What Is Lennox-Gastaut Syndrome?
Epilepsy is a central nervous system disorder. In the brain, epilepsy creates brief yet strong electrical bursts. Epilepsy can take the form of Lennox-Gastaut syndrome (LGS). Children with Lennox-Gastaut syndrome (LGS), also known as childhood epileptic encephalopathy, have numerous seizure types, mental regression or retardation, and abnormal electroencephalography results showing a specific pattern of brain activity known as slow spike-and-wave. The recurring seizures (epilepsy) start early in life.
What Are the Typical Features Seen in Lennox-Gastaut Syndrome?
Tonic seizures, which lead to involuntary muscle contraction, are the most prevalent type of seizure. These seizures frequently happen while one is sleeping, but they can sometimes happen when the individual is awake and result in unexpected falls. Atypical absence seizures, which result in a momentary loss of consciousness, are also frequent. Numerous affected people also experience episodes known as "drop attacks," which result in unexpected falls and serious or life-threatening injuries. Drop attacks can be brought on by either an aberrant muscle contraction (tonic) or a sudden lack of muscle tone (atonic). Less frequently reported seizures in those with Lennox-Gastaut syndrome include other forms. Lennox-Gastaut syndrome-related seizures frequently do not respond well to anti-epileptic drug therapy. Even while the duration of each Lennox-Gastaut syndrome-related seizure event is often brief, more than two-thirds of those who are affected have extended periods of seizure activity (also known as status epilepticus) or bouts of several seizures that happen in a cluster.
Even before their first seizure, the majority of kids with Lennox-Gastaut syndrome have intellectual impairments or learning issues. If seizures are severe or frequently occurring, these issues may get worse with time. Some affected youngsters can experience behavioral issues and various neurological impairments. Many people also have delays in the development of motor abilities like sitting and crawling. Most persons with Lennox-Gastaut syndrome need assistance with daily activities due to their seizures and intellectual disabilities. Only a small portion of affected adults live on their own. Lennox-Gastaut syndrome patients are more likely to pass away than others their own age. The increased risk is partially brought on by poorly controlled seizures and injuries from falls.
What Causes Lennox-Gastaut Syndrome?
The cause of Lennox-Gastaut syndrome is not always known by doctors. In some instances, it might be brought on by:
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Oxygen deficiency during labor.
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Severe brain damage associated with pregnancy or childbirth, such as those caused by low birth weight or early delivery.
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Brain infections such as encephalitis, rubella, or meningitis.
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Infantile spasms, or West's syndrome, are infantile seizures that first appear in children.
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Cortical dysplasia is a condition where some brain nerve fibers do not align properly as the unborn child develops.
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With tuberous sclerosis, the brain and other organs throughout the body can develop non-cancerous tumors.
What Are the Symptoms of Lennox-Gastaut Syndrome?
Depending on the kind of seizure the patient is having, Lennox-Gastaut syndrome symptoms can change. Among the signs of a tonic seizure are:
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Stiffness in the trunk, legs, or arms.
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Seizure affects both sides of the body.
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Patients can fall.
Atonic seizure symptoms include:
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Abrupt decline in muscle tone.
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Falling to the ground and becoming limp with a momentary loss of consciousness.
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Sagging eyelids.
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Nodding of the head.
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Jerking.
Symptoms of an atypical absence seizure include:
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Muscle wasting in the head, abdomen, arms, or legs frequently causes a sagging posture over time.
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Myoclonic (slight) jerking movements.
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Loss of consciousness.
The following can be signs of myoclonic seizures:
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Muscle spasms that can be:
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Sudden.
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Brief.
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Involuntary.
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Shock-like.
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Varying in frequency and intensity.
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Affects either the entire body or just a specific area of it.
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Sometimes it is severe enough to make it difficult to move, speak, or eat.
Patients with Lennox-Gastaut syndrome frequently experience some degree of behavioral or mental difficulties. This might comprise:
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Delays in development.
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Disruptions in behavior.
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Comprehending information with difficulty.
How to Diagnose Lennox-Gastaut Syndrome?
A diagnosis of Lennox-Gastaut syndrome requires the identification of a clinical trio. A peculiar electroencephalography brain wave pattern (slow spike-and-wave pattern) and several seizures of various sorts make up this triad, Although this pattern may not be present in every electroencephalography and some degree of behavioral and cognitive problems. A precise diagnosis of Lennox-Gastaut syndrome is challenging due to the possibility that some of these symptoms may not be present at the disorder's inception. Lennox-Gastaut syndrome has a wide range of probable causes, which further complicates the diagnosis.
Lennox-Gastaut syndrome is often diagnosed after a careful clinical examination, full patient history review, and comprehensive physical and neurological examination, which may include the use of cutting-edge imaging methods like magnetic resonance imaging (MRI) and electroencephalography (EEG).
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Electroencephalography records the electrical activity of the brain. Such EEG testing often identifies the specific brain wave pattern in people with Lennox-Gastaut Syndrome.
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A three-dimensional image of the brain's anatomy is created during an MRI scan, which enables medical professionals to evaluate the brain's structure and maybe identify the source of seizure activity.
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Multiple genetic causes of epilepsy are being tested using epilepsy gene panels, which are beginning to be used in evaluation procedures.
How Is Lennox-Gastaut Syndrome Treated?
Treatment for Lennox-Gastaut syndrome can be quite challenging. In order to enhance seizure control and other related problems, a combination of seizure drugs and other treatments may be employed.
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The drug Valproate is used as the first-line treatment for diverse seizure types. Clobazam, Lamotrigine, Topiramate, Cannabidiol, and Rufinamide are some more anticonvulsant drugs. The United States Food and Drug Administration (FDA) approved Cannabidiol in June 2018 for the treatment of Lennox-Gastaut syndrome seizures in people two years of age and older. The substance lacks the euphoria that is typically associated with marijuana and only includes trace amounts of the psychoactive component.
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The ketogenic diet, vagus nerve stimulation, and epilepsy surgery are further treatment options. In epilepsy surgery, typically a corpus callosotomy, deep in the brain, a band of nerve fibers known as the corpus callosum joins the two hemispheres of the brain together and it facilitates communication between the two hemispheres of the brain, but it also aids in the propagation of seizure impulses from one side to the other. The corpus callosum is sliced during a corpus callosotomy procedure, which stops the spread of seizures. In order to maximize seizure control, medication may be taken with the other treatments. Children who initially make progress can eventually develop a medication tolerance or experience uncontrollable seizures.
Conclusion:
Children and adults with Lennox-Gastaut Syndrome, as well as those who care for them, continue to face many difficulties. In order to find improved cures of all kinds, a lot more study is required.
Families, medical professionals, and researchers need to give new developments in seizure detection, recording, and alerting, as well as other precautions and safety equipment, more consideration. For people with Lennox-Gastaut Syndrome and their carers, these kinds of therapies and services can enhance the quality of life and safety management.
