New in the treatment of epilepsy. New methods of treating epilepsy. Relevance of the topic of new treatment for epilepsy

A new study has confirmed that fewer than two-thirds of patients with newly diagnosed epilepsy are seizure-free at 1 year. The risk-free rate in this new study was essentially unchanged from 64.0% in a smaller study published in 2000.

"Despite the introduction of many new antiepileptic drugs over the past two decades, the overall outcomes of people with newly diagnosed epilepsy have not changed fundamentally," Patrick Kwan, MD, PhD, professor, neurology, Monash University, Melbourne, Australia, told Medscape Medical news.

A "paradigm shift" in treatment and research strategies is needed to improve long-term outcomes for patients with epilepsy, said Dr Kwan, who was at the University of Melbourne during the study.

The original study included 470 patients with newly diagnosed epilepsy at the Western Hospital, Glasgow, Scotland, who were first treated between 1982 and 1998. In the current study, this period continued until 2012.

The new analysis included 1,795 patients, 53.7% men and a mean age of 33 years. About 21.5% had generalized epilepsy and 78.5% had focal epilepsy.

Following diagnosis of epilepsy, clinicians considered seizure type, adverse drug effects, and interaction profiles when selecting an appropriate antiepileptic drug (AED). The majority of patients in the study (98.8%) experienced two or more attacks before starting treatment.

During the first 6 months after initiation of treatment, patients were seen in the epilepsy clinic every 2–6 weeks. Thereafter, they attended follow-up visits at least every 4 months.

Patients were asked to record the number of seizures they had between clinic visits and to describe these events.

Freedom of seizure was defined as the absence of seizures in at least the previous year. The overall 1-year freedom of forfeiture rate was 63.7%. The majority of patients who became attack-free (86.8%) achieved this by taking one AED.

This rate of 86.8% is lower than the proportion of patients in the earlier study whose seizures were controlled by monotherapy (90.5%).

In a new study, patients with generalized epilepsy responded better to AED therapy than those with focal epilepsy.

Patients who did not achieve a year of seizure freedom by taking the first AED were more likely to have uncontrolled epilepsy with each additional AED (odds ratio, 1.73; 95% confidence interval, 1.56–1.91; P<0, 001 после корректировки для классификации болезни, возраст и пол). В то время как вторая схема AED могла бы сделать на 11% больше этих пациентов без припасов, пособие было уменьшено более чем на половину для третьего режима. И попробовав четвертый - или более - AED предоставил менее 5% дополнительной вероятности свободы захвата.

There was an increase

The use of new AEDs increased significantly during the study. Early, older drugs such as carbamazepine, valproate and phenytoin were used much more often, but by the end of the study drugs such as valproate, levetiracetam and lamotrigine were more common.

But the proportion of patients who were attack-free was similar for subgroups categorized into the three AED initiation periods (1982 to 1991, 1992 to 2001, and 2002 to 2012).

Newer AEDs are not necessarily better tolerated than older drugs, Dr. Kwan comments. The notion that these newer drugs have fewer side effects is "probably not true," but they may be easier to use because they don't require comprehensive drug monitoring, he said.

From his own practice, Dr. Kwan could see that new epilepsy drugs were not having a "huge impact" on patient outcomes, but he thought the study would show at least some improvement.

However, despite the "dramatic change" in drugs used, with a shift from older to newer agents, he and his colleagues were surprised by how little change there was as a result.

"It wasn't just that there was little change, there was no change," he said.

The researchers analyzed treatment results using the International League Against Epilepsy's 2010 definition of grip freedom. By this definition, freedom of seizure may be the absence of cramps for three times the pretreatment interval between cramps or at least the past year, whichever is greater.

The reason for the update was that some patients experience infrequent seizures, "so being seizure-free for a year may have nothing to do with the medication," Dr. Kwan explained.

This analysis yielded results similar to those obtained using the original definition of being seizure-free at 1 year.

The new study also confirmed that the prognosis of AED treatment was associated with factors such as the number of seizures that occurred before treatment, a family history of epilepsy in first-degree relatives, and a history of recreational drug use.

While the study found that the level of parish freedom has not changed since the onset at the population level, Dr. Kwan noted that this may not be the case at the individual level.

“In terms of the frequency of seizures and their severity in individual patients, new drugs may make a difference and this could have an impact on people's lives, but we did not measure that.”

Epilepsy is a "very complex disorder" that is not just one disease, making it "very difficult to find a magic bullet" that targets everyone and makes a "huge impact" on outcomes, Dr. Kwan said.

However, it is important to develop more effective treatments for epilepsy, and this requires a change in thinking and perhaps "more risky approaches," he said. He added that this change in thinking must come from "all stakeholders", including funding bodies, research groups and the pharmaceutical industry.

Clinicians should refer patients whose two drugs fail to reach a specialist center, where they can be considered for non-arc therapy, such as recirculation surgery and brain stimulation techniques, Dr. Kwan said.

"Do it early, don't leave it too late," he said. “There is evidence that the earlier you treat these patients, the better the outcome.”

Disconcerting Conclusions

Some of the new findings are sobering and somewhat discouraging, W. Allen Hauser, MD, professor emeritus of neurology and epidemiology, Columbia University Sergius Center, New York, writes in an accompanying editorial. In an interview with Medscape Medical News, Dr. Houser elaborated on what he found so embarrassing.

“Until now, efforts have been made to develop new and effective antiseptic drugs,” he said. “I suspect there have been 20 or more new drugs marketed in the US or Europe in the last 30 years, or both. And during this time, at least with new epilepsy, which is what this study relates to, it seems to us that we are not doing anything better.”

He noted "dramatic changes" in medications over the course of the study, with new drugs largely replacing older ones.

“But in terms of objective outcomes, which in terms of epilepsy controls for seizure, there really was no change.”

That the new drugs do not increase the percentage of patients who are seizure free should not be surprising because, for the most part, seizure drugs were developed to try to prevent seizures rather than address the underlying cause, Dr. Houser said.

“People have insults to the brain, such as a stroke or severe head injury, and then develop epilepsy, and the ideal would be to develop something that would prevent any process that would lead to epilepsy. The drugs available to us, as far as we know, only suppress seizures, they do nothing in terms of preventing the process of epileptic development."

Dr. Houser also noted that there is little evidence that tolerability has improved with the advent of new drugs. So while the hope is to find an agent that stops seizures and has no side effects, "it doesn't look like there has been any improvement with drugs in either arena," he said.

It is possible, Dr. Hauser said, that a two-thirds grip freedom level represents a “ceiling” for initial epilepsy control.

However, he agreed that most new drugs have some benefit. For example, they have improved bioavailability and pharmacokinetics, making them easier to administer, he said.

Dr Kwan has received research grants from the National Health and Medical Research Council of Australia, the Australian Research Council, the US National Institutes of Health, the Hong Kong Research Grants Council, the Innovation and Technology Foundation, the Health Sciences and Healthcare Research Foundation, and the Health and Medical Foundation. research. He and/or his institution have also received speaker or consulting fees and/or research grants from Eisai, GlaxoSmithKline, Johnson & Johnson, Pfizer, and UCB Pharma. Dr. Hauser is a member of the Neuropace Epilepsy Sudden Unexpected Death Surveillance Committee and a member of the editorial boards of Acta Neurologica Scandinavia, Epilepsy Research and Neuroepidemiology.

What medications are prescribed for epilepsy

In pathological conditions, antiepileptic drugs can avoid death and prevent recurrent seizures. To treat the disease, anticonvulsants and tranquilizers are selected. The prescription of drug therapy depends on the severity of the pathology, the presence of concomitant ailments and the clinical picture.

Main objectives of treatment

Complex therapy for epilepsy is primarily aimed at reducing its symptoms and the number of seizures and their duration. Treatment of pathology has the following goals:

  1. Pain relief is necessary if seizures are accompanied by pain. For these purposes, painkillers and anticonvulsants are systematically taken. To alleviate the symptoms accompanying attacks, the patient is recommended to eat foods rich in calcium.
  2. Prevent new recurrent seizures with appropriate tablets.
  3. If it is not possible to prevent subsequent attacks, then the main goal of therapy is to reduce their number. Medicines are taken throughout the patient’s life.
  4. Reduce the intensity of seizures in the presence of severe symptoms with respiratory failure (its absence after 1 minute).
  5. Achieve a positive result followed by discontinuation of drug therapy without relapse.
  6. Reduce side effects and risks from the use of medications for epilepsy attacks.
  7. Protect those around you from the person who poses a real threat during a seizure. In this case, drug treatment and observation in a hospital setting are used.

The method of complex therapy is selected after a full examination of the patient, determining the type of epileptic seizures, the frequency of their recurrence and severity.

For these purposes, the doctor carries out a complete diagnosis and sets priority treatment areas:

  • exclusion of “provocateurs” that cause a seizure;
  • neutralization of the causes of epilepsy, which are blocked only through surgery (hematoma, neoplasm);
  • establishing the type and form of the disease using the global list of classification of pathological conditions;
  • prescribing medications against certain epileptic seizures (monotherapy is preferred; in the absence of effectiveness, other drugs are prescribed).

Correctly prescribed medications for epilepsy help, if not eliminate the pathological condition, then control the course of seizures, their number and intensity.

Drug therapy: principles

The effectiveness of treatment depends not only on the correctness of the prescription of a particular drug, but also on how the patient himself behaves and follows the doctor’s recommendations. The primary goal of therapy is to choose a drug that can eliminate seizures (or reduce their number) without causing side effects. If a reaction occurs, the doctor must promptly adjust the treatment.

The dosage is increased only in extreme cases, as this may negatively affect the patient's daily lifestyle. Therapy should be based on the following principles:

  1. At first, only one medication from the first group is prescribed.
  2. The dosage is observed, the therapeutic as well as toxic effect on the patient’s body is monitored.
  3. The medicine and its type are selected taking into account the form of epilepsy (seizures are divided into 40 types).
  4. In the absence of the expected result from monotherapy, the doctor may prescribe polytherapy, that is, drugs from the second group.
  5. You should not suddenly stop taking medications without first consulting your doctor.
  6. When prescribing a medication, the person’s material capabilities and the effectiveness of the drug are taken into account.

Compliance with all principles of drug treatment provides a real opportunity to obtain the desired effect from therapy and reduce the symptoms of epileptic seizures and their number.

Mechanism of action of anticonvulsants

Convulsions during seizures are the result of pathological electrical functioning of areas of the cerebral cortex. Reducing the excitability of neurons and stabilizing their state leads to a decrease in the number of sudden discharges, thereby reducing the frequency of attacks.

In epilepsy, anticonvulsants act by the following mechanism:

  • “irritation” of GABA receptors. Gamma-aminobutyric acid has an inhibitory effect on the central nervous system. Stimulation of GABA receptors reduces the activity of nerve cells during their generation;
  • blockade of ion channels. The electrical discharge changes the membrane potential of neurons, which appears at a certain ratio of calcium, sodium, and potassium ions at the edges of the membrane. Changing the number of ions reduces epiactivity;
  • a decrease in the content of glutamate or a complete blockade of its receptors in the area of ​​redistribution of the electrical discharge from one neuron to another. Neutralization of the effects of neurotransmitters makes it possible to localize the epileptic focus, preventing it from spreading to the entire brain.

Each antiepileptic drug may have several or one mechanism of therapeutic and prophylactic action. Side effects from the use of such drugs are directly related to their purpose, since they do not work selectively, but on all parts of the nervous system as a whole.

Why treatment is sometimes not effective

Most people who suffer from epileptic seizures must take medications to reduce their symptoms throughout their lives. This approach to therapy is effective in 70% of cases, which is a fairly high figure. In 20% of patients the problem remains forever.

If drug therapy is not effective, doctors decide on surgical treatment. In some situations, the vagal nerve ending is stimulated or a diet is prescribed.

The effectiveness of complex therapy depends on factors such as:

  1. Medical qualifications.
  2. Timely, correct diagnosis.
  3. Quality of life of the patient.
  4. Compliance with all doctor's advice.
  5. The appropriateness of using prescribed medications.

Some patients refuse drug therapy due to fear of side effects and worsening general condition. No one can rule out this, but a doctor will never recommend medications until he has established which of them can do more harm than good.

Groups of medications

The key to successful treatment is an individual approach to the prescription of the drug, its dosage and duration of treatment. Depending on the nature of the pathological condition and its forms, medications of the following groups can be used:

  • anticonvulsants for epilepsy. They help relax muscle tissue, so they are taken for focal, temporal, cryptogenic, and idiopathic pathologies. Medicines in this group neutralize primary and secondary generalized attacks;
  • anticonvulsants can also be used in the treatment of children with myoclonic or tonic-clonic seizures;
  • tranquilizers. Suppresses excessive excitability. Most often used for mild seizures in children. Drugs in this group may aggravate the course of epilepsy during the first weeks of their use;
  • sedatives. Not all attacks in people pass without consequences; very often after and before them the patient becomes annoying, irritable, and depressed. In this situation, he is prescribed a sedative and consultation with a psychologist;
  • injections. Used for affective distortions and twilight states.

All modern drugs against epileptic seizures are divided into first and second row, that is, the basic group and new generation drugs.

Anticonvulsant medications for seizures

Some drugs can be purchased at a pharmacy without a doctor's prescription, others only if one is available. Any medications should be taken only as prescribed by a doctor, so as not to cause the development of complications and side effects.

List of popular antiepileptic drugs:

All medications for the treatment of pathological syndrome can be taken only as prescribed by a doctor, after a full examination. In some situations, medications are not used at all. Here we are talking about short-term and one-time attacks. But most forms of the disease require drug therapy.

Medicines of the newest generation

When prescribing the drug, the doctor must take into account the etiology of the disease. The use of new drugs is aimed at eliminating many different causes that provoke the development of the pathological syndrome with minimal risk of side effects.

Modern drugs for the treatment of epilepsy:

Medicines of the first group should be taken 2 times a day, every 12 hours. For single use, it is better to take the tablets before bedtime. When using drugs 3 times, it is also recommended to maintain a certain interval between the use of “pills”.

If adverse reactions occur, you must consult a doctor; you cannot refuse medications, nor ignore various ailments.

Possible effects of anticonvulsants

Most medications can only be purchased with a prescription, as they have many side effects and, if overdosed, can pose a threat to the patient's life. Only a specialist is allowed to prescribe medications, after a full examination and tests.

Improper use of tablets can provoke the development of the following conditions:

  1. Staggering while moving.
  2. Dizziness, drowsiness.
  3. Vomiting, feeling of nausea.
  4. Double vision.
  5. Allergies (rashes, liver failure).
  6. Breathing problems.

As patients age, they become much more sensitive to the medications used. Therefore, they need to be tested from time to time for the content of active components in the blood plasma and, if necessary, adjust the dosage together with the treating doctor. Otherwise, the likelihood of side effects increases.

Some products contribute to the breakdown of drugs, as a result of which they gradually accumulate in the body, provoking the development of additional diseases, which significantly worsens the patient’s condition.

The main condition for drug therapy is that all anticonvulsants should be used in accordance with recommendations and prescribed taking into account the general condition of the patient.

Drugs for epilepsy - a review of effective drugs

Epilepsy is a chronic disease that manifests itself in different ways and differs in symptoms and treatment methods.

For this reason, there are no tablets that would suit all patients with epilepsy.

All types of this disease have one thing in common - an epileptic seizure, which varies in clinical picture and course.

Specific treatment is selected for a specific seizure, and individual medications for epilepsy are selected.

Is it possible to get rid of epilepsy forever?

Epilepsy can be completely cured if the disease has an acquired form. The disease is unique in nature.

It is not uncommon for patients to experience behavioral changes along with the attacks.

There are three types of epilepsy:

  • Hereditary type.
  • Acquired. This type is a consequence of traumatic brain injury. Also, this type of epilepsy can occur due to inflammatory processes in the brain.
  • Epilepsy can occur without an identified cause.

Some types of epilepsy (including, for example, benign) cannot be registered in an adult. This type is a childhood disease and after a few years the process can be stopped without the intervention of doctors.

Some doctors are of the opinion that epilepsy is a chronic neurological disease that occurs with regular repetition of attacks and irreparable disorders are inevitable.

The course of epilepsy is not always progressive, as practice shows. Seizures leave the patient, and thinking ability remains at an optimal level.

It is impossible to say for sure whether it is possible or not to get rid of epilepsy forever. In some cases, epilepsy can be completely cured, but sometimes this cannot be done. Such cases include:

  1. Epileptic encephalopathy in a child.
  2. Severe brain damage.
  3. Meningoencephalitis.

Circumstances that influence the outcome of treatment:

  1. How old was the patient when he had his first seizure?
  2. The nature of the attacks.
  3. The state of the patient's intelligence.

An unfavorable prognosis exists in the following cases:

  1. If therapeutic measures are ignored at home.
  2. Significant delay in treatment.
  3. Features of the patient.
  4. Social circumstances.

Did you know that epilepsy is not always a congenital pathology? Acquired epilepsy - why does it occur and how to treat it?

Is it possible to completely cure epilepsy? You will find the answer to this question here.

The diagnosis of epilepsy is made based on a complete examination of the patient. Diagnostic methods are briefly described at the link.

Anticonvulsants for epilepsy: list

The main list of anticonvulsants for epilepsy is as follows:

  1. Clonazepam.
  2. Beclamid.
  3. Phenobarbital.
  4. Carbamazepine.
  5. Phenytoin.
  6. Valproate

The use of these drugs relieves various types of epilepsy. These include temporal, cryptogenic, focal and idiopathic. Before using any medications, it is necessary to study everything regarding complications, because These drugs often cause adverse reactions.

Ethosuximide and Trimethadone are used for minor seizures. Clinical experiments have confirmed the rationality of using these drugs in children, because they cause the least number of adverse reactions.

Many drugs are quite toxic, so the search for new drugs does not stop.

It is due to the following factors:

  • Long-term use is required.
  • Seizures occur frequently.
  • It is necessary to carry out treatment in parallel with mental and neurological diseases.
  • The number of cases of the disease in older people is increasing.

The greatest amount of effort in medicine goes to treating diseases with relapses. Patients have to take medications for many years and become accustomed to the drugs. At the same time, the disease operates against the background of the use of medications and injections.

The main goal of correct prescription of drugs for epilepsy is to select the most appropriate dosage, which can help keep the disease under control. In this case, the medicine should have a minimum number of side effects.

An increase in outpatient appointments makes it possible to more accurately select the dosage of medications against epilepsy.

Which drug to choose for the treatment of epilepsy

Persons suffering from epilepsy are prescribed only one drug. This rule is justified by the fact that if you take several medications at once, their toxins can be activated. First, the medicine is prescribed in the smallest dosage to monitor the body's reaction. If the drug does not work at all, then the dosage is increased.

First of all, doctors opt for one of the following drugs:

  • Benzobarbital;
  • Ethosuximide;
  • Carbamazepine;
  • Phenytoin.

These products have proven their effectiveness to the maximum.

If for some reason these drugs are not suitable, then they are chosen from the second group of drugs.

Second-line drugs of choice:

These drugs are not popular. This is due to the fact that they do not have the desired therapeutic effect, or work with pronounced side effects.

How to take the pills

Epilepsy is treated for quite a long time, prescribing drugs in fairly large dosages. For this reason, before prescribing a particular drug, conclusions are drawn about what the expected benefits of this treatment are and whether the positive effect will outweigh the harm from adverse reactions.

Sometimes the doctor may not prescribe medications. For example, if consciousness switches off shallowly, or the attack was singular and for the first time.

Taking “new” medications for epilepsy should be done in the morning and evening, and the interval between taking the drug cannot be less than twelve hours.

To make sure you don’t miss your next pill dose, you can set an alarm.

If you have epilepsy, it is important to follow a proper diet. Nutrition for epilepsy in adults is characterized by reduced carbohydrate intake.

If intolerance to the medication occurs, you should immediately inform your doctor. If the case is severe, you should immediately call an ambulance.

Anticonvulsants for epilepsy - list of modern drugs

Epilepsy is a serious chronic disease that requires long-term treatment. Most often, the pathology manifests itself in early childhood, but the development of the disease in adults is not excluded. Epilepsy is a consequence of disorders that arise in the brain as a result of the influence of certain factors. Thus, in children, the most common cause of epilepsy is hypoxia during fetal development or during childbirth. In adults, the disease is a consequence of head trauma, neuroinfection, tumor, etc. An important factor in the development of epilepsy is heredity. If there are episodes of epilepsy in the family, a person is at risk of developing pathology.

The main treatment for epilepsy is drug therapy. Antiepileptic and anticonvulsant drugs are used in treatment. Their appointment is carried out by the attending physician. You cannot take such drugs on your own without control. Epilepsy is a very serious disease, and with inadequate treatment it can lead to disruption of brain activity. To receive quality treatment, you should contact the Yusupov Hospital. Experienced neurologists and epileptologists work here who treat epilepsy of various types.

When to use anticonvulsants for epilepsy

In the treatment of epilepsy, drug therapy is successfully used, which shows positive results in more than 70% of cases. Medicines can reduce the intensity of attacks and reduce their number. With the help of drug therapy, complete elimination of attacks can be achieved. Also, for the treatment of epilepsy, a special diet, a special regime of work and rest, and physiotherapy may be prescribed. However, they will only be an addition to the main drug therapy. Treatment of epilepsy begins after an accurate diagnosis has been established and is carried out only by a specialist.

The clinical manifestations of epilepsy are very diverse. There are both convulsive and non-convulsive epileptic seizures. In each case, a specific medication will be used, which is effective specifically for attacks of this type. If seizures occur, the patient is prescribed anticonvulsants.

The algorithm for treating a patient for epilepsy is as follows:

  1. monotherapy is prescribed: treatment begins with one drug;
  2. the dosage is gradually increased to achieve the required therapeutic effect;
  3. adding a drug from another group if the first one turned out to be ineffective (switching to polytherapy);
  4. compliance by the patient with the established doctor’s prescriptions: the average duration of therapy is 2-5 years from the moment the attacks cease;
  5. gradual withdrawal of the drug: reduction in the dosage of medications should be monitored by the attending physician. Discontinuation of the drug may take about a year. During the process of reducing the dosage, the patient will need to undergo examinations to monitor the condition.

First-line anticonvulsants for epilepsy

Anticonvulsants are used in the treatment of idiopathic and focal epilepsy with primary and secondary generalized seizures. The drugs show high effectiveness in the treatment of tonic-clonic and myoclonic seizures. Anticonvulsants help relax muscles, eliminate seizures, and reduce the intensity of an epileptic attack.

Modern medications for epilepsy are divided into first-line and second-line drugs. The first line is drugs for basic therapy, the second is drugs of a new generation.

Therapy begins with one first-line drug. Several anticonvulsant drugs are not recommended because their unjustified use can provoke resistance to drug therapy and increases the risk of side effects. At the beginning of therapy, drugs are used in small dosages to assess the body's response to the medication. Then the dosage is increased until the desired result is achieved.

First-line anticonvulsants include:

  • sodium valproate;
  • carbamazepine;
  • lamotrigine;
  • topiramate.

These drugs show maximum effectiveness in the treatment of seizures in patients with epilepsy.

New generation drugs for epilepsy

The advantages of new antiepileptic drugs are less toxicity, good tolerability and ease of use. The use of new generation drugs does not require constant monitoring of the concentration of the drug in the blood.

Initially, the drugs were used as additional therapy when the main medication was insufficiently effective, as well as in the case of drug-resistant epilepsy. Currently, new generation epilepsy drugs are approved for use as monotherapy.

New generation drugs include:

  • felbamate;
  • gabapentin;
  • tiagabine;
  • oxcarbazepine;
  • levetiracetam;
  • zonisamide;
  • clobazam;
  • vigabatrin.

Treatment of epilepsy in Moscow

In Moscow, successful treatment of epilepsy is performed at the Yusupov Hospital. Neurologists and epileptologists at the Yusupov Hospital are the best specialists in their field. Doctors use evidence-based medicine methods that have shown the greatest effectiveness in treating epilepsy. Neurologists constantly study modern innovations in medicine, so they are aware of the latest effective developments in the treatment of pathology. The use of modern methods in working with patients, as well as the extensive experience of doctors, allows us to achieve maximum results in the treatment of pathology.

At the Yusupov Hospital, drug therapy is tailored strictly individually based on examination data and taking into account all the patient’s characteristics. Adequate therapy helps to significantly improve the patient’s condition, reduce the number of attacks and achieve long-term remission of the disease.

You can make an appointment with neurologists and epileptologists, get information about the work of the diagnostic center, or clarify other questions of interest by calling the Yusupov Hospital.

The most effective drugs for epilepsy

  1. Phenytoin
  2. Phenobarbital
  3. Lamotrigine
  4. Benzobamyl
  5. Sodium valproate
  6. Primidon

Epilepsy is a disease that primarily affects the human brain, is chronic, causes seizures and loss of consciousness. The most dangerous periods are attacks, when, due to unconsciousness and convulsions, there is a possibility of swallowing the tongue and subsequent suffocation.

To prevent seizures, as well as to ensure that epilepsy bothers a person as little as possible, it is necessary to competently and carefully approach the choice of drug treatment.

Let's look at the most effective drugs for treating epilepsy. First of all, it should be noted that none of the following drugs should be taken without a doctor’s recommendation and a thorough examination. It is also necessary to take into account that the sooner the diagnosis, the greater the likelihood that you will not need to take medications for the rest of your life, and the remission will be longer.

Phenytoin

    Indications. Belongs to the hydantoin group. Its main effect is aimed at slightly slowing down the reactions of nerve endings, thus stabilizing neuronal membranes. Phenytoin is often prescribed to people with epilepsy who experience frequent seizures.

Application and dosage. Adults are prescribed 3 to 4 mg per kg per day, gradually increasing the dosage to 300–400 mg per day after meals. Children begin to be given the drug at a dose of 5 mg per kg per day, not exceeding 300 mg.

Side effects. May cause unpleasant side reactions such as vomiting, trembling, headache, involuntary eye movement, stupor.

Contraindications. Phenytoin can be taken during pregnancy only with the permission of your doctor.

  • Analogues. Diphenylhydantoin, Dilantin, Diphenin.
  • The cost of this medicine in Russia is 3,000 rubles for 200 tablets of 100 mg. In Ukraine, you can purchase the drug for 200 UAH. (60 tablets).

    Phenobarbital

    The drug Phenobarbital belongs to the group of barbiturates, and is actively used to treat the initial stages and to maintain remission in epilepsy.

      Indications. This drug has a mild sedative effect, which is not always sufficient during acute epilepsy. Because of this, Phenobarbital is often taken in combination with other drugs.

    Application and dosage. Children, depending on age, are prescribed from twenty mg 2-3 times a day. Adults from 20 to 150 mg per day, depending on the degree of the disease, 1-3 times a day.

    Side effects. Decreased activity of the nervous system, allergies, pressure surges.

    Contraindications. Do not use in the first three months of pregnancy, or during breastfeeding. Alcohol and drugs are prohibited when taking Phenobarbital.

  • Analogues. Dormiral, Luminal and Barbital.
  • The price of the medicine in the Russian Federation is 12 rubles for 6 tablets of 100 mg. In Ukraine - from 5 UAH for the same packaging.

    A medication in this group should be taken for a certain amount of time to achieve the desired effect, since the most active sedative effect will manifest itself as a result of the accumulation of the drug in the body. You should not suddenly stop taking the medication: this can worsen the disease and provoke attacks.

    Lamotrigine

      Indications. One of the most powerful drugs for the treatment of epilepsy is the drug Lamotrigine. When prescribed correctly, the course can almost completely stabilize the functioning of the nervous system without interfering with the necessary release of amino acids.

    Application and dosage. Children from two years old - 2-10 mg per kg per day, adults - 25-150 mg per day.

    Side effects. Causes a rash.

    Contraindications. Due to its high effectiveness and strong effect on the body, when taking Lamotrigine, it is necessary to exclude work that requires concentration and quick reaction.

  • Analogues. Lamitor, Convulsan, Lameptil, Vero-Lamotrigine, Lamictal, Triginet, Seizar, Lamolep.
  • You can buy the medicine in the Russian Federation for an average of 230 rubles (30 tablets of 25 mg each). In Ukraine, for the same pack you will need to pay 180 UAH.

    Benzobamyl

      Indications. The drug Benzobamil has a milder and non-toxic effect on the nervous system, which is why it is often prescribed to children suffering from epilepsy.

    Application and dosage. Use after meals. Dosage for children is from 5 to 10 mg 2-3 times a day, for adults - 25 mg 3 times a day.

    Side effects. Desire to sleep, fatigue, apathy.

    Contraindications. Due to the strong effects on the body, it is prohibited for people with liver, kidney and heart problems.

  • Analogues. Difenin, Benzonal, Carbamazepine, Konvulex.
  • When taking this drug, you must especially strictly monitor your blood pressure.

    The cost of Benzobamil in the Russian Federation is about 100 rubles for 50 tablets of 100 mg, and in Ukraine - from 50 UAH.

    Sodium valproate

      Indications. Epileptic seizures and behavioral disorders.

    Application and dosage. Start using 10 mg per kg per day, gradually increasing the dose.

    Side effects. Decreased blood clotting, poor circulation, rash, increased body fat, worsened clarity of consciousness, etc.

    Contraindications. Sodium valproate is prohibited for hepatitis, pregnancy and glucocorticoids, heart, liver and pancreas problems.

  • Analogues. Valprocom, Depakin, Konvulex.
  • Sodium valproate differs from many antiepileptic drugs in that it not only affects the neural system of the brain, preventing the manifestation of symptoms of epilepsy - seizures and convulsions, but also emotionally calms a person, increases the amount of the hormone of happiness and generally improves the condition during crises.

    The cost of the medicine in the Russian Federation is 450 rubles for 30 tablets of 500 mg. In Ukraine - 250 UAH.

    Primidon

      Indications. The drug Primidone is prescribed during severe stages of epilepsy.

    Application and dosage. Adults start taking 125 mg per day after meals, gradually increasing to 250 mg. Children are prescribed from 50 mg per day, increasing to 125 mg.

    Side effects. The desire to constantly sleep, allergies, rashes, causeless anxiety, apathy.

    Contraindications. Elderly, children, pregnant women, as well as people with kidney and liver problems.

  • Analogues. Hexamidine, Misolin.
  • Primidone has a strong inhibitory effect on damaged neurons, allowing to reduce or eliminate seizures, but does not affect undamaged areas of the brain. The drug must be taken only after consultation with a doctor, as this drug is addictive and addictive.

    Primidone can be purchased in Russia for 400 rubles (50 tablets of 250 mg). In Ukraine the cost is 250 UAH.

    Above we described the most used and effective medications for epilepsy. For proper treatment and prevention of attacks, it is necessary not only to accurately select the medicine, but also to correctly determine the dosage. You cannot change the drug yourself, as well as the frequency and dose of administration. Any action not agreed with a doctor may cause deterioration in health.

    There are also a number of folk remedies for treating and stabilizing the condition of epilepsy, but the effectiveness of these methods has not yet been proven by doctors. The main thing to remember is that every fifth person today suffers from this disease, but many manage to live, study and work fully with the help of properly selected medications.

    About the causes, symptoms and treatment of epilepsy in this video:

    Epilepsy pills

    Epilepsy is a chronic disease that has dozens of different forms and manifestations, which differ in symptoms and, accordingly, treatment principles. Therefore, there are no universal pills for epilepsy.

    All forms of manifestation of this disease have one thing in common - an epileptic seizure, which can vary in clinical manifestations and course. For each type of epileptic seizure, conservative treatment is selected separately, highlighting a specific group of drugs for epilepsy.

    Treatment Goals

    The overall goal of treatment for epilepsy can be divided into several basic principles:

    • Pain relief for attacks occurs when the patient experiences pain during an attack. To do this, they regularly take anticonvulsants and painkillers. To alleviate the course of seizures, a diet with a high calcium content is prescribed;
    • Preventing the occurrence of new attacks is achieved by prescribing appropriate medications;
    • Reducing the frequency of attacks is the main goal of treatment if it is not possible to prevent the occurrence of new ones. Lifelong drug therapy is used;
    • Reducing the duration of seizures becomes a treatment priority in the presence of severe seizures with breath holding for more than a minute;
    • Achieve discontinuation of medications without resumption of attacks;
    • Reduce the risks and side effects of drug treatment;
    • To protect society from aggressive actions when a person suffering from this pathology poses a threat to himself and those around him. Compulsory inpatient observation and drug therapy are used.

    The basic principles of treatment are selected after a detailed examination of the patient, establishing the form of the seizure, its severity, and frequency of occurrence. To do this, the attending physician carries out the necessary diagnostic procedures and identifies the main areas of therapy:

    • Elimination of factors leading to an attack;
    • Exclusion of causes of attacks that can only be eliminated surgically (tumor formations, hematomas, etc.);
    • Determination of the type and type of attack using the international classification list;
    • Prescription of drugs against epilepsy of a certain group. It is advisable to use monotherapy with drugs of the same series. Only if treatment is ineffective, use complex therapy.

    Correctly selected medicine for epilepsy in humans allows, if not cure the disease, then makes it possible to control its course.

    Drug treatment

    Depending on the type and form of attacks, the basic rules of therapy are followed in the treatment of epilepsy.

    Doses of drugs for epilepsy in children and adults are different and depend on body weight. At first, the minimum dosage is prescribed to check the side effects of the medicine. Then gradually increase to achieve the desired therapeutic effect.

    It should be noted that abrupt cessation of taking pills for epilepsy is unacceptable. It is necessary to stop taking it gradually, gradually reducing the dose and switching to another group of drugs for the treatment of epilepsy.

    The right medication for epilepsy in people can achieve treatment goals, reduce side effects and reduce the frequency of seizures. Often, medications for epilepsy are taken for life.

    When taking pills, many patients are afraid of the development of side effects and their toxic effect on internal organs. Therefore, all prescriptions are made only by the attending physician under strict supervision, and if a side effect occurs, the drug is discontinued and replaced with another. Currently, there are many drugs of choice for treating and reducing the severity of seizures. They all have different indications for use and side effects. Individual calculation of the dosage and duration of taking the tablets allows you to minimize the development of side effects.

    Let's consider the main medications for epilepsy, which are used both separately and in complex therapy.

    Anticonvulsants for epileptic seizures

    Anticonvulsants or anticonvulsants – stop muscle cramps of various origins, which helps reduce the frequency, severity and duration of convulsive attacks. Their main pharmacological action is aimed at reducing the frequency of neuron firing. There are three main mechanisms of action:

    • Increased activity of inhibitory neurons;
    • Inhibition of excitatory neurons;
    • Disturbance in the conduction of nerve impulses.

    Anticonvulsants are prescribed if focal and generalized seizures are accompanied by clonic, tonic and myoclonic seizures.

    List of main anticonvulsants for epilepsy:

    • Barbiturates and their derivatives. The most common is phenobarbital, a glutamic acid inhibitor that has an inhibitory effect on the neurons of the epileptic focus. Phenobarbital has a non-selective depressant effect on the central nervous system;
    • Benzodiazepine derivatives act on GABA (gamma-aminobutyric acid) receptors and enhance the activity of inhibitory neurons. The most common drugs in this group are diazepam, clonazepam, nitrozepam;
    • Fatty acid derivatives (valproic acid, gamma-aminobutyric acid) prevent the reuptake of GABA and have an inhibitory effect on active neurons;
    • Hydantoin derivatives. These include phenytoin and its analogues. It has an anticonvulsant effect without a pronounced hypnotic effect. The mechanism of action is based on stabilizing the nerve cell and limiting the spread of excitation;
    • Carboxamide derivatives (carbamazepine) – limit the spread of electrical potential along neurons;
    • Oxazolidine derivatives. Trimethadione is used for mild epileptic seizures (absence seizures). There is information about its teratogenic effect on the body, so the use of the drug is limited. Trimethadione is prescribed only in cases of resistance to other drugs;
    • Succinimide derivatives (ethosuximide) are used to treat absence seizures. Ethosuximide is a calcium channel blocker. The drug has anticonvulsant activity like trimethadione, but is less toxic. Effectiveness has been proven in the treatment of myoclonic seizures.

    Side effects of anticonvulsants are associated with inhibition of the central nervous system and are expressed:

    • Drowsiness;
    • Dizziness;
    • Severe asthenic syndrome;
    • Cognitive impairment;
    • Motility disorders up to ataxia;
    • Memory impairment.

    Tranquilizers

    Tranquilizers are psychotropic substances that are intended

    to suppress excitability.

    Tranquilizers have hypnotic, sedative, muscle relaxant and anticonvulsant effects. The use of this group of drugs leads to a decrease in anxiety in patients. Therefore, they are more often prescribed in the treatment of epileptic seizures in children. Epilepsy pills of this group can cause addiction and physical dependence when taken for a long time.

    Side effects of benzodiazepines are associated with their sedative and muscle relaxant effects. These include:

    • Drowsiness;
    • Dizziness;
    • decreased attention and concentration;
    • decreased concentration.
    • Decreased libido;
    • The occurrence of depressive states.

    Neurotropic agents

    Neurotropic drugs are psychoactive substances that affect the central and peripheral nervous system. The mechanism of their action is associated with inhibition or stimulation of impulse transmission in various parts of the nervous system, as well as an increase or decrease in the sensitivity of the nerve endings of the peripheral nervous system.

    Neurotropic substances include many types of narcotic substances of plant and synthetic origin. For medical purposes, only ephedrine, morphine, and codeine are used. The development of addiction to this group of drugs limits their use in the treatment of seizures.

    Racetams

    Racetams are psychoactive nootropic substances that have an activating effect on glutamate receptors of inhibitory neurons. This drug group is promising for the treatment of partial and generalized seizures.

    Sedatives

    Sedatives are used in cases of severe agitation of the patient and the development of depressive states. This group is prescribed in complex therapy with anticonvulsants. Patients calm down, their sleep normalizes, and the feeling of anxiety goes away. It should be noted that in case of severe manic-depressive syndrome, drugs from the group of tranquilizers should be considered.

    Basic drugs for the treatment of epilepsy

    There are such concepts as a list of first- and second-line drugs for epilepsy.

    The first-line anti-epilepsy drug is prescribed as monotherapy, while its therapeutic effect and side effects are monitored.

    If treatment with one drug is unsuccessful, then additional drugs for epilepsy are prescribed (second-line drugs). Moreover, the list of first- and second-line epilepsy pills depends on the type and form of seizures.

    The list of pills for epilepsy can be divided into the first and second row according to their effectiveness.

    First line drugs:

    • Carbamazepine and its analogs. Used for severe seizures accompanied by psychomotor impairment. Effectiveness for minor forms of the disease has not been proven. The drug is also effective for depression;
    • A new generation drug for epilepsy, benzobarbital, is an analogue of phenobarbital and has less hypnotic and sedative effects when used in patients. In combination with other drugs, it is used for the treatment of non-convulsive and polymorphic seizures;
    • Valproic acid is widely used in adults and children for various types of disease. The drug is effective against generalized seizures (minor and major) and focal motor seizures. For minor forms of the disease, they are limited only to the administration of valproic acid;
    • Ethosuximide is the latest generation epilepsy drug, has minimal toxic effects and is widely used for the treatment of epilepsy throughout the world. Used to treat minor forms of the disease;
    • Phenytoin is used in the treatment of generalized tonic-clonic and complex focal seizures. The drug also has a pronounced analgesic effect.

    The above remedies are used primarily to prescribe treatment for epilepsy. If a significant side effect develops or there is no therapeutic effect, the attending physician selects a second-line drug. These medications for epilepsy are prescribed gradually under the supervision of a doctor, due to their mild effect or the presence of a significant side effect.

    The most common drugs are:

    • Phenobarbital has a pronounced anticonvulsant effect. The drug is limited in use due to significant side effects: depression of the central nervous system, mental retardation in children, carcinogenic effects.
    • Carbamazepine drugs (carboxamide) can cause severe forms of anemia;
    • Tiagabine blocks GABA reuptake and is used to treat treatment-resistant focal seizures. However, monotherapy with tiagabine is ineffective. Positive results can be achieved when complex treatment is prescribed;
    • Lamotrigine is used to treat focal seizures. Side effects are associated with the presence of allergic reactions, depression of the central nervous system;
    • Topiramate is a fructose derivative. It has limited use, especially in children, as it can cause delayed psychomotor development, personality disorders, and hallucinations;
    • Therapy with clonazepam can cause persistent addiction, especially in people who have previously abused alcohol, so this drug is not included in their list of prescriptions;
    • Gabapentin has limited use due to the risk of status epilepticus if the drug is abruptly discontinued;
    • Nitrozepam has a pronounced inhibitory effect on the central nervous system;
    • Diazepam has a pronounced teratogenic effect.

    Second-line drugs are prescribed with caution, usually under inpatient observation. The attending physician notes the presence of side effects and the severity of the therapeutic effect.

    It is worth noting that many medicines are used in modern medicine. The choice of one drug or another depends on the type and form of epileptic seizures. A patient suffering from epilepsy, as well as his family and friends, must strictly follow the doctor’s recommendations and know the name of the epilepsy pills and their dose. The effectiveness of treatment is achieved through the correct use of all medications.

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    In a few years, people with drug-resistant epilepsy will be able to take a new drug to suppress seizures only when needed, much like people with headaches take painkillers today.

    Worldwide, approximately 50 million people suffer from epilepsy.

    Of these, only 70% respond well to antiepileptic drugs (AEDs).

    After 2-5 years of successful treatment, drugs can be discontinued in 70% of children and 60% of adults without subsequent exacerbations. However, millions of patients who do not respond to drug therapy must, in some cases, undergo surgical treatment.

    Researchers from University College London in the UK are confident that a new anticonvulsant drug that can be taken “as needed” will be a real lifesaver for the 30% of patients who do not respond to standard medications.

    The new treatment, which has so far only been tested in rodents, makes nerve cells more sensitive to certain substances in the brain that are normally inactive.

    The lead author of the study, the developer of the new therapy, Professor Dimitri Kullmann from the Institute of Neuroscience at University College London, explains how his drug works: “First, we inject a modified virus into the area of ​​the brain where seizure activity begins. The virus instructs brain cells to produce a protein that is activated by the substance CNO (clozapine-N-oxide). This substance can be taken in tablet form. The activated protein suppresses the excessive excitability of the cells responsible for seizures, but only in the presence of CNO."

    Today, severe epileptic seizures are treated with drugs that suppress the excitability of all brain cells, and this leads to serious side effects. If the dose required by the patient is very high, then patients have to be hospitalized.

    “If we could transfer our new method into clinical practice, which we hope to do over the next decade, we could treat such patients with CNO tablets on an “as needed” basis, after having given one single injection of the virus for the entire time,” says Professor Kullmann .

    Many people with drug-resistant epilepsy experience episodes of petit mal seizures. The professor envisages taking the new drug in these situations, as well as in cases where the patient feels an approaching severe attack.

    Factors that increase the risk of seizure activity in this group of patients include illness, lack of sleep, certain periods of the menstrual cycle, etc. In these situations, taking CNO is also advisable to prevent the worst-case scenario.

    A team of scientists is working on alternative methods of delivering the drug. One of them will be an injection that will quickly and effectively stop an attack that has already begun. Work is also underway on an automatic drug delivery system, in which CNO will be administered according to the same principle as in insulin pumps for diabetics.

    “Fully reversible” treatment without permanent side effects

    Professor Kullmann claims that the “new method is completely reversible”, so any side effects that may occur while taking the drug will go away once it is stopped.

    The professor also answered questions about possible side effects associated with the introduction of a modified virus into the body of patients.

    He said: “The modified virus has been rendered harmless so it is unable to produce new viruses and spread. The injection is a short surgical procedure in which a small hole is drilled into the patient's skull, a needle is inserted through the lining of the brain, and medication is delivered directly to the problem area. "This operation is much simpler and safer than what patients with drug-resistant epilepsy usually face - they sometimes have to remove several cubic centimeters of brain tissue."

    Another advantage of CNO is that this substance has a short lifespan (only a few hours), and only affects areas of the brain tissue treated with the virus. This avoids problems in other areas of the brain that are common with other antiepileptic drugs, and also prevents permanent damage from more invasive treatments.

    “Short-acting drugs are rarely used for epilepsy. However, because they affect the entire brain and have a limited role, they are used only as second-line drugs for acute treatment of seizures in a hospital setting. Limitations of short-acting drugs include potentially dangerous respiratory depression. Our approach avoids such complications,” concluded Professor Kullmann.

    Problem commission “Epilepsy. Paroxysmal states" of the Russian Academy of Sciences and the Ministry of Health of the Russian Federation

    Russian Antiepileptic League

    EPILEPSY

    and paroxysmal

    state

    EPILEPSY AND PAROXYZMAL CONDITIONS

    2017 Vol. 9 No. 1

    www.epilepsia.su

    Included in the list of leading peer-reviewed journals and publications of the Higher Attestation Commission 1:

    DOI: 10.17749/2077-8333.2017.9.1.006-025

    and paroxysmal states

    International League Against Epilepsy Classification of Epilepsy: Revision and Update

    Avakyan G. N., Blinov D. V., Lebedeva A. V., Burd S. G., Avakyan G. G.

    Federal State Budgetary Educational Institution of Higher Education "Russian National Research Medical University named after. N.I. Pirogov" Ministry of Health of Russia, Moscow

    The International League Against Epilepsy (ILAE), 30 years after the last revision, approved the ILAE Working Classification of Seizure Types 2017 and the ILAE Classification of Epilepsy 2017. According to the ILAE 2017 Classification of Seizure Types, seizures can be focal or generalized, decisive The onset of the attack is important. Focal seizures are divided into focal seizures with preserved consciousness and focal seizures with impaired consciousness. Several new types of generalized seizures have been added to the classification. Seizures are also classified according to the presence or absence of a motor component. Separately, attacks with an unspecified onset are identified. The 2017 ILAE Classification of Epilepsy provides three levels of categorization: seizure type (defined by the 2017 ILAE Classification of Seizure Types), epilepsy type (focal, generalized, combined generalized and focal, unspecified), and epileptic syndrome. The etiological diagnosis should be clarified as data is obtained during diagnosis. Epileptic syndrome may have more than one etiological factor. The term “benign” has been replaced by the terms “self-limiting” and “pharmacoreactive” as appropriate. The term “age-related and epileptic encephalopathy” has been introduced, which can be used either partially or completely depending on the clinical situation. The 2017 ILAE Classification of Seizure Types and the 2017 ILAE Classification of Epilepsy will be useful in both routine clinical practice and research, helping to improve the management of patients with epilepsy and their quality of life.

    Keywords

    International League Against Epilepsy, MPEL, ILAE, International League Against Epilepsy, classification of seizure types, classification of epilepsy, epilepsy, epileptic syndrome, focal seizure, generalized seizure, convulsions, onset of seizures, absence seizures, terminology, EEG.

    Article received: 02/06/2017; in revised form: 03/07/2017; accepted for publication: March 31, 2017

    Avakyan G. N., Blinov D. V., Lebedeva A. V., Burd S. G., Avakyan G. G. Classification of epilepsy by the International League Against Epilepsy: revision and update 2017. Epilepsy and paroxysmal conditions. 2017; 9 (1): 6-25. DOI: 10.17749/2077-8333.2017.9.1.006-025.

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    ILAE CLASSIFICATION oF THE EPILEPSIEs: THE 2017 REVISION AND UPDATE

    Avakyan G. N., Blinov D. V., Lebedeva A. V., Burd S. G., Avakyan G. G.

    N. I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, Moscow Summary

    The International League Against Epilepsy (ILAE) has developed and approved the Operational classification of seizure types 2017 and the Classification of the Epilepsies 2017. According to the ILAE 2017 Operational classification of seizure types, seizures can be initially divided into focal or generalized; and the seizure onset is of crucial importance in this case. Focal seizures are optionally subdivided into focal aware seizures and focal impaired awareness seizures. Several new types of generalized-onset seizures were implemented. Specific motor and non-motor classifiers may be added. Unknown onset seizures are placed separately. Three levels of diagnostics are specified in the 2017 ILAE Classification of the Epilepsies: seizure type (defined in accordance with the 2017 ILAE Operational classification of seizure types), epilepsy type (focal, generalized, combined generalized and focal, unknown), and epilepsy syndrome . An etiologic diagnosis should be considered at each step along the diagnostic pathway. A patient's epilepsy may be classified into more than one etiological category. The term "benign" is replaced by the terms "self-limited" and "pharmacoresponsive" to be used where appropriate. The term "developmental and epileptic encephalopathy" can be applied in whole or in part where appropriate. The 2017 Operational classification of seizure types and the 2017 ILAE Classification of the Epilepsies can be helpful both in routine clinical practice and scientific research that will assist in improving epilepsy care and quality of life of patients with epilepsies .

    International League Against Epilepsy, ILAE, Classification of seizure types, Classification of the Epilepsies, Epilepsy, Epilepsy syndrome, focal seizures, generalized seizures, convulsion, onset seizures, absanses, terminology, EEG.

    Received: 02/06/2017; in the revised form: 03/07/2017; accepted: 03/31/2017.

    Conflict of interests

    The authors declare no conflict of interests and no need for financial disclosure regarding this manuscript. All authors equally contributed to this article. For citation

    Avakyan G. N., Avakyan G. G. ILAE Classification of the epilepsies: the 2017 revision and update. Epilepsy i paroksizmal "nye sostoyaniya / Epilepsy and paroxysmal conditions. 2017; 9 (1): 6-25 (in Russian). DOI: 10.17749/2077-8333.2017.9.1.006-025.

    Corresponding author

    Address: 1 Ostrovityanova St., Moscow, Russia, 117997. E-mail address: [email protected](Avakyan G. N.).

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    Introduction

    Epilepsy is one of the most common disorders of the nervous system, which has a significant impact on the quality of life of the patient and his family members. The prevalence in developed countries is 5.8 people. per 100 population, in developing countries - 10.3 people. per 1000 population in urban settlements and 15.4 people. per 1000 population in rural areas. In the Russian Federation, the prevalence is 3.2 people. per 1000 population (European part - 3.1; Siberia and the Far East -3.4; large cities - 3.1; small towns and rural areas - 3.7 people per 1000 population, respectively). Almost 80% of people with epilepsy live in low- and middle-income countries. Three quarters of them do not receive proper treatment; Often they, like their family members, suffer from discrimination.

    Epilepsy occurs in people of any age and has extremely diverse manifestations. That's why

    the creation of a unified classification initially presented difficulties. In the international professional community of specialists dealing with the problem of epilepsy, the most authoritative organization is recognized as the International League Against Epilepsy (ILAE), created in 1909, an organization of doctors and scientists of various specialties, whose mission is to help provide medical workers, patients, governments and the public around the world with the educational and research resources needed to understand, diagnose and treat people with epilepsy. Today ILAE has national branches in more than 100 countries, and the number of ILAE members exceeds 100,000 people. The structural division of ILAE in Russia is the Russian Anti-Epileptic League (RPEL), Russian League Against Epilepsy (RLAE) (RLAE President - MD, Prof. G. N. Avakyan). The first sha-

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    When developing a treatment plan for a patient with seizures, it should be necessary to determine the type of seizures in accordance with the principles of classification. This explains the high attention that ILAE pays to the issues of definition and classification of epilepsy.

    definition of epilepsy

    According to the consensus of the ILAE and the International Bureau for Epilepsy (IBE), epilepsy is a disease that includes various disorders and conditions. It was decided to abandon the term “disorder” or “group of disorders” because this term indicates functional impairment of varying duration, while the term “disease” implies a longer-term impairment of function. Also, serious conditions such as cancer or diabetes are recognized as diseases, and include many different disorders. The definition of epilepsy was updated and supplemented by ILAE in 2014. Previously, epilepsy was understood as a brain disorder characterized by a persistent predisposition to epileptic seizures, that is, the presence of two unprovoked epileptic seizures with an interval of more than 24 hours. Adopted by the ILAE Executive Committee in December 2013, the ILAE's official position on the operational definition of epilepsy for clinical diagnosis was published in early 2014. According to this definition, epilepsy is considered a brain disease that meets the following criteria: 1) at least two unprovoked (or reflex) epileptic seizures with an interval of more than 24 hours; 2) one unprovoked (or reflex) attack and a probability of recurrence of attacks close to the general risk of relapse (>60%) after two spontaneous attacks in the next 10 years; 3) diagnosis of epileptic syndrome.

    The definition of an epileptic seizure has not changed; an epileptic seizure is understood as transient clinical manifestations of pathological excessive or synchronous neural activity of the brain. In this case, an attack associated with the influence of any transient factor on the normal brain, which temporarily reduces the seizure threshold, is not classified as epilepsy. Synonyms for the term “provoked attack” are “reactive attack” or “acute symptomatic attack.” It is necessary to distinguish between the cause and provoking factors, since some conditions (causes) can create a long-term prerequisite for epileptic seizures. For example, a brain tumor, unlike a stroke, can cause repeated seizures. Recurrent reflex epileptic seizures (for example, in response to flashes of light) are provoked by

    and paroxysmal states

    stupas, which are attributed to epilepsy. Although these seizures are induced by a specific factor, the tendency to have recurrent seizures when exposed to that factor is consistent with the conceptual definition of epilepsy because there is a pathological predisposition to such seizures. However, a seizure following a concussion accompanied by fever or alcohol withdrawal (examples of provoked seizures) is not considered epilepsy according to the ILAE. The term "unprovoked" implies the absence of a temporary or reversible factor lowering the seizure threshold and causing a seizure at the specified time point. The ILAE recognizes that the terminology of unprovoked and provoked seizures needs further clarification and work in this direction is ongoing.

    The definition of epilepsy has now added a criterion such as the risk of relapse. If the risk of relapse is high, then even after one unprovoked attack, the management of a patient with epilepsy should be followed. Examples include: a distant symptomatic attack in the presence of epileptiform changes on the electroencephalogram (EEG), a single epileptic attack at least 1 month later. after a stroke or a single attack in a child with structural pathology of the central nervous system. In this case, the first attack may represent status epilepticus, which in itself is not a criterion for epilepsy. In most cases, the risk of recurrence is unknown. After two unprovoked attacks it is approximately 60-90%. In children with epileptiform changes on the EEG, the risk of relapse within 2-3 years after the first seizure is up to 56-71%. Moreover, the degree of risk progressively decreases over time after the last epileptic seizure.

    In the context of the definition of epilepsy, the ILAE pays particular attention to the question of when and under what circumstances the diagnosis of epilepsy can be withdrawn. The ILAE working group tried to define criteria that would allow some patients to refuse the diagnosis of epilepsy and the associated societal attitude. The term "cure" was not endorsed by the working group because it indicates that the risk of seizure recurrence is no greater than in healthy individuals, but that such a low risk is never achieved in patients with a history of epilepsy. On the other hand, the term "remission" was also not approved because it does not indicate the absence of disease and is not clear enough for the population. The ILAE Working Group has approved the use of the term "permit". This term indicates that the patient no longer has epilepsy, but at the same time, the occurrence of seizures in the future cannot be ruled out with certainty. As criteria for the resolution of epilepsy, work

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    The ILAE group recommends using a certain age in patients with age-dependent epilepsy syndrome or freedom from epileptic seizures for 10 years in patients who have not used antiepileptic drugs (AEDs) for at least 5 years.

    Previous classifications

    Given the difficulties of definition and differences of opinion that have historically developed regarding epilepsy, there is no single generally accepted classification. Attempts to clarify the classification began to be made by the ILAE, starting from the creation of this organization in 1909, and intensified in the early 60s of the last century, when G. Gastaut proposed a new concept of classification. Several other classifications were proposed around the same time, and none of them gained complete dominance in the international community. One of these classifications was that of W. G. Penfield. He classified epileptic syndromes in various active cerebral processes according to morphological principles and causes of occurrence. Famous scientists R. L. Marsland, R. N. Dijong, D. Zhants and Z. Servit presented their classification options. Based on the concept of G. Gastaut, in 1964 the ILAE adopted the International Classification of Epilepsy, which was based on a clinical and phenomenological approach. The 1969 ILAE classification has become more widespread, based on six criteria, including the clinical type of seizures, the type of seizures on the EEG, changes in the interictal EEG, the anatomical substrate, etiology and age of the patients. In this classification, four groups of seizures were identified: partial, generalized, unilateral (or predominantly unilateral), and unclassifiable. Seizures in which the first clinical symptoms of the disease indicated activation of anatomical and/or functional neuronal systems limited to a region or one hemisphere of the brain with the corresponding localization of the EEG discharge were considered partial. Partial seizures were divided into simple and complex. Seizures during which both hemispheres were involved in the epileptic process were considered generalized. Convulsive and non-convulsive seizures were also distinguished.

    Since there is no clear comparison between the clinical manifestations of epilepsy and epileptic syndromes, there is a need for at least two separate classifications - a classification of seizure manifestations and a classification of epilepsy that reflects aspects beyond the seizure manifestations, such as time of onset, neuroimaging findings, etiology, prognosis and etc.

    In 1981, IAE presented the Standardized Classification and Terminology of Epileptic Seizures, which has become most widespread in the professional community (see Table 1). In 1985, the ICAE introduced the Classification of Epilepsies and Epileptic Syndromes, which was quickly followed by a revised version, ratified by the ICAE General Assembly in 1989. The 1989 IAE classification of epilepsies and epileptic syndromes is widely used throughout the world, having a major impact on the management of patients with epilepsy and research (see Table 2).

    It is worth noting significant changes in approaches to achieving consensus in the process of developing the IAE position on fundamental issues, which include terminology and classifications. The previously prepared document required ratification at the IAE General Assembly by a vote of the heads of IAE national branches participating in the meeting. The impressive successes of recent years in the development of means of communication have made it possible to involve a much wider range of experts at the stage of discussion and approval of documents. Since 2013, IAE has used a new process to prepare and approve documents that reflect its positions on key topics. A group of IAE experts prepares the first version of the document, after which it is posted publicly on the IAE official website to collect comments and additions. A separate group of experts is created to review and incorporate into the document relevant comments received from the interested community. In parallel, an expert assessment is carried out by the editorial board of the journal to which the document is sent for publication. If necessary, the process goes through several iterations.

    During the time that has passed since the approval of the International Classification of Epileptic Seizures in 1981, proposals for changes have been periodically considered, a number of which have been adopted. Thus, in 2010, it was proposed to replace “cryptogenic” forms of epilepsy with “probably symptomatic”, “partial” (seizures and forms of epilepsy) with “focal”, and the word “convulsions” with “attacks”. The division of partial (focal) seizures into simple and complex (depending on the impairment of consciousness) was also excluded. It is also necessary to note the proposal to divide epileptic seizures into self-limited (generalized and focal) and ongoing.

    Classification approaches developed in the 1980s took into account mainly clinical manifestations and EEG data. Meanwhile, over the past 30 years, scientific advances, mainly in the field of neuroimaging and genetics,

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    I. Partial (focal, local) seizures

    A. Simple partial seizures (without impairment of consciousness)

    1. Motor seizures

    a) focal motor without march

    b) focal motor with march (Jacksonian)

    c) adversive

    d) postural

    e) phonatory (vocalization or speech stopping)

    2. Somatosensory seizures or seizures with special sensory symptoms (simple hallucinations, eg flashes of flame, ringing)

    a) somatosensory

    b) visual

    c) auditory

    d) olfactory

    d) taste

    e) dizzy

    3. Seizures with vegetative-visceral manifestations (accompanied by epigastric sensations, sweating, redness of the face, constriction and dilation of the pupils)

    4. Seizures with mental dysfunction (changes in higher nervous activity); rarely occur without impairment of consciousness, more often manifest as complex partial seizures

    a) dysphasic

    b) dysmnestic (for example, a feeling of “already seen”)

    c) with impaired thinking (for example, daydreaming, impaired sense of time)

    d) affective (fear, anger, etc.)

    e) illusory (for example, macropsia)

    e) complex hallucinatory (for example, music, scenes)

    B. Complex partial seizures (with impaired consciousness, can sometimes begin with simple symptoms)

    1. Simple partial seizure followed by loss of consciousness

    a) begins with a simple partial seizure (A.1 -A.4) followed by loss of consciousness

    b) with automatisms

    2. Begins with a disturbance of consciousness

    b) with motor automatisms

    B. Partial seizures with secondary generalization (can be generalized tonic-clonic, tonic, clonic)

    1. Simple partial seizures (A), turning into generalized ones

    2. Complex partial seizures (B), turning into generalized ones

    3. Simple partial seizures, turning into complex and then generalized

    II. Generalized seizures (convulsive and non-convulsive)

    A. Absence seizures

    1. Typical absence seizures

    a) only with impaired consciousness

    b) with a weakly expressed clonic component

    c) with an atonic component

    d) with a tonic component

    d) with automatisms

    e) with a vegetative component

    2. Atypical absence seizures

    a) changes in tone are more pronounced than with typical absence seizures

    b) the onset and (or) cessation of seizures does not occur suddenly, but gradually

    B. Myoclonic seizures (single or multiple myoclonic seizures)

    B. Clonic seizures

    D. Tonic seizures

    D. Tonic-clonic seizures

    E. Atonic (astatic) seizures

    III. Unclassified epileptic seizures

    Seizures that cannot be included in any of the above groups due to lack of necessary information, as well as some neonatal seizures, for example, rhythmic eye movements, chewing, swimming movements

    Table 1. The ILAE 1981 Classification of Epileptic Seizures.

    1. Localization-related forms (focal, local, partial) of epilepsy and epileptic syndromes

    1.1. Idiopathic (with age-dependent onset)

    1.1.1. Benign epilepsy of childhood with central temporal spikes

    1.1.2. Childhood epilepsy with occipital paroxysms on EEG

    1.1.3. Primary reading epilepsy

    1.2. Symptomatic

    1.2.1. Chronic progressive partial epilepsy of childhood (Kozhevnikov syndrome)

    1.2.2. Syndromes characterized by seizures caused by specific precipitating factors (including partial seizures due to sudden excitement or emotional influence)

    1.2.3. Temporal lobe epilepsy

    1.2.4. Frontal lobar epilepsy

    1.2.5. Parietal lobar epilepsy

    1.2.6. Occipitolobar epilepsy

    1.3 Cryptogenic

    2. Epilepsy and syndromes with generalized seizures

    2.1. Idiopathic (with age-dependent onset)

    2.1.1. Benign familial neonatal seizures

    2.1.2. Benign idiopathic neonatal seizures

    2.1.3. Benign infantile myoclonic epilepsy

    2.1.4. Childhood absence epilepsy (pycnolepsy)

    2.1.5. Juvenile absence epilepsy

    2.1.6. Juvenile myoclonic epilepsy (impulsive petit mal seizure)

    2.1.7. Epilepsy with generalized tonic-clonic seizures on awakening

    2.1.8. Other generalized epilepsies (not listed above)

    2.1.9. Epilepsy with specific provoking factors (reflex seizures, startle epilepsy)

    2.2. Cryptogenic or symptomatic

    2.2.1. West syndrome (infantile spasms)

    2.2.2. Lennox-Gastaut syndrome

    2.2.3. Epilepsy with myoclonic absence seizures

    2.2.4. Epilepsy with myoclonic-astatic seizures

    2.3. Symptomatic

    2.3.1. Nonspecific etiology

    2.3.1.1. Early myoclonic encephalopathy

    2.3.1.2. Early infantile epileptic encephalopathy with areas of suppressed bioelectrical activity on the EEG

    2.3.1.3. Other symptomatic generalized forms of epilepsy not listed above

    2.3.2. Specific syndromes (include diseases in which seizures are an early and main manifestation of the disease)

    3. Epilepsy and syndromes uncertain as to whether they are focal or generalized

    3.1. With genetically isolated and focal seizures

    3.1.1. Neonatal seizures

    3.1.2. Severe myoclonic epilepsy of early childhood

    3.1.3. Epilepsy with continuous peak waves on EEG during non-REM sleep

    3.1.4. Acquired epileptic aphasia (Landau-Kleffner syndrome)

    3.1.5. Other forms not listed above

    3.2. Without certain generalized and focal signs

    4. Special syndromes

    4.1. Situation-related seizures

    4.1.1. Febrile seizures

    4.1.2. Isolated single seizures or isolated status epilepticus

    4.1.3. Attacks associated exclusively with acute exposure to metabolic or toxic factors, as well as sleep deprivation, alcohol, medications, eclampsia, etc.

    Table 2. The ILAE 1989 International classification of the epilepsies and epileptic syndromes.

    had a great influence on understanding the nature of epilepsy. Therefore, there is a need to update the ILAE International Classification.

    2017 ILAE working classification of seizure types.

    The Seizure Type Classification Task Force was established by the ILAE in 2015. The Task Force selected the 1981 classification and its subsequent modifications as the basis for developing the updated Classification. The main motivations for revising the 1981 classification of epileptic seizures were the following:

    1. A number of seizure types, such as tonic seizures or epileptic spasms, can have either a focal or generalized onset.

    2. Lack of awareness of onset makes it difficult to classify seizures and presents difficulties for discussion in the context of the 1981 classification.

    3. Retrospective descriptions of seizures often do not include characterization of the patient's persistence or change in consciousness, which, although a cornerstone for many seizure types, presents certain challenges.

    5. A number of terms used, such as “mental”, “partial”, “simple partial”, “complex partial” and “dyscognitive”, do not have a clear understanding among society or a high level of recognition among the professional community.

    5. A number of important seizure types are not included in the classification.

    Among the goals and objectives of the Working Group was the creation of an easy-to-use classification that could serve as a communication platform for clinicians, researchers, as well as other interested parties - epidemiologists, health education workers, regulators, insurance companies, patients and their family members, etc. Therefore, the seizure types were classified so that the classification was understandable to everyone, including patients and their families, and applicable to all ages, including newborns.

    The seizure classification structure used since 1981 was retained. A number of definitions have been confirmed. Seizures are defined as “transient signs and/or symptoms of excessive or synchronous neuronal activity in the brain.” Focal seizures were defined by the 2010 ILAE as “occurring in network structures limited to one hemisphere. They can be discretely localized or have a wider distribution. Focal seizures may occur in subcortical structures.” Primary generalized seizures were

    and paroxysmal states

    defined as “initially occurring simultaneously, with rapid involvement of bilaterally located network structures.”

    However, following the 2010 decisions, the term “focal” (“focal”) was used instead of the term “partial”, since this term is more understandable in the context of determining the location of the lesion at the onset of an attack. The term “convulsion” was also excluded, since in a number of languages ​​this term is synonymous with the term “seizures” and the motor context is not clear enough. The terms “conscious / impaired consciousness,” “hyperkinetic,” “cognitive,” and “emotional” were added to the updated classification. The following are definitions of these terms as they apply to this classification.

    The term “conscious/conscious” reflects the ability to be aware of oneself and what is happening around oneself during an attack. The Classification contains two terms that define consciousness - “awareness” and “consciousness”. Their definitions are given in the table below: the developers of the 2017 ILAE Classification of Seizure Types indicate that “impaired awareness” and “impairment of consciousness” are synonymous, therefore, when adapting the classification into Russian, “aware / impaired awareness” is translated as “in consciousness / consciousness is impaired."

    The term “hyperkinetic seizures” was added to the category of focal seizures. Hyperkinetic activity involves agitated, rapid movements of the legs that mimic kicking or pedaling. The term "cognitive" has replaced the term "mental" and refers to cognitive disturbances during the attack, such as aphasia, apraxia or agnosia, and to phenomena such as déjà vu, jame vu (the opposite of déjà vu), illusions or hallucinations. . The term “emotional” refers to emotional manifestations that accompany non-motor seizures, such as fear or joy. It also applies to affective expressions of emotion during gelastic or dacrystic attacks (violent laughter or crying).

    The 2017 ILAE Working Classification of Seizures has been developed in two versions - a basic and an extended version. Figure 1 shows the Basic Working Classification of Seizures ILAE 2017, Figure 2 shows the Expanded Working Classification of Seizures ILAE 2017. The basic classification does not show subtypes of convulsive seizures. You can use both basic and advanced classifications, depending on the desired level of detail.

    Although the classification is presented in the form of a diagram, it does not represent a hierarchical structure, that is, a number of levels may be skipped. The first step is to determine whether the initial manifestations of an attack are focal or

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    [Bilateral tonic-clonic 1 with focal onset

    Unclassified*

    Figure 1. Basic working classification of seizure types of the International League Against Epilepsy 2017 Note." Due to lack of information or inability to assign to other categories.

    Fugure 1. The Basic ILAE 2017 Operational Classification of Seizure Types. Note." Due to inadequate information or disability to place in other categories.

    Focal debut

    Motor debut

    Automatisms

    Atonic*

    Clonic

    Epileptic spasms*

    Hyperkinetic

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    Vegetative

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    Cognitive

    Emotional

    Sensory

    Generalized debut

    Motor

    Tonic-clonic

    Clonic

    Myoclonic

    Myoclonic-tonic-clonic

    Myoclonic-atonic

    Atonic

    Epileptic spasms

    Non-motor (absence seizures)

    Typical

    Atypical

    Myoclonic

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    Unspecified debut

    Unclassified*

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    Figure 2. Expanded working classification of seizure types of the International League Against Epilepsy 2017 Notes." State of consciousness is usually not determined; * due to lack of information or inability to assign to other categories.

    Fugure 2. The Expanded ILAE 2017 Operational Classification of Seizure Types.

    Notes."Degree of awareness is usually not specified;" due to inadequate information or disability to place in other categories.

    generalized. If it is not possible to assess the onset of an attack, such an attack refers to attacks with an unspecified onset.

    In focal seizures, determination of the state of consciousness is optional. Retained consciousness means that the person is aware of himself and his environment during the attack, even if

    he is motionless. Focal seizures with preservation of consciousness correspond to simple partial seizures in the previous terminology. Focal seizures with impaired consciousness correspond to complex partial seizures in the previous terminology. Impaired consciousness during any phase of a focal attack gives grounds to classify it as a focal attack with impaired consciousness.

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    Focal seizures with preserved consciousness or with impaired consciousness can be further characterized as seizures with motor onset or non-motor onset, reflecting their first manifestations or symptoms.

    Seizures should be classified based on their earliest characteristic manifestations, with the exception of focal seizures with behavioral inhibition, for which cessation of motor activity is the dominant feature during the attack. When classifying a focal seizure, it is permissible to exclude references to the state of consciousness in cases where this is not applicable or the state of consciousness is unknown. In this case, seizures are classified directly based on the characteristics of the presence/absence of movements at the onset of the attack. Atonic convulsions and epileptic spasms usually do not have specific disorders of consciousness. Cognitive attacks refer to disorders of speech or other cognitive functions - déjà vu, hallucinations, illusions and disorders of consciousness. Emotional attacks include anxiety, fear, joy, other emotions, or the onset of a state of passion without subjective experiences. Some of the components of emotional attacks are subjective and must be clarified with the patient or their caregiver. Absence seizures are classified as atypical if they have a slow onset or completion or significant changes in tone against the background of atypical, slow generalized spike waves on the EEG. An attack is unclassifiable if there is a lack of information or, based on available information, it is not possible to assign the attack to a particular type in other categories.

    Within this classification, compared to the previous version, new types of focal seizures are epileptic spasms, tonic, clonic, atonic and myoclonic seizures, which were previously regarded as exclusively generalized seizures. The list of motor manifestations of seizures consists of the most common types of focal motor seizures, but other less common types, such as focal tonic-clonic seizures, may also be included. Focal automatisms, autonomic seizures, behavioral inhibition, cognitive, emotional and hyperkinetic seizures are new types of seizures introduced into this classification. Autonomic attacks are accompanied by sensations in the gastrointestinal tract, sensations of heat or cold, hot flashes, pins and needles, palpitations, sexual arousal, respiratory distress or other autonomic effects. Bilateral tonic-clonic seizures with focal onset also present

    and paroxysmal states

    represent a new type of seizures, their previous name was secondary generalized seizures.

    Compared to the 1981 classification, new types of generalized seizures were introduced into the updated classification, such as absences with eyelid myoclonus, myoclonic-atonic and myoclonic-tonic-clonic seizures, although tonic-clonic seizures with clonic onset were mentioned in the publication 1981 It would be logical to classify seizures with eyelid myoclonus as motor seizures, but since eyelid myoclonus is the most significant clinical manifestation of absence seizures, they were placed in the category of absence/nonmotor seizures. Epileptic spasms are seizures that can be classified as either focal or generalized or unspecified seizures; differentiation may require video-EEG monitoring.

    In focal seizures, determining the state of consciousness is optional. Preserved consciousness means that the patient is aware of himself and is oriented in space during the attack, even if he remains motionless. To clarify the state of consciousness, you can ask the patient whether he adequately perceived the events occurring during the attack, or whether the understanding was impaired, for example, does he remember the presence of a person who entered the room during the attack? You can also ask the patient to describe his behavior during the attack. It is important to distinguish the state during an attack from the state after an attack, when consciousness returns to the patient in the post-attack period. Focal seizures with preservation of consciousness or with impaired consciousness can be characterized (not necessarily) by the presence or absence of a motor component at its onset as having a motor onset or a non-motor onset, respectively. The name of a focal seizure does not necessarily indicate characteristics of consciousness. Thus, if the state of consciousness at the time of the onset of the attack remains unknown, it is possible to classify the attack directly according to the characteristics of the motor component as motor or non-motor, although it is permissible not to indicate the state of consciousness.

    Focal seizures in their motor manifestations can be atonic (focal decrease in tone), tonic (sustained focal increase in tone), clonic (focal rhythmic contraction), or epileptic spasms (focal flexion or extension of the arms and flexion of the torso). The distinction between clonic and myoclonic seizures seems somewhat arbitrary, but clonic seizures are characterized by sustained, regular, spaced-out repeated muscle contractions, whereas in myoclonus the contractions are less regular and shorter in duration. Less common are focal seizures with hyperkinetic

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    ical activity or with automatisms. Automaticity is a more or less coordinated, repetitive, purposeless motor activity. To verify a focal seizure with automatisms, witnesses of the seizure can be asked whether the patient during the seizure demonstrated repeated, purposeless fragments of his behavior that might appear normal in other circumstances? Some automatisms may overlap with other movement disorders, such as pedaling or hyperkinetic activity, and as a result the classification may be ambiguous. The 2017 ILAE Working Classification of Seizure Types grouped pedaling with hyperkinetic seizures rather than with automatism seizures. With focal seizures, automatisms can be observed in the absence of seizures.

    Focal motor seizures with inhibition of behavioral reactions are characterized by cessation of motor activity and lack of response. Because the brief behavioral disturbance that occurs early in many types of seizures is nonspecific and difficult to identify, a focal seizure with behavioral inhibition should contain this component as a predominant component throughout the entire episode.

    Impairment of consciousness during an attack does not provide sufficient grounds to classify an attack as cognitive, because impairment of consciousness can accompany any focal seizure.

    Focal sensory attacks can be characterized by the appearance of olfactory, visual, auditory, gustatory, vestibular sensations, as well as feelings of heat or cold.

    In practice, other types of focal seizures are sometimes encountered, for example, focal tonic-clonic seizures. However, their prevalence is not high enough to be called a specific type of seizure. Rather than including the term “other” in each category, the ILAE working group decided to recommend reverting to a higher level category such as “motor onset seizures” or “nonmotor onset seizures” if the next level the details are unclear or the observed type of seizure does not belong to any of those given in the classification.

    The classification of generalized-onset seizures is similar to the 1981 classification, but contains several new types of seizures. During generalized attacks, consciousness is usually impaired, so this characteristic is not used in their classification. The division of seizures into “motor” and “non-motor (absences)” was chosen as the main one. This approach makes it possible to classify

    including those attacks that cannot be characterized in any way except by the principle of classifying them as motor or non-motor. However, when the name of the attack clearly indicates the presence or absence of a motor component, for example, “generalized tonic seizure,” the term “motor” or “non-motor” may not be used. Also, for seizures that can only have a generalized onset (for example, absence seizures), it is permissible to omit the term “generalized”.

    The term "tonic-clonic seizures" replaced the previously used French term "grand mal". Since a new type of seizure has appeared in the classification, characterized by myoclonic movements preceding tonic and clonic ones (myoclonic-tonic-clonic seizures), it is important to correctly determine the initial phase of the attack. In tonic-clonic seizures, the initial phase is the tonic phase. The clonic phase of a tonic-clonic seizure is usually characterized by muscle twitching with a regularly decreasing frequency during the seizure. During a tonic-clonic seizure, loss of consciousness occurs before or simultaneously with the tonic or clonic phases. In some cases, tonic-clonic seizures may begin with nonspecific sensations of impending convulsions or short-term bending of the head or limbs. None of these symptoms prevent these attacks from being classified as attacks with a generalized onset, since biological processes never exhibit perfect synchrony.

    The onset, course and end of generalized clonic seizures are characterized by sustained rhythmic twitching of the head, neck, face, trunk and limbs on both sides. Generalized clonic seizures are much less common than tonic-clonic seizures and usually occur in infancy. They should be distinguished from restlessness in anxiety states and tremors in panic attacks.

    Generalized tonic seizures manifest as bilateral tension or elevation of the limbs, often accompanied by tension in the neck muscles. This classification assumes that tonic activity is not accompanied by clonic movements. Tonic activity may be a stable, unnatural posture, possibly involving stretching or bending of body parts, sometimes accompanied by tremors of the limbs. Tonic activity is difficult to differentiate from dystonic activity, which is characterized by prolonged contractions of both agonist and antagonist muscles, causing athetoid or "twisting" movements that can lead to unnatural postures.

    Generalized myoclonic seizures can occur alone or in combination with to-

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    nic or atonic activity. Unlike clonus, with myoclonus the repetitive movements are briefer and more irregular. Myoclonus as a symptom can be of both epileptic and non-epileptic nature.

    Generalized myoclonic-tonic-clonic seizures begin with several myoclonic jerks followed by tonic-clonic activity. These types of seizures are usually observed in patients with juvenile myoclonic epilepsy, and sometimes with other generalized epilepsies. It is difficult to differentiate between myoclonic and clonic jerks, but if they are not too long to be considered clonic, they are classified as myoclonic.

    A myoclonic-atonic attack is characterized by short-term twitching of the limbs or torso, followed by a drop in muscle tone. These seizures, formerly called myoclonic-astatic seizures, are most often seen in Douz syndrome but can also occur in Lennox-Gastaut syndrome and a number of other syndromes.

    Atonic generalized attacks are often accompanied by the patient falling onto his buttocks or forward onto his knees and face. Recovery usually takes a few seconds. Typical for tonic or tonic-clonic seizures, on the contrary, is falling backwards.

    Epileptic spasms were previously called infantile spasms. The term "infantile spasms" remains relevant for epileptic spasms occurring in infancy. An epileptic spasm is a sudden flexion, stretching, or combination of stretching and flexion of predominantly proximal or trunk muscles. They usually group in clusters and occur most often in infancy.

    The group of non-motor generalized seizures (absences) still includes typical and atypical absences, since these two types of seizures are associated with characteristic changes in the EEG, epileptic syndromes with appropriate therapy and prognosis. According to the 1981 classification, absence seizures should be classified as atypical if there are tone disturbances that are more pronounced than with typical absence seizures, or the onset and cessation of the attack is not sudden. EEG data may be required to differentiate between typical and atypical absence seizures.

    Myoclonic absence seizures include absences with rhythmic myoclonic movements with a frequency of 3 times per second, leading to holding in the air and gradual raising of the upper limbs, associated with generalized peak-wave discharges with the same frequency on the EEG. The duration is usually 10-60 seconds. Impaired consciousness may not be obvious. Myoclonic absence seizures may

    and paroxysmal states

    may be genetically determined, or may also occur without known causes.

    Eyelid myoclonus is characterized by myoclonic jerking of the eyelids, upward deviation of the eyes, often provoked by light or closing the eyes. Eyelid myoclonus may be associated with absence seizures, but can also represent motor seizures without absence seizures, making their classification difficult. In the 2017 ILAE Working Classification of Seizure Types, eyelid myoclonus is included in the group of non-motor seizures with a generalized onset (absence seizures), which may seem counterintuitive. However, the dominant basis for this decision was precisely the connection between eyelid myoclonus and absence seizures. Absence seizures with eyelid myoclonus, seizures, and paroxysmal EEG activity upon closing the eyes or exposure to light constitute the triad of Jeavons syndrome.

    Seizures with an unspecified onset most often include tonic-clonic seizures, the characteristics of the onset of which remain unknown. If additional information is obtained in the future, this will allow the seizure type to be classified as focal onset or generalized onset. Other possible types of seizures that fall into this category include epileptic spasms and seizures with behavioral inhibition. To clarify the nature of the onset of epileptic spasms, video-EEG monitoring may be required, but this is important for making a prognosis - seizures with a focal onset may respond better to therapy. Seizures with behavioral inhibition of unknown onset may be focal seizures with behavioral inhibition and impaired consciousness, or absence seizures.

    Seizures may remain unclassified due to lack of information or failure to be classified into other categories. Unless an episode is clearly a seizure, it should not be classified as an unclassified seizure. To a large extent, this category is reserved for atypical events that are related to seizures but not classified in other categories.

    The authors note that each seizure classification is associated with some degree of uncertainty. The working group adopted the general principle of an 80% confidence level. If there is >80% confidence that the onset was focal or generalized, the attack should be classified in the appropriate category. If there is no such confidence, the attack should be regarded as an attack with an unspecified onset.

    Table 3 summarizes the terms used in the updated 2017 ILAE Seizure Classification. Where possible, the same terminology from the 2001 ILAE glossary has been retained, but a number of new terms have been introduced.

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    Term Definition Source

    Typical absence seizure Sudden onset, interruption of current activity, absent gaze, possible short-term deviation of the eyes. Usually the patient does not respond to treatment. Duration - from a few seconds to U min. with very fast recovery. EEG demonstrates generalized epileptiform discharges during a seizure (although the method is not always available). Absence, by definition, is an attack with a generalized onset. The term is not synonymous with "vacant gaze", which can also occur with seizures. Adapted from

    Atypical absence seizure Absence with changes in tone that are more pronounced than in typical absence seizure; onset and/or cessation are not sudden and are often associated with slow, irregular, generalized spike-wave activity on the EEG. Adapted from

    Inhibition See behavioral inhibition New

    Atonic (seizure) Sudden loss or decrease in muscle tone without a visible preceding myoclonic or tonic component lasting ~1-2 seconds, including muscles of the head, trunk, face or extremities

    Automatism More or less coordinated motor activity, which usually occurs against the background of a disorder of cognitive functions, often followed by amnesia. Often resembles controlled movements and may represent altered motor activity that occurred before the attack

    Autonomic (autonomous) attacks A clear change in the function of the autonomic nervous system, including changes in pupil diameter, sweating, changes in vascular tone, thermoregulation, disorders of the gastrointestinal tract and cardiovascular system Adapted from

    Aura A sudden, patient-specific subjective phenomenon that may precede an attack

    Consciousness (“Aware”) Awareness of oneself or the ability to navigate the surrounding space New

    Bilateral Involving the left and right sides, although the presentation of bilateral seizures may be either symmetrical or asymmetrical New

    Clonic (seizure) A twitching, symmetrical or asymmetrical, that is regularly repeated and involves the same muscle groups Adapted from

    Cognitive Refers to thinking and higher cortical functions such as language, spatial perception, memory and praxis. The previous term for similar use in the context of defining seizure type was "mental" New

    Consciousness Both subjective and objective aspects of the state of mind, including awareness of oneself as a unique entity, perception, responses and memory New

    Dacrystic (attack) Accompanied by episodes of crying, which may not necessarily be associated with sadness

    Dystonic (attack) Accompanied by sustained contractions of both agonistic and antagonistic muscles, causing athetoid or twisting movements that may cause unnatural postures Adapted from

    Emotional attacks Attacks with emotion or the appearance of emotion as an early characteristic, such as fear, spontaneous joy or euphoria, laughter (gelastic) or crying (dacrystic) New

    Epileptic spasms Sudden flexion, stretching, or alternating flexion and stretching of predominantly proximal and trunk muscles, which is usually more prolonged than a myoclonic seizure, but not as prolonged as a tonic seizure. Grimaces, head nods, or small eye movements may occur. Epileptic spasms often develop in clusters. Infantile spasms in infancy are the best known form, but epileptic spasms can occur at any age. Adapted from

    Table 3. Terms used in the updated 2017 ILAE Working Classification of Seizure Types.

    Table 3. Glossary of terms used in ILAE 2017 Operational Classification of Seizure Types. Note. “New” - a new definition created in ILAE2017 Operational Classification of Seizure Types.

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    and paroxysmal states

    Epilepsy A disease of the brain defined by any of the following conditions: (1) at least two unprovoked (or reflex) seizures, >24 hours apart; (2) one unprovoked (or reflex) seizure and a probability of seizure recurrence similar to the overall risk of relapse (>60%) after two spontaneous seizures in the next 10 years; (3) diagnosis of epileptic syndrome. Epilepsy is considered resolved in patients who have reached a certain age with an age-dependent epileptic syndrome or in the absence of epileptic seizures for 10 years in patients who have not used antiepileptic drugs (AEDs) for at least 5 years

    Myoclonus of the eyelids Twitching of the eyelids with a frequency of at least 3 times per second, usually with deviation of the eyes upward, usually lasting<10 сек., часто провоцируется закрытием глаз. В части случаев может сопровождаться кратковременной потерей ориентации Новый

    Fencing fit A type of focal motor seizure involving extension of one arm and bending of the other at the elbow, simulating fencing with a rapier. Also called "supplementary motor area spasms" New

    “Figure 4” seizures Seizures characterized by extension of one arm perpendicular to the torso (usually the contralateral epileptogenic zone in the brain) and flexion of the other arm at the elbow, forming a figure “4” New

    Focal (attack) Occurring in network structures limited to one hemisphere. It can be discretely localized or have a wider distribution. Focal seizures may occur in subcortical structures

    Bilateral tonic-clonic seizures with focal onset A type of seizure with focal onset, with preservation or impairment of consciousness, can be motor or non-motor, then characterized by developing bilateral tonic-clonic activity. Previous term - “secondary generalized seizures with partial onset” New

    Gelastic (fit) Bursts of laughter or giggles, usually without a corresponding affective background

    Generalized (attack) Initially occurring simultaneously, with rapid involvement of bilaterally located network structures

    Generalized tonic-clonic Bilateral symmetrical, sometimes asymmetrical, tonic contractions followed by bilateral clonic muscle twitching, usually associated with autonomic phenomena and impaired consciousness. These seizures involve the network structures of both hemispheres from the very beginning. Adapted from

    Hallucinations Compiling a perceptual composition without appropriate external stimuli, including visual, auditory, somatosensory, olfactory and/or gustatory stimuli. Example: the patient hears and sees people talking

    Inhibition of behavioral reactions Inhibition or pause in activity, freezing, immobility, characteristic of attacks with inhibition of behavioral reactions New

    Immobility See behavioral inhibition New

    Impaired awareness See "awareness". Weakened or lost consciousness is a sign of focal seizures with impaired consciousness, formerly called complex partial seizures New

    Impairment of consciousness See “impaired consciousness” New

    Jacksonian seizure Traditional term for the spread of clonic jerks unilaterally through adjacent parts of the body

    Motor Any form of muscle involvement. Motor activity can consist of both increased contraction (positive) and decreased muscle contraction (negative) when producing movements

    Note. “New” is a new term developed during the creation of the 2017 ILAE Working Classification of Seizure Types.

    Table 3 (cont.). Glossary of terms used in ILAE 2017 Operational Classification of Seizure Types. Note. “New” - a new definition created in ILAE 2017 Operational Classification of Seizure Types.

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    Myoclonic (attack) Sudden, brief (<100 мс) непроизвольное одиночное или множественное сокращение мышц или групп мышц с переменной топографией (аксиальная, проксимальная, мышцы туловища, дистальная). При миоклонусе движения повторяются менее регулярно и с меньшей продолжительностью, чем при клонусе Адаптировано из

    Myoclonic-atonic Generalized type of seizures with myoclonic jerks preceding the atonic motor component. This type of seizure was previously called myoclonic-astatic. New

    Myoclonic-tonic-clonic One or more bilateral twitching of the trunk muscles, followed by the development of a tonic-clonic seizure. The initial twitching can be thought of as a short period of clonus or myoclonus. Seizures of this type are characteristic of juvenile myoclonic epilepsy. Isolated from

    Non-motor Focal or generalized seizure in which motor (motor) activity is not manifested New

    Spread The spread of seizure activity from one brain center to another or the involvement of additional network structures of the brain New

    Response The ability to adequately respond with movement or speech to a presented stimulus New

    Seizure Transient appearance of signs and/or symptoms associated with abnormal excessive or synchronous neuronal activity in the brain

    Sensory attack A subjectively perceived sensation not caused by corresponding stimuli in the external world

    Spasm See "epileptic spasm"

    Tonic (attack) Sustained increasing muscle contraction lasting from several seconds to several minutes

    Tonic-clonic (seizure) A sequence consisting of a tonic contraction phase followed by a clonic phase

    Unconscious The term "unconscious" can be used as a shorthand term for "impaired consciousness" New

    Unclassified May apply to a seizure type that is not described in the 2017 PAE Classification due to insufficient information or unusual clinical features. If an attack is not classified due to insufficient information about its onset, it can be classified to a limited extent based on the data available for interpretation. New

    Lack of response Inability to adequately respond with movement or speech to a presented stimulus New

    Versive (attack) Long-term forced conjugate rotation of the eyes, head and torso or their deviation laterally from the central axis

    Table 3 (continued). Terms used in the updated 2017 ILAE Working Classification of Seizure Types.

    Note. “New” is a new term developed during the creation of the 2017 ILAE Working Classification of Seizure Types.

    Table 3 (cont.). Glossary of terms used in ILAE 2017 Operational Classification of Seizure Types. Note. “New” - a new definition created in ILAE2017 Operational Classification of Seizure Types.

    Seizure classification algorithm

    To facilitate the use of the 2017 PAE Working Classification of Seizure Types, practitioners have developed the following algorithm, which contains expert advice on the sequence of actions and criteria for assessing seizures.

    1. Onset: Determine whether the seizure is focal or generalized using the 80% confidence level. At confidence level<80% начало следует расценивать как неуточненное.

    2. Consciousness: For focal seizures, it is necessary to decide whether to classify according to state of consciousness or to abandon the use of the criterion of consciousness for classification. “Focal seizures with preservation of consciousness” correspond to “simple partial seizures”, “focal seizures with impaired consciousness” correspond to “complex partial seizures” in the old terminology.

    3. Impaired consciousness at any time: a focal seizure is a “focal seizure with

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    impairment of consciousness” if consciousness is impaired at any time during the attack.

    4. The principle of onset dominance: it is necessary to classify a focal attack, taking into account the first sign or symptom (with the exception of inhibition of behavioral reactions).

    5. Inhibition of behavioral reactions: in a “focal attack with inhibition of behavioral reactions,” inhibition of behavioral reactions is a characteristic feature of the entire attack.

    6. Motor/non-motor: “focal seizure with preserved consciousness” or “focal seizure with impaired consciousness” can be further classified based on characteristics of motor (motor) activity. Conversely, a focal seizure may be classified based on characteristics of locomotor (motor) activity without specifying the state of consciousness, for example, “focal tonic seizure.”

    7. Optional terms: Some terms, such as motor or non-motor, may be omitted if the seizure type clearly indicates them.

    8. Additional characteristics: After classifying the type of attack based on initial manifestations, it is recommended to add descriptions of other signs and symptoms from among the proposed characteristics or in free form. Additional characteristics cannot change the type of seizures. Example: focal emotional attack with tonic activity of the right arm and hyperventilation.

    9. Bilateral or generalized: The term “bilateral” should be used for tonic-clonic seizures that involve both hemispheres and “generalized” for seizures that appear to initially develop simultaneously in both hemispheres.

    10. Atypical absence seizure: An absence seizure is atypical if it has a slow onset or completion, marked changes in tone, or spike waves.<3 Гц на ЭЭГ.

    11. Clonic or myoclonic: prolonged rhythmic twitching is referred to as clonic and regular short twitching is referred to as myoclonus.

    12. Eyelid Myoclonus: Absence with eyelid myoclonus is a violent jerking of the eyelids during an absence seizure.

    ILAE Classification of Epilepsy 2017

    At the same time as the 2017 ILAE Working Classification of Seizure Types, the 2017 ILAE Classification of Epilepsy was presented, which is the first comprehensive Classification of Epilepsy since the ratified ILAE Classification of 1989. It represents the result of the work of commissions and workers

    and paroxysmal states

    groups ^AE during the 28 years that have passed since the adoption of the previous classification.

    The 2017 IAE classification of epilepsy is multilevel and intended for use in clinical practice (see Fig. 3). The different levels of classification—seizure type, epilepsy type, and epileptic syndrome—are provided because practitioners around the world may have widely varying access to necessary resources, such as diagnostic tools, etc. Where possible, IAE recommends making a diagnosis at all three levels and additionally establishing the etiology of epilepsy.

    Classification levels

    The starting point in the structure of the 2017 IAE Classification of Epilepsy is the type of seizure. It is assumed that by this stage the doctor has already carried out a differential diagnosis of epileptic seizures with non-epileptic conditions and has established that the attack is precisely epileptic. The classification of seizure types is determined in accordance with the principles of the new Working Classification of Seizure Types 2017 IAE, given above.

    The second level of the 2017 IAE Classification of Epilepsy involves determining the type of epilepsy. It is assumed that the patient's diagnosis of epilepsy is based on the 2014 IAE definition of epilepsy, also given above. In addition to the already widely used definitions of “focal epilepsy” and “generalized epilepsy”, the 2017 IAE Classification of Epilepsy introduced a new type of “combined generalized and focal epilepsy”, as well as a category of “Epilepsy unspecified”. Some epilepsies may involve more than one type of seizure.

    Focal epilepsy includes unifocal and multifocal disorders, as well as seizures involving one hemisphere. With focal epilepsy, such types of seizures can be observed as focal with preservation of consciousness, focal with impaired consciousness, focal motor seizures, focal non-motor seizures, bilateral tonic-clonic seizures with a focal onset. Interictal EEG usually shows focal epileptiform discharges. However, the diagnosis should be made on the basis of clinical data, using EEG results as additional data.

    Patients with generalized epilepsy may have seizure types such as absences, myoclonic, atonic, tonic and tonic-clonic seizures, etc. The diagnosis of generalized epilepsy is made on the basis of clinical data confirmed by EEG results, where there are typical discharges recorded between attacks.

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    Figure 3. Structure of the 2017 ILAE Classification of Epilepsy Note. "Assessed by the onset of the attack.

    Fugure 3. Framework for Classification of Epilepsies ILAE 2017. Note. "Denotes onset of seizure.

    pami - generalized peak-wave activity. Caution should be exercised when making a diagnosis in patients who have generalized tonic-clonic seizures and a normal EEG. In this case, to make a diagnosis of generalized epilepsy, additional data should be available, such as the presence of myoclonus or a relevant family history.

    The 2017 PAE Classification of Epilepsy introduced a new group of combined generalized and focal epilepsies, since there are patients who have both generalized and focal epileptic seizures. The diagnosis is also made on the basis of clinical data confirmed by EEG results. Performing an EEG during a seizure may be helpful, but is not necessary. Interictal EEG may contain generalized spike-wave activity, but the presence of epileptiform activity is not necessary for diagnosis. Common examples in which both types of seizures occur are Dravet syndrome and Lennox-Gastaut syndrome.

    The type of epilepsy may also be the final level of detail for the diagnosis, which is acceptable in cases where the clinician cannot determine the epileptic syndrome. Examples of such a diagnosis are: a patient with temporal lobe epilepsy who has focal epilepsy of unspecified etiology; a 5-year-old child with generalized tonic-clonic seizures and generalized

    peak-wave activity on the EEG, for which it is not possible to determine the epileptic syndrome, but it is possible to make an unambiguous diagnosis of “generalized epilepsy”; a 20-year-old woman with focal seizures with impaired consciousness and absences with the presence of both focal discharges and generalized peak-wave activity on EEG and MRI without features, for whom it seems possible to be diagnosed with “combined generalized and focal epilepsy.”

    The term “unspecified epilepsy” is used in cases where it is understood that a patient has epilepsy, but the doctor cannot determine whether the type of epilepsy is focal or generalized because there is not enough information. Information may be insufficient for various reasons. Perhaps there is no access to an EEG, or the EEG study turned out to be uninformative. If the seizure type is unspecified, then the epilepsy type may also be unspecified for the same reasons, although the two definitions do not necessarily have to be consistent. For example, a patient could have several symmetrical tonic-clonic seizures without focal components and a normal EEG. Thus, the type of seizures at the onset remains unspecified and the type of epilepsy also remains unspecified.

    The third level is the diagnosis of epilepsy syndrome. The syndrome is a group of signs including seizure types, EEG and

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    walkers in neuroimaging, which tend to accompany each other. They often have characteristics that depend on the patient's age, such as age of onset and remission (when applicable), attack triggers, time of onset during the day, and sometimes prognosis. The syndrome may also be associated with concomitant conditions, such as intellectual and mental dysfunction, as well as specific findings with instrumental research methods (EEG and neuroimaging). Defining the syndrome is important to clarify the etiology, treatment and prognosis. Many syndromes, such as childhood absence epilepsy, West syndrome and Dravet syndrome, are well known, but it should be noted that IAE never set out to develop a formal classification of epilepsy syndromes.

    Etiology

    In the 2017 IAE Classification of Epilepsy, when distributing etiological groups, emphasis was placed on those groups that may be important for choosing treatment tactics. These are structural, genetic, infectious, metabolic and immune etiologies, as well as unknown etiologies. In this case, the patient’s epilepsy can be classified into more than one etiological category. For example, epilepsy in a patient with tuberous sclerosis may have both a structural and genetic etiology. Knowledge of the structural etiology is critical for the choice of surgical intervention, and the genetic one for genetic counseling of family members and the selection of innovative methods of targeted drug therapy.

    Comorbid conditions

    There is growing evidence that epilepsy may be associated with comorbid conditions such as learning problems, psychological and behavioral disorders. Comorbid conditions vary in type and severity, from subtle learning difficulties to severe disorders of intellectual and mental functions, such as autism spectrum disorders, depression and problems with adaptation in society. In more severe cases of epilepsy, there may be a range of comorbidities, including motor deficits such as cerebral palsy, scoliosis, insomnia and gastrointestinal disorders. As with etiological classification, when diagnosing a patient with epilepsy, it is important that the presence of comorbid conditions is given due attention at an early stage, in order to ensure their early identification, diagnosis and proper control.

    and paroxysmal states

    Changes in terminology and definitions

    ILAE experts note the need to clarify the term “epileptic encephalopathy.” It should be used not only for severe epilepsy in infancy and childhood, but for patients of any age with epilepsy of any severity, both genetic etiology and others (for example, structural etiology, hypoxic-ischemic damage to the central nervous system or stroke) . It is also appropriate (where applicable) to use the expanded definition of “age-related and epileptic encephalopathy”. This allows one or both definitions to be used together, making it easier for both practitioners and family members of patients with epilepsy to understand the disease. The term “age-related encephalopathy” can be used in cases where there is a developmental disorder without frequent epileptic seizures associated with regression or slowdown in further development (“developmental encephalopathy”). The term "epileptic encephalopathy" may be applicable in cases where there is no preexisting developmental delay and the genetic mutations themselves do not cause the delay. The term "age-related and epileptic encephalopathy" can be used in cases where both factors play a role (it is often impossible to recognize which of them is dominant). Many patients with such disorders were previously classified as having "symptomatic generalized epilepsy." However, this term will no longer be used because it has been applied to a highly heterogeneous group of patients: patients with age-related encephalopathy and epilepsy (ie, static mental retardation and mild epilepsy), with epileptic encephalopathy, with age-related and epileptic encephalopathy, and some patients with generalized epilepsy or combined generalized and focal epilepsy. The new 2017 ILAE Classification of Epilepsy will make it possible to more accurately determine the type of epilepsy in such patients.

    ILAE experts expressed serious concern about the ongoing underestimation of the impact of comorbid conditions on the patient's life, especially in mild forms of epilepsy, such as benign epilepsy with centrotemporal spikes (BECTS) and childhood absence epilepsy. Although generally benign, BECTS may be associated with transient or long-term effects on cognition. There is evidence of an association of childhood absence epilepsy with an increased risk of early pregnancy. Based on this, ILAE experts made a replacement

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    the term “benign” with the terms “self-limiting” and “pharmacoreactive”, that is, giving a positive reaction in response to pharmacotherapy. The term “self-limited” means the probable independent resolution of the epileptic syndrome. The term "pharmacoactive" means that the epileptic syndrome is likely to be controlled with appropriate antiepileptic therapy. However, not all patients with such syndromes will respond to AED therapy. Since PAE does not have a formal classification of epilepsy syndromes, it cannot fully influence their names, but PAE experts expect that the term “benign” in the names of syndromes will be replaced by other specific terms. The terms “malignant” and “catastrophic” will also be eliminated from the lexicon, as they have alarming and psychologically devastating connotations.

    Conclusion

    Since the clinical manifestations of epilepsy and epileptic syndromes do not have a one-to-one correspondence with each other, that is, one type of seizures can be observed in different types of epilepsy and, conversely, a combination of epileptic seizures can be observed in an epileptic syndrome, there is a need for at least two separate classifications - classification of seizures and classification of epilepsy. Classification of epileptic

    Seizures 1981 and the 1989 Classification of Epilepsies and Epileptic Syndromes of the International League Against Epilepsy (ALE) have become widespread among practitioners and in scientific research. Scientific achievements that have taken place since then, incl. in the field of genetics and neuroimaging, as well as advances in the development of communication tools, have dictated the need to update approaches to the classification of epilepsy. In 2014, PAE approved a revised definition of epilepsy, according to which epilepsy is considered a brain disease that meets three criteria: 1) at least two unprovoked (or reflex) epileptic seizures with an interval of more than 24 hours; 2) one unprovoked (or reflex) attack and a probability of recurrence of attacks close to the general risk of relapse (>60%) after two spontaneous attacks in the next 10 years; 3) diagnosis of epileptic syndrome. At the same time, the concept of the degree of risk of relapse was introduced and the criteria for resolution of epilepsy were clarified. Thirty years after the last revision, the 2017 PAE Working Classification of Seizure Types and the 2017 PAE Classification of Epilepsy were introduced. At the same time, clarifications were made to the terminology. It is expected that these tools will be useful both in routine clinical practice and in research, helping to improve the management of patients with epilepsy and their quality of life.

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    Avakyan Gagik Norairovich - Doctor of Medical Sciences, Honored Scientist of the Russian Federation, Professor of the Department of Neurology, Neurosurgery and Medical Genetics, State Budgetary Educational Institution of Higher Professional Education Russian National Research Medical University named after. N.I. Pirogova. Address: st. Ostrovityanova, 1, Moscow, Russia, 117997. E-mail: [email protected].

    Blinov Dmitry Vladislavovich - candidate of medical sciences, State Budgetary Educational Institution of Higher Professional Education, Russian National Research Medical University named after N. I. Pirogov. Address: st. Ostrovityanova, 1, Moscow, Russia, 117997. E-mail: [email protected].

    Lebedeva Anna Valerianovna - Doctor of Medical Sciences, Professor of the Department of Neurology, Neurosurgery and Medical Genetics of the Medical Faculty of the Federal State Budgetary Educational Institution of Higher Education Russian National Research Medical University named after. N.I. Pirogov Ministry of Health of Russia, 117997, Moscow, st. Ostrovityanova, 1. E-mail: [email protected].

    Burd Sergey Georgievich - Doctor of Medical Sciences, Professor of the Department of Neurology, Neurosurgery and Medical Genetics of the Medical Faculty of the Federal State Budgetary Educational Institution of Higher Education Russian National Research Medical University named after. N.I. Pirogov Ministry of Health of Russia, 117997, Moscow, st. Ostrovityanova, 1. E-mail: [email protected].

    Epilepsy is a rare disease, but those who suffer from it need medical help. As of 2016, not a single method has yet been found to eliminate seizures in patients. They can happen at the most unexpected time and in any place, so doctors must be prepared for such cases.

    To find effective ways to combat the consequences of epilepsy, scientists from China and the USA have joined forces. They conducted one large study, the purpose of which was to identify effective methods for blocking seizures in representatives of different ages.

    The experiment involved animals and rodents. Thanks to him, it was possible to find out that the first signs of the disease in humans appear during puberty. It falls on the ages of 12−16 years (girls) and 13−18 years (boys).

    It turned out that at puberty the child develops GABA receptors, which help suppress the activity of the nervous system. One consequence of this is that neurons in the “olfactory” part of the brain (the hippocampus) go to sleep. Against the background of such changes, the epileptic seizure passes.

    A study conducted by Chinese and American scientists showed that rodents with GABA receptors have a 30-40% lower chance of developing epilepsy than other mice from the control group.

    It should be noted that the most well-known GABA receptor is “α4βδ”, and it has already been proven that it can eliminate seizures in patients. In the near future, doctors hope to use it to develop effective methods for the prevention and treatment of people suffering from epilepsy.

    It should also be noted that people with this disease are still labeled as “asocial individuals” who do not fit into normal society. Now, of course, everything is much simpler with this than in the same 70s.

    At that time in Great Britain there was a law prohibiting the marriage of a healthy person and a sick person. According to the European Convention on Human Rights, this is a gross violation.

    Let us recall that epilepsy is a neurological disease, which is characterized by convulsive attacks with the discharge of white foam from the mouth. In Russia it is often called “falling disease.” It occurs not only in humans, but also in mice, cats, and dogs.