Lennox-Gastaut syndrome is the name given to a rare epilepsy syndrome. The difficult-to-treat form of epilepsy primarily affects children between the ages of 2 and 6.
What is Lennox-Gastaut syndrome?
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. The condition particularly affects 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 in detail for the first time in the 1950s and were involved in its research. In doing so, they distinguished the condition from other forms of epilepsy. There is a presumption that for every 100 children affected by epilepsy, five will have 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 prior to onset, which subsequently progresses to LGS.
Causes
There is no single cause for Lennox-Gastaut syndrome, so several reasons for its onset can be considered. One in five children suffers from West syndrome, another severe form of epilepsy, before the onset of LGS. It is not uncommon for newborn infants to present with convulsions or generalized epileptic seizures prior to onset. 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 LGS. The most common triggers include metabolic diseases, tuberous sclerosis, toxoplasmosis, meningitis (meningitis) or encephalitis (inflammation of the brain). Other causes include pronounced brain organic disorders due to a lack of oxygen during the birth process or premature birth, as well as various traumatic brain injuries. In many cases, no underlying disease can be identified in Lennox-Gastaut syndrome. In medicine, this is then referred to as idiopathic or cryptogenic LGS.
Symptoms, complaints, and signs
Lennox-Gastaut syndrome usually appears between the ages of 2 and 6 years. Occasionally, onset does not occur until after 8 years of age. Since there are considerable parallels to West syndrome, a relationship between the two diseases is suspected. A typical symptom of Lennox-Gastaut syndrome is the repeated occurrence of epileptic seizures several times a day. Characteristic are different types of seizures, the variety of which is not observed in any other epilepsy syndrome. Most often, affected children suffer from tonic seizures, which usually occur during sleep and are accompanied by stiffening of the muscles. When tired, myoclonic seizures are also common, with sudden onset of muscle twitching. Other symptoms of LGS include atonic seizures, grand mal seizures, focal and tonic-clonic seizures, and atypical absences. Usually, seizures last only a few seconds. Some children also suffer from apathy, lack of reaction and confusion. Another problem is falls due to the epileptic seizures, which in turn can lead to injuries. For this reason, affected children are advised to wear a crash helmet. Another accompanying symptom of Lennox-Gastaut syndrome is cognitive disability, behavioral problems, and delays in overall body development.
Diagnosis and course of the disease
Diagnosis of Lennox-Gastaut syndrome is considered difficult. For example, the symptoms often have similarities to other disorders. In addition, there is no single cause of the syndrome. In order to differentiate LGS from other epilepsy syndromes, the examining physician clarifies the age of the child at the first appearance of the symptoms. He also looks at the clinical picture, the frequency and variability of the epileptic seizures, and possible delays in physical and psychological development. A sleep EEG is considered an important diagnostic method. Thus, the typical tonic seizures mostly appear during sleep.A differential diagnosis to the so-called pseudo-Lennox syndrome, in which the tonic seizures are absent, is also important. Magnetic resonance imaging (MRI) of the brain can be performed to identify brain-organic triggers. If the movement patterns of the epileptic seizures appear predominantly on a particular side of the brain, this indicates that they are damaged. Because Lennox-Gastaut syndrome is difficult to treat, the disease rarely has a positive outcome. LGS is fatal in about five percent of all cases. Neurological deficits also often occur, causing delays in the child’s intellectual development.
Complications
Lennox-Gastaut syndrome usually causes patients to suffer increased epileptic seizures. In the process, these can significantly reduce and limit the affected person’s quality of life. In most cases, the seizures also lead to severe pain and further restrictions in everyday life. It is not uncommon for relatives and parents to be affected by Lennox-Gastaut syndrome as well, due to psychological complaints or depression. Patients also suffer from severe fatigue and not infrequently muscle twitching. Likewise, there is a versatility, so that the affected persons can end up with disorders of coordination and concentration. As a result, the development of the children is also not infrequently severely restricted and delayed. In most cases, Lennox-Gastaut syndrome also causes behavioral problems and other disabilities in the patient. Motor and cognitive abilities are also not infrequently disturbed by Lennox-Gastaut syndrome. In childhood, affected individuals may also suffer from teasing or bullying as a result. Lennox-Gastaut syndrome is treated with the help of medication. Usually, no particular complications occur. 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 their pediatrician. If symptoms persist, the child must be examined and, if necessary, treated with medication. If behavioral problems or signs of a cognitive disability occur, the doctor should also be consulted so that a diagnosis can be made quickly. If Lennox-Gastaut syndrome is present, the child must be monitored closely. In the absence or inadequacy of treatment, falls and other complications can occur as a result of the epileptic seizures. In addition, the condition often has a negative impact 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, surgical intervention must be performed to prevent greater discomfort. Since Lennox-Gastaut syndrome can progress quite differently, medical clarification is always necessary.
Treatment and therapy
Compared with other forms of epilepsy, Lennox-Gastaut syndrome is difficult to treat. However, treatment is still considered easier than for West syndrome. However, treatment success cannot be guaranteed even with early diagnosis. Most sufferers receive medication to control the seizures. Agents such as valproate, felbamate, benzodiazepines, topiramate, levetiracetam and lamotrigine are used. One problem, however, is that even these drugs cannot always guarantee freedom from seizures. In therapy-resistant epilepsies, the implementation of a ketogenic diet is considered useful. Thus, the protein-balanced and carbohydrate-limited diet led to an improvement of the symptoms in one out of three cases. If a treatable brain-organic lesion is responsible for Lennox-Gastaut syndrome, there is the option of surgical intervention as part of epilepsy surgery. Thus, surgical removal of the damage can resolve the seizures.
Outlook and prognosis
The prognosis of Lennox-Gastaut syndrome is unfavorable. There is damage to the brain that is irreparable. As a result, the patient’s quality of life is significantly impaired. Without medical care, life-threatening conditions may result.The epileptic seizures must be monitored and controlled by a doctor to reduce the risk of complications. In a treatment, an attempt is made to minimize the occurrence of the seizures by administering drugs. If the given active substances are well absorbed and processed by the organism, a favorable course of the disease can be achieved overall. Nevertheless, the drugs are fraught with side effects. In addition, a seizure can occur again at any time during the patient’s lifetime. Freedom from symptoms is not guaranteed. In some patients, surgery may be performed. Surgery is also associated with risks and side effects. Nevertheless, this treatment measure is a good option for some affected persons, for a long-term relief of the 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 overall health. However, it must be taken into account that this is a highly complex intervention in the human brain. If surrounding regions are damaged in the process, severe irreversible dysfunctions in the organism may result.
Prevention
Preventive measures against Lennox-Gastaut syndrome are not known. Thus, there is no single cause for the occurrence of the epileptic seizures.
Follow-up
Because Lennox-Gastaut syndrome is incurable, regular and comprehensive follow-up is required. Affected individuals usually suffer from a number of complications and discomforts, which in the process can lead to the death of the affected individual in the worst case scenario. The disease should therefore be detected and treated at a very early stage to prevent further complaints or complications. Patients should see a doctor regularly to check the setting of the medication and possible side effects. Psychological support for relatives may also be advisable. In addition, relatives should be trained to recognize an epileptic seizure and take appropriate action. In the event of a seizure, emergency medical attention should be called immediately, as it can be life-threatening.
What you can do yourself
Lennox-Gastaut syndrome is associated with epileptic seizures and often manifests in young children, so caregivers bear the greatest responsibility with regard to appropriate medical care and patient support. First of all, the parents accompany the sick child regularly to medical examinations. In addition, the parents learn first aid measures and the correct handling of the children during epileptic seizures. Since these occur in very severe form, young children are exposed to an increased risk of accidents. Therefore, it is recommended for patients to wear a helmet to protect the head from serious injuries. In severe cases, parents also equip the child with joint protectors, for example on the knees or hands. The epileptic seizures significantly limit the well-being of the sufferers. In addition, the children also suffer from impaired cognitive development, so that attendance at special care facilities is often essential. Later, the patients usually attend a special school in order to support them according to their individual perceptive abilities. In addition, social contacts with other children often have a beneficial effect on the patients’ quality of life. Because parents are exposed to enormous stress from the ill child and his epileptic seizures, they often develop depression, which always requires therapy.
