Lennox-Gastaut syndrome (LGS) is the name given to a severe form of epilepsy. It is also called Lennox syndrome and is considered difficult to treat. Particularly affected by the disease are children between the ages of two and six, who suffer from frequent epileptic seizures.
Lennox-Gastaut syndrome was named after the American neurologist William G. Lennox (1884-1960) and the French physician Henri Gastaut (1915-1995). Both physicians described the disease for the first time in the fifties for the first time and dealt with their research. They distinguished the suffering from other types of epilepsy.
It is suspected that out of every 100 children affected by epilepsy, five 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. Other sufferers have previously had epilepsy, which subsequently passes into LGS.
There is no consistent cause for Lennox-Gastaut syndrome, so there are several reasons for its onset. One in five children suffers from the onset of LGS under West Syndrome, another form of severe epilepsy. Not infrequently, spasms or generalized epileptic seizures are found in newborn children.
In about two-thirds of all affected children, Lennox-Gastaut syndrome is caused by damage to the brain. Likewise, other diseases or developmental disorders may be responsible for the LGS. The most common triggers include metabolic diseases, tuberous sclerosis, toxoplasmosis, meningitis (encephalitis) or encephalitis (brain inflammation).
Other causes include pronounced organic brain disorders due to a lack of oxygen in the course of the birth process or a premature birth, as well as different craniocerebral traumas. In many cases, Lennox-Gastaut syndrome can not detect any triggering underlying disease. In medicine, there is talk of an idiopathic or cryptogenic LGS.
Lennox-Gastaut syndrome usually occurs between the ages of 2 and 6 years. Sometimes it comes only after the age of 8 to the outbreak. Since there are significant parallels to the West Syndrome, a relationship between the two diseases is suspected.
A typical symptom of Lennox-Gastaut syndrome is a recurrence of epileptic seizures that occur several times a day. Characteristic are different types of seizures whose diversity can not be observed in any other epilepsy syndrome. The children most often suffer from tonic seizures, most of which appear in their sleep and are accompanied by muscle stiffness.
In addition, tiredness often causes myoclonic seizures, which can lead to sudden onset of muscle twitching. Other complaints of the LGS are atonic seizures, grand mal seizures, focal and tonic-clonic seizures as well as atypical absences. As a rule, the seizures last only a few seconds.
Some children also suffer from apathy, lack of response 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 affected children put on a crash helmet. Another side effect of Lennox-Gastaut syndrome is cognitive disability, behavioral problems and delays in the overall development of the body.
The diagnosis of Lennox-Gastaut syndrome is considered difficult. Thus, the symptoms often have similarities to other diseases. In addition, there is no common cause of the syndrome. To differentiate the LGS against other epilepsy syndromes, the examining doctor clarifies the age of the child at the first appearance of the symptoms.
In addition, he deals with the clinical picture, the frequency and diversity of epileptic seizures and possible delays in physical and psychological development. An important diagnostic method is sleep EEG. Thus, the typical tonic seizures usually appear in sleep.
Also of importance is a differential diagnosis to the so-called pseudo-Lennox syndrome, in which the tonic seizures fail. To find brain organic triggers, a magnetic resonance imaging (MRI) of the brain can be performed. If the patterns of epileptic seizures appear predominantly on a particular side of the brain, this indicates their damage.
Because the Lennox-Gastaut syndrome is difficult to treat, it rarely leads to a positive course of the disease. Thus, the LGS ends in about five percent of all cases deadly. Frequently, there are also neurological deficits that cause delays in the mental development of the child.
Patients usually suffer more from epileptic seizures due to Lennox-Gastaut syndrome. These can significantly reduce and limit the quality of life of those affected. In most cases, the seizures also lead to severe pain and further restrictions in everyday life. The relatives and parents are also often affected by Lennox-Gastaut syndrome due to mental illness or depression.
The patients also suffer from a pronounced tiredness and not infrequently from muscle twitching. Likewise, there is a sense of accomplishment, so that those affected can end up in disturbances of coordination and concentration. The development of the children is also often severely limited and delayed. In most cases, the Lennox-Gastaut syndrome also leads to behavioral problems and other disabilities of the patient.
Motor and cognitive abilities are also often disturbed by Lennox-Gastaut syndrome. In childhood, those affected can also suffer from teasing or bullying. Treatment of Lennox-Gastaut syndrome is medicated. As a rule, no special complications occur. However, in most cases it does not lead to a completely positive course of the disease.
If the child suddenly suffers from cramping or prolonged tiredness, this indicates a serious condition that needs to be clarified. Parents of affected children should discuss the condition with the pediatrician. If symptoms persist, the child must be examined and, if necessary, treated with medication. If any abnormal behavior or signs of cognitive disability come along, 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.
Lack of or inadequate treatment may result in falls and other complications due to epileptic seizures. In addition, suffering often has a negative effect on the mental state of the child. Therapeutic treatment reduces the risk of serious mental illness. The drug treatment is supported by physiotherapeutic measures. In severe cases, surgery must be performed to prevent major discomfort. Since the Lennox-Gastaut syndrome can be very different, a medical examination is always necessary.
Compared to other types of epilepsy, Lennox-Gastaut syndrome is difficult to treat. However, treatment is still considered to be easier than in West Syndrome. However, the success of the treatment can not be guaranteed even at an early diagnosis.
Most sufferers receive medication to fight the seizures. Active ingredients such as valproate, felbamate, benzodiazepines, topiramate, levetiracetam and lamotrigine are used. One problem, however, is that even medicines can not always guarantee seizure freedom.
In refractory epilepsies, performing a ketogenic diet is considered useful. Thus, the protein-balanced and carbohydrate-limited diet resulted in one of three cases of illness to improve the symptoms.
If a treatable brain organ lesion is responsible for the Lennox-Gastaut syndrome, there is the option of surgery for epilepsy surgery. This way, seizures can be repaired by surgically eliminating the damage.
The prognosis of Lennox-Gastaut syndrome is unfavorable. There is damage to the brain that is irreparable. This significantly affects the quality of life of the patient. Without medical care, life-threatening conditions can occur. Epileptic seizures must be monitored and controlled by a physician to reduce the risk of complications. In a treatment, the administration of drugs tries to minimize the occurrence of seizures. If the given active ingredients are well absorbed and processed by the organism, overall a favorable disease course can be achieved.
Nevertheless, the drugs are associated with side effects. In addition, a seizure can occur again at any time over the life span. Complaints are not guaranteed. Some patients may undergo surgery. An operation is also associated with risks and side effects. Nevertheless, for some sufferers, this treatment is a great way for long-term relief of the condition.
In a surgical procedure, the damaged areas of the brain are removed. If no further complications occur, the affected person experiences a clear improvement in his general state of health. However, it has to be considered that this is a highly complex intervention in the human brain. If surrounding regions are damaged, it can lead to strong irreversible dysfunctions in the organism.
Preventive measures against Lennox-Gastaut syndrome are not known. So there is no uniform cause for the occurrence of epileptic seizures.
Because Lennox-Gastaut syndrome is incurable, regular and comprehensive follow-up is required. Sufferers usually suffer from a number of complications and complaints, which in the worst case can lead to the death of the person affected. The disease should therefore be detected and treated very early, so it does not lead to further complaints or complications.
Patients should consult a doctor regularly to check the medication and possible side effects. Even a psychological care of the relatives can be recommended. In addition, the relatives should be trained to recognize and take appropriate action in the event of an epileptic seizure. In the event of a spasm, the emergency doctor should be called immediately, as this can be life-threatening.
Lennox-Gastaut syndrome is associated with epileptic seizures and is often seen in infants, so caregivers have the greatest responsibility for proper medical care and care for patients. First, the parents regularly accompany the ill child to medical examinations. In addition, the guardians learn first aid measures and the correct handling of children in epileptic seizures.
Since these occur in very strong form, toddlers are exposed to an increased risk of accidents. Therefore, it is advisable for the patient wearing a helmet to protect the head from serious injury. In severe cases, the parents also provide the child with joint protectors, such as on the knees or hands.
The epileptic seizures clearly limit the well-being of the patients. In addition, the children also suffer from impaired cognitive development, so that visiting special care facilities is often indispensable. Later, patients usually attend a special school to promote them according to their individual perceptions. In addition, social contacts with other children often have a beneficial effect on the quality of life of the patients. Because parents are exposed to a tremendous burden from the ill child and its epileptic seizures, they often develop depression, which is always in need of therapy.