Epileptic encephalopathy in children. How to treat and what is the prognosis?

What is epileptic encephalopathy and how does it manifest itself in children? What treatment is needed and what is important for parents to know?

Epileptic encephalopathy is a type of epilepsy that can lead to brain damage.

Epileptic encephalopathy is a severe variant of the course of the disease, in which, in most cases, not only epileptic seizures are observed, but also cognitive impairment of varying severity.

These diseases make their debut, in most cases, at an early age against the background of normal or already existing impaired development. Medicine knows several dozens of different EE, we will introduce you to the most common of them in this article.

Types of epileptic encephalopathies

• Myoclonic encephalopathy of early age

Early myoclonic encephalopathy is a rare age-related form of epilepsy. The diagnosis can be established by means of an EEG, since it shows the specific epileptic activity of the brain, which is characteristic of this particular EE. Unfortunately, this type of disease, in most cases, is resistant to drug treatment with antiepileptic drugs.

• West syndrome

West syndrome is an age- dependent epileptic syndrome belonging to the group of infantile epileptic encephalopathies and characterized by a special type of seizures – infantile spasms. This disease can lead to serious cognitive impairment, and therefore it is extremely important to diagnose as soon as possible and prescribe the appropriate treatment for the patient.

The cause of West’s syndrome is brain damage, which most often appears during hypoxia (oxygen starvation of the brain) during childbirth. Infectious diseases, structural pathologies of the brain, genetic disorders, and others can also cause damage.

In most cases, West syndrome in children requires aggressive medical treatment to prevent further cognitive impairment in the child.

• Drave ‘s syndrome 

Dravet syndrome is a hereditary childhood epileptic encephalopathy that manifests itself with epileptiform seizures and leads to cognitive impairment. Girls are more likely to suffer from this disease than boys. As a rule, up to 5 months, parents do not notice any deviations in the development of the child and he has convulsions, but after 5 months, encephalopathy makes itself felt.

During the syndrome, the child has a large number of various seizures: focal, febrile, myoclonic and generalized convulsive seizures. In addition, other syndromes appear in children: problems with motor skills, tremor, dysarthria, ataxia, and others.

Unfortunately, most children suffering from this syndrome are not susceptible to drug therapy. However, in some cases it is possible to achieve a reduction in the number of seizures thanks to antiepileptic drugs.

• Lennox -Gastaut syndrome

Lennox – Gastaut syndrome is characterized by numerous seizures of various types and mental retardation of varying degrees. Every fifth child who suffers from this ES was previously diagnosed with West syndrome.

Children with this syndrome may experience various types of seizures: tonic, myoclonic paroxysms, atypical absences , atonic paroxysms, and others. An important feature of the syndrome is the presence of mental retardation in the child, which at the beginning of the disease may not be noticeable, but after the child’s condition worsens if the diagnosis is not made in time and treatment is not started.

At the moment, there is a certain list of drugs that are considered the most effective for the treatment of this syndrome.

• Landau- Kleffner syndrome

Landau- Kleffner syndrome (LAS, acquired aphasia) is a rare disease characterized by epileptic seizures with gradual or sudden regression of speech development. The disease is more common in boys and manifests itself between the ages of 3 and 8 years.

In children suffering from this syndrome, various types of epileptic seizures can be observed, however, a feature of the disease is the regression of speech development. Regression in this case can occur both very quickly and slowly.

The child begins to gradually speak in shorter sentences, use fewer words, and also ceases to understand the speech addressed to him.

In most cases, the cause of this disease remains unknown, however, some studies have recorded that this deviation begins to manifest itself in children with severe brain damage in the temporal lobe.

• Electrical status epilepticus of slow wave sleep (ESES)

Electrical status epilepticus of slow -wave sleep (ESES) is one of the syndromes that belongs to epileptic encephalopathies. ESES is an age- dependent status and is considered difficult to diagnose, since in some cases it is not accompanied by characteristic clinical manifestations. Most often, this deviation is recorded in children aged from 2 years of age.

ESES is manifested by various types of epileptic seizures, however, in addition, patients may experience general cognitive regression, problems with understanding, regression of speech skills, problems with concentration, dysfunction of the oral cavity, and others.

The goal of treating ESES is to stop further brain damage and deterioration in the patient’s condition and cognitive abilities. For this, both medical treatment and surgery can be applied.

Treatment of epileptic encephalopathies

Treatment of epileptic encephalopathies, in most cases, is aggressive and may include both drug treatment and other methods: the appointment of a strict diet and surgery. Treatment is always selected individually and depends not only on the type of encephalopathy, but also on the individual characteristics of the patient: concomitant diseases, the degree of general regression, etc.

The prognosis regarding the further development of the disease will always be individual. However, in the case of epileptic encephalopathies, in no case should one delay diagnosis and treatment, because the progression of the disease can lead to irreversible consequences.