Lennox-Gastaut Syndrome - NORD (National Organization for Rare Disorders) (2023)

Lennox-Gastaut Syndrome

NORD gratefully acknowledges James W. Wheless, MD, Professor and Chief of Pediatric Neurology, Le Bonheur Chair in Pediatric Neurology, University of Tennessee Health Science Center; Director, Le Bonheur Comprehensive Epilepsy Program & Neuroscience Institute, Le Bonheur Children's Hospital, for assistance in the preparation of this report.

Synonyms of Lennox-Gastaut Syndrome

  • LGS

General Discussion

Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures. Children with Lennox-Gastaut syndrome may also develop cognitive dysfunction, delays in reaching developmental milestones and behavioral problems. Lennox-Gastaut syndrome can be caused by a variety of underlying conditions, but in some cases no cause can be identified. Lennox-Gastaut syndrome can be difficult to treat because it is resistant (refractory) to many kinds of antiseizure medications. Research is ongoing to identify and assess new therapies for Lennox-Gastaut syndrome.

There is no consensus in the medical literature on the exact definition of Lennox-Gastaut syndrome. Generally, three findings are necessary for the diagnosis: multiple generalized seizure types; a slow spike-and-wave pattern (less than 2.5 Hz) on EEG; and cognitive dysfunction. The International League Against Epilepsy (ILAE) Task Force most recently classified the disorder as an epileptic encephalopathy. Epileptic encephalopathies are a group of disorders in which seizure activity leads to progressive cognitive dysfunction.

NORD Video: Lennox-Gastaut Syndrome, Tracy’s Story

Scroll back up to restore default view.

Signs & Symptoms

The symptoms of Lennox-Gastaut syndrome usually begin during infancy or childhood, most often between 3 to 5 years of age. Multiple types of seizures, which are basically electrical disturbances in the brain, affect children with Lennox-Gastaut syndrome. Most affected individuals experience multiple types of seizures, multiple times throughout the day. As affected individuals grow older, the types and frequency of seizure activity may change.

The most common types of seizures associated with Lennox-Gastaut syndrome are tonic and atonic seizures. Tonic seizures cause increased muscle tone and muscle stiffness. They are characterized by sustained muscle contractions that can cause mild abnormalities such as a slight bend of the body and brief interruption of breathing or more significant problems such as muscle spasms of the face and flexion or extension of the arms and legs. Affected children may extend their arms over their heads similar to a ballet dancer. Tonic seizures are usually brief (lasting between a few seconds and a minute) and are especially prevalent at night during sleep, but can also occur during the day. There is usually a brief loss of consciousness during a tonic seizure. Tonic seizures that occur when awake can cause affected individuals to fall.

Atonic seizures cause a sudden loss of muscle tone and limpness. They can cause the head to drop or nod, problems with posture or sudden falls. Atonic seizures are also known as drop attacks. Atonic seizures can lead to injuries of the head and face because of sudden, unexpected falls. When sitting, affected individuals may collapsed forward or backward at the waist. Atonic seizures may only partially affect consciousness and usually last only a few seconds.

A third type of seizure commonly associated with Lennox-Gastaut syndrome is atypical absence seizures. This type of seizure is associated with a period of unconsciousness usually marked by unresponsive staring. Absence seizures usually begin and end abruptly and the affected individual usually resumes activity with no memory of the episode. Absence seizures do not cause convulsions and may be so mild that they go unnoticed. They usually last only a couple to several seconds. If the child is developmentally delayed, the parents may only notice a subtle change in function or responsiveness.

Additional types of seizures can affect individuals with Lennox-Gastaut syndrome less often. These include myoclonic seizures, which are characterized by abnormal, jerky movements and may occur alone or in conjunction with atypical absence seizures; tonic-clonic seizures (once known as grand mal seizures), which last a couple of minutes and are characterized by stiffening of the limbs and then jerking of the limbs and face; and partial or focal seizures, which involve electrical abnormalities in a limited area of the brain and come in a variety of forms. Some individuals with Lennox-Gastaut syndrome experience prolonged, uninterrupted seizure activity that lasts for more than 30 minutes (nonconvulsive status epilepticus). Nonconvulsive status epilepticus may be associated with a child being unaware or inattentive and, in some cases, may be so subtle that it goes unnoticed. Nonconvulsive status epilepticus requires medical intervention.

(Video) Tracy Dixon Salazar: Personal Story from the New Era of Patient Advocacy

Intelligence is usually, but not always, affected in children with Lennox-Gastaut syndrome. Affected children may experience varying degrees of cognitive dysfunction and delays in reaching developmental milestones such as sitting, crawling or walking. Children with Lennox-Gastaut syndrome may develop normally before the onset of seizures, and then lose previously acquired skills (psychomotor regression).Because the seizures associated with Lennox-Gastaut syndrome are usually resistant to treatment, intellectual impairment and learning problems may worsen over time. Children with Lennox-Gastaut syndrome may also develop behavioral problems ranging from hyperactivity and irritability to autistic symptoms and psychosis.

In some cases, individuals with Lennox-Gastaut syndrome may have been initially affected by infantile spasms. Infantile spasms, which are also known as West syndrome, are characterized by sudden, involuntary contractions of the head, neck, and trunk and/or uncontrolled extension of the legs and/or arms. (For more on West syndrome, please see the Related Disorders section below.)

Causes

In approximately 70-80 percent of patients, Lennox-Gastaut syndrome has an identifiable cause. These cases may be referred to as symptomatic Lennox-Gastaut syndrome. Examples of conditions that can cause Lennox-Gastaut syndrome include abnormal development of the brain cortex (cortical dysplasia), congenital infections, stroke, trauma, reduced oxygen supply that occurs before birth (perinatal hypoxia), infections of the central nervous system such as encephalitis or meningitis and a rare, genetic disorder called tuberous sclerosis. Approximately 17-30 percent of individuals with Lennox-Gastaut syndrome have a previous history of West syndrome. In general, these cases tend to be more severe.

Lennox-Gastaut syndrome may also be classified as cryptogenic, in which the cause is unknown or cannot be determined after evaluation. Cryptogenic cases are presumed to result from an unidentified condition (secondary Lennox-Gastaut syndrome). Individuals with cryptogenic Lennox-Gastaut syndrome do not have a previous history of seizure activity, prior neurological problems or cognitive impairment before the development of the disorder. Cryptogenic cases generally have a later onset than symptomatic cases.

In some cases of Lennox-Gastaut syndrome no associated condition is present or presumed and the cause of the disorder is unknown.

Although the cause of Lennox-Gastaut is known in most cases, the exact underlying mechanisms that ultimately bring about the various seizures that characterize the disorder are unknown. Researchers have not discovered any genes that are associated with Lennox-Gastaut syndrome, although the disorder may have a genetic component that contributes to its development. More research is necessary to determine the specific factors, including any potential genetic factors that are involved in the development of Lennox-Gastaut syndrome.

Affected Populations

Lennox-Gastaut syndrome affects males slightly more often than females. Lennox-Gastaut syndrome is estimated to occur in .1-.28 people per 100,000 and is believed to account for 1-4 percent of all cases of childhood epilepsy. The annual incidence in children is estimated to be 2 per 100,000 children. Onset of Lennox-Gastaut syndrome is usually between 2-7 years with a peak onset between 3 to 5 years.

Related Disorders

Symptoms of the following disorders can be similar to those of Lennox-Gastaut syndrome. Comparisons may be useful for a differential diagnosis.

West syndrome is a type of epilepsy characterized by spasms, an abnormal brain wave pattern (interictal EEG) called hypsarrhythmia and cognitive dysfunction. The spasms that occur may range from violent jackknife or “salaam” movements where the whole body bends in half, or they may be no more than a mild twitching of the shoulder or eye changes. These spasms usually begin in the early months after birth and can sometimes be helped with medication. There are many different causes of West syndrome and if a specific cause can be identified, a diagnosis of symptomatic West syndrome can be made. If a cause cannot be determined, a diagnosis of cryptogenic West syndrome is made. Some infants with West syndrome may eventually develop Lennox-Gestaut syndrome. (For more information on this disorder, choose “West” as your search term in the Rare Disease Database.)

Epilepsy is a general term for a group of neurological disorders characterized by recurrent seizures, and associated with abnormal electrical discharges in the brain. It is characterized by loss of consciousness, convulsions, spasms, sensory confusion, and disturbances in the autonomic nervous system. Attacks may be preceded by an “aura”, a feeling of unease or sensory discomfort; the aura marks the beginning of the seizure in the brain. This is typically in partial-onset seizures. There are many different types of epilepsy and the exact cause is generally unknown. Epilepsy can also occur as part of larger syndromes. Types of epilepsy or disorders associated with epilepsy include Rett syndrome, Angleman syndrome, Landau-Kleffner syndrome, Dravet syndrome, and the neuronal ceroid lipofuscinoses. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)

Diagnosis

Lennox-Gastaut syndrome is defined as having a clinical triad that must be identified for a diagnosis. This triad consists of multiple seizures of different types, a distinctive EEG brain wave pattern (slow [1.5- to 2.5-Hz] spike-and-wave pattern)(Note this pattern may not be present on every EEG.) and some degree of cognitive impairment and behavioral abnormalities. However, these symptoms may not all be present at the onset of the disorder, making an accurate diagnosis of Lennox-Gastaut syndrome difficult. The wide variety of potential causes of Lennox-Gastaut syndrome also complicates the diagnosis.

A diagnosis of Lennox-Gastaut syndrome is usually made based upon a thorough clinical evaluation, a detailed patient history and a complete physical and neurological evaluation including advanced imaging techniques, such as electroencephalography (EEG) and magnetic resonance imaging (MRI). During an EEG, the brain’s electrical impulses are recorded. In individuals with LGS, such EEG testing typically reveals the distinctive brain wave pattern (slow [1.5- to 2.5-Hz] spike-and-wave pattern). During a MRI scan, three-dimensional images are produced that reflect the brain’s anatomy; such scanning helps physicians examine brain structure and potentially locate the cause of the seizure activity. Epilepsy gene panels that assay for multiple genetic causes are starting to become part of the evaluation process.

(Video) Erica Mumm: Personal Story from the New Era of Patient Advocacy

Standard Therapies

Treatment
No specific therapy for Lennox-Gastaut syndrome is effective in all cases and the disorder has proven particularly resistant to most therapeutic options. The three main forms of treatment of Lennox-Gastaut syndrome are anti-epileptic drugs (AEDs), dietary therapy (typically the ketogenic diet) or device/surgery (VNS therapy or corpus callosotomy). Rarely, resective surgery is an option.

Treatment may require the coordinated efforts of a team of specialists. Pediatricians, neurologists, pediatric neurologists, surgeons, and/or other healthcare professionals may need to systematically and comprehensively plan an affected child’s treatment. Families need to work with healthcare professionals to develop a treatment plan that covers various potential situations such as seizure emergencies, routine medical illnesses, or what to do if an affected individual misses a dosage of medication. Families should also keep a list of which medications can possibly worsen Lennox-Gastaut syndrome. An affected individual’s treatment regimen will need repeated revisions throughout a person’s life as the types and frequency of seizures may change and the effectiveness of a particular therapy can lessen. Other healthcare providers are frequently consulted, including social workers, neuropsychologists, psychiatrists and rehabilitation services (occupational, physical and speech therapy).

AEDs are usually given to individuals with Lennox-Gastaut syndrome, but the individual response is highly variable. In some cases, it is possible that treatment with AEDs may help reduce or control various types of seizure activity associated with LGS. However, because individuals with Lennox-Gastaut syndrome have different types of seizures, they often require therapy with multiple types of AEDs. Such medications may include clonazepam, sodium valproate, topiramate, lamotrigine, felbamate (closely monitored), clobazam,rufinamide or cannabidiol. However, such drugs may have limited success in treating seizure activity in some individuals with the disorder. In addition, AEDs may be associated with significant side effects, especially in individuals who receive multidrug, high-dose regimens. AEDs can also become less effective over time. Being on multiple medications, which may cause sedation, can sometimes worsen seizure control.

Valproate (valproic acid) is generally considered the first-line therapy for Lennox-Gastaut syndrome because it is effective against a wide spectrum of seizures. Valproate is usually first given alone (monotherapy) and if ineffective another drug such as lamotrigine, topiramate, rufinamide, clobazam or cannabidiol may be added.

A variety of specific drugs have been approved by the Food and Drug Administration (FDA) for the treatment of Lennox-Gastaut syndrome including topiramate (Topamax). Topiramate has been approved as an add-on (adjunctive) therapy for children and adults. The drug is manufactured by Ortho-McNeil Neurologics.

The FDA has also approved the anticonvulsant drug felbamate (Felbatol) for the treatment of seizures in children with Lennox-Gastaut syndrome. Due to the occurrence of rare but serious side effects from the drug, physicians should become familiar with the medication and know how to monitor for side-effects before prescribing the medication. This drug, while effective, is not typically first or second line because of the side-effect concerns. The drug is manufactured by Meda Pharmaceuticals, Inc.

In addition, the FDA has approved the lamotrigine (Lamictal) as an add-on (adjunctive) therapy (i.e., as a medication to be used in association with other appropriate anticonvulsant medications) for the treatment of generalized seizures associated with Lennox-Gastaut syndrome. For more information, contact GlaxoSmithKline Inc.

In 2008, the FDA approved rufinamide (Banzel) for the use as an adjunctive (add-on) treatment for seizures associated with Lennox-Gastaut syndrome. Rufinamide decreases seizure frequency in some individuals and seems to be particularly effective for atonic or drop attack seizures. Banzel is manufactured by Eisai Inc.

Clobazam (Onfi) was approved by the FDA in 2011 to treat the seizures associated with Lennox-Gastaut syndrome. Onfi is manufactured by Catalent Pharma Solutions LLC for Lundbeck.

In 2018, Epidiolex (cannabidiol or CBD) was approved to treat seizures associated with Lennox-Gastaut syndrome in patients two years of age and older. This is the first FDA-approved drug that contains a purified drug substance derived from the Cannabis plant. Epidiolex is manufactured by Greenwich Biosciences.

Additional therapies that have been used to treat individuals with Lennox-Gastaut syndrome include the ketogenic diet, VNS Therapy and various surgical techniques. These options are generally reserved for individuals who have been treated with at least 2 – 3 approved medications without an adequate response, and are typically combined with drug therapy (The exception is dietary therapy, which is typically added to drug therapy, but rarely maybe successful by itself in this population.).

(Video) Day 1: Advancements in Treating Rare Diseases

The ketogenic diet may reduce seizure activity in some individuals with Lennox-Gastaut syndrome. The ketogenic diet is a high fat, low carbohydrate diet that makes the body burn fat for energy instead of sugar (glucose). It is a strict diet that requires rigid compliance and commitment. The ketogenic diet can have side effects and individuals following the diet should be routinely monitored by their physicians and a trained nutritionist. The effectiveness of the ketogenic diet varies from one individual to another. Researchers do not understand why the diet is effective in treating seizures or why it is effective for some people, but not others.

Some individuals with Lennox-Gastaut syndrome, especially those who have not responded to other forms of therapy, may be treated with surgical therapies including complete corpus callosotomy or vagus nerve stimulation.

A corpus callosotomy is a surgical procedure in which the cerebral hemispheres are disconnected by cutting the corpus callosum, which is a large bundle of nerves that connects the two halves (hemispheres) of the brain and allows them to share information. This procedure does not include the cutting of brain tissue. This procedure is generally reserved for individuals who suffer from intractable seizures that lead to injuries (e.g., drop seizures or frequent generalized tonic-clonic seizures) or are potentially life-threatening. It is most effective for atonic, tonic and tonic-clonic seizures.

Vagus nerve stimulation is a procedure in which a device called a pulse generator is inserted into the chest and a wire is run underneath the skin to the vagus nerve in the neck. The pulse generator is similar to a pacemaker and transmits mild, electrical impulses to the brain via the vagus nerve. These impulses prevent seizures from occurring. The intensity and timing of the nerve impulses are determined based upon each individual’s needs. This is combined with drug therapy and most effective for drop seizures and generalized tonic-clonic seizures.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [emailprotected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/for-patients-and-families/information-resources/news-patient-recruitment/

For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com

For more information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Contact for additional information about Lennox-Gastaut syndrome:

James W. Wheless, MD
Professor and Chief of Pediatric Neurology
Le Bonheur Chair in Pediatric Neurology
University of Tennessee Health Science Center
Director, Le Bonheur Comprehensive Epilepsy Program & Neuroscience Institute
Le Bonheur Children’s Hospital
49 N Dunlap Avenue, FOB 3rd Floor
Memphis, TN 38105
901-287-5207 (Wilhelmina Alfonso)
901-287-5325 (fax)
[emailprotected]

Supporting Organizations

  • American Epilepsy Society
    • 135 South LaSalle Street
    • Suite 2850
    • Chicago, IL 60603
    • Phone: (312) 883-3800
    • Email: [emailprotected]
    • Website: http://www.aesnet.org
  • CURE: Citizens United for Research in Epilepsy
    • 430 W. Erie
    • Suite Suite 210
    • Chicago, IL 60654
    • Phone: (312) 765-7118
    • Toll-free: (800) 765-7118
    • Email: [emailprotected]
    • Website: http://www.CUREepilepsy.org
  • Epilepsy Foundation
  • Genetic and Rare Diseases (GARD) Information Center
  • LGS Foundation, Inc.
  • NIH/National Institute of Neurological Disorders and Stroke

References

TEXTBOOKS
Pellock JM, Nordli DR, Sankar R, Wheless JW (ED’s.) Pellock’s Pediatric Epilepsy, 4th Edition.Demos Medical. NYC, NY. 2017: 451-466.

Acosta MT, Pearl PL. Lennox-Gastaut syndrome. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:549-550.

JOURNAL ARTICLES
Gresham J, Eiland LS, Chung AM. Treating Lennox-Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide. Neuropsychiatr Dis Treat. 2010;6:639-645.

Wheless JW. Managing severe epilepsy syndromes of early childhood. J Child Neurol. 2009;24:24S-32S.

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

Stafstrom CE. Update on the management of Lennox-Gastaut syndrome with a focus on rufinamide. Neurophyschiatr Dis Treat. 2009;5:547-551.

Wheless JW, Clarke DF, Arzimanoglou A, Carpenter D. Treatment of pediatric epilepsy: European expert opinion, 2007. Epileptic Disord. 2007;9:353-412.

Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol. 2003;20:426-441.
Trevathan E. Infantile spasms and Lennox-Gastaut syndrome. J Child Neurol. 2002;17:2S9-2S22.

Frost M, Gates J, Helmers SL, et al. Vagus nerve stimulation in children with refractory seizures associated with Lennox-Gastaut syndrome. Epilepsia. 2001;42:1148-1152.

INTERNET
Cherian KA, Glauser TA, Lennox-Gastaut Syndrome. Medscape. Updated: Nov 09, 2018. Available at: http://emedicine.medscape.com/article/1176735-overview Accessed June 2, 2020.

National Institute of Neurological Disorders and Stroke. Lennox-Gastaut Syndrome Information Page. Last Update 2019-03-27. Available at https://www.ninds.nih.gov/Disorders/All-Disorders/Lennox-Gastaut-Syndrome-Information-Page Accessed June 2, 2020.

(Video) CORDs Rare Disease

Years Published

1992, 1993, 1994, 1995, 1996, 1998, 2002, 2011, 2014, 2017, 2020

The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.

(Video) INFANTILE SPASMS: KNOW THE SIGNS AND TAKE ACTION

The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.

National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100

FAQs

Is Lennox-Gastaut syndrome a rare disease? ›

Lennox-Gastaut syndrome affects an estimated 1 to 2 per million people. This condition accounts for less than 5 percent of all cases of childhood epilepsy. For unknown reasons, it appears to be more common in males than in females.

How long do people with Lennox-Gastaut live? ›

What's the outlook for this condition? The biggest risks from Lennox-Gastaut syndrome come from damage to the brain due to uncontrolled seizures or falls that happen because of seizures. Because of those risks, people with LGS have a death rate between 3% and 7% over 10 years after their diagnosis.

Is Lennox-Gastaut syndrome considered a disability? ›

Lennox-Gastaut syndrome is a typical childhood-onset, severe epileptic encephalopathy associated with a serious intellectual disability in 20%-60% patients at the time of the onset of seizures, the proportion of which will increase to 75%-95% at five years after the onset of seizures.

What triggers Lennox-Gastaut syndrome? ›

Lennox-Gastaut syndrome can be caused by a variety of conditions, including brain malformations, tuberous sclerosis, perinatal asphyxia, severe head injury, central nervous system infection, and inherited genetic and inherited degenerative or metabolic conditions. In 30-35 percent of individuals, no cause can be found.

How many people have Lennox-Gastaut syndrome? ›

Approximately 13,400 children under the age of 18 in the United States are estimated to have LGS. Approximately 34,300 adults 18 years and older in the United States are estimated to have LGS. This means approximately 48,000 children and adults in the United States currently have LGS.

What is the most serious type of epilepsy? ›

Overview. A grand mal seizure causes a loss of consciousness and violent muscle contractions. It's the type of seizure most people picture when they think about seizures. A grand mal seizure — also known as a generalized tonic-clonic seizure — is caused by abnormal electrical activity throughout the brain.

Is Lennox-Gastaut progressive? ›

Lennox-Gastaut syndrome is a progressive epilepsy syndrome that causes tonic and atypical absence seizures and intellectual disability. It is difficult to treat, although some newer treatments are being investigated.

How rare is LGS? ›

How common is it? "Lennox-Gastaut syndrome accounts for only 2 to 5% of childhood epilepsies." Yet children with this type of epilepsy are well-known to both pediatric and adult neurologists. Seizures in people with LGS are hard to control and continue throughout life.

What medication is used to treat Lennox-Gastaut syndrome? ›

Valproate (valproic acid) is generally considered the first-line therapy for Lennox-Gastaut syndrome because it is effective against a wide spectrum of seizures.

Can Lennox-Gastaut syndrome cause death? ›

The mortality rate is 5%. Those with LGS are 24 times more likely to die prematurely. Premature death in LGS is often due to SUDEP*, seizures, injury, or the underlying brain disorder. LGS occurs secondary to many different causes including injury, brain malformations, infections, and genetic factors.

What are the 4 types of epilepsy? ›

There are four main types of epilepsy: focal, generalized, combination focal and generalized, and unknown. A person's seizure type determines what kind of epilepsy they have.
...
The three primary seizure types are:
  • generalized seizures.
  • focal seizures.
  • unknown seizures.
21 Nov 2019

Can adults have Lennox-Gastaut syndrome? ›

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy with childhood onset that usually continues through adolescence and into adulthood. In the long term, patients with this condition still have intractable seizures, intellectual disability, behavioral problems, and physical comorbidities.

How do seizures lead to death? ›

A seizure may cause a person to have pauses in breathing (apnea). If these pauses last too long, they can reduce the oxygen in the blood to a life-threatening level. In addition, during a convulsive seizure a person's airway sometimes may get covered or obstructed, leading to suffocation.

Which drug will a nurse expect to see prescribed for a patient with Lennox-Gastaut syndrome? ›

Lamotrigine is indicated as an adjunctive therapy for partial seizures, primary generalized tonic–clonic seizures, and generalized seizures of Lennox–Gastaut syndrome in patients two years of age or older.

What brain conditions cause seizures? ›

Health conditions that cause seizures
  • epilepsy.
  • diabetes.
  • meningitis, an infection of the membranes that surround the brain.
  • encephalitis, inflammation of the brain.
  • dementia, including Alzheimer's disease.
  • a stroke.
  • in rare cases, a brain tumor.
9 Sept 2021

What type of seizure causes you to stop breathing? ›

Tonic-clonic seizures, formerly known as grand mal seizures, comprise two stages: a tonic phase and a clonic phase. These intense seizures can be frightening to experience or observe, as extreme muscle spasms may temporarily arrest breathing.

What are the symptoms of LGS? ›

Patients with LGS experience many different types of seizures including:
  • Tonic - stiffening of the body.
  • Atonic - temporary loss of muscle tone and consciousness, causing the patient to fall.
  • Atypical absence - staring episodes.
  • Myoclonic - sudden muscle jerks.

How do you say Lennox-Gastaut syndrome? ›

Lennox-Gastaut syndrome (LGS) visual mnemonic - YouTube

What does an epileptic cry sound like? ›

The person will usually emit a short, loud cry as the muscles in the chest contract and the air rushes between the vocal cods, making a sound. This cry does not indicate pain.

What does a seizure feel like in your head? ›

If the abnormal electrical activity involves a large area of the brain, the person may feel confused or dazed, or experience minor shaking, muscle stiffening, or fumbling or chewing motions. Focal seizures that cause altered awareness are called focal unaware seizures or complex partial seizures.

How long can a seizure last before brain damage? ›

A seizure that lasts longer than 5 minutes, or having more than 1 seizure within a 5 minutes period, without returning to a normal level of consciousness between episodes is called status epilepticus. This is a medical emergency that may lead to permanent brain damage or death.

Is there a cure for LGS? ›

There is no cure for LGS but numerous treatments for seizures are available. The goal of seizure treatment in LGS is to minimize the seizures, treatment side effects, and the number of medications as well as to attain the best quality of life for the individual with LGS and their loved ones.

When was Lennox-Gastaut syndrome discovered? ›

LGS was described in 1966 by the Marseille School in France, where Gastaut et al. (1966) generously proposed the term Lennox syndrome to denote a childhood-onset epilepsy characterized by frequent tonic and absence seizures.

Is genetic epilepsy curable? ›

There's no cure for epilepsy. But there are many options to treat epilepsy.

Is LGS a disability? ›

LGS is a lifelong neurodevelopmental disorder and seizures are the main feature, are frequent, and are debilitating. Therapies may help. However, they do not completely stop seizures. Persons with LGS have varying degrees of intellectual disability and behavioral issues.

How many stages of epilepsy are there? ›

Seizures take on many different forms and have a beginning (prodrome and aura), middle (ictal) and end (post-ictal) stage.

Can adults develop LGS? ›

Although onset after age 8 is rare, LGS has been diagnosed into adolescence and adulthood [4], [5], [6].

What does a focal aware seizure look like? ›

Patients experiencing a complex focal seizure may stare blankly into space, or experience automatisms (non-purposeful, repetitive movements such as lip smacking, blinking, grunting, gulping or shouting).

What are drop seizures? ›

Atonic seizures are a type of seizure that causes sudden loss of muscle strength. These seizures are also called akinetic seizures, drop attacks or drop seizures. The sudden lack of muscle strength, or tone, can cause the person to fall to the ground. The person usually remains conscious, and may not always fall down.

Are myoclonic jerks seizures? ›

Myoclonic seizures are a type of seizure that causes sharp, uncontrollable muscle movements. They're usually minor and brief, but can happen with very severe seizure disorders. They're most common with childhood seizure conditions, but can also happen in adults.

What are the 3 major groups of seizures? ›

There are now 3 major groups of seizures.
  • Generalized onset seizures:
  • Focal onset seizures:
  • Unknown onset seizures:

What is the difference between epilepsy and seizures? ›

Epilepsy vs Seizures

A seizure is a single occurrence, whereas epilepsy is a neurological condition characterized by two or more unprovoked seizures.

What is the difference between grand mal and petit mal epilepsy? ›

Tonic-clonic seizures may cause a person to lose consciousness, this may cause them to fall to the ground, have muscle jerks or spasms, and cry out. They are also called grand mal seizures. Absence seizures cause rapid blinking or staring into space for a few seconds. They are also called petit mal seizures.

Videos

1. 7th International LGS Family Conference: Katherine Junger, PhD, - Caring for the Caregiver
(LGS Foundation (Lennox-Gastaut Syndrome))
2. Early infantile epileptic encephalopathy - causes, symptoms, diagnosis, treatment, pathology
(Osmosis)
3. Lily 2018 - Epilepsy in mitochondrial disease
(The Lily Foundation)
4. Matty Moo's Story
(Epilepsy Foundation)
5. Zogenix: our commitment to developing new treatments for Patients living with rare diseases.
(CDKL5 Alliance)
6. AES Professor's Rounds: Pediatric Epilepsy Case Studies
(American Epilepsy Society)
Top Articles
Latest Posts
Article information

Author: Sen. Ignacio Ratke

Last Updated: 01/10/2023

Views: 5919

Rating: 4.6 / 5 (56 voted)

Reviews: 87% of readers found this page helpful

Author information

Name: Sen. Ignacio Ratke

Birthday: 1999-05-27

Address: Apt. 171 8116 Bailey Via, Roberthaven, GA 58289

Phone: +2585395768220

Job: Lead Liaison

Hobby: Lockpicking, LARPing, Lego building, Lapidary, Macrame, Book restoration, Bodybuilding

Introduction: My name is Sen. Ignacio Ratke, I am a adventurous, zealous, outstanding, agreeable, precious, excited, gifted person who loves writing and wants to share my knowledge and understanding with you.