LGS stands for Lennox-Gastaut Syndrome. Lennox-Gastaut syndrome is a rare epilepsy syndrome. This form of epilepsy, which is difficult to treat, mainly affects children between the ages of 2 and 6 years.
What is Lennox-Gastaut Syndrome?
A sleep EEG is an important diagnostic method. The typical tonic seizures usually appear during sleep. See AbbreviationFinder for abbreviations related to LGS.
Lennox-Gastaut syndrome (LGS) is the name for a severe form of epilepsy. It is also called Lennox syndrome and is considered difficult to treat. Children aged two to six years, who suffer from frequent epileptic seizures, are particularly affected by the disease.
Lennox-Gastaut syndrome was named after the American neurologist William G. Lennox (1884-1960) and the French doctor Henri Gastaut (1915-1995). Both doctors described the disease in detail for the first time in the 1950s and started researching it. They differentiated the condition from other forms of epilepsy.
It is believed that out of 100 children affected by epilepsy, five will develop Lennox-Gastaut syndrome. Boys are more likely to suffer from LGS than girls. Some of the children show no abnormalities before the onset of the disease. Others suffer from epilepsy beforehand, which then progresses to LGS.
There is no uniform cause for the Lennox-Gastaut syndrome, so that various reasons for its onset can be considered. One in five children has West syndrome, another serious form of epilepsy, before developing LGS. It is not uncommon for newborn children to have convulsions or generalized epileptic seizures beforehand.
In around two-thirds of all affected children, Lennox-Gastaut syndrome is caused by brain damage. Other diseases or developmental disorders can also be responsible for LGS. The most common triggers include metabolic diseases, tuberous sclerosis, toxoplasmosis, meningitis (inflammation of the meninges) or encephalitis (inflammation of the brain).
Other causes are pronounced organic brain disorders due to a lack of oxygen during the birth process or premature birth, as well as various craniocerebral trauma. In many cases, no underlying disease can be identified in Lennox-Gastaut syndrome. In medicine, there is then talk of an idiopathic or cryptogenic LGS.
Symptoms, Ailments & Signs
The Lennox-Gastaut syndrome usually appears between the ages of 2 and 6 years. Sometimes it does not break out until after the age of 8. Since there are significant parallels to West syndrome, a relationship between the two diseases is suspected.
A typical symptom of the Lennox-Gastaut syndrome is a repeated occurrence of epileptic seizures that appear several times a day. It is characterized by different types of seizures, the variety of which cannot be observed in any other epilepsy syndrome. Most often, the affected children suffer from tonic seizures, which usually appear in them while they are sleeping and are associated with stiffening of the muscles.
Myoclonic seizures, in which there are sudden onset muscle twitches, are also common with fatigue. Other LGS symptoms include atonic seizures, grand mal seizures, focal and tonic-clonic seizures, and atypical absences. The seizures usually only last a few seconds.
Some children also suffer from apathy, unresponsiveness, and confusion. Another problem is falls due to epileptic seizures, which in turn can lead to injuries. For this reason, it is recommended that the children affected put on a crash helmet. Another side effect of the Lennox-Gastaut syndrome are cognitive disabilities, behavioral problems and delays in the overall development of the body.
Diagnosis & course of disease
Lennox-Gastaut syndrome is difficult to diagnose. The symptoms often show similarities to other diseases. In addition, there is no consistent cause of the syndrome. In order to be able to differentiate LGS from other epilepsy syndromes, the examining doctor clarifies the age of the child when the symptoms first appear.
He also deals with the clinical picture, the frequency and variety of epileptic seizures and possible delays in physical and psychological development. A sleep EEG is an important diagnostic method. The typical tonic seizures usually appear during sleep.
A differential diagnosis of the so-called pseudo-Lennox syndrome, in which tonic seizures do not occur, is also important. In order to find out organic brain triggers, a magnetic resonance imaging (MRI) of the brain can be carried out. If the movement patterns of the epileptic seizures appear predominantly on a certain side of the brain, this indicates damage.
Because Lennox-Gastaut syndrome is difficult to treat, the disease rarely progresses positively. The LGS ends fatally in about five percent of all cases. Neurological deficits are also common, causing delays in the child’s mental development.
Patients with Lennox-Gastaut syndrome usually suffer more from epileptic seizures. These can significantly reduce and limit the quality of life of those affected. In most cases, the seizures also lead to severe pain and other limitations in everyday life. It is not uncommon for relatives and parents to be affected by Lennox-Gastaut syndrome due to psychological problems or depression.
The patients also suffer from severe fatigue and not infrequently from muscle twitching. Likewise, it comes to an adeptness, so that those affected can end up with disorders of coordination and concentration. The development of the children is often severely restricted and delayed as a result. In most cases, the Lennox-Gastaut syndrome also leads to behavioral problems and other disabilities in the patient.
Motor and cognitive abilities are also often impaired by Lennox-Gastaut syndrome. In childhood, those affected can also suffer from teasing or bullying. Treatment of Lennox-Gastaut syndrome is carried out with the help of drugs. Usually there are no special complications. However, in most cases there is not a completely positive course of the disease.
When should you go to the doctor?
If the child suddenly suffers from cramps or persistent fatigue, this indicates a serious illness that needs to be clarified. Parents of affected children should discuss the symptoms with the pediatrician. If symptoms persist, the child must be examined and, if necessary, treated with medication. If there are behavioral problems or signs of cognitive disability, it is best to consult the doctor as well so that a diagnosis can be made quickly. If Lennox-Gastaut syndrome is present, the child must be closely monitored.
If there is no or insufficient treatment, falls and other complications can occur as a result of the epileptic seizures. In addition, the condition often has a negative effect on the child’s psychological state. Therapeutic treatment reduces the risk of serious mental illness. Drug treatment is supported by physiotherapeutic measures. In severe cases, an operation must be performed to avoid greater discomfort. Since the course of the Lennox-Gastaut syndrome can vary greatly, a medical examination is always necessary.
Treatment & Therapy
Compared to other forms of epilepsy, Lennox-Gastaut syndrome is difficult to treat. However, treatment is still considered to be easier than for West syndrome. However, the success of the treatment cannot be guaranteed even with an early diagnosis.
Most sufferers receive medication to control the seizures. Active ingredients such as valproate, felbamate, benzodiazepines, topiramate, levetiracetam and lamotrigine are used. One problem, however, is that medication cannot always guarantee freedom from seizures.
In the case of therapy-resistant epilepsy, a ketogenic diet is considered sensible. The protein-balanced and carbohydrate-limited diet led to an improvement in the symptoms in one out of three cases.
If a treatable organic brain lesion is responsible for the Lennox-Gastaut syndrome, there is the option of an epilepsy surgery. The seizures can be eliminated by surgically removing the damage.
Outlook & Forecast
The prognosis of Lennox-Gastaut syndrome is unfavorable. There is brain damage that cannot be repaired. This significantly affects the patient’s quality of life. Without medical care, life-threatening conditions can occur. The epileptic seizures must be monitored and controlled by a doctor to reduce the risk of complications. In one treatment, medication is administered to try to minimize the occurrence of seizures. If the given active ingredients are well absorbed and processed by the organism, a favorable course of the disease can be achieved overall.
However, the medication does have side effects. In addition, a seizure can occur again at any time over the lifespan. A freedom from complaints is not guaranteed. Surgery can be done in some patients. Surgery is also associated with risks and side effects. Nevertheless, this treatment measure is a good option for some sufferers for long-term relief of symptoms.
In a surgical procedure, the damaged areas of the brain are removed. If no further complications occur, the affected person experiences a significant improvement in their general state of health. It should be noted, however, that this is a highly complex intervention in the human brain. If surrounding regions are damaged, severe irreversible dysfunctions in the organism can occur.
Preventive measures against Lennox-Gastaut syndrome are not known. There is no uniform cause for the occurrence of epileptic seizures.
Since Lennox-Gastaut syndrome is incurable, regular and comprehensive follow-up care is required. Those affected usually suffer from a number of complications and symptoms, which in the worst case can lead to the death of the person concerned. The disease should therefore be recognized and treated at a very early stage to prevent further symptoms or complications.
Those affected should see a doctor regularly to check the medication and possible side effects. Psychological support for relatives can also be recommended. In addition, relatives should be trained to recognize and take appropriate measures in the event of an epileptic seizure. In the event of a cramp, an ambulance should be called immediately, as this can be life-threatening.
You can do that yourself
Lennox-Gastaut syndrome is associated with epileptic seizures and often presents in young children, leaving caregivers with the greatest responsibility for providing appropriate medical care and care for patients. First, the parents regularly accompany the sick child to medical examinations. In addition, the guardians learn first-aid measures and how to deal with the children in the event of epileptic seizures.
Since these are very pronounced, small children are exposed to an increased risk of accidents. It is therefore advisable for patients to wear a helmet to protect their heads from serious injuries. In severe cases, the parents also equip the child with joint protectors, for example on the knees or hands.
The epileptic seizures significantly reduce the well-being of the patient. In addition, the children also suffer from impaired cognitive development, so that attending special care facilities is often indispensable. Later, the patients usually attend a special school in order to promote them according to their individual comprehension. In addition, social contact with other children often has a beneficial effect on the patients’ quality of life. Since the parents are under enormous stress from the sick child and its epileptic seizures, they often develop depression that always requires therapy.