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LGS (Lennox-Gastaut Syndrome)

Lawrence W. Brown, MD

Lawrence W. Brown, MD is Associate Professor of Neurology and Pediatrics at the Children’s Hospital of Philadelphia (CHOP) and the Perelman School of Medicine of the University of Pennsylvania. Dr. Brown is director of the Pediatric Neuropsychiatry Program, a multidisciplinary clinic for children whose complex learning and behavioral issues have a neurobiological basis. This population includes Tourette syndrome, high functioning autism and complex ADHD with co-morbidities, as well as those with epilepsy, genetic syndromes and other neurological disorders. The pediatric neuropsychiatry program has allowed him to broaden his basic interest in the relationship between behavior and the developing nervous system. Related research interests include clinical drug trials and genetic studies in Tourette syndrome. The latter is funded through an RO1 from the National Institutes of Health as part of an international consortium to develop a world-wide data bank of individuals with Tourette syndrome. In addition, Dr. Brown is a senior clinician in CHOP’s Pediatric Regional Epilepsy Program where he continues to be productive as principal investigator on numerous clinical drug trials in pediatric epilepsy. He is also a senior clinician in the Sleep Disorders Center with a major interest in how sleep and its disorders interact in children with neurological and developmental disorders.

Dr. Brown is the immediate past president of the Child Neurology Foundation (and currently serves as project director of a national medical transition initiative), an active member of the Child Neurology Society, the American Epilepsy Society, the Academy of Pediatrics (where he served two terms as Chairman of the Section of Neurology) and the American Academy of Neurology (where he served sequentially as fellow, advisor and faculty at the Palatucci Advocacy Leadership Forum). He is a member of the editorial boards of Pediatric Neurology and Clinical Neurology. Locally, he served for many years as chairman of the Professional Advisory Board and the executive board of the Epilepsy Foundation of Eastern Pennsylvania. Dr. Brown’s publications include associate editorship of Schwartz’s Clinical Handbook of Pediatrics, Fifth Edition, as well as all previous editions. He is the recipient of multiple honors and awards, including the Fritz E. Dreifuss International Scholar Award presented by the Epilepsy Foundation of America, and a teaching sabbatical at the Westmead Children’s Hospital in Sydney, Australia. Dr. Brown recently served on the American Academy of Pediatrics Subcommittee on ADHD which revised guidelines for diagnosis and treatment of ADHD. He currently serves on the Advisory Committee to the Coordinating Center for Children and Youth with Epilepsy which is funded by the Maternal Child Health Bureau of the Health Resources and Services Administration. Among many other professional and community lectures, he was the keynote speaker of the first national meeting of the LGS Foundation in 2011.

SUMMARY

Lennox-Gastaut syndrome (LGS) is a severe childhood form of epilepsy defined by multiple, often medication-refractory seizures (usually “drop attacks”, atypical absence seizures and tonic seizures), a characteristic EEG pattern (slow waking background, slow spike wave) and paroxysmal fast rhythms in sleep) as well as cognitive, behavioral and psychiatric symptoms. This triad of mixed seizures, abnormal EEG and encephalopathy represents one of the most difficult to treat. LGS is also a physically dangerous epilepsy syndrome of childhood because of the frequent falls, injuries, and cognitive impairment that can severely limit quality of life. Neurologists are aware of different causes of LGS from cortical malformations  to meningitis  and almost any condition that causes brain injury or disrupts normal neural networks. New treatments are available, including medications specifically approved for the disorder. Complete end to all seizures is unusual.

Lennox-Gastaut syndrome (LGS) is a severe childhood form of epilepsy described by:

  • multiple, often medically difficult to manage seizures
  • a specific electroencephalography (EEG) pattern; and
  • broad brain dysfunction or encephalopathy (causing cognitive, behavioral, psychiatric symptoms)

 

DESCRIPTION

Lennox-Gastaut syndrome (LGS) is a clinical condition which refers to a triad of signs and symptoms. These include (1) multiple seizure types, often medically refractory, with (2) a typical EEG pattern and (3) frequent moderate to severe cognitive disabilities. The most common seizure patterns are tonic, atypical absence, and “drop attacks” or atonic seizures, but generalized tonic-clonic and focal seizures can occur as well. The EEG abnormalities may vary, but usually there is high voltage, generalized slow spike and wave activity (usually less than 2.5 Hertz) in addition to bursts of generalized paroxysmal fast activity (GPFA) during sleep; waking background is often slow and disorganized.  The degree of cognitive delay and behavioral dysfunction vary, but are extremely common and often severe. These challenges represent an epileptic encephalopathy which weakens learning, social interaction and quality of life in general. Seizure types may change over time, slow spike wave and GPFA may not be appear on every EEG (especially routine waking studies), but cognitive and behavioral disabilities are permanent. There are children who fulfill the diagnosis for LGS whose seizures are well controlled and have minimal learning, social and behavioral challenges. However, many LGS patients wear protective headgear to prevent serious injuries from unprotected falls, and suffer from the long term effects of regular seizures and multiple drugs usage.
LGS is a clinical condition involving three specific signs and symptoms:

  • Multiple seizure types, often difficult to medically manage. The most common seizure patterns are tonic, atypical absence and “drop attacks.” Generalized tonic-clonic and focal seizures can occur, as well.
  • A specific EEG pattern that shows abnormal brain activity including:
  1. high voltage (or increased activity),
  2. generalized slow spike and wave activity (usually less than 2.5 Hertz),
  3. bursts of specific activity called Generalized Paroxysmal Fast Activity (GFPA) during sleep waking background that is often slow and disorganized
  • Moderate to severe cognitive disabilities are common. The brains of these children have existing encephalopathy (or brain dysfunction) that limits their ability to learn, social interactions, and their quality of life.

 

SYMPTOMS

LGS is a diagnosis based on the triad of seizure types plus EEG pattern as well as cognitive-behavioral dysfunction. None of these problems are specific to LGS, nor is there is single cause that leads to the syndrome, but it is important to make the diagnosis since it has great impact on treatment and prognosis. In sleep, the most typical seizure type in LGS is the generalized tonic seizure (often brief stiffening of all extremities). “Drop attacks” during wakefulness often occur dozens of times daily. They may include a brief tonic phase followed by loss of tone and falling. More subtle atonic seizures may present as head nods. Atypical absence seizures can be difficult to identify since they can have gradual onset and unclear termination; in other words, it is not always clear when they begin or end. Additionally, they are defined by brief episodes of loss of awareness, sometimes with head and eye deviation or subtle automatisms, such as eye flutter or muscle twitching. Not uncommon for children with LGS is to have periods of non-convulsive status epilepticus which can last for many minutes or days. This episode is characterized by altered awareness with almost continuous atypical absence seizures which may be interrupted by intermittent tonic or other seizure types. The presence of focal seizures may confuse the diagnosis, especially if the EEG does not show typical features of slow-spike wave or the decremental pattern called GPFA (generalized paroxysmal fast activity).

The following define typical seizures seen in LGS patients:

  • Generalized tonic seizures are often the brief stiffening of arms and legs in sleep.
  • Atonic-akinetic seizures or “drop attacks” occur many times daily when a child is awake. They may include a brief tonic phase, loss of muscle tone, and a fall. Sometimes these seizures may present as head nods.
  • Atypical absence seizures can be difficult to identify because they gradually begin and end. They can appear to be a loss of awareness, head and eye deviation (turn) or subtle automatisms, such as eye flutter or muscle twitching.
  • Non-convulsive status epilepticus which can last for many minutes or days; and can look like altered awareness and continuous atypical absence seizures. These seizures may be interrupted by intermittent tonic or other seizure types.
  • Focal or partial seizures may also be present.

 

Variable degree of cognitive delay is common in LGS. However, this may not be clear at the time of diagnosis. This can make it difficult to understand the reason for what may appear to be increasingly obvious failure to make progress or actual regression, as it could be due to:

  • Underlying brain structure
  • Epileptic encephalopathy caused by the frequent seizures
  • Side effects of anti-seizure medication often prescribed as polypharmacy.

Unfortunately, almost all children with LGS have clear cognitive impairment within 5 years of diagnosis. The few LGS patients with normal intelligence usually continue to have major problems in daily life due to poor attention, hyperactivity, impulsivity, slow mental processing, and social difficulties.

LGS must be distinguished from other epilepsy syndromes, including:

  • Early-onset childhood absence epilepsy does not have other seizure types, except for generalized tonic-clonic seizures. One difficulty may be caused by the fact that tonic seizures are inconsistent and may not be seen because they usually occur at night. Children with absence epilepsy should not have slow and disorganized EEG background and the frequency of spike-wave discharges are faster (approximately 3 Hertz) than those seen in LGS.
  • Children with myoclonic-astatic epilepsy (Doose syndrome) are developmentally normal and typically have normal waking EEG background prior to onset of seizures and an epileptic encephalopathy.
  • There is an overlap between LGS and Dravet syndrome , but the latter typically starts much earlier (within the first year of life) with atypical, prolonged febrile seizures followed by more focal seizures.
  • Another important distinction is between LGS and atypical benign partial epilepsy. “Pseudo Lennox syndrome” can be distinguished by the persistently normal background, sleep activation of central-temporal spikes, and lack of tonic seizures.

 

LGS can be considered an age-related epileptic encephalopathy. It typically does not start in infancy, but other early onset epileptic encephalopathies may evolve into LGS. For example, it is not uncommon for infantile spasms, even if initially well-controlled, to subsequently demonstrate the variable seizure types and EEG patterns, as well as developmental delays which define LGS. Eventually, as the child becomes an adolescent and enters adulthood, daytime seizures often improve with persistence of only sleep-related tonic seizures or development of focal seizures. In addition, the EEG may evolve and no longer fit the expected criteria with improved waking EEG background and absence of inter-ictal slow spike wave discharges. Interestingly, night-time tonic seizures often continue and still show the same diffuse fast rhythms of GPFA . It is important for adult practitioners to remember the life story of the individual and to put the current picture into context.  It can be so common for adults to have focal epilepsy that a lack of  awareness of the syndrome’s presentation can lead to a wrong diagnosis and inadequate treatment. LGS should be considered in a patient with medically resistant seizures, especially if they include falling.

LGS should be considered as the diagnosis in all individuals with medically resistant seizures, especially if they include falling.

PHYSICAL ABNORMALITIES

There are no specific physical abnormalities that are associated with this epilepsy syndrome. However, LGS can be seen in a variety of chromosomal disorders, genetic-metabolic syndromes such as tuberous sclerosis and brain injury conditions (i.e. hypoxic-ischemic encephalopathy) that can help to determine the specific etiology of the disorder and lead to specific interventions.

CAUSATION

Since LGS is an age-related epileptic response to many different causes, the diagnosis suggests a broad differential diagnosis. Cerebral malformations, infections, hypoxic-ischemic injury, trauma, tuberous sclerosis and even progressive metabolic disorders are among the causes which can explain up to 75% of cases.

The following are examples of various conditions that explain 75% of seizure disorders:

  • Cerebral malformations (abnormalities within child’s brain structure)
  • Infections (such as meningitis)
  • Hypoxic-ischemic injury (brain injury that limits the amount of blood and oxygen delivered to certain areas of the brain)
  • External trauma or injury
  • Genetic – metabolic disorders (such as tuberous sclerosis)
  • Other chromosomal disorders

 

Approximately 1 out of 4 children do not have a specific cause identified for their seizure disorder.

Age-related epilepsy often represents different stages of response to disrupted brain development. For example, Ohtahara syndrome which presents in the newborn period with tonic seizures, developmental delay, and an EEG showing burst suppression is caused by severe brain malformation or damage. This condition often evolves to West syndrome with infantile spasms and a chaotic EEG pattern (hypsarrhythmia) at 4-8 months of age. Over time, approximately 1/3 of children with West syndrome will eventually progress to LGS in childhood.

The diagnosis of LGS does not exclude other developmental or neuropsychiatric diagnoses. While some children with LGS  have neurodevelopmental delays from the time of first diagnosis, even if they appear normal at the time when seizures are first recognized,  the large majority of LGS patients are struggling with cognitive, behavioral, or social disabilities within several years. In addition to intellectual disability, many children will fulfill criteria for ADHD or autistic spectrum disorders. The diagnosis of LGS does not prevent medical treatment of these other conditions. Caregivers or providers should not be discouraged by theoretic concerns of seizure activity from stimulants or neuroleptics (typical treatment medications for ADHD and autism spectrum disorders).

ADDITIONAL CONCERNS AND COMPLICATIONS

Cognitive decline or intellectual disability (mental retardation) is present in 75-90% of children. Moderate to severe cognitive impairment is common in adults with LGS (96% in one study). Cognitive decline can occur over time, particularly in those with frequent seizures and many episodes of status epilepticus. However, it is unclear whether it is the frequent seizures causing worsening intellectual abilities or limitations that become more obvious due to lack of expected brain development. Behavioral disorders and psychiatric problems are also very common in children with LGS. These co-existing conditions further complicate the management of seizures and quality of life.

Laboratory investigations

Children present with seizures and developmental delays before they are eventually diagnosed with LGS. The work-up of epilepsy is discussed in another section. Obviously, the formal diagnosis of LGS requires a compatible EEG, but it is important to recognize that the characteristic features are inconsistent and may not be seen on every routine or even 24 hour video EEG. Once the diagnosis of LGS is made, it may be necessary to revisit the work-up for the child’s epilepsy. For example, an MRI performed in the first year of life may need to be repeated with specific epilepsy protocols since a 3 Tesla study may now show abnormal myelination patterns or areas of cortical dysplasia not previously appreciated.

EEG and magnetic resonance imaging (MRI) are two tests neurologists use to diagnosis LGS. When thinking about the use of these tests, it is important to understand:

  • While a LGS diagnosis requires a specific EEG pattern, the pattern may not be seen on every routine or even 24 hour video EEG.
  • Once the diagnosis of LGS is made, it may be necessary to revisit the initial tests to see if the child’s condition has changed. For example, an MRI performed in the first year of life may be repeated later to see if there have been any changes in the child’s brain structure.

 

DIAGNOSTIC STANDARDS

As stated above, Lennox-Gastaut syndrome is a severe form of childhood onset epilepsy defined by (1) multiple seizure types, often medically refractory, with (2) a typical EEG pattern and (3) frequent moderate to severe cognitive disabilities. Most often it is the result of a brain insult during early life, but occasionally it can develop in an otherwise previously healthy child. Seizures associated with LGS may follow severe earlier seizure syndromes such as West syndrome or occur on their own. Any seizure pattern may be present, but typically include tonic, atypical absence and “drop attacks” or atonic seizures. The electrographic abnormalities may vary, but classically there is high voltage and generalized slow spike and wave activity (usually less than 2.5 Hertz) in addition to bursts of generalized paroxysmal fast activity during sleep; waking background is often slow and disorganized.

THERAPEUTIC INTERVENTION

There have been several new drugs recently approved by the Food and Drug Administration (FDA) for treatment of seizures associated with LGS. Until 2011 there were only four medications that were so designated including topiramate, lamotrigine,clonazepam and felbamate. Interestingly, none of these were the typical first choice of most clinicians who would start with valproate (which was never specifically tested for FDA registration in this population, so it has never been officially approved).Valproate can be effective for all of the seizure types typically associated with LGS. It is a complex drug which has significant side effects ranging from tremor to reduction in platelet count and function as well as hyperammonemi a. Rare life-threatening side effects include liver failure and pancreatitis.

Topiramate also has a broad spectrum of action which can help the multiple seizure types of LGS. It has been shown to reduce drop attacks and has a safety profile that many consider better than lamotrigine or felbamate. Even when dosages are increased slowly, there is a high rate of central nervous system (CNS) side effects, such as somnolence or sleepiness, mental slowing and ataxia (unstable gait) in addition to word-finding difficulties and weight loss. Lamotrigine has effects against multiple seizure types in addition to mood stabilizing properties, but unusual pharmacologic effects and interactions make it a challenging drug to use. It is rarely associated with life-threatening rash as long as the drug is introduced slowly and drug interactions (particularly with valproate) are considered.

Felbamate was effective for LGS when it was first introduced, and one of the first new agents that promoted alertness as well as weight loss; however, the risk of aplastic anemia and hepatic failure have relegated it to a last-ditch adjunctive drug for difficult to manage epilepsy. Clonazepam  represented the most commonly employed representative of the benzodiazepine class of medications. It is particularly effective in myoclonic seizures, but it is limited by the common problem of tolerance as well as withdrawal seizures. In addition, sedation and drooling make it unappealing first line agents. Therefore, it is rarely used as a long term option, although various benzodiazepines (including clonazepam) are still used for short term relief at the time of seizure flurries.

Clobazam, a unique 1,5 benzodiazepine derivative long available in Europe, was finally introduced to the US in 2011. Its different structure appears to be responsible for a lower rate of sedation and broader spectrum of action as well as longer half-life  than other benzodiazepines which all share a 1,4 structure. In a short time it has proven itself to be a rapidly effective agent with an apparently lower rate of tolerance than other drugs in its class. It was shown to significantly reduce drop attacks and other seizure types. Rufinamide is the other newly approved drug. It is structurally unrelated to any other anti-seizure compound and has good efficacy against various seizure types associated with LGS.

Until 2011, only four medications were designated in the treatment of LGS:

  1. Topiramate can help control the multiple seizure types of LGS. It has been shown to reduce “Drop Attacks”, and has a safety profile that many consider better than lamotrigine or felbamate. Side effects may include sleepiness (somnolence), mental slowing and unstable gait (ataxia) in addition to word-finding difficulties and weight loss.
  2. Lamotrigine is effective with multiple seizure types, as well as being mood stabilizing. It is a challenging drug to use because of the varying ways it works in a person’s body, and the large number of medication interactions. It is rarely related to a life-threatening rash, as long as the drug is introduced slowly and drug interactions (particularly with valproate) are taken into account.
  3. Clonazepam is the most commonly used benzodiazepine. It is very effective in myoclonic seizures, but it is limited by the common problem of tolerance, as well as withdrawal seizures. In addition, sedation and drooling stop many providers from initially prescribing it initially. It is rarely used as a long term option
  4. Felbamate was very successful for LGS when it was first introduced, and one of the first medications that promoted alertness as well as weight loss. However, the risk of aplastic anemia and hepatic failure have reserved its use to a final drug choice for difficult-to-manage seizures.

 

There is no single treatment which provides complete or even satisfactory control of LGS. Seizures frequently persist even with multiple medications directed at the various seizure types. Furthermore, polypharmacy (multiple drug therapy) often contributes to side effects due to drug interactions. These interactions may worsen cognitive and behavioral abilities. Not only are the drug interactions between seizure medications responsible, but also interactions with other drugs used by psychiatrists and neurologists – such as antidepressants and neuroleptics — that may induce or inhibit the same mechanisms. Even if there are no significant drug interactions, the burden of multiple medications on various body organs may lead to side effects involving critical functions of central nervous system (CNS), liver, kidneys and bone marrow.

Since seizures are frequently refractory to all medications and combinations, it is important to consider other approaches. One of the most important alternative strategies is the ketogenic diet. This high fat dietary approach has been available for almost 100 years. Recent advances in understanding and creative thinking have improved its safety and tolerability. It has shown definite effectiveness against all of the seizure types associated with LGS at rates that meet or exceed any new medication. However, it is a rigorous treatment that requires the support of a comprehensive medical team (including nurses and dieticians),  a major commitment by a family, and willingness of the child to avoid potentially dangerous loss of seizure control by cheating on the diet with excessive carbohydrates.

The vagus nerve stimulator (VNS) has similarly achieved a strong position as an adjunctive treatment for seizures associated with LGS, at rates comparable to those of the ketogenic diet. Although FDA-approved only for partial seizures in patients above 12 years of age, the VNS has evidence for safety and efficacy involving all seizure types at any age. Although a surgical intervention, it is a minor procedure usually performed as day surgery. It is a long-term commitment since the device must be ramped up slowly to avoid side effects such as coughing, voice alteration, and pain. The VNS has few side effects but it can cause dysphagia , increased drooling, or exacerbation of obstructive sleep apnea.

Resective surgery is an uncommon consideration in LGS because the cause of seizures is rarely restricted to an area of the brain able to be removed. However, corpus callosotomy is a reasonable treatment for refractory drop attacks. This is a disconnection of the bundle of nerve fibers that connect the two hemispheres; different technical approaches have been recommended which emphasize either partial or complete disconnection. Anterior two-thirds disconnection is the most frequently performed procedure, but complete callosotomy is often performed for those with drop attacks and severe mental disabilities.

Since seizures are frequently difficult to manage, it is important to explore treatments besides medication, including:

  • Ketogenic diet
  • Vagus nerve stimulator
  • Corpus callosotomy or resective surgery

 

PROGNOSIS

Although it would be reassuring if one could provide an optimistic outlook on the future of a child with a new diagnosis of LGS, it more often has a devastating course and requires heroic team effort with many clinical resources. Up to 90% of children with LGS have developmental challenges including variable intellectual disability, behavioral abnormalities,  and autistic features. Most have a static encephalopathy, but the disorder can be progressive depending on cause or it can appear progressive due to severity of seizures or side effects of medication. More than 80% will continue to have seizures as adults, although seizure types may evolve and the EEG patterns that helped to define the disorder may disappear. More than most other epilepsy syndromes, LGS carries a greater long-term mortality; several studies have estimated this as 3-7%. While it may not be true in the individual case, the diagnosis of LGS often means a life of dependency.

PREVENTION

There is no simple way to prevent the evolution of LGS since it is usually related to significant brain malformations or abnormalities, genetic-metabolic syndromes, or injuries. However, early and effective treatment may reduce the degree of disability caused by epileptic encephalopathy (either from potentially controllable frequent seizures or effects of polypharmacy).

CARETAKER AUGMENTATION

Since the probability of outgrowing epilepsy and becoming a fully independent adult is unlikely, it is especially important that parents and caregivers prepare for and coordinate transition to adulthood. The majority of children with LGS have refractory epilepsy, cognitive and behavioral disabilities, and some also have additional challenges such as cerebral palsy, blindness or hearing impairment associated with the particular cause of their epilepsy. Medical transition is only one part of a more complex process that ideally leads to maximal independence and optimal treatment.

Patients and their families may have grown up with a child neurologist and be unwilling to change a long-standing relationship. However, there are issues better managed by adult epileptologists, including drug-drug interactions with hormonal contraception, bone health, risks of long term obesity in adult-onset diabetes, and management of complex needs with far fewer resources than were available during childhood (i.e. special education, coordinated therapies and behavioral services). Furthermore, parents may be increasingly challenged by the care needs of their adult children even if they are not suffering from their own age-related conditions.

Transition should start very early with financial and legal considerations, such as estate planning, special needs trusts, and  preparation of a will that includes “child care” for a disabled adult. These decisions are important to consider soon after an LGS diagnosis because of the risk of something happening to the parents before the child reaches adulthood. Parents must consider whether their child with LGS needs guardianship at the age of 18 years. This is a legal process that recognizes that the individual is incapable of making medical and financial decisions so that the parents can continue to help to make decisions for the individual. Living arrangements must be considered. It helps to have the youth with LGS on waiting lists for group homes or residential facilities even before they or the parents are ready since it may take years before one’s name comes up.

Important quality of life considerations in planning for transitioning a youth with LGS to adult health care provider:

  • Financial: estate planning or special needs trusts
  • Legal: preparation of a will or guardianship
  • Community-based services: child care, or respite care throughout the patient’s life span
  • Living arrangements: group homes or residential facilities

 

GLOSSARY/ DEFINITIONS

Atypical absence seizures – non-convulsive arrest of activity and staring, typically with slow onset and return to baseline often associated with irregular slow-spike wave on EEG.

Drop attacks – a combination of seizures with atonia (loss of motor tone) and myoclonic (sudden jerking of a group of muscles) that can lead to loss of muscle control. These unprotected falls are associated with injuries including chin lacerations, fractured teeth, concussion, etc. and may require protective head gear.

Non-convulsive status epilepticus – prolonged state of reduced awareness associated with epileptic EEG abnormalities often associated with abnormal speech, intermittent eye deviation, automatisms or twitching. This can be difficult to identify in a child with delayed development.

Tonic seizures – rigidity without additional motor component, usually associated with generalized paroxysmal fast activity (electrodecremental response) on EEG; these are most common during non-REM sleep.

 

REFERENCES

Arizimaoglou A, French J, Blume WT et al Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management and trial methodology. Lancet Neurol 8: 82-93, 2009.

Camfield PR. Definition and natural history of Lennox-Gastaut syndrome. Epilepsia 52:3-9, 2011.

Camfield PR, Gibson PA, Douglass LM. Strategies for traitining to adult care for youth with Lennox-Gastuat syndrome and related disorders. Epilepsia 52 (suppl 5): 21-27, 2011.

Conry JA, Ng Y, Paolicchi JM. Clobazam in the treatment of Lennox-Gastaut syndrome. Epilepsia 50: 1159-1166, 2009.

Ferlazzo E, Nikaronova M, Italiano D. Lennox-Gastaut syndrome in adulthood: clinical and EEG features. Epilepsy Res: 89: 271-279, 2010.

Glauser T, Kluger G, Sachdeo R et al. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurol 70: 1950-1958, 2008.

VanStraten AF, Ng Y. Update on the management of Lennox-Gastaut syndrome. Pediatr Neurol 47:153-161, 2012.

www.lgsfoundation.org. This is the most comprehensive and independent patient advocacy voice for information and support of individuals with LGS.

www.livingwithlgs.com. This is a commercial website sponsored by Eisai which includes an excellent video that describes the challenges of living with LGS as well as video which focuses on other important topics including sleep hygiene, behavioral challenges, identifying a team of experts, and family dynamics.

www.lgstogether.com This is a commercial website sponsored by Lundbeck which offers a patient forum to share ideas and solutions.

Family Stories

THE BOND OF LOVE CAN NEVER BE BROKEN
by Kathy Leavens

 

KL5Words were so empty in the beginning days of my grief. No one had the magic words that made everything better. You have no idea how much those words have come to mean to me over time.

Life as a young mom moves so fast. Raising four boys and being a single mom to kids with special needs certainly didn’t slow it down any.  Life holds many surprises but mine seemed to be playing out a series of tragic events – one after another. I kept thinking, “This is the last lesson, right?”  During the most difficult days the love I have for my children was the glue that held me together. I was searching for the positive outcome but it seemed like the more I fought my lessons only got harder.

My family was growing. At home were Brian age 10 and Eric 9 then in September 1995 I just had my third son, Matthew and 1998 brought the addition of my fourth son, Jacob. I loved my perfect family! What a lucky mom to have all boys!

A mother’s instinct is so valuable. That instinct can hold so many clues to what is KL4going on with our children. Immediately following Matthew’s birth my own instinct was telling me something was terribly wrong. I had no idea what to expect as I started to question Matthew’s episodes I was witnessing. After I insisted on an evaluation by the “experts”, we were given a generic diagnosis of seizures. Today, I believe that Infantile Spasms was the true diagnosis. Infantile Spasms having never being properly treated, resulted in Lennox Gastaut syndrome at the age of 14. Everyone in the home knew how to react to the multiple tonic clonic seizures that we expected daily. Seizure free days were heaven not only for Matt but for the whole family.

I thought learning to live with the unpredictability of seizures and seeking a cure for Matt was the hardest thing I was to face as a mom.  Years later, my oldest son, 16 at the time, made the most common mistake of all and fell asleep at the wheel of his car. The accident left him with a complete spinal cord injury, a high school athlete faced with never walking again. I wasn’t sure he wouldn’t give up on life. That was all I could think, but Brian had other questions. First, how and where was the passenger that was traveling with him? Next, “did I hurt anyone else”?  I knew he would be more than alright. His friend walked away from the accident with no injuries.  I don’t think I have ever heard him complain. He is truly my inspiration.

KL6As we settled in to our new routines I will never forget the next moment of heart wrenching pain that came 5 years later. Brian had moved out and was living on his own, Eric was away at college, Matthew continued with seizures, and my youngest in elementary school. It was Eric’s sophomore year, a psychology major – wanting to change the world before he even hit 20 years old. My world stopped that cold night in December when two police officers came to my door instead of Eric arriving home to celebrate his birthday and Christmas break. My heart shattered as they spoke, they were at my house asking questions and not giving much detail. But my heart already knew.  Eric had been involved in a car accident and I would never again hear my son’s voice, give him hugs or say goodbye. How is it fair that Eric had suffered fatal injuries and his passion for change in the world would never be realized?

My world turned grey and every day I woke to the same thing, missing Eric and the feeling my shattered heart would never heal.  Learning to live my “new normal” was difficult as Matt’s seizures only increased and became more severe.  We tried every drug available, in every possible combination and dose. With no options left, brain surgery would hopefully provide relief for Matthew and stop the seizures that only got worse with each passing day. Matthew loved life and just wanted to play, laugh and sing! Brain surgery gave us a short break in the seizures! Twenty days seizure free! Only to have the seizures return.KL3

Every night I would crawl into bed next to Matt so he could get a bit of sleep before the seizures started for that night. I wanted my hand on him to feel him as he breathed, be immediately able to intervene and stop the next seizure. While lying there resting, I closed my eyes only to plead, beg and cry for that one miracle….just one please!  I couldn’t help but believe that my children deserved it. I was going to beg and keep begging until we got just one. I’m relentless like that! Sometimes we are given miracles unexpectedly and they might not even look how we imagine they should. Part of the deal I was making was that I will keep fighting, find the next treatment and we would hang on until someone finds a cure. Until then I will protect Matthew the best I can. It was my job to make sure nothing else bad happened. Life had a completely different idea and brought me a few more lessons.

KL1The miracle I begged for came alright, but not until after my youngest son was in for the fight of his life.  I could not believe the words I was hearing, Jacob had Ewings Sarcoma, a bone cancer that does not have the best outcomes. Childhood cancer takes priority and demands decisions in care. The treatment plan was put in motion immediately.  Jacob, quite a trooper, defied all the odds and chemotherapy was successful! He won a battle I was told we had no hope of winning. Today Jacob is a 5 year cancer survivor!

Things were finally looking up! Maybe just maybe, I would be granted two wishes? Instead, the plan was for LGS, seizures and SUDEP to take my child. Matthew had a pure heart and only wanted to enjoy life in spite of unrelenting seizures. How could this happen? How could one parent feel the pain of a broken heart twice in her lifetime? Life was feeling like a series of unbelievably bad jokes that just kept coming. I had everything under control, did my research, asked every question and made every call I could. I even put every preventative measure in place that I could think of to stop this from happening.  I had no choice but to look up and ask, REALLYKL2

All these events, as horrible as they seem, including the ones that you have experienced have actually given us the gift of growth. Finding your way is one of the most valuable lessons in life.

The lessons taught me that I am strong. So are you;

I am a survivor. So are you;

I am proud of the work I have done to be standing here today. So should you ;

I can fight when I have to but I don’t have to fight all the time. Neither do you;

I had to accept that ultimately in life we are not in control – a power greater than you or I has the final say.

Given the option to change anything, I wouldn’t. My heart was shattered the day my children went before me but I believe that all the difficult moments I have survived have led me to my purpose of being an example for others. I am here to fight beside you. You can overcome anything life asks you to face,  you can Stand Strong and Believe in a positive outcome, and you can believe that the darkest moments just might bring the brightest light to not only your life but the people’s lives you touch”.

Today, I am simply here as a mom, a supporter in your fight of finding the best for your loved one. I am no expert, but I can share my experiences and encourage you to make that call, ask that question and never give up hope for the best outcome, even if it means accepting an outcome that doesn’t appear to be everything you asked for. Through helping you, I get the amazing gift of putting my heart back together piece by piece. So thank you for allowing me to know you, to feel the emotion of your walk and know that I share in your passion.

If you are still reading I have to say the most important thing is that you know deep down, the bond of Love is truly never broken, its lasts through even death. Love is the cord that connects our souls. It can never be severed. It’s the best lesson that my children have taught me! One I could only have learned by them going before me. Please,  love each other, don’t be scared of what you face, be proactive, ask for help from others along the way, put everything in place you can and then please do one thing for me and my children….LIVE and ENJOY every day you are given!

Resources

UnderstandingLGSInfoGraphic

View the high-resolution Understanding LGS Infographic here.

LGSTipIG

View the high-resolution LGS Tip Sheet here.

 

LGSFoundation logo

www.lgsfoundation.org 

LGS Foundation: Parents/Young Adults

www.adultsandlgs.org 

 

LGSTogetherLogo

www.LGSTogether.com

LGSConnections

https://www.youtube.com/user/LGSConnections 

 

 

LivingWLFG

http://www.livingwithlgs.com/

 

LGSHope

http://www.lgshope.com/resources/Lennox-Gastaut-Syndrome.aspx

 

-LGS UK Lennox-Gastaut Syndrome Facebook page

https://www.facebook.com/lgsfoundationuk/ 

-LGS/Epilepsy Dads Facebook page

https://www.facebook.com/groups/469879779757589/