This type of epilepsy is uncommon and occurs in between one and five in every 100 children with epilepsy. However, it is the most common cause of intractable (difficult to treat) childhood epilepsy. The most common time for it to start is between three and five years of age.
Symptoms
The seizures experienced can vary.
The most common type is atonic seizures or ‘drop attacks’. During these, the child will suddenly fall to the ground which may at first be mistaken as tripping up or poor balance. These seizures usually happen many times a day and are very upsetting as the child is often injured during them.
Another common seizure type is atypical absence seizures, which can last from 10 seconds to several minutes. During these seizures the child will appear vacant or blank. They may have other features like head nodding, or rapid blinking. The child may have some awareness of what is going on around them.
These seizures can be very frequent and can even merge to be constant, forming a state called ‘non-convulsive status epilepticus’. During these episodes the child will ‘not be with it’, drool, be unable or slow to speak, need help with feeding and be floppy or wobbly.
Another frequent seizure type is tonic seizures. They may only happen at night. There is a general stiffening of the arms and legs.
Children with Lennox-Gastaut syndrome may have other types of seizures including generalised tonic-clonic seizures, partial motor seizures, complex partial seizures and myoclonic seizures.
All children with Lennox-Gastaut syndrome will develop learning difficulties which will be moderate to severe. Some children will have developmental delay or learning difficulties before the seizures start.
About two out of ten children will develop Lennox-Gastaut syndrome after having West syndrome (infantile spasms).
Diagnosis
The diagnosis becomes clearer with time. The electroencephalogram (EEG) is very helpful as typical abnormalities are seen, even when the child is not having any obvious seizures.
Some children may have a cause which can be identified. These include a genetic condition called tuberous sclerosis (a structural brain malformation, brain damage due to problems before or around the time of birth), meningitis as a young baby or a head injury. In about half of all children with Lennox-Gastaut syndrome, no cause can be found.
Treatment
Treatment of Lennox-Gastaut syndrome is very difficult. The drugs which may be effective in some cases include sodium valproate, lamotrigine, topiramate, clobazam and phenytoin.
Sometimes the ketogenic diet may be effective.
Courses of corticosteroid drugs are sometimes used when seizures are particularly difficult to control.
In children who have repeated drop attacks surgery may be helpful. Corpus callostomy and Vagus Nerve Stimulation (VNS) are two surgical procedures that may be considered.
Prognosis (outlook)
The long term prognosis is poor in terms of seizure control and intellectual development.
A very small number of children will outgrow their seizures by their teenage years. However, the remaining children will continue to have seizures, often on a daily basis, into adult life. These patients will be dependent on others for all their life.
Some children will develop problems like repeated or severe chest infections which may shorten their life.
Support organisations
Lennox-Gastaut Support Group, 9 South View, Burrough on the Hill, Melton Mowbray, LE14 2JJ, telephone 01664 454 305.
Tuberous Sclerosis Association, P O Box 12979, Barnt Green, Birmingham, B45 5AN, telephone 0121 445 6970 www.tuberous-sclerosis.org
Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, www.cafamily.org.uk
Because this page is written by an epilepsy healthcare professional and not by Epilepsy Action, it falls outside the requirements of the Information Standard.
Our thanks
Epilepsy Action is indebted to Dr Richard Appleton, a Consultant Paediatric Neurologist who specialises in children’s epilepsy, and to Dr Rachel Kneen, Consultant Paediatric Neurologist and Dr Stewart Macleod, Specialist Registrar in paediatric neurology, at Alder Hey at Alder Hey Children’s Hospital, Liverpool, who have kindly prepared the information on this page.
This information is exempt under the terms of The Information Standard.
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Updated July 2009To be reviewed December 2013
Comments: read the 5 comments or add yours
Comments
Hello,
I thought that I would leave a comment as there is very little support for LGS in the UK at the moment. My Beautiful daughter hasn't been fully diagnosed with LGS, but we have been told this is the case as she suffers multiple types of seizures throughout the day. She has cluster attacks of tonic clonic seizures and 6 - 8 drop fits a day. Also suffers myoclonic jerks morning and night.
We are feeling like we are at the star of a journey. She has been through alot in the last 3 months and tiff is currently on 3 drugs, eplilim and lamortrigne, and runfinamide.
But no luck as of yet. we are hoping that something will kick in and give her a few days fit free. I have contacted the LGS support group on this page and after speaking to Lesley. I feel alot better and know that we are not alone. Also my outlook for Tiffany has changed as I feel the doctors tell you the worse case scenario so much it sinks in. Hoping that the drugs will take effect when they get up to the right level.
Tiff is 3 and half and talks in full sentences, feeds herself and goes to the toilet. We have been told all this will stop. I refuse to let this happen and will continue to keep her independent. Times have been hard but I know this is far from over.
I just hope the future holds a cure.
Jon
Hello
My son George started with LGS aged 4yrs 3months he is now 15.He didn't loose any of the skills he had all ready learnt.He is a very happy boy with lots of friends at school. He went to a small main stream school until year 6 now he goes to a special school and loves it. He can do basic reading and maths and has come on a lot since starting at the special school i wish he had gone there early.He is on 3 drugs epilim chrono ,lamotrigine and clobazam and has a VNS, he still has seizures every night but not during the day unless he is going through a bad patch then he usually end up going into hospital for 2 weeks and has been in intensive care. I hope your daughter is doing ok ,if you want anyone to talk to you can ring me or E-mail.
carol
It breaks me that I am helpless. My daughter hurts herself sometimes during a fit often bleeding.
Hello, my daughter Emily had her first fibril seizure at about 8 months old due to a high fever. We thought that was going to be all. She seemed fine until she was about 2. It seemed as though she had an occasional shiver but soon escalated into obvious seizures. She used to be able to feed herself, talk, sing, play, and was almost potty trained. But LGS robs the life quality of our children! We have tried everything we know to do: She has been on multiple medication and "cocktail" mixtures. At one time she was taking 38 1/2 pills per day with an average of about 10seizures still per day. She is currently taking on a daily basis: Clonapin, Lamictol, Vimpat, Banzel, 100mg of Vitamin B-6, and Diastat (as needed). We put her on the "strict" Ketogenic diet for 2 years and at first we did see some improvement but the LGS proved resistant to the diet. Then she got the VNS (Vegus Nerve Stimulator) Implanted in her chest. NO HELP THERE, so, we went to Cleveland Clinic in hopes of a Miracle and ....nadda:(
Emily is now 7, still wearing diapers, limited speech if any, needs assistance with everything. However, she has the soul of an angel. She is sweet, kind, and extremely loving. LGS a roller coaster ride that never ends! The changes in the severity and types of seizures are so irregular. We also notice “ticks” associated with how this has changed her neurologically. She went through a aggressive period but luckily it was brief. She went through a time of clapping her hands repeatedly, smacking her lips, shrugging her shoulders, pacing, hopping, and them our least favorite; the high pitch repeated shrills. Sometimes even in her sleep. She is now doing two different habitual actions one is saying “yeah” in a rhythmic pattern (which is the only thing we catch her saying currently). The other is a “farting” noise with her hands clasped together. We have had to install multiple gates throughout our house. At the top of our stairs, the bottom of the stairs, the family room…..ect. We are prisoners of our own home, but if we don’t do this…SHE WILL HAVE MORE INJURIES. I can’t tell you how many times she has taken a nose dive down our hard wood stairs. I am so grateful for the permanent swing gates that we found and installed.
It took us seven years to conceive so Emily means the world to us as I am sure your children do to you. I just really feel that this terrible disease has very little awareness and more needs to be done to research a cure. Our children are suffering!
Hello,
My son has LGS, but diagnosis has been really slow. He is 12 now and really only got his diagnosis 18 months ago - and this despite the fact that it was ruled out as a diagnosis when he was 5. We were told he had Severe Learning Dsiabilities and Autism, but his Epilepsy didn't really feaure in discussions as for many years he only had absence seizures that, although they occured 8 or so times every day and could last for 20 minutes, seemed umproblematic and were not affected by any of the drugs that were tried.
He started having other types of seizures 5 yesrs ago - and we have been on a rollercoaster since then. Drugs have been tried and have been ineffective or have been effective for only limited periods. As he grows, he is harder and harder to manage. He is much loved by his siblings - but really hard to manage within the family as his autism and LD require lots of accommodation anyway and his Epilepsy makes things even more exhausting.