What Is Lennox-Gastaut Syndrome?
Lennox-Gastaut syndrome occurs in approximately 3-6% of children with epilepsy and is more common in boys than girls. Its onset begins at about 3-5 years of age and in almost half of the cases the cause is unknown. Clinically, in addition to epileptic seizures, children begin to show cognitive deficits as well as slow spike waves. In almost all cases, cognitive deficits are progressive.
Patients show learning difficulties, memory loss, and psychomotor fluctuations. Half of the patients who survive to adulthood are severely mentally handicapped and only a very small percentage of them can be independent. There is almost always a cognitive delay in Lennox-Gastaut syndrome.
About 5% of patients with Lennox-Gastaut syndrome die from the disease or its complications before they have been ill for 10 years. Symptoms often worsen during adolescence and adulthood, causing many mental problems and developmental disorders. Thus, current therapies focus on improving the patient’s quality of life.
Symptoms of Lennox-Gastaut syndrome
Lennox-Gastaut syndrome is a severe childhood epilepsy that manifests itself in conjunction with impaired intellectual capacity and developmental problems. Seizures usually begin to occur around the age of four. Seizures can vary in type, but the most common are:
- Toned scenes: body stiffness, rotating eyes, dilated pupils, and variable breathing rhythm.
- Atonic seizures : brief loss of muscle condition and consciousness, causes a person to fall suddenly (which can be very dangerous).
- Atypical absent episodes: absent gaze, focus on a particular site, unresponsiveness to external stimuli.
- Myoclonic seizures: sudden muscle tremors.
Sometimes the scenes are regular and sometimes there are short periods when they don’t appear. Most children with Lennox-Gastaut syndrome experience some form of impairment in intellectual activity or information processing, as well as developmental delay and behavioral disorders.
Problems associated with the disease
Lennox-Gastaut syndrome is usually associated with other serious behavioral disorders, such as:
- To hyperactivity
- To aggression
- Autistic tendency
- Personality disorders
- For regular psychotic symptoms
Common health complications include neurological diseases such as tetraplegia, hemiplegia, extrapyramidal symptoms, and delayed motor development. An early onset can occur and thus it is impossible to say whether it is a continuation of West syndrome or not. However, symptoms can also appear in late childhood, adolescence, or even adulthood.
Patients may experience slow spike-wave complexes in EEG diagnosis, delay in mental health, difficulty dealing with crises, and poor response to anticonvulsant drugs. Children have an uncertain prognosis about their mental development and crisis management. However, there is no single latent pathology – Lennox-Gastaut syndrome has many different causes.
Causes and treatment
The most common reasons are:
- Hereditary diseases
- Neurocutaneous diseases
- Post-traumatic hypoxic-ischemic encephalopathy
- Meningitis
- Brain malformations
- Birth asphyxia
- Severe brain injury
- Central nervous system infections
- Degenerative diseases
- Hereditary metabolic syndromes
In 30-35% of cases, the cause is unclear. The treatment is very difficult as it is very resistant to traditional means. Valproate and benzodiazepine (i.e., clonazepam, nitrazepam, clobazapam) are selected as the first medication, depending on the patient’s most common type of epileptic seizure.
Generally, more than one drug is prescribed to reduce or alleviate symptoms. For example, a patient may mix lamotrigine, valproate, and topiramate. Some children see progress, but usually they develop tolerance to drugs over time, so they lose the ability to keep seizures under control.
Treatment lasts a lifetime because there is no cure for the disease. The main goal is to improve the patient’s quality of life by reducing the number of regular epileptic seizures, although complete reversal is not possible. In addition to medication, there are other treatments such as a ketogenic diet, vagus nerve stimulation, and surgical procedures.
The current long-term prognosis is depressing and the mortality rate is 10 before the age of 11 years. The good thing is that scientists are working hard every day to improve those numbers and have already made some big strides in recent years thanks to advances in technology.
Learn more
Experts agree on the specificity, regularity and thought-provoking nature of the scenes they observed. But when it comes to the type of scene, different opinions begin to emerge. In fact, it is common for them to be short-lived and inconspicuous.
Another ongoing trait is usually a severe mental change that is seen in the patient and is related to both intelligence and personality. The mental delay remains the same or worsens in most cases. This is usually associated with atrophy of the brain, which can be confirmed using gaseous enography or computed tomography.
However, the decline in learning ability appears to be related to the regularity of seizures, the existence of prolonged confusing states in the form of status epilepticus, and psychotic symptoms, not to mention school exclusion and over-therapy. The patient’s mental state is often affected by pre-psychotic changes or psychotic personality changes and autism.
