What is the treatment for Lennox-Gastaut syndrome?
Alva-Moncayo E, Ruiz-Ruiz A. The value of topiramate used with conventional schemes as an adjunctive therapy in the treatment of Lennox-Gastaut syndrome. Revista de Neurologia. 2003; 36:453–7. [Google Scholar] Angelini L, Broggi G, Riva D, et al. A Case of Lennox-Gastaut Syndrome Successfully Treated by Removal of a Parietotemporal Astrocytoma.
What is the prognosis for Lennox Gastaut syndrome?
Surgery. If medications and other treatments aren't reducing the number of seizures, your doctor may suggest surgery. The vagus nerve stimulator is a small device placed in the arm or near the ...
What are the causes of Lennox Gastaut syndrome?
· Lennox-Gastaut syndrome can be very difficult to treat. A combination of seizure medications and other treatments may be used to improve seizure control and other associated conditions. The medication valproate is generally considered a first-line therapy for …
When was rufinamide approved for the treatment of Lennox-Gastaut syndrome (LGS)?
Family training and support is a key element in a successful treatment plan. Parents and caregivers must know how to watch for and respond to seizures. Lennox-Gastaut syndrome treatment will generally include physical therapy, occupational therapy and speech therapy.
Can Lennox-Gastaut be cured?
The prognosis for individuals with Lennox-Gastaut syndrome varies. There is no cure for the disorder. Complete recovery, including freedom from seizures and normal development, is very rare.
How long can you live with Lennox-Gastaut?
Their mean age was 23.0 ± 3.8 years (range 18.7–35.8 years) and the mean follow-up duration was 19.3 ± 6.0 years (range 8.3–32.5 years)....Clinical profiles.CharacteristicsValues>60 months, n (%)21 (30.9)Brain MRI, n (%)Normal22 (32.4)Atrophy only11 (16.2)14 more rows
Can LGS be cured?
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.
What is life expectancy for LGS?
The progressive intellectual impairment associated with Lennox-Gastaut syndrome means that many patients require full-time care throughout life. The short-term mortality of Lennox-Gastaut syndrome is approximately 4-7%.
Is Lennox-Gastaut syndrome fatal?
People with Lennox-Gastaut syndrome have a higher risk of death than their peers of the same age. Although the increased risk is not fully understood, it is partly due to poorly controlled seizures and injuries from falls.
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.
Is Lennox-Gastaut syndrome 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.
Is Lennox-Gastaut syndrome a rare disease?
Lennox-Gastaut syndrome (LGS) is a rare and severe kind of epilepsy that starts in childhood. Children with LGS have seizures often, and they have several different kinds of seizures. This condition is hard to treat, but researchers are looking for new therapies.
What age does Lennox-Gastaut syndrome start?
LGS is most common in children between the ages of 2 and 7 years old. Boys are slightly more likely to be affected than girls. In most cases the cause for LGS is known.
Is Fintepla approved for LGS?
U.S. FDA Approves FINTEPLA® ▼(fenfluramine) Oral Solution for Treatment of Seizures Associated with Lennox-Gastaut Syndrome (LGS)
What is Dravet Syndrome and Lennox-Gastaut syndrome?
Introduction. Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are rare central nervous system diseases [1,2] considered serious epileptic encephalopathies, generally resistant to treatment, that cause different types of epileptic seizures as well as cognitive and behavioural changes [3-6].
What is the diagnosis of Lennox-Gastaut syndrome?
Lennox-Gastaut syndrome ( LGS) is a rare and severe kind of epilepsy that starts in childhood. Children with LGS have seizures often, and they have several different kinds of seizures. This condition is hard to treat, but researchers are looking for new therapies. Finding practical and emotional support is key to help you give your child ...
What are the symptoms of LGS?
Symptoms. Children with LGS have frequent and severe seizures. And they often have different kinds of seizures, including: Atonic seizures. Also called "drop attacks," because the person loses muscle tone and can fall to the ground. Their muscles may jerk.
What are the developmental problems of LGS?
They can also have behavioral problems. Each child develops differently, and it's impossible to predict how a child with LGS will do.
Can LGS cause behavioral problems?
They can also have behavioral problems. Each child develops differently, and it's impossible to predict how a child with LGS will do. While most children have ongoing seizures and some form of learning disability, some may respond well to treatment and have fewer seizures.
What is the name of the brain problem where some nerve fibers in the brain don’t line up right during development
A brain problem called cortical dysplasia, where some nerve fibers in the brain don’t line up right during development in the womb. Tuberous sclerosis, where non-cancerous tumors form in many places throughout the body, including the brain. Genetics.
Can a child with LGS have seizures?
While most children have ongoing seizures and some form of learning disability, some may respond well to treatment and have fewer seizures.
Why is Lennox-Gastaut syndrome so difficult to treat?
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.
What medications are used for Lennox-Gastaut syndrome?
Such medications may include clonazepam, sodium valproate, topiramate, lamotrigine, felbamate (closely monitored), clobazam,rufinamide or cannabidiol.
What are the three findings 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.
Can Lennox-Gastaut syndrome affect children?
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.
Is intelligence affected by Lennox-Gastaut syndrome?
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.
Can Lennox-Gastaut syndrome cause intellectual impairment?
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.
Is Lennox-Gastaut syndrome cryptogenic?
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.
Can Lennox-Gastaut syndrome be controlled?
The seizures in Lennox-Gastaut syndrome can be difficult to control, as they often don’t respond to anti-epileptic medications. A combination of anti-seizure medications to control the different seizure types may help to provide partial relief. Rescue therapies, a different set of medications, may be given to help stop or shorten clusters ...
Is Lennox-Gastaut syndrome a sudden death?
Regular medical care is needed to adjust treatment to changing seizure patterns. Children and adults with Lennox-Gastaut syndrome are at an increased risk of premature mortality, often from sudden unexpected death in epilepsy (SUDEP), injury, seizures, or as a consequence of the underlying brain disorder.
What causes Lennox-Gastaut syndrome?
These causes include structural brain differences caused by an acquired brain injury or infection, genetic syndromes that cause differences in brain development, or hereditary metabolic disorders.
Is Lennox-Gastaut syndrome a genetic disorder?
In many cases (approximately 70%), Lennox-Gastaut syndrome is secondary to an identifiable cause. These causes include structural brain differences caused by an acquired brain injury or infection, genetic syndromes that cause differences in brain development, or hereditary metabolic disorders. In a quarter to a third of cases, no cause can be ...
What is a generalized tonic clonic seizure?
Generalized tonic-clonic seizures (also called grand mal seizures) Many children with the condition have developmental delays, cognitive impairments or behavioral problems. These problems may be noticed before the first appearance of seizures.
What is the best treatment for seizures?
Rescue therapies, a different set of medications, may be given to help stop or shorten clusters of seizures when they occur. Implantable devices such as Vagus Nerve Stimulation (VNS) or Responsive Neurostimulation (RNS) may be considered when medications are not effective in controlling seizures.
Can corpus callosotomy be used for atonic seizures?
Corpus callosotomy surgery to separate the two halves of the brain may be effective for atonic seizures, tonic clonic seizures, and tonic seizures and may significantly reduce seizure frequency and duration in patients with LGS. Family training and support is a key element in a successful treatment plan.
What is the goal of Lennox-Gastaut syndrome?
The goals of treatment for patients with Lennox-Gastaut syndrome (LGS) are the same as for all patients with epilepsy: the best quality of life with the fewest seizures (ideally, none), the fewest adverse treatment effects, and the least number of medications.
When was Rufinamide approved?
In February 2015, rufinamide was approved in pediatric patients aged 1 to 4 years based on a pharmacokinetic bridging study of a Phase III clinical trial which demonstrated the pharmacokinetic and safety profiles are consistent with those seen in ages ≥ 4 years. [ 31] Benzodiazepines.
Is topiramate safe for LGS?
Topiramate. In a multicenter, double-blind, placebo-controlled trial, topiramate (Topamax) was found to be safe and effective as adjunctive therapy for patients with LGS. [ 20] .
Is ketogenic diet good for epilepsy?
In general, the benefits of the diet for people with epilepsy include fewer seizures, less drowsiness, better behavior, and need for fewer concomitant AEDs. Based on multiple open-label and, most recently, randomized controlled studies, the ketogenic diet appears to be a useful therapy for patients with LGS.
Do LGS patients wear helmets?
Some patients with LGS wear protective helmets with face guards to maximize protection of the forehead, nose, and teeth (see the image below). Unfortunately, some patients with LGS do not tolerate the helmet with face guards, and even if tolerated, helmets often are uncomfortable and rarely are cosmetically acceptable.
Is zonisamide safe for children with LGS?
A multicenter study of zonisamide for long-term adjunctive therapy in 62 children with LGS found that it was safe and effective; 4.8% of children had 100% seizure freedom, and 22.6% had >75% reduction in their seizures, independent of seizure type.
What is the incidence rate of Lennox-Gastaut syndrome?
LGS is a rare disease; it has an estimated incidence rate of 0.1 to 0.28 per 100,000 overall, with an incidence rate of 2 per 100,000 in children, accounting for approximately 2–5% of all childhood epilepsies, with a slight male preponderance [ 3 ].
What are the treatments for LGS?
Six treatments are specifically indicated as adjunct therapies for the treatment of seizures associated with LGS in the US: lamotrigine, clobazam, rufinamide, topiramate, felbamate and most recently cannabidiol.
What is LGS in medical terms?
Lennox-Gastaut syndrome (LGS), a childhood-onset severe developmental and epileptic encephalopathy (DEE), is an entity that encompasses a heterogenous group of aetiologies, with no single genetic cause.
Is drug refractory seizures a hallmark of LGS?
Drug-refractory seizures are a hallmark and treatment is further complicated by its multiple morbidities, which evolve over the patient’s lifetime. This review provides a comprehensive overview of the current and future options for the treatment of seizures associated with LGS.
When was Rufinamide approved?
As such, rufinamide was approved in the EU in 2007 and in the US in 2008 as adjunctive therapy for the treatment of seizures associated with LGS in children and adults aged 4 years and older, which was expanded to include patients 1 year of age and older in 2015 in the US and in 2018 in the EU [ 65, 66 ].
What is fenfluramine used for?
fenfluramine acts via multiple receptors to exert its therapeutic effects for the treatment of seizures, although the exact mechanisms are still being elucidated. Fenfluramine increases extracellular levels of serotonin (5-HT) through interaction with serotonin transporter proteins, and exhibits agonist activity at serotonin 5HT-2 receptors (5HT1D and 5HT2C) [ 97 – 100 ]. In addition, at the in vitro and in vivo level, fenfluramine activity at 5-HT receptors is complemented synergistically by functional activation of sigma-1 receptors, resulting in improved cognitive functions of spatial and contextual learning via activity at sigma-1 receptors in mouse models [ 101 ]. Fenfluramine was previously prescribed as an anorexigen, but it was withdrawn in 1997 based on reports of increased risks of cardiac valvulopathy and pulmonary hypertension in adult patients treated for obesity; however, the fenfluramine doses were much higher compared with a licensed maximum dose for DS, and it was frequently given off-label with phentermine, which itself has an impact on valvular disease [ 102 – 104 ]. In contrast, the benefit/risk profile of fenfluramine given at low dose for the treatment of seizures appears to be positive, and fenfluramine has been approved by the FDA in the US for the treatment of seizures associated with DS in patients age 2 years and older [ 98 ]. In December 2020 fenfluramine was approved by the EMA in the EU for the treatment of DS.
Is cognitive impairment included in LGS?
In contrast, cognitive impairment ( intellectual disability and associated behavioural problems) is not always present at the outset of LGS and therefore this part of the ‘triad’ is not always included in the diagnostic criteri a. b Brain damage can be the result of hypoxia at birth or head injuries, among others.
Watch: The Latest Treatment Options for LGS
Scott Perry, MD, Medical Director of Neurology & Genetics Epilepsy Center at Cook Children’s Hospital speaks on the latest treatments available for those living with Lennox-Gastaut Syndrome. Dr. Perry also offers insight on how your medical team can determine the best options for your loved one with LGS.
Clinical Trials in LGS
Research is ongoing to find novel, innovative treatments for LGS. Clinical trials are research studies performed in those with LGS. They scientifically evaluate a medical or surgical intervention.
Costs
A 2015 report showed that average yearly medical costs are about $64,000 for LGS. This number depends on insurance status. Hospitalizations and home health services are a big expense. Many medication options are generic and are relatively inexpensive. Newer medications, such as cannabidiol, are more expensive but are generally covered by insurance.
Outlook
LGS always persists into adulthood. Certain types of seizures are more common in adults than others with LGS. Over time, those with LGS experience developmental slowing, plateauing or regression. This culminates in moderate to severe intellectual disability in more than 90% of patients. Behavior disorders are common in childhood and adolescence.
What is the diagnosis of Lennox-Gastaut syndrome?
Lennox-Gastaut syndrome or LGS is a severe and rare form of epilepsy which starts in childhood. Children suffering from Lennox-Gastaut syndrome or LGS have frequent and various types of seizures. The seizures commonly start between the ages of 2 and 6. Patients suffering from Lennox-Gastaut syndrome or LGS will also have moderate to severe developmental delays and learning difficulties. They also may have behavioral problems.
Can Lennox-Gastaut syndrome be predicted?
It is not possible to predict the course of this condition in a child with Lennox-Gastaut syndrome, as each child develops differently. Many children have constant seizures and some type of learning disability, whereas there are some children who respond well to treatment and have decreased number of seizures.
Can Lennox-Gastaut syndrome cause behavioral problems?
Patients suffering from Lennox-Gastaut syndrome or LGS will also have moderate to severe developmental delays and learning difficulties. They also may have behavioral problems. It is not possible to predict the course of this condition in a child with Lennox-Gastaut syndrome, as each child develops differently.
What causes Lennox-Gastaut syndrome in children?
Lack of oxygen or insufficient oxygen during birth can cause Lennox-Gastaut syndrome in child. Brain infections, such as meningitis, encephalitis and rubella can also cause the child to have Lennox-Gastaut syndrome. Acute injuries to the brain in association with pregnancy or birth, such as premature birth or low birth weight.
What are the behavioral problems of Lennox-Gastaut syndrome?
Children suffering from Lennox-Gastaut syndrome or LGS have severe, frequent and different types of seizures including: Atonic Seizures: These are also known as “drop attacks,” as there is loss of muscle tone which results in the patient falling to the ground.
Why is it important to talk to other families about Lennox-Gastaut syndrome?
Talking with other families who are facing the same challenges helps in reducing the feelings of isolation.
What is the best medication for seizures in children?
It takes time to find out the right treatment for the child. Medicines used in treating seizures are: Clobazam (Onfi), rufinamide (Banzel), divalproex sodium (Depakote), lamotrigine (Lamictal ) and topiramate (Topamax). There’s no single drug which completely controls the seizures.
Symptoms
Signs and symptoms
Classification
Causes
Epidemiology
Diagnosis
Treatment
Medical uses
- 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 or clobazam may be added. In 2008, the FDA approved rufinam...
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