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Lennox-Gastaut Syndrome

Introduction

Lennox-Gastaut syndrome (LGS) is one of the most severe forms of childhood epilepsy and is characterized by several different seizure types and developmental delay. It typically develops between one and eight years of age, although the average onset is 3 years. There are several known causes for LGS, such as brain injury associated with the pregnancy and birth, severe brain infections (encephalitis, meningitis, toxoplasmosis, and rubella), genetic brain diseases (tuberous sclerosis, metabolic brain diseases), rare childhood diseases, and developmental malformations of the brain. However, in one-third of the cases, there is no known cause for LGS.

LGS was first described in 1770 by Dr. Tissot when he reported an 11 year old boy with frequent drop attacks, myoclonus, and progressive functional impairment. In 1938, the classic EEG pattern was identified and in 1950, Dr. Lennox found and described a clinical correlation between the typical EEG pattern and patients with multiple epileptic episodes.

 

Features and Characteristics

Seizures

Seizures are always of several different types. The most common seizures associated with LGS are tonic, atonic, and atypical absence seizures. The frequency may vary from 9 to 70 seizures a day. Often times, LGS begins with infantile spasms, where the arms stretch out, the head may nod forward, and the eyes look upwards. Infantile spasms typically develop around ages three to eight months and then evolve into the mixed seizure pattern seen in individuals with LGS.

Status epilepticus (a term used to describe a seizure that lasts 30 minutes or longer, or repeated seizures without regaining consciousness) occurs in approximately 50% of those with LGS. The seizures may persist for days, weeks, or even months. Factors which can lead to "status" include sudden withdrawal from medication, illness, fever, and infections. Status is usually nonconvulsive in children with LGS. It is characterized by confusion, stupor, or dementia, and greatly impaired balance and coordination. Convulsive status, on the other hand, may lead to brain damage if not treated immediately. Often times, Valium is given to control these types of seizures.

Developmental Delay

Most children with LGS experience some level of impaired intellectual functioning. Approximately two-thirds of children with LGS show signs of intellectual disability either before or at the time of diagnosis. Other children tend to show signs within a couple years of the onset of the seizures. Some causes of impairment may be the underlying condition causing the epilepsy, the sedative effects of the anti-epileptic medication, and the abnormal electrical activity of the brain from uncontrolled seizures.

Behavioral Disturbances

Behavioral issues include poor social skills and attention seeking behavior. This may be the result of the condition causing LGS, the effects of the medication, uncontrolled epilepsy, or difficulty with interpreting information and a lower level of understanding concepts.

 

Diagnosis

LGS is diagnosed by some or all of the following: the presence of a mixed seizure pattern, some degree of developmental delay or intellectual disability, and an EEG (slow spike-and-wave discharges are typically seen).

 

Treatment

LGS is one of the most drug-resistant forms of childhood epilepsy. As a result, seizures are treated with anticonvulsant medication, however, complete seizure control is not always achieved. It is difficult to find the best dose and the type of medication, and some drugs lose their effectiveness. A combination of drugs are often used, the most common being sodium valproate (Epilim), clonazepam (Rivotril), clobazam (Frisium), and clobazam ethosuximide (Zarontin).

Steroids may also be used to control seizures. The two most commonly used steroids are ACTH and Prednisolone. Side effects may include increased appetite, weight gain, irritability, high blood pressure, high blood sugar, and low blood potassium. Treatment is usually only for a period of a few weeks. When the steroids are discontinued, the side effects disappear.

The ketogenic diet is also sometimes used to treat seizures. It is high in fat and low in carbohydrates. As with any special diet, there are potential side effects of the ketogenic diet including a deficiency of essential dietary requirements. However, it is the most effective dietary treatment for epilepsy.

Surgery may become an option for those who are not responding to anticonvulsants and have one specific developmental abnormality of the brain.

Vagus Nerve Stimulation (VNS) has proven to be helpful for several children with intractable epilepsy. VNS is a mild electrical stimulation of the vagus nerve (the nerve that carries information to the brain). A small generator (similar to a pacemaker), which sends a pattern of stimulation to the vagus nerve through a lead, is implanted under the skin in the upper left chest. The lead is attached to the left vagus nerve in the lower left side of the neck. The generator can then be programmed with different frequencies of stimulation to meet the needs of the individual. A recent study (Lundgren et al, 1998) showed that 6 out of 16 children treated with VNS had at least a 50 percent reduction in seizure frequency. In addition, the study indicated that VNS reduced seizure severity and improved quality of life. Another study (Ben-Menachem et al, 1999) reported that five of eight patients with LGS had greater than a 50 percent reduction in seizures. Since VNS is a new form of treatment, long-term side effects are not known. However, short-term effects include tingling in the neck, hoarseness, throat pain, ear pain, tooth pain, and in some cases, nausea and vomiting.

 

What to Expect

For most people, LGS is permanent; complete recovery from seizures and normal development are not likely. However, approximately 5 percent of those with LGS will outgrow their seizures by teenage years and will attain normal or near normal intelligence and abilities. The following factors can be associated with a poorer prognosis: onset before the age of three years, LGS is preceded by infantile spasms, high seizure frequency, frequent status epilepticus, and an EEG with slow and persistent background rhythm.

The disorder typically persists into adulthood, causing behavioral issues in 25% of those affected by LGS. Almost all adult individuals with LGS have disabilities such as learning issues, loss of previously developed skills, language difficulties, and impaired organization of movement. Approximately 50% of chronically-affected adults are completely dependent, while 17% are completely independent.

What’s most important to know in terms of a prognosis for your child, however, is that all children are affected differently and the range of severity varies from child to child.

 

Personal Stories

My name is Gayle, mother to Christopher who is 10 years old. He was diagnosed with Lennox-Gastaut syndrome at age 2 years. I noticed the seizures when he was 22 months old, but after much personal searching and questioning, I actually believe he was having them during sleep at least 4-6 months before that time. I believed at the time that he was experiencing night terrors. I now believe it was seizure activity that woke him since he would tremble uncontrollably and be very difficult to get back to sleep. He was diagnosed mostly by the typical slow spike waves on his EEG. He was started immediately on anticonvulsants or antiepileptic drugs, of course, with little effect - typical of LGS. From there we tried ACTH injections for approximately 8-10 weeks and then the Ketogenic diet, both with limited success.

He is currently on Felbatol and Topamax for seizures and Inderal for behavior issues. He still has seizure activity on a daily basis and larger seizures every one to two weeks. This is the best control he has ever had. When he was four years old, I asked his neurologist what his prognosis was, and he told me that they would probably never get 100% control of seizures, but that when he reached his twenties, they may be reduced to a few a month. He also said that he would probably have to live in a group home because he would never be independent.

Christopher is currently at age 3 or 4 years developmentally, except with language. His motor skills are much more developed, but with very limited impulse control (meaning he has little to almost no fear, and has been known to wander and get into a heap of trouble). He also has autistic behaviors, except that he is very social and lovable. He has the most beautiful smile and is almost always happy. Now that he is a bit more verbal, he talks or sings most of the time. Of course, we don't always know what the conversations mean. But, after so many years with virtually no communication, I welcome anything. You can only imagine the joy when he actually puts a few words together that actually convey something he wants or needs! And, you can bet he usually gets what he has requested! He is not toilet trained, despite all our best efforts.

The thing that is so interesting about this syndrome is that every child is different and has different complexities. For some, the most difficult thing is the constant seizures. For us, it is more just the constantness of his care. He can never be left without a trained eye watching out for his safety (and ours for that matter). He also has some tough behavioral issues and can be quite aggressive at times. These are the most challenging aspects of this syndrome for us, not the seizures (although they are also a constant in our lives).

What do I love most about Christopher? It is of course his joy, his exuberance for life, his determination to go after something that catches his eye, and most of all, his absolute unconditional and never-ending capacity to love! He is a blessing to our family and anyone who takes the time to know him. - Gayle Parks


We did not have a diagnosis for several years, and that was probably the most frustrating thing! I did not like the pediatric neurologist that was treating my child, so I sought out another one, who was some distance away. I can't stress how important a good relationship between the parents and pediatric neurologist is!

Our daughter was normal at birth (normal apgar scores) and had normal development until her 3rd DPT shot. Immediately, she slept for 13 hours and awoke fussy. She did not have a high fever, just a slight one. Within 48 hours, she started having infantile spasms, which appeared like an infant startle reflex, but different because her eyes rolled back in her head. It was determined she was having seizures. (We have filed a claim with the National Vaccine Compensation Program.) Immediately, phenobarbital was started. All development stopped, and she lost what she had attained to that point. More drugs were added, and she was basically a zombie; no smiles and no eye contact for at least a year. She also cried a lot. Over the years, the various drugs had different side effects, some of them behavioral, some of them life threatening. That has been such a "roller coaster" for us.

Shortly before my daughter turned 2 years old, my husband left our family. There is a high rate of divorce amongst couples with LGS kids. Luckily for my daughter and me, I remarried 2 years later. The 2 years of being a single parent were very difficult.

Many LGS kids have sleeping problems. We had years of poor sleep; she would basically catnap. I believe her seizures would wake her up. Her brain waves were much worse on the sleep portion of her EEG. We went through a lot of different solutions to try and improve this. One thing that helped was a special weighted blanket (available through an occupational therapy supply company) that weighed 25 lbs. It was comforting to our daughter, and she was not able to get up.

Our daughter was tactile defensive and occupational and physical therapies were very beneficial. We saw big gains with this. We also did alternative therapies such as: patterning (Doman-Delacato), acupuncture, chiropractic, cranial sacral, etc., with added success. We also did the ketogenic diet for about one year.

After a week long status epilepticus, Lauren lost her ability to walk, swallow, hold objects, and move her own bowels. This took us by surprise. The doctors do not dwell on the potential for losses like this. It happened when she was 9 years old. She is now in a wheelchair and is G-tube fed. Prior to that, she enjoyed food (finger fed herself) and was able to walk and run. She has always been in diapers.

At 10 years old, Lauren was implanted with a Vagus Nerve Stimulator. The LGS cases who have received implants have a much higher rate of success with this device than the general epilepsy population. We have seen a 90% reduction in her seizures. The results are slow to begin with, but increase over time. I highly recommend investigating this device for anyone with an LGS diagnosis. We've been able to reduce her medications and we've seen an increase in cognitive skills. After a year with the implant, her swallowing improved and she was able to move her bowels on her own again. We were not sure if those functions would return again.

What we love best about our daughter is her "pure essence." She is often described as pure love. She does not have an ego, and is very loving. Since we removed the drugs that caused aggression, she doesn't exhibit any "bad" behaviors. The most frustrating thing is that she has always been non-verbal. It's so hard to know what is wrong. You have to really learn to read her non-verbal cues, which are very subtle. - Paulette Lane


"Kayla" is a beautiful, happy, five-year-old girl, soon to be six. She greeted the world at 37 weeks gestation, weighing 4 lbs. 14 oz with apgars of 9 at one minute and 9 at five minutes. I experienced hypertension and Kayla had intrauterine growth retardation (IUGR). One ear of Kayla's did not fully form (a problem known as microtia), however, neurological problems are not usually correlated with this issue. My husband and I were ecstatic and Kayla progressed normally for several months.

Around 6 months of age, Kayla started having crying spells where she was inconsolable. Usually an easy-going, happy baby, I thought it was teething. At nine months of age or so, I was studying the developmental baby books to see if she was within range of "typically developing" children. The pediatrician kept responding that all was fine when I asked her questions about Kayla's progress. Around twelve months of age, I saw an ad for a local child find where they evaluate your child to see if they might have any delays. My concerns were confirmed; they saw significant delays in her development.

I switched pediatricians and was waiting for our first appointment when Kayla began waking up at night crying and jerking in her crib. In panic, I asked to be seen earlier by the developmental pediatrician. He had us video the events, and he thought they looked like infantile spasms. He, too, confirmed that our child had global developmental delays.

Referred to a neurologist, Kayla quickly was admitted for three days of thorough testing in the hospital (EEGs, an EKG, an MRI, blood work, a spinal tap, a skin biopsy) and we had to repeat "our story" 15 different times (I think they were looking for inconsistencies).

The bottom line: "abnormal EEG" and a prescription for valproic acid. This was the start of the medication cycle and continued decline of our beautiful daughter. She developed a mixed seizure disorder, and at the home day care where she had been since I returned to work, was isolated to a playpen because of her frequent falls.

At first we were given no diagnosis. The neurologist told us that he did not know what was wrong and that only time would tell. He did not outline a plan, and was reticent in sharing information. He soon added Dilantin and we weaned the valproic acid. His reluctance to treat us as intelligent partners in the decisions surrounding our daughter’s care led us to request a referral to a new neurologist. Two years after the first series of tests and doctors visits, Kayla was officially given the diagnosis of LGS. By then, our medication trials included the addition of Lamictal to Dilantin, mono-therapy with Dilantin, and then 2.5 years on the ketogenic diet. There was another medication or two in there somewhere, but I can't recall them now.

Except for the valproic acid, each medication would work to control Kayla's seizures for a brief period of time, but then she would begin breaking through. The ketogenic diet was a desperate measure for us that proved to be great for Kayla. We were able to completely wean medication and control all clinical seizures for the duration of the diet. We stopped the diet because of cheating and foraging by Kayla, and because we had seen some indications of possible seizure activity. We elected to try Topamax and are having great results. Subsequent steps will most likely include the Vagus Nerve Stimulator.

Kayla is almost six years old, and is entering an integrated kindergarten. She is primarily non-verbal, but is developing some vocal approximations for some words (bi - bike, ba ba - bye bye, mu - more, ya ya- yes, na - no) and a few signs. She is a happy little girl with a huge smile and no fear of anything or anyone. She is very affectionate and loves music. She can carry a tune quite well, using her own "words" to sing the tune. One of her latest favorites is "Oh my darling, oh my darling, oh my darling Clementine …"

She has mild hypotonia, functions between 18 - 24 months in most skill areas, but with constant repetition, is clearly learning new skills. Her receptive language has shown considerable improvement over the past year. She is not potty trained, but we are working on having her sit still on the potty. She shows little interest in TV or books. Favorite toys are those that play music or have some cause and effect (balls, cars, noisy games).

I spent many months angry at life for what seemed at the time, to be ruining my child. When a parent learns that they have a child with a disability, they go through a mourning process for the child they lost. You see, we all imagine that our child will be healthy because we take such care to ensure our pregnancy goes well. We eat right, get regular medical care, they are born healthy and then WHAM! At first this is all consuming, and then we start to move on. Occasionally we are stuck again, sometimes when we least expect it. Once I was sitting in traffic and a little girl skipped by with her mom, laughing and talking. That little girl looked to be about my daughter’s age, and I just started sobbing in the car.

It is important to note that it is not all sorrow. Kayla is a wonderful "teacher" and has changed me in a way that nothing else could ever have done. I take nothing for granted and find joy in small moments. I celebrate her successes and am thankful for the opportunity to share her life. She has taught me understanding, patience and compassion as no one else has or ever could. As I wrote in response to some negative press regarding special education children, it will never be my child who speaks an unkind word or deliberately hurts someone. There is much to rejoice in that truth!

As stressful as my job is, I know it is just a job, although several years ago it was a career and essential to my being. I don't know how long I will be able to keep up a job, my family, and Kayla's care. I just take it as it comes. Trust me, some days I wish wouldn't come, but there is always tomorrow and the possibility of a better day.

I have many new friends, and a few of my old ones - the ones that really were my friends still are and I know will always be my friends. I find the world in general to be a cruel place, with pockets of warmth and caring. I'm learning that the direct approach to strangers is the best; "Yes, my child is developmentally delayed, non-verbal and tired. What is your problem?" Nonetheless, finding the snappy come-back is not always easy; we too have "bad" days.

The best advice I can give is live in the present. Work at your marriage, your relationships with any other children you have, your friends, and remember to try to find some time for yourself. (Yeah, I know that sounds like a joke…TIME?! SELF?!) - Anonymous by request

 

Resources

If you are interested in meeting other parents and individuals who are involved in raising a child with Lennox-Gastaut syndrome, the following listserv is available:

LGS List - To subscribe, simply click here: http://lyris1.telelists.com/htbin/lyris.pl?enter=lennox-g&text_mode=0

For more information on Lennox-Gastaut syndrome, please visit these helpful sites and references:

 

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