Lennox-Gasto syndrome

Lennox-Gastaut syndrome is one of the forms of epilepsy. Let's consider the features of the pathology, diagnostic and treatment methods, as well as the prognosis for recovery.

Generalized epilepsy occurs due to CNS lesions during intrauterine development and negative factors that occur during and after the birth process. Neurological disorder is most often diagnosed in children aged 1-5 years, usually in boys. Very often it is the result of the transformation of childhood encephalopathy and West syndrome. Therefore, it is related to age-dependent and therapy-resistant forms of neuralgia.

The disease is characterized by a combination of atonic and tonic seizures, delayed mental development and atypical absences. As the patient grows older, the nature of the seizures changes, secondary generalized and partial seizures appear. In most cases, the disorder has a poor prognosis, since it is almost impossible to completely cure.

Causes Lennox-Gastaut syndrome

What provokes the development of serious neurological disorders is still unknown. The causes of Lennox-Gastaut syndrome are associated with epileptic activity in the brain, the structure of the membrane of brain cells and their chemical properties. Due to the extensive clinical manifestations, the disorder is difficult to reduce to one factor. Thus, in 70% of cases, the cause remains unidentified, despite a comprehensive neurological examination.

There are a number of factors that can trigger a generalized form of epilepsy:

• Anomalies in brain development.
• Congenital pathological diseases of the central nervous system and brain.
• Premature birth and asphyxia during the birth process.
• CNS trauma in the perinatal period.
• History of severe infectious diseases (rubella, meningitis, encephalitis).
• Traumatic brain injuries and circulatory disorders caused by them.
• Tumors and abscesses of the brain.
• Frequent psycho-emotional stress.
• Overwork and stress.
• Change in climatic conditions.

Depending on the patient's age, the doctor may assume certain causes of PH syndrome. If the disease appeared before the age of 20, the cause is most likely related to brain damage during pregnancy or childbirth. If the attacks occurred after the age of 25, they may be related to brain tumors or circulatory disorders. Let's consider possible causes of the syndrome by age category:

Age	Cause
0-2 years	Hypoxia and ischemia in the perinatal period Birth injuries Metabolic and genetic disorders Idiopathic disorders Acute infection
2-12 years	Traumatic brain injuries Infections
12-18 years old	Idiopathic disorders Various injuries Arteriovenous malformations Withdrawal syndrome from alcohol or strong drugs
18-35 years old	Injuries Brain tumors Alcoholism and drug addiction
>35 years old	Tumors and vascular lesions of the brain Alcoholism Metabolic disorders Injuries

According to medical research, focal seizures are based on damage to one or more areas of the brain, unlike generalized seizures. But both types of disorders require a thorough diagnostic examination.

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Pathogenesis

The determining factor in the development of the syndrome is diffuse encephalopathy in the brain. Pathogenesis is represented by a clear mechanism of pathophysiological processes. The onset of attacks is associated with the functioning of the frontal lobes of the brain, where many biochemical reactions occur. The frequency and nature of epileptic seizures depend on their condition. Another factor is the child's age, upon reaching which the frontal lobes are formed and mature. This explains the coincidence of the period of the onset of the disease and the maturation of the frontal lobes.

According to the symptoms and clinical course, the defect is related to encephalopathy of epileptic nature. That is, this disease is considered a transitional form between epilepsy and complete epileptic status. In 30% of cases, the symptoms are not associated with existing pathologies of the brain, which may indicate the idiopathic nature of the disease. This requires differential diagnostics, as it may indicate serious damage to the central nervous system and brain. In other cases, these are symptomatic attacks caused by serious brain damage. Disorders can be caused by infectious diseases, injuries or difficult childbirth.

At the initial stages of the syndrome development, focal signals are recorded in the patient's brain, which indicate the participation of subcortical structures in the pathological process. Wave activity of the brain is of considerable importance, as it negatively affects its functioning and causes cognitive impairments that manifest as epileptic seizures.

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Symptoms Lennox-Gastaut syndrome

The neurological disorder most often develops in male patients aged 2-8 years. Symptoms of Lennox-Gastaut syndrome are characterized by sudden onset of seizures without apparent cause.

The main symptoms of the disease:

• Delayed or absent psychomotor development
• Intellectual development disorders
• Behavioral disorders (hyperactivity, aggression)
• Early puberty and hypersexuality
• Autism and antisocial traits
• Chronic psychoses

But in most cases, the disorder does not have pronounced neurological signs. Symptoms depend on the localization and extent of the pathology. Most often, patients experience motor symptoms, that is, spasms of various muscle groups. The attacks are short-lived and depend on the muscle tissues involved. Let's consider them in more detail:

• Tonic – the muscles of the trunk, neck and limbs are involved in the pathological process. This manifests itself as sharp bends of the trunk, nods, drawing up of the legs or stretching of the arms. Convulsions last from a few seconds to a minute, most often occurring during sleep or awakening.
• Atypical absences – are not accompanied by convulsions, but are characterized by loss of consciousness, half-open mouth, twitching of facial muscles, lips and eyelids. They occur very often, as a rule, during wakefulness, that is, during the day. They last less than five seconds, so they are not noticeable to others.
• Epileptic seizures of falls - shuddering, abrupt lifting of shoulders and throwing out of arms, squatting, bending of the body, falling forward. The seizures are fleeting, without impairment of consciousness.

In addition to the above-described seizures, the following may occur: myoclonic (spasms of the muscles of the face and limbs) and generalized tonic-clonic. Polymorphism of symptoms is a distinctive feature of Lennox-Gastaut syndrome. Each type of seizure has its own diagnostic picture.

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First signs

The main symptom of the generalized form of epilepsy is seizures of various nature. The first signs appear as short-term spasms of different parts of the body. Patients are characterized by mental retardation, they begin to write, read and speak later. In old age, mental retardation may be observed. The state of stupor is characterized by a smooth transition to epilepsy, personality and cognitive disorders.

Attacks manifest as involuntary twitching of the whole body or limbs, sudden tension of the muscles of the trunk, arms or legs and their contraction. Falls caused by temporary loss of muscle tone are especially dangerous. Attacks most often occur at night. Most patients exhibit impulsiveness, lack of self-preservation instinct, and self-attention.

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Lennox-Gastaut syndrome in children

The first symptoms of Lennox-Gastaut syndrome in children appear at 1-2 years of age. The main clinical manifestations are cerebellar ataxia, impaired coordination of movements and speech, and intention tremor. By the age of 10, patients lose the ability to move independently. Impaired coordination of movements may be combined with diffuse muscle hypotonia, impaired eye movement, and decreased tendon reflexes. At later stages, mental and intellectual development disorders appear.

Children with generalized epilepsy have seizures of varying frequency and severity (atonic, absence, and tonic). The cause of the disease may be congenital anomalies in the development of the brain and central nervous system, birth injuries, brain tumors, and other pathologies. Depending on the cause, the following forms of Lennox-Gastaut syndrome are distinguished:

• Secondary (classical) – occurs against the background of brain damage (birth injuries), diseases, tumors.
• Primary (idiopathic) – has no clearly established cause.

Most pediatric patients have intellectual disabilities and delayed psychomotor development. This manifests itself as behavioral disorders, impulsivity, lack of self-preservation, autism, and a need for increased attention. Due to developmental delays, the likelihood of normal intelligence is extremely low. The main problem is associated with the lack of skill formation and complete or partial cessation of information acquisition. According to medical statistics, half of patients do not have self-care skills, and a quarter of those who survive to adolescence face problems in the emotional sphere and with socialization.

Autistic tendencies, hyperactivity, attention deficit, frequent mood swings, a tendency to aggression, and problems with social adaptation may also occur. At the same time, there is a certain relationship between the child's age and the onset of attacks. The earlier the syndrome begins to manifest itself, the more significant the decrease in intelligence.

The disease is diagnosed based on the analysis of generalized seizures. The doctor's task is to correct both tonic seizures and identify atypical absences. The prognosis for children is severe, but variable. Thus, complete relief of the disorder with the help of drug therapy occurs in 10-20% of cases. Myoclinic seizures without severe pathologies of the central nervous system and brain are amenable to therapy, unlike tonic seizures with a strong decrease in intelligence.

Lennox-Gastaut syndrome in adults

Involuntary jerking, loss of consciousness, and intermittent loss of muscle tone are symptoms that indicate Lennox-Gastaut syndrome. In adults, it can be caused by head injuries, acute cerebrovascular accidents or hemorrhages, various tumors, cysts, clusters, and aneurysms of the brain. Meningitis, encephalitis, and toxic brain damage due to drugs or alcohol can also trigger the disorder.

In most cases, generalized epileptic seizures are accompanied by impaired consciousness, i.e. the patient does not remember what happened to him. Seizures in adults are divided into several groups depending on the accompanying symptoms:

• Absences are sudden loss of consciousness for 5-15 seconds. If fainting is accompanied by other symptoms (eye roll, eyelid and nose twitching, lip licking, rapid heartbeat and breathing), then this is a complex absence.
• Myoclonic – pronounced muscle contractions, twitching, throwing back the head, falling to the knees.
• Tonic-clonic - tonic seizures cause a fall, convulsive contraction of the larynx and masticatory muscles, and arching of the body. The seizure lasts from 15 seconds to 1-2 minutes.
• Tonic – muscle spasms from 5 to 30 seconds (extension of the neck, limbs and torso).
• Astatic – sudden loss of muscle tone in any part of the body. This can manifest as a drooping jaw, a falling head, or a complete fall of the body, freezing in one position for a couple of seconds.
• Clonic – occur extremely rarely, are similar to tonic-clonic seizures, but occur without the first phase.

Based on this, the signs of Lennox-Gastaut syndrome in adults do not always manifest as seizures or loss of consciousness. If the attacks last longer than 30 minutes or the attacks occur one after another, this indicates a severe epileptic status, which is life-threatening. The main danger of the disease is that neurons die during seizures. This can lead to an epileptic personality change. Patients become aggressive, rude, pedantic, tactless, sullen, emotional. At the same time, a slowdown in thinking is observed.

Neurological disorder in adults is incurable. But with a correctly established type of seizures and diagnosis, the pathology can be stopped with medication. Regular use of antiepileptic drugs reduces epileptic seizures to a minimum, allowing you to return to normal life.

Complications and consequences

Lennox-Gastaut syndrome is a complex disease, the complete cure of which is impossible. The consequences of the pathology make themselves known throughout life, even if the attacks are stopped with medication. Most often, patients face the following problems:

• Mental retardation
• Violation of labor and social adaptation
• Intellectual and behavioral disorders
• Mental changes
• Frequent injuries and accidents due to attacks
• Psychological problems

In most cases, the consequences are social. If a sick child has an attack at school, he or she may become an outcast among peers. Although the disorder is not contagious, not all people react normally to its manifestations. Physical activity is subject to restrictions. Exhausting physical activity can provoke an attack, causing injury or more dangerous irreversible consequences.

Special attention is paid to women with the syndrome who are planning a pregnancy. The gynecologist must know the diagnosis of the expectant mother already at the stage of planning the child in order to create all the conditions for preserving the fetus. Medical and genetic consultation is mandatory. The consequences of the neurological disorder cause a number of difficulties in everyday life, but by following the doctor's recommendations and drug therapy, the patient's condition can be improved.

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Complications

If the disease was detected in childhood, then as the child grows older, the symptoms of the syndrome not only persist, but without proper treatment they begin to progress. Complications are associated with persistent intellectual impairment, periodic seizures, and loss of the ability to care for oneself.

Let's consider the main complications of the generalized form of epilepsy:

• Epileptic status – there is little time between seizures, and many different disorders in the functioning of internal organs appear. Most often, complications affect the cardiovascular and respiratory systems, which can cause death.
• Injuries – during an attack, muscles involuntarily tense or relax, which leads to falls and injuries. A sharp fall is a risk of limb fractures or a compression fracture of the spine. Due to convulsions, damage occurs on the tongue and cheeks, since the patient is able to bite them during an attack.
• Mental disorders – patients often live in a state of depression, are asocial and isolated from society.
• Neurogenic pulmonary edema – abnormal functioning of the nervous system causes an increase in blood pressure. Edema develops due to increased pressure in the left atrium and oxygen starvation.
• Sudden death - death may occur during an attack accompanied by extensive convulsions.
• The syndrome is especially dangerous for pregnant women, as its attacks can cause premature birth or miscarriage. In addition, the use of anticonvulsants during pregnancy threatens the development of congenital defects in the child.

In addition to the above complications, problems may arise when taking antiepileptic drugs. This is encountered by 7-25% of patients of different ages. Most often, these are toxic complications (constipation, low body temperature and muscle tone), allergic reactions (shortness of breath, urticaria, increased fatigue, dermatitis) and metabolic complications (sleep disorders, irritability and nervousness, apathy).

The main goal of the treatment prescribed by the doctor is to improve the patient's quality of life and minimize the occurrence of seizures. Refusal of treatment or independent change of the prescribed therapy scheme leads to the fact that Lennox-Gastaut syndrome is transformed into a more severe form of epilepsy.

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Diagnostics Lennox-Gastaut syndrome

If various neurological symptoms make themselves known over a long period of time, then it is worth contacting a doctor, as these may be signs of a serious pathology. Diagnosis of Lennox-Gastaut syndrome is based on the clinical manifestations of the disease and anamnesis. It consists of:

1. Collection of anamnesis and analysis of complaints:

• When did symptoms of the syndrome first appear?
• How did the birth proceed, was it difficult, protracted or traumatic?
• Is there a hereditary predisposition?
• Correspondence of development level to age.

2. Neurological examination:

• Presence of CNS pathologies outside of an attack.
• Questionnaire to determine the level of mental development.

3. Instrumental and laboratory diagnostics

• Tests
• Electroencephalography.
• Computed tomography.
• Magnetic resonance imaging.

A consultation with an epileptologist is mandatory. The doctor pays attention to the presence of generalized seizures, identifies tonic seizures and atypical absences. The examination is carried out during wakefulness and sleep. This allows identifying cognitive impairments in the functioning of the brain.

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Tests

If neurological disorders are suspected, the patient must undergo a whole series of diagnostic procedures. The tests are included in this complex and are:

• Biochemical and general blood analysis
• Blood sugar test
• Spinal tap
• Tests for infectious diseases
• Liver and kidney function tests

A standard blood test can determine whether the seizures are related to hypoglycemia, hyper/hypocalcemia, or hyper/hyponatremia. If biochemical abnormalities are detected, their correction will be prescribed. Tests can identify less common causes of the disease, such as thyrotoxicosis, lead or arsenic intoxication, and intermittent porphyria.

Signs of Lennox-Gastaut syndrome in elderly patients may indicate acute cerebrovascular accident or be the consequences of a previous infarction. Therefore, laboratory diagnostics will be aimed at confirming these assumptions. If generalized tonic-clonic seizures occur in patients without deviations from the central nervous system, this may indicate sleep deprivation. This occurs in students, people working night shifts, and military personnel. If the tests do not reveal any deviations after a single seizure, then further treatment is not required.

If after the entire complex of tests no pathologies are revealed, then most likely the seizures are idiopathic. Such a diagnosis is made with multiple seizures, which may be associated with serious damage to the central nervous system or tumors in the brain.

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Instrumental diagnostics

Examination for suspected generalized epilepsy consists of a complex of various procedures. Instrumental diagnostics are necessary to confirm the results of tests and other types of research. It is represented by the following methods:

1. Electroencephalography is a recording of brain activity, that is, a measurement of brain waves. It is performed during sleep and wakefulness in order to track the frequency of seizures. In Lennox-Gastaut syndrome, diffuse slow sharp waves are observed.
   • Interictal EEG – reveals seizure activity. This manifests as slowing of background activity, spike-wave complexes with a frequency of <2.5 Hz and paroxysms of fast rhythms.
   • Ictal EEG – depends on the type of seizure. Tonic seizures manifest as paroxysms of fast activity >2- Hz, atypical absences – slow spike-waves with a frequency of <2.5 Hz, myoclonic – polyspikes, and atonic – paroxysms of fast activity, spikes (slow, waves).
2. Videoelectroencephalography – is performed in a hospital setting, since the patient must be under constant video surveillance by a doctor. It is prescribed both for primary diagnostics and after ineffective therapy.
3. Computer tomography - scanning of the brain refers to visualization methods. Determines the presence of pathological changes, makes it possible to examine the condition of soft tissues and bone structures in real time.
4. Magnetic resonance imaging – this method is prescribed to patients with unexplained seizures, significant mental or motor problems. With the help of MRI, it is possible to determine the need for surgical treatment.

In addition to the above-described instrumental methods, positron emission tomography can be used in the diagnostic process. It reveals scars and damage in the brain in areas where attacks occur. Single-photon emission computed tomography is performed if surgical intervention is necessary. With its help, the doctor determines which part of the brain is subject to removal. Both methods are done in conjunction with MRI.

Differential diagnosis

Any neurological disorders require a comprehensive diagnostic approach. This is explained by the fact that many pathologies have similar symptoms. Differential diagnostics of Lennox-Gastaut syndrome is carried out with:

• Non-epileptic events.
• Epileptic encephalopathies.
• Epilepsy with myoclonic-astatic seizures.
• Recklinghausen's disease.
• Neurofibromatosis.
• Louis-Bar syndrome.
• Tuberous sclerosis.

The task of doctors is to exclude any conditions that cause generalized epileptic seizures, and these can be:

• Migraine - severe headaches with increased anxiety and convulsions can be mistaken for neurology.
• Fainting is a short-term loss of consciousness during which the blood flow to the brain is reduced. It is often misdiagnosed as an epileptic seizure, even though patients do not have rhythmic contractions and relaxations of muscles.
• Panic disorder – partial seizures can sometimes resemble panic disorder. Symptoms include: rapid heartbeat, increased sweating, shortness of breath, chest pain, chills, trembling, fear of death.
• Sleep disorders - narcolepsy is accompanied by a sudden loss of muscle tone and increased daytime sleepiness, so it can be mistaken for signs of the syndrome.

Based on the results of diagnostic measures, the doctor selects the most effective and safe method of treatment.

Treatment Lennox-Gastaut syndrome

The main problem that doctors face when treating Lennox-Gastaut syndrome is its resistance to any type of therapy. Therefore, even with timely diagnosis and a properly composed regimen, the prognosis remains serious. But thanks to a comprehensive approach, the patient's condition can be improved. The following methods are used for this:

• Surgery – surgical intervention is aimed at removing the tumor or tuberous sclerosis, i.e. the organic substrate of the pathology. In some cases, dissection of the corpus callosum and stimulation of the vagus nerve are used.
• Drug therapy – the patient is prescribed a number of broad-spectrum anticonvulsants. At first, monotherapy is effective with the gradual addition of other drugs. If improvements are observed, then drugs that are effective against certain types of seizures are added to relieve pathological symptoms. It is mandatory to take drugs to improve metabolic processes in the brain.
• Physiotherapy includes massages, exercise therapy, acupuncture and other procedures aimed at preventing attacks of the syndrome and minimizing their pathological impact on the body. To correct developmental delays, regular sessions with a psychologist, teachers and speech therapist are held.

But even the use of the above-described complex does not guarantee an improvement in the patient's condition, which is observed in 14% of cases. According to medical statistics, about 10% of children die during the first 10 years of life. In 100% of cases, varying degrees of behavioral and intellectual impairment are diagnosed.

In addition to the above methods, there are a number of experimental methods for treating generalized epilepsy:

• Deep brain stimulation – the generation of impulses is directed to the part of the brain that produces epileptic seizures. The results of the method showed a positive outcome.
• Neuroimplantation – stimulating devices are implanted into the brain, which detect seizures through electrical stimulation of the brain and stop them. This method has been developed to stimulate the trigeminal nerves, which are involved in suppressing seizures.
• Stereotactic radiosurgery – using focused beams of radiation, lesions deep in the brain are removed. This method eliminates the need for open surgery, minimizing trauma to the patient.

Particular attention is paid to stopping seizures that are accompanied by falls. Since this aggravates the degree of epileptic encephalopathy. As the patient grows older, the main directions of treatment remain unchanged, only the need for drugs that reduce the side effects of the syndrome increases. However, complete seizure control is unlikely.

Drug treatment

Treatment of Lennox-Gastaut syndrome requires mandatory drug therapy for patients of all ages. Medicines are selected by a doctor based on diagnostic results. When choosing medications, the type of attacks, their duration and other features of the disease are taken into account. The basic drugs are derivatives of valproic acid. In addition to them, benzodiazepines, succinimides and other drugs can be prescribed. If there is absolute resistance to the above drugs, then hormonal therapy is carried out in combination with anticonvulsants.

Let's look at popular antiepileptic drugs:

1. Sodium valproate

Anticonvulsant in tablet form 300/500 mg. Active ingredient - sodium valproate, auxiliary: hypromellose, copolyvidone, magnesium stearate, titanium dioxide, opadry and others. The mechanism of action is associated with an increase in the concentration of the inhibitory neurotransmitter GABA in the central nervous system. Active components affect the potassium and sodium channels of neuronal membranes.

After oral administration, it is rapidly and completely absorbed in the small intestine. About 95% of sodium valproate binds to blood proteins and is distributed throughout the body. It is metabolized in the liver and excreted as metabolites in the urine. The half-life is 12-16 hours.

• Indications for use: generalized epileptic seizures, absences, tonic-clonic seizures, atonic and myoclonic seizures. Tablets can be used as adjuvant therapy for focal and partial seizures.
• The method of administration and dosage depend on the clinical response to the drug and the symptoms of the syndrome. At the initial stages of therapy, the minimum dosage is indicated to control seizures. Adults are prescribed 600 mg per day with a gradual increase in the dose every 5-7 days until the seizures completely stop. The maintenance dose is 1000-2000 mg per day, the maximum daily dose is 2500 mg. The dose for children and elderly patients is calculated by the doctor for each patient individually.
• Side effects are temporary and dose-dependent. Most often, patients complain of headaches and dizziness, nausea, gastrointestinal irritation, and metabolic disorders. Reactions from the hematopoietic and cardiovascular systems are possible; to eliminate them, you must consult a doctor.
• The drug is contraindicated in case of individual intolerance to sodium valproate and other components. It is not used in chronic and acute hepatitis, severe dysfunction of the kidneys and pancreas, porphyria and a tendency to bleeding.
• Overdose symptoms include nausea, vomiting, respiratory depression, headaches, and dizziness. Drowsiness, tachycardia, increased intracranial pressure, and seizures may occur. There is no specific antidote, so symptomatic therapy is indicated.

2. Depakine

An anticonvulsant drug that acts on the central nervous system. It is available in tablet form with the active substance valproic acid. It is effective in the treatment of various forms of epilepsy, including Lennox-Gastaut syndrome. It has sedative and muscle relaxant properties, increases the level of gamma-aminobutyric acid in the central nervous system. This leads to a decrease in excitability and seizure activity of the motor areas of the cerebral cortex. The tablets improve mood and have an antiarrhythmic effect.

• Indications for use: generalized and minor epileptic seizures, focal partial seizures with simple and complex symptoms. Convulsive syndrome in organic diseases of the brain and behavioral disorders, tics, febrile seizures. Can be used in manic-depressive psychosis, bipolar disorder and West syndrome.
• The tablets are taken orally, 2-3 times a day, with water. The dosage is selected by the doctor for each patient individually, while the patient's body weight should be more than 25 kg. For adults and adolescents, 20-30 mg / kg is prescribed, with a gradual increase to 200 mg per day with a break of 3-4 days.
• Side effects: nausea, vomiting, aching pain in the epigastrium, upset stomach and pancreas, allergic reactions, headaches, tremor of the extremities, visual disturbances. To eliminate the above reactions, it is recommended to reduce the dosage of the drug and consult a doctor.
• Contraindicated in case of hypersensitivity to the components of the drug, acute/chronic hepatitis, pancreatic dysfunction, liver failure. Not prescribed to patients with hemorrhagic diathesis, thrombocytopenia, in the first trimester of pregnancy and during lactation, as well as for children under three years of age. With special caution used in leukopenia, anemia, mental retardation, thrombocytopenia and organic lesions of the central nervous system. Depakine is prohibited during pregnancy, as in 2% of cases it can provoke congenital defects in the fetus (neural tube malformations, spina bifida).
• In case of overdose, a comatose state, a sharp decrease in blood pressure, and respiratory dysfunction are possible. To eliminate these symptoms, gastric lavage and osmotic diuresis are performed. It is also necessary to monitor the patient's blood pressure, pulse rate, and respiration. If necessary, hemodialysis is performed.

3. Carbamazepine

Antiepileptic, normothymic and antidepressant agent. Available in tablet form of 200 mg, 30 and 100 pieces per package.

• Indications for use: psychomotor epilepsy, severe seizures, post-traumatic and post-encephalitic seizures, trigeminal neuralgia. Contraindicated for use in liver damage and cardiac conduction disorders.
• The tablets are taken orally according to the dosage prescribed by the doctor. For adult patients, as a rule, 100 mg is prescribed 2-3 times a day with a gradual increase in dosage to 800-1200 mg per day. The daily dose for children is calculated as 20 mg/kg, i.e. from 100 to 600 mg per day depending on age.
• Side effects are rare, as the drug is well tolerated. In rare cases, nausea, vomiting, loss of appetite, headaches, impaired coordination of movements and drowsiness occur. To reduce adverse reactions, it is necessary to reduce the dosage of the drug.

4. Phenobarbital

Antiepileptic drug. It is often used as a sleeping pill, as it has a calming effect in small doses. It is available in tablet and powder form and is suitable for treating children and adults.

• Indications for use: generalized tonic-clonic seizures, pathologies of the nervous system with motor excitations and uncoordinated movements, convulsive reactions, spastic paralysis. In combination with vasodilators and antispasmodics, it can be used as a sedative or hypnotic for neurovegetative disorders.
• Treatment of Lennox-Gastaut syndrome is long-term, the drug is started with a minimum dosage of 50 mg 2 times a day and gradually increased to 500 mg per day. The dosage for children is calculated depending on the patient's age. The drug should be discontinued gradually, since abrupt withdrawal can cause a number of epileptic seizures.
• Side effects: decreased blood pressure, skin allergic reactions, CNS depression, changes in blood count.
• Contraindicated for use in severe kidney and liver damage, alcohol and drug addiction, muscle weakness. Not prescribed for treatment in the first trimester of pregnancy and during lactation.

5. Oxcarbazepine

An antiepileptic drug whose action is based on stabilizing the membranes of overexcited neurons. Inhibits serial discharges of neurons, reducing synaptic conduction of impulses.

• Indications for use: monotherapy and combination treatment of generalized epileptic seizures with or without loss of consciousness, secondary generalization, tonic-clonic seizures.
• The dosage and duration of use depend on the symptoms of the neurological disorder and medical indications. In monotherapy, the drug is taken 2 times a day, gradually increasing the dosage. The drug should be discontinued gradually to prevent the occurrence of side effects. Adults are prescribed 600 mg per day in 2 doses, for children 3-5 mg / kg. As part of combination therapy, the initial dose is 300 mg 2 times a day.
• Contraindicated for use in case of hypersensitivity to active components, during pregnancy and lactation, for the treatment of children under two years of age.
• Side effects are manifested by the following symptoms: drowsiness, headaches, dizziness, nausea, increased fatigue, vomiting, nausea, decreased appetite, tremor. In case of overdose, the same symptoms appear. Symptomatic and supportive therapy is indicated to eliminate it. There is no specific antidote.

Most antiepileptic drugs used for Lennox-Gastaut syndrome are prescribed simultaneously with other antispasmodic or sedative drugs. If the patient responds well to the drugs and there are improvements in his condition, then they can be completely abandoned only after 5-10 years, provided that the attacks have not made themselves known for the last two years.

Vitamins

With any neurological disease, the body experiences severe stress and a deficiency of nutrients. Vitamins for Lennox-Gastaut syndrome and other forms of epilepsy are necessary to maintain and strengthen the immune system. Vitamins and minerals are substances necessary for normal life. They should be taken only under medical supervision, since instead of the expected benefit, the course of the disease can be aggravated.

The need for vitamins is due to the fact that some hereditary pathologies can provoke epileptic seizures. For example, with a deficiency and metabolic disorder of vitamin B6, seizures can appear in early childhood. In addition, prolonged use of various antiepileptic drugs affects the level of vitamins B, C, D and E in the blood, as well as folic acid, beta-carotene. A deficiency of these substances can provoke behavioral disorders.

Let's consider which vitamins should be used in the treatment of generalized epilepsy:

• B1 (thiamine)

When it enters the body, it is transformed into the enzyme thiamine pyrophosphate, which is necessary for the normal functioning of the nervous system. It supplies the brain and the entire nervous system with nutrients, regulates the water-salt balance and is responsible for the hormonal regulation of nerve tissue. The main feature of this enzyme is that it utilizes lactic and pyruvic acid. If the utilization process is insufficient, the nervous system does not receive the hormone acetylcholine, which, together with norepinephrine, regulates the work of nerve cells throughout the body.

Contained in such products: legumes, green vegetables, bread, grain crops, nuts, berries, fruits, seaweed, herbs, root vegetables, meat, dairy products. That is, it is very difficult to create a diet in which B1 is not present. Since it is quickly used up, it must be constantly replenished. In addition, it is necessary to improve the absorption of vitamin B5.

• B2 (riboflavin, lactoflavin)

A water-soluble vitamin, necessary for the formation of antibodies and red blood cells, growth regulation and normal functioning of reproductive functions. Responsible for the health of nails, hair and skin. Deficiency of this substance negatively affects the condition of tissues rich in vessels and capillaries, for example, brain tissue. It can provoke cerebral insufficiency of varying severity, general weakness, dizziness, increased tendon and muscle reflexes.

B2 is found in the following products: offal (liver, kidneys), egg white, fish, cheese, porcini mushrooms, cottage cheese, buckwheat, milk, meat, legumes, leafy greens and vegetables. The body does not accumulate this substance in reserve, so its excess is excreted in the urine. Regular consumption of the above products will help normalize the level of B2 in the body.

• B5 (pantothenic acid)

Responsible for the metabolism of fats, amino acids and carbohydrates, synthesizes vital fatty acids. When entering the body, it is transformed into pantethine, which is part of coenzyme A, responsible for acetylation and oxidation. B5 is necessary for the absorption and metabolism of vitamin B9. A deficiency of this substance is manifested by the following symptoms: irritability, sleep disorders, increased fatigue, headaches and muscle pain, numbness of the limbs, dyspepsia.

The body's need for this vitamin is fully satisfied with normal nutrition, since B5 is found in foods of plant and animal origin: buckwheat, oatmeal, peas, garlic, egg yolk, greens, bran bread, cauliflower, carrots. In addition, it is synthesized by intestinal flora.

• B6 (pyridoxine, pyridoxal, pyridoxamine, pyridoxal phosphate)

Participates in the formation of red blood cells and the process of glucose absorption by nerve cells. Participates in protein metabolism of amino acids. Deficiency of B6 causes convulsions, irritability, increased anxiety, dermatitis and other skin reactions, depressive state. Many patients experience decreased appetite, frequent attacks of nausea and vomiting, polyneuritis.

High levels of B6 are found in unrefined grains of cereal crops, leafy greens, yeast, wheat, rice and buckwheat groats, legumes, carrots, bananas, fish, cod and cattle liver and other offal. As well as in egg yolk, cabbage and walnuts.

• B7 (biotin, vitamin H, coenzyme R)

Improves the functioning of the nervous system, participates in the breakdown of fatty acids and fat burning. Vitamin deficiency causes the following symptoms: nervousness, various skin rashes, drowsiness, lethargy, irritability, high blood sugar and cholesterol. The substance is found in yeast, tomatoes, soy, egg yolk, milk, cauliflower and mushrooms.

• C (ascorbic acid)

It is necessary to strengthen the body during long-term use of antiepileptic drugs that are metabolized in the liver. It is found in citrus fruits and vegetables. Rose hips, red peppers, kiwi, black currants, tomatoes, and onions are rich in vitamin.

• E (tocopherol)

Reduces the frequency of epileptic seizures in patients of all ages. Rejuvenates the body, activates tissue respiration processes, reduces blood clotting, improves microcirculation and prevents blood stagnation. Makes the walls of blood vessels smooth, protecting them from the deposition of cholesterol plaques. Contained in vegetable oils, sprouted wheat and corn grains, legumes, pearl barley, oatmeal, buckwheat, eggs, seafood and fish.

Uncontrolled use of vitamins in Lennox-Gastaut syndrome is as dangerous as their deficiency. Any vitamin complexes are taken under strict medical supervision. This is due to the fact that some vitamins can reduce the effectiveness of antiepileptic drugs.

Physiotherapy treatment

Physiotherapy is indicated for the prevention and warning of neurological disorders. Its essence lies in the impact on the body of various physical factors: electromagnetic fields, water, temperature, ultrasound, etc. Physiotherapy is rightfully considered the oldest direction of medicine, which is constantly being improved and developed. The advantage of this method is that it has a minimum of contraindications, is safe and rarely causes side effects.

Physiotherapy is used as an additional method of treatment. It can be used independently at the initial stages of the disease, but in most cases it is carried out in combination with medicinal methods and means.

For Lennox-Gastaut syndrome, the following physiotherapy procedures are performed:

• Acupuncture
• Hydrotherapy (massage shower, hydrogen sulphide, carbon dioxide and radon baths)
• Electrophoresis with drugs
• Hyperbaric oxygenation (oxygen under high pressure)
• Ultra high frequency therapy
• Inductothermy
• Regional barotherapy

The main principle is a course of treatment, since single procedures do not provide a long-term effect. Physiotherapy is carried out daily or every 2-3 days, the course of treatment lasts from 6 to 20 days. This ensures a comprehensive approach to the disease, since many body systems are involved in the pathological process, requiring support and stimulation.

Despite the positive effect of physiotherapy, the method has contraindications: malignant neoplasms, stage 3 hypertension, atherosclerosis of the cerebral vessels, diseases of the hematopoietic system, severe exhaustion of the body, fever, psychosis, epilepsy with frequent seizures. The effectiveness of physiotherapy treatment is significantly higher if there was a course of drug therapy before that, which stopped the attacks.

Folk remedies

Generalized epileptic seizures accompanying Lennox-Gastaut syndrome appeared in people even before the development of modern medicine and the description of this pathology. Various methods were used to eliminate them, but special preference was given to folk treatment.

Let's look at effective recipes of alternative medicine:

• An excellent preventative measure against attacks of any severity is onion juice. Eating ½ an onion a day can reduce attacks and make them recur less frequently.
• Spinach juice has a similar effect. Wash a bunch of fresh leaves thoroughly and grind them in a blender. Drink 100 ml of juice before each meal throughout the day. The juice should be freshly prepared.
• Pour 100 g of the roots of the meadow pasque flower with 500 ml of alcohol and let it brew in a dark, cool place for 10 days. Then strain and take ½ glass (diluted with water) 3 times a day an hour before meals. This recipe is contraindicated in chronic kidney disease. It is also not recommended to change the proportions of the ingredients, since the plant is poisonous and can cause side effects.
• Juice from dandelion roots and leaves has antiepileptic properties. Rinse fresh leaves and roots of the plant well under cold water, cut into small pieces and scald with boiling water. The raw materials must be chopped in a blender or through a meat grinder. Strain the resulting plant liquid through cheesecloth, dilute with water in a 1:1 ratio and boil over low heat for 3-5 minutes. Take 1-3 spoons of the medicine 2-3 times a day 20 minutes before meals. The drug can be stored in the refrigerator, but not longer than three days.
• Pour 750 ml of boiling water over 30 g of crushed peony roots and let it brew in a closed container for 1-1.5 hours. After that, strain the infusion and take 50 ml before meals 2-3 times a day.

It is contraindicated to use any folk remedies without a doctor's permission. In addition, it is worth paying attention to the fact that herbal decoctions and infusions can increase the acidity of gastric juice and lower blood pressure. Therefore, during the period of their use, you need to carefully plan your diet.

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Herbal treatment

Many medicines contain herbal components, as they have a minimum of contraindications and side effects, effectively combat various diseases. Herbal treatment refers to alternative medicine methods and allows you to fully use the value of plants.

Herbal Therapy Recipes for Generalized Seizure Syndrome:

• Pour 250 ml of boiling water over 30 g of rue herb and let it brew for 1-2 hours, strain. Take 30 ml 2-3 times a day.
• Pour 350 ml of boiling water over 100 g of dry wormwood herb and let it brew for 3-4 hours in a closed container, strain. Take 150 ml 2 times a day before meals.
• To reduce the frequency of attacks, strengthen and tone the body, an infusion of 100 g of juice from the green tops of oats is suitable. The medicine is taken 2-3 times a day before meals.
• Take equal proportions of hop cones, peppermint, lemon balm, sweet clover, sweet woodruff and elecampane root. Mix the mixture thoroughly, add 250 ml of boiling water to 30 g of the mixture and leave to infuse until cool. Take 2 times a day, regardless of meals.
• Take equal proportions of calendula and chamomile flowers, valerian root, rose hips and rowan berries. Pour 250 ml of boiling water over 50 g of the mixture and simmer for 5 minutes. The medicine should be infused for 20-30 minutes, after which it is filtered and taken ½ cup 3-4 times a day.

The above recipes can be used only after permission from the attending physician. Their independent use can cause a number of uncontrolled side effects.

Homeopathy

There are many different methods used to treat neurological disorders. Homeopathy is considered an alternative therapy because not all doctors agree with its effectiveness and safety. However, there are a number of medications that can help with Lennox-Gastaut syndrome.

The most commonly used substances to stop epileptic seizures are:

• Arnica 3x
• Argentum metallicum and nitricum 6
• Hyoscyamus 3
• Kalium bro-matum b
• Cuprum metallicum 6
• Silicea 6
• Sulfur 6
• Platinum 6
• Nux vomica 6

They are taken 8 peas 4-5 times a day for a year. The above-described complex helps to relieve attacks and reduce their frequency. In addition, there are more effective collections, for example, the complex developed by homeopath Voronsky:

First day

• In the morning before meals: argentum nitricum 12, vipera 12, 8-10 peas each.
• Ignacia 30, cuprum metallicum 30, naia 12, cuprum aceti-cum 6, 8-10 peas each.
• Veratrum album 3, hemlock virosa 3, artemisia 3, cactus 3x 8-10 peas 2-3 times a day.
• Vipera 12, 5-8 peas before bed.

Second day

• Before breakfast: argentum nitricum 12, magnesium phosphoricum 3, 8-10 granules.
• Belladonna 6, agaricus 6, ignatia 3 (30), colocynthus 3x – 8-10 peas.
• Hyoscyamus 3x, arnica 2, absinthium feta 3x, valerian feta 2x – 8-10 granules.
• Before bed – magnesium phosphoricum 3 x 7-8 peas.

Day three

• In the morning before meals: argentum nitricum 12, zincum metallicum 3 – 8-10 grains
• Arsenicum 30, phosphorus 30, aurum bromatum 30, zinc cyanatum 30 – 8-10 grains.
• Calcium phosphoricum 6, curare 6, potassium phosphoricum 6, mentha piperita 3x - 8-10 granules.
• In the evening before bed: zinc metallicum 3, 7-8 peas each.

The duration of the course is determined by the doctor, individually for each patient. But, as a rule, for stable elimination of cramps, 3-4 cycles are done with a 1-2 day break.

Surgical treatment

Surgical treatment of generalized epilepsy is possible when diagnostic results show that seizures occur in small, clearly defined areas of the brain. At the same time, the affected areas do not disrupt vital functions, i.e., they do not affect speech, hearing, vision, etc. Surgical treatment is performed in 20% of patients with various forms of epilepsy. Its main goal is to improve the quality of life and minimize the number of seizures.

Indications for surgery:

• Attacks of an atonic nature, sudden falls without convulsions.
• Partial seizures with secondary generalization and loss of consciousness.
• Partial seizures with preserved consciousness.
• Progressive sclerosis of the hippocampus of the temporal lobe.

The preoperative planning process and the surgical methods themselves are being improved every day. The operation becomes as safe as possible thanks to visualization methods and monitoring of the entire process. Before treatment, the patient undergoes a number of diagnostic tests to determine whether the operation is necessary. The main ones are:

• MRI – identifies pathological areas of brain tissue where seizures occur.
• Video EEG monitoring is the observation of a patient in a hospital setting with continuous recording of brain activity.
• Ambulatory EEG monitoring is a study of the state of the brain and central nervous system in the patient’s daily life.

These tests allow for precise identification of the affected tissues. If their results reveal that pathological activity is spreading over several areas, more invasive monitoring methods are used.

Types of operations performed:

• Temporal lobe resection – during the procedure, the epileptogenic focus is excised or removed. Most often, it is located in the anterior or masial region. If it is necessary to remove brain tissue behind the temporal lobe, then extratemporal resection is performed.
• Lesionectomy – this method is aimed at eliminating isolated damage, i.e. injured areas or areas with pathologies. These can be tumors or malformations of blood vessels.
• Callosotomy of the corpus callosum is the partial or complete removal of the nerve connections between the hemispheres to prevent the spread of tumors that cause the syndrome. The operation is performed on patients with severe, uncontrolled forms of epilepsy, which are accompanied by atonic seizures.
• Functional hemispherectomy is the complete removal of one hemisphere of the brain. It is most often performed on children under 13 years of age, in whom one of the hemispheres has abnormal functioning.
• Vagus nerve stimulation – an electronic device is implanted under the skin that stimulates the vagus nerve, which is responsible for communication between the brain and internal organs. After such an operation, seizure activity is significantly reduced.
• Multiple subpial transection – removal of pathological foci that cannot be removed without significant neurological risk. The doctor makes multiple transections in tissue to prevent the spread of epileptic impulses. This method allows maintaining normal brain function.
• Neurostimulator implantation (RNS) – a neurostimulator is implanted under the skin in the skull area. The device is connected to a pair of electrodes located on the brain tissue that responds to seizures. The neurostimulator monitors abnormal activity and stimulates the brain, normalizing its function and preventing an attack.

Depending on the type of surgery chosen, its effectiveness is estimated at 50-80%. In some patients, attacks disappear completely, in others they become less pronounced and occur less frequently. But regardless of the results of the surgery, the patient still needs to take the medications prescribed by the doctor, but in a reduced dosage.

Like any operation, surgical treatment of Lennox-Gastaut syndrome is associated with certain risks. First of all, these are postoperative infections, bleeding or reactions to the drugs used, anesthesia. There is a risk of neurological disorders, that is, loss of vision, memory, motor activity. It is worth remembering that even a carefully planned and successfully performed operation cannot guarantee recovery.

Prevention

The principles of prevention of Lennox-Gastaut syndrome have not been developed, as the pathology has unpredictable causes (trauma, tumors, meningitis) causing it. Prevention is aimed at alleviating the patient's condition and improving his quality of life.

Preventive measures:

• A good night's rest - poor or fragmented sleep can cause attacks. It is very important to regulate your sleep and wakefulness patterns.
• Nutrition – various types of food allergies cause not only seizures, but also headaches, gastrointestinal discomfort, and migraines. Carefully monitor your diet and carefully choose foods rich in vitamins and minerals.
• Bad habits – alcohol, smoking and drug addiction are contraindicated for people with epileptic seizures of any severity, as they can provoke them.
• Relaxation and physical health – meditation and deep breathing reduce cramps and relieve anxiety. Regular exercise increases tone and improves overall well-being.
• Emotional and psychological background – many patients and their relatives need professional psychological support. This will help normalize relationships, learn to accept the disease and fight its manifestations.

In addition to the above recommendations, it is necessary to strictly adhere to the drug therapy prescribed by the doctor, since the course of the disease depends on it. It is strictly contraindicated to independently adjust the dosage or take any drugs. It is also recommended to wear a special medical bracelet. This will help doctors and others to navigate if medical assistance is needed. The patient must be involved in an active social life. Since uncontrolled seizures affect life, causing bouts of apathy and depression.

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Forecast

The disease has a poor prognosis and is almost refractory to drug therapy. About 10% of cases end in the death of patients during the first ten years of life. In 80-90%, attacks continue into old age. The following factors are unfavorable in terms of prognosis: high frequency of paroxysms, convulsive syndrome against the background of mental retardation, detection of pathology in early childhood.

Lennox-Gastaut syndrome is a serious diagnosis, the high mortality rate of which is associated with trauma during seizures with falls. At the same time, 95% of patients have serious mental and neurological deviations, various degrees of mental retardation, 40% lack the ability to self-care.

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