thereafter. Following the patient clinically when there may be any sign of increased infection, bruising, bleeding or just becoming ill during that time period may be ther most important thing to follow. Blood tests then can confirm whether any changes such as anemia have occurred. There is no absolute way to do this where all physicians agree. |
| What is the number one med you have you had the best success in prescribing?—Julie |
| Rather than my #1 med I'll tell you that valproate is the world wide prefered medication for the initial treatment of LGS. This is because of reported experience. We certainly do not have the perfect agent and that is why treatmnent varies. It is different for different persons because of their predominant seizure type and may actually change over the years in the some individual |
| How would you make a decision as to which medication to decrease if a patient was on 5 medications?—Phyllis Reed |
| I personallly ask caregivers the question of which med was the best or did the least. That helps us all decide on which to attempt to remove. One problem is that almost any change can lead to improvement or decline in the short run and good record keeping, observation and perseverance is required. There is a tremendous variablity in seizure frequency over time. |
| Is it possible for LGS to survive into adulthood or become misdiagnosed by neurologist specialists (Professors)?—HeadStorms |
| Yes. One must look at the entire history of epilepsy including the time of onset and seizure types throughout one's lifetime to diagnose epilepsy syndromes, including LGS. |
| How often should labs be done after the fisrt year?—We do it every month. Denae |
| Sometimes we don't have the advantage of seeing earlier EEGs but that also helps. |
| What is your experience with VNS?—Stacie Brass |
| Our experience with VNS is that it has helped some of our patients with their drop spells and other seizure types, but not all benefit. |
| Is it true that when weaning off some medications you can go through "withdrawal" where the seizures increase?—Michelle |
| Absolutely, Seizures may increase or decrease with medication changes. Adding or subtracting. |
| How prevalent are psychological issues in individuals with LGS?—LGS Foundation |
| LGS patients frequently have multiple handicaps and intellectual disabilities. That being said, behavor difficulties are much more problematic thatn in the general population.Special school and behavior programs are an important part of the total care of these individuals. |
| Is there a point in age when certain seizure types can change and some eventually stop over the yrs?—Natalie |
| Some studies suggest that partial seizures become more prominent as persons get older but they continue with drop spells and sometimes tonic seizures seen at night. |
| Can LGS patient eeg's ever show normal?—Julie |
| It is exceedingly rare for an EEG in LGS to be called "normal" |
| Is there any benefit to trying drugs that didn't help before a second time? Like several years later?— Lauren Drake |
| Yes, over time seizures and responses may change and different combinations may work better at different times in ones life. |
| What if you have never tried either of the two, banzel or felbatol . . .which would you prescribe next?—Julie |
| I today would probably try Banzel first, because of the side effffect profile, but I would need to know much more about the individual patient and circumstances, allergies, seizure type, etc. |
| How does LGS differ from other forms of childhood epilepsy?—Epilepsy Foundation |
| LGS is one of the prototypical "encephalopathic childhood epilepsies". |
| It is 5:30PM (EST) and we will be closing out this live chat session. Thank you, Dr. Pellock for your time and we appreciate all that have joined today's live chat. Epilepsy Foundation |
| I also want to thank all of you who have submitted these excellent questions and helped all understand more about LGS. Thanks to the Epilepsy Foundation staff for their hard work. And, most importantly, thanks to all of you who care for these children and adults. Have a good evening |
