Posts Tagged Lennox-Gastaut Syndrome

[WEB PAGE] Types of Epilepsy | MyEpilepsyTeam

 

Article written by Kelly Crumrin

Epilepsy is a spectrum of neurological disorders that involve recurring seizures caused by abnormal electrical activity in the brain. Diagnosing the specific type of epilepsy helps you and your doctor better know what to expect regarding seizure progression, any challenges in learning and physical development, and which treatments will be most effective.

Each type of epilepsy involves specific types of seizures and other symptoms.

Localized epilepsies and syndromes

Some types of epilepsy are referred to as localized, which means they are known by the location in the brain where seizures originate. A related term is focal epilepsy, which indicates seizures that have abnormal electrical activity focused in one area of the brain. Other types of epilepsy are known as syndromes, or collections of specific signs and symptoms that point to a certain medical condition. Each type of epilepsy has unique characteristics. Some syndromes are considered benign, meaning children will eventually become seizure-free with age, while other types of epilepsy are lifelong conditions.

Temporal lobe epilepsy (TLE)

Approximately 60 percent of people with epilepsy have temporal lobe epilepsy (TLE), or seizures that originate in the temporal lobe of the brain. The temporal lobe is located on the sides of the brain, near the ears, and is responsible for processing sound and language as well as memories relating to sound and vision. About one-third of TLE or other cases of focal epilepsy are resistant to anti-epileptic drugs (AEDs). Surgery may be recommended, and vagus nerve stimulation may also be therapeutic.

TLE usually develops between late adolescence and early adulthood, often after a head injury or febrile (fever-induced) seizure. For women with TLE, hormonal changes during the menstrual cycle can increase seizure activity. Focal onset seizures are most common for people with TLE, though some people may experience prolonged seizures or, in rare circumstances, status epilepticus. Read more about seizure types and symptoms.

Frontal lobe epilepsy (FLE)

Frontal lobe epilepsy is the second most common form of focal epilepsy after TLE. FLE may be inherited, or it may be caused by a structural problem such as a birth defect, an abnormal blood vessel, trauma, or scaring caused by infection. In about 50 percent of FLE cases, no cause is ever determined.

The frontal lobes of the brain are large, and some of its functions include reasoning, paying attention, regulating emotion, organizing, and solving problems. Researchers have not discovered the functions of many areas. It is possible for a seizure to begin in the frontal lobe and proceed without symptoms before spreading to other parts of the brain, leading to a tonic-clonic seizure. FLE seizure symptoms can vary widely, depending on the function of the affected part of the lobe. Learn more about seizure symptoms and types.

FLE is usually responsive to medications, but in cases where AEDs are ineffective, surgery or vagus nerve stimulation may help.

Occipital lobe epilepsy and parietal lobe epilepsy

Epilepsy that originates in the occipital and parietal lobes is much less common than TLE and FLE. Seizures that begin in these lobes are usually idiopathic – of unknown cause. The occipital lobe is in the back of the brain and is primarily responsible for vision. The parietal lobe is located on the top and upper sides of the head and is known as the “association cortex” because it is where perception becomes reality. Sounds are recognized as words, visual images are created, and touch becomes associated with an object.

In both types of epilepsy, AEDs are the first treatment option. If medication fails, surgery may be recommended.

Panayiotopoulos syndrome (PS)

Also known as early onset occipital epilepsy, PS commonly begins in early childhood. Typically, onset is between the ages of 3 and 10. PS affects boys and girls equally, and it is idiopathic (cause unknown). As many as 6 percent of children who have nonfebrile (not caused by fever) seizures have PS. PS frequently stops two to three years after the first seizure.

Children with PS will have focal seizures that can spread to a generalized seizure. Seizures in PS often last 20 to 60 minutes, and more than half occur during sleep. Pale skin, a sick feeling, and vomiting are typical symptoms during a PS seizure. Some children may also have tonic-clonic movements. Read more about types of seizures.

If seizures are infrequent, medication may not be needed. However, if they are needed, AEDs are usually effective at controlling PS seizures. Neurologists often teach parents how to initiate rescue therapy and create an emergency plan for children with PS.

Benign rolandic epilepsy (BRE)

Also known as benign epilepsy with centrotemporal spikes (BECTS), BRE usually begins around ages 6 to 8. Boys are slightly more likely to have BRE than girls. BRE accounts for approximately 15 percent of all epilepsies in children.

Benign rolandic epilepsy is characterized by numbness, twitching, or tingling of the face or tongue. Seizures may inhibit speech and cause drooling. The child remains conscious during the seizure. Seizures are not frequent and occur mostly at night. AEDs may be prescribed if the seizures happen during the day or disrupt sleep, but many children do not need medication. Seizures stop by early adolescence in almost all children with BRE.

Generalized epilepsies and syndromes

In contrast to localized types of epilepsy discussed above, generalized types of epilepsy feature seizures that do not originate, or do not remain confined, in one lobe or area of the brain. Generalized epileptic syndromes tend to be idiopathic – of unknown cause. Idiopathic generalized epilepsies account for one-third of epilepsy cases.

Juvenile myoclonic epilepsy (JME)

JME, also known as Janz syndrome, begins between the ages of 8 and 26, but most commonly between the ages of 12 and 16. Absence seizures may be the first type of seizure most people with JME experience, although this type happens less often. Mild myoclonic seizures, generalized tonic-clonic (GTC), or clonic-tonic-clonic seizures (GTC seizures that begin with a clonic phase) seizures are the most common types. Myoclonic seizures tend to occur immediately upon waking in the morning. Photosensitivity – seizures triggered by flashing or flickering light – affects 40 percent of people with JME. Photosensitive seizures usually show on an electroencephalography (EEG) test. Read more about seizure types.

Most cases of juvenile myoclonic epilepsy are treatable with AEDs. Most people with JME need to remain on medication for life.

Childhood absence epilepsy (CAE)

CAE accounts for 2 to 8 percent of childhood epilepsy. Childhood absence epilepsy typically begins between the ages of 3 and 11, most frequently between ages 5 and 8. One-third of children with CAE have a family history of seizures, suggesting that the cause may be genetic. Siblings of children with CAE have a 1-in-10 chance of developing epilepsy.

Children with CAE experience absence seizures (formerly known as petit mal seizures). The child is not aware or responsive during seizures, and may stare, blink, or roll their eyes up. You may notice a chewing motion or other repetitive movements. Seizures last about 10 seconds, after which the child immediately returns to normal. The child is usually not aware they have had a seizure. Seizures may be infrequent or happen as often as 100 times a day. One-third of children with CAE have concentration and memory problems before seizures start; these issues often improve after AEDs are started. Rarely, children who have very frequent seizures may develop learning difficulties.

If AEDs are not effective, the ketogenic diet may help children with CAE. At least two-thirds of children with CAE respond to treatment, and their seizures will cease by mid-adolescence. However, 10 to 15 percent of children with CAE will develop other seizure types during adolescence – typically myoclonic seizures, generalized tonic-clonic seizures, or both. Read more about seizure types.

Juvenile absence epilepsy (JAE)

JAE is similar to childhood absence epilepsy; however, it starts later in childhood (generally between ages 9 and 13) and is usually a lifelong condition. Two percent of people with epilepsy have juvenile absence epilepsy (JAE). Although it is rare to have a family history of seizures, the cause of JAE is thought to be genetic.

People with JAE experience absence seizures lasting from 10 to 45 seconds. Seizures may happen infrequently or 100 times a day. Seizures often happen during exercise. Seventy-five percent of those with JAE will also have tonic-clonic seizures. The risk of absence status epilepticus (also known as nonconvulsive status epilepticus), in which seizures can last minutes or even hours, is higher in people with JAE.

Children with JAE generally develop normally, though they may experience learning difficulties if they have frequent seizures. One-third of children with JAE have concentration and memory problems before seizures start; these issues often improve after AEDs are started. AEDs work well to treat JAE and must be taken for life.

Lennox-Gastaut syndrome (LGS)

LGS is an uncommon epilepsy syndrome; between 2 and 5 percent of children with epilepsy have LGS. Lennox-Gastaut syndrome often starts between the ages of 3 and 5. Atonic seizures, also known as drop attacks, are most common in children with Lennox-Gastaut syndrome (LGS). Seizures may happen multiple times a day and cause the child to suddenly drop to the ground. Drop attacks may be perceived as a trip or the result of poor balance. Injuries are common, making the seizures very upsetting for the child. Atypical absence seizures and tonic seizures are also common, especially at night, but children with LGS may experience other types of seizures as well.

LGS is very difficult to treat and is often referred to as intractable or refractory. Some AEDs can be effective, and the ketogenic diet may help. Surgery may be recommended if diet and medication do not work, though surgical treatment will not stop the seizures altogether.

Children with LGS have moderate to severe learning difficulties, with some children exhibiting developmental delays before their first seizure. Twenty percent of children who have West syndrome (infantile spasms) will develop LGS.

Progressive myoclonic epilepsies

The progressive myoclonic epilepsies are a group of rare syndromes characterized by a combination of tonic-clonic and myoclonic seizures. Disorders that fall under this category include Lafora disease, mitochondrial encephalopathies, severe myoclonic epilepsy of infancy (also referred to as Dravet syndrome), and Unverricht-Lundborg disease (also known as Baltic myoclonus). The cause is often hereditary but may be unknown. Progressive myoclonic epilepsies affect males and females equally and start at different ages, depending on the specific condition. Read more about seizure types.

In progressive myoclonic epilepsies, seizures are difficult to control. As the condition progresses, people with PME accumulate cognitive (relating to thinking and memory) and motor (relating to movement) disabilities.

Medications may be successful at first, but effectiveness declines over months or years as the disease progresses.

Other epilepsy syndromes

Epilepsy is a broad spectrum involving dozens of neurological disorders. While those described above are the most commonly diagnosed, there are many other syndromes that cause epilepsy. Examples include Angelman syndrome, Doose syndrome (myoclonic astatic epilepsy), Dravet syndrome, neurocutaneous syndromes (such as Sturge-Weber syndrome), Rasmussen’s syndrome, and Rett syndrome.

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[WEB SITE] FDA approves marijuana based medication for epilepsy treatment

 

An advisory panel from the United States Food and Drug Administration (FDA) has recommended the approval of a novel epilepsy drug that is made up of ingredients from marijuana. The agency normally follows the recommendations of the advisory panels regarding approvals and rejections of applications of new drugs. The recommendation statement came yesterday (19th April 2018).

If this drug gets a green light, it is expected to become the first cannabis-derived prescription medicine to be available in the US. The drug is named Epidiolex and is made by GW Pharmaceuticals from Britain. It contains cannabidiol or CBD that is derived from cannabis. However the drug is not seen to cause any intoxication among the users.

Marijuana plant flowering outdoors. Image Credit: Yarygin / Shutterstock

Marijuana plant flowering outdoors. Image Credit: Yarygin / Shutterstock

The use of only one of the components of cannabis also makes it different from medical marijuana that is approved for pain management and other conditions around the world and in the United States. Synthetic forms of chemicals in the cannabis plant are also used to treat nausea among cancer patients and in AIDS patients to prevent weight loss.

Dr. Igor Grant, director of the Center for Medicinal Cannabis Research at the University of California San Diego welcomed this new recommendation from the panel saying, “This is a very good development, and it basically underscores that there are medicinal properties to some of the cannabinoids… I think there could well be other cannabinoids that are of therapeutic use, but there is just not enough research on them to say.”

As of now the panel has recommended the use of this new drug for two types of epilepsy only – Lennox-Gastaut syndrome and Dravet syndrome. These are notoriously difficult to treat and most people continue to have seizures despite treatment. Multiple seizures may occur in a day and this makes the children with these conditions vulnerable for developmental and intellectual disabilities. Lennox-Gastaut syndrome can appear in toddlers at around ages 3 to 5 and Dravet syndrome is usually diagnosed earlier. Nearly 30,000 children and adults suffer from Lennox-Gastaut syndrome and similar numbers of people are diagnosed with Dravet syndrome. Due to the small population of diagnosed patients Epidiolex was filed and classified under orphan drug status.

An orphan drug is one that is developed for a relatively rare disease condition. The FDA provides special subsidies and support for development of orphan drugs and often speed tracks their approval process.

The recommendation from the advisory panel is based on the results of three randomized, double-blind, placebo-controlled trials that included patients of both these disease conditions. The agency statement says, “The statistically significant and clinically meaningful results from these three studies provide substantial evidence of the effectiveness of CBD for the treatment of seizures associated with LGS and DS.” They drug causes liver damage but the report says that this could be managed effectively.

The FDA will conduct a final vote for approval of this drug in June. Oral solution of the drug for a small group of patients with these conditions would be allowed.

Reference: https://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/PeripheralandCentralNervousSystemDrugsAdvisoryCommittee/UCM604736.pdf

 

via FDA approves marijuana based medication for epilepsy treatment

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[WEB SITE] Epilepsy Drug With Marijuana-Based Ingredient Could Be Available In The US This Year

 By Allan Adamson Tech Times

A new class of epilepsy drugs based on a marijuana ingredient could be become available in the United States as early as the second half of 2018 pending approval from the Food and Drug Administration.

Epidiolex

GW Pharmaceuticals, the maker of the drug called Epidiolex, announced on Wednesday the promising results of a clinical study of the drug.

A group of 171 individuals were randomly assigned to either receive Epidiolex treatment or placebo. The participants were between 2 and 55 years old with a condition called Lennox-Gastaut syndrome. They were also suffering from seizures existing drugs cannot efficiently control.

The participants on average had tried and discontinued use of six anti-seizure treatments and were experiencing 74 “drop” seizures per month. This particular seizure involves the entire body, head and trunk, and often leads to fall and other injuries.

LGS Patients Taking Epidiolex Sees Significant Reduction Seizures

Results of the study, which was reported in the journal Lancet,  showed that over a period of 14 weeks, 44 percent of the patients taking the drug saw significant reduction in seizures. The rate is significantly higher compared with the 22 percent in the placebo group. More of those who were given the experimental drug also experienced a 50 percent or greater reduction in drop seizures.

“LGS is one of the most difficult types of epilepsy to treat and the majority of patients do not have an adequate response to existing therapies,” said Elizabeth Thiele, from Harvard Medical School. “These results show that Epidiolex may provide clinically meaningful benefits for patients with LGS.”

Epidiolex is based on pure marijuana-derived cannabidiol or CBD. The cannabis compound has been known for its medical benefits sans making people feeling “stoned.”

Adverse Events Linked To Use Of Epidiolex

Adverse events associated with use of the drug include diarrhea, decreased appetite, sleepiness, vomiting, and fever. Once given the go-signal to be marketed in the United States, the drug is intended to be used as a prescription drug to be dispensed by doctors.

“Add-on cannabidiol is efficacious for the treatment of patients with drop seizures associated with Lennox-Gastaut syndrome and is generally well tolerated. The long-term efficacy and safety of cannabidiol is currently being assessed in the open-label extension of this trial,” investigators wrote in their report.

GW Pharmaceuticals has not yet disclosed the pricing of the drug, but Justin Gover, GW’s chief executive officer, said that the company is already in talks with health insurers about coverage.

via Epilepsy Drug With Marijuana-Based Ingredient Could Be Available In The US This Year : Health : Tech Times

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[WEB SITE] Cannabidiol shows promise to reduce seizures for people with difficult-to-treat epilepsy

Taking cannabidiol may cut seizures in half for some children and adults with Lennox-Gastaut syndrome (LGS), a severe form of epilepsy, according to new information released today from a large scale controlled clinical study that will be presented at the American Academy of Neurology’s 69th Annual Meeting in Boston, April 22 to 28, 2017. Cannabidiol is a molecule from the cannabis plant that does not have the psychoactive properties that create a “high.”

Nearly 40 percent of people with LGS, which starts in childhood, had at least a 50 percent reduction in drop seizures when taking a liquid form of cannabidiol compared to 15 percent taking a placebo.

When someone has a drop seizure, their muscle tone changes, causing them to collapse. Children and adults with LGS have multiple kinds of seizures, including drop seizures and tonic-clonic seizures, which involve loss of consciousness and full-body convulsions. The seizures are hard to control and usually do not respond well to medications. Intellectual development is usually impaired in people with LGS.

Although the drop seizures of LGS are often very brief, they frequently lead to injury and trips to the hospital emergency room, so any reduction in drop seizure frequency is a benefit.

“Our study found that cannabidiol shows great promise in that it may reduce seizures that are otherwise difficult to control,” said study author Anup Patel, MD, of Nationwide Children’s Hospital and The Ohio State University College of Medicine in Columbus and a member of the American Academy of Neurology.

For the randomized, double-blind, placebo-controlled study, researchers followed 225 people with an average age of 16 for 14 weeks. The participants had an average of 85 drop seizures per month, had already tried an average of six epilepsy drugs that did not work for them and were taking an average of three epilepsy drugs during the study.

Participants were given either a higher dose of 20 mg/kg daily cannabidiol, a lower dose of 10 mg/kg daily cannabidiol or placebo as an add-on to their current medications for 14 weeks.

Those taking the higher dose had a 42 percent reduction in drop seizures overall, and for 40 percent, their seizures were reduced by half or more.

Those taking the lower dose had a 37 percent reduction in drop seizures overall, and for 36 percent, seizures were reduced by half or more.

Those taking the placebo had a 17 percent reduction in drop seizures, and for 15 percent, seizures were reduced by half or more.

There were side effects for 94 percent of those taking the higher dose, 84 percent of those taking the lower dose and 72 percent of those taking placebo, but most side effects were reported as mild to moderate. The two most common were decreased appetite and sleepiness.

Those receiving cannabidiol were up to 2.6 times more likely to say their overall condition had improved than those receiving the placebo, with up to 66 percent reporting improvement compared to 44 percent of those receiving the placebo.

“Our results suggest that cannabidiol may be effective for those with Lennox-Gastaut syndrome in treating drop seizures,” said Patel. “This is important because this kind of epilepsy is incredibly difficult to treat. While there were more side effects for those taking cannabidiol, they were mostly well-tolerated. I believe that it may become an important new treatment option for these patients.”

There is currently a plan to submit a New Drug Application to the FDA later this year.

Source: Cannabidiol shows promise to reduce seizures for people with difficult-to-treat epilepsy

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