Lennox-Gastaut syndrome in children and adults

Multiple tonic, clonic, atonic and myoclonic seizures are the main signs characterizing the Lennox Gastaut syndrome. In general, the disease is characterized by psychomotor backwardness of the patient, which in time only worsens. The syndrome of Lennox Gasto is difficult to cure and is no less difficult to treat.

[1], [2]

Epidemiology

The incidence of Lennox Gastaut syndrome is considered to be relatively low. Nevertheless, the distinctive features of seizures allow to speak about their high prevalence: it is not less than 5% of people of all age categories suffering from epilepsy, and close to 10% of epilepsy patients in infancy and adolescence.

[3], [4], [5], [6], [7], [8], [9]

Causes of the lennox Gastaut syndrome

To date, the exact causes that lead to the appearance of the child's syndrome Lennox Gastaut, are not established. There are only a number of factors that, as specialists suggest, can give an impetus to the development of pathology.

The following risk factors are considered:

• the state of hypoxia in the fetus during the period of intrauterine development;
• negative processes in the child's brain related to premature birth or fetal lag in development;
• infectious pathologies in the brain (eg, meningoencephalitis, rubella);
• infantile convulsive syndrome, or Vest syndrome;
• focal-cortical dysplasia of the brain;
• Burneville disease.

If Lennox Gastaut's syndrome appeared at an older age, then the causes may be tumor processes in the brain, or cerebrovascular disorders. Provocative factors that contribute to the development of the syndrome after 25 years are head injuries, alcohol abuse, drug use, severe vascular pathology.

[10], [11]

Pathogenesis

Until now, there is no information that indicates possible violations of the physiological processes that precede the appearance of the Lennox Gastaut syndrome. However, there is evidence that the condition of the frontal lobes is of great importance in the development of the symptoms of the syndrome. It can be concluded that in the formation of pathology, these brain structures are primarily involved.

At the stage of development of the Lennox Gastaut syndrome, focal discharges and spike-wave complexes are often detected, and the role of subcortical structures in the course of the pathological reaction is clearly manifested.

The significant interictal activity of spike waves, presumably, is of great importance in the appearance of cognitive disorders - it is these disorders that predominantly occur along with convulsive seizures. Lennox's syndrome Gasto is considered one of the variants of epileptogenic encephalopathy - so to speak, an intermediate form of epilepsy and epileptic status.

The relative impact of spike-wave activity, possibly, affects the ability of neuron networks to generate delayed oscillations associated with the release of GABA. This process leads to the interruption of fast activity.

[12], [13], [14], [15], [16]

Symptoms of the lennox Gastaut syndrome

For Lennox Gastaut's syndrome, the symptomatic triad is typical:

• common delayed spike-waves with EEG;
• psychomotor delay;
• a large number of generalized seizures.

The most common syndrome is found in childhood - from 2 to 8 years, mainly in boys.

The first signs can arise unexpectedly, spontaneously, or because of obvious damage to the brain.

The Lennox Gastaut syndrome in children begins with cases of sudden fall. In older children, the first symptoms may be behavioral disorders. Over time, seizures occur more often, epileptic seizures occur, the intellect decreases, personality disorders are observed, in some cases, psychoses are diagnosed.

The appearance of tonic seizures is the most characteristic feature of the Lennox Gastaut syndrome. Such attacks are in most cases short-lived and last only a few seconds.

There are such kinds of similar attacks:

• axial (reduction of the flexor muscles of the neck and trunk);
• axial-rhizomelic (lifting with reduction of proximal parts of hands, neck muscular tension, lifting of the shoulders, opening the mouth, "rolling up" the eyes, short-term respiration);
• global (with a sharp drop in the patient from the standing position).

Attacks of the syndrome may be asymmetrical or lateral. In some cases, tonic convulsions are followed by automatisms. The tonic stage in most cases occurs at the stage of falling asleep, but it can also occur during the day.

Infants have a delayed or suspended psychomotor development. In 50% of patients, behavioral disorders are noted:

• hyperactivity;
• emotional lability;
• aggression;
• autism;
• associativity;
• disturbed sexual activity.

In addition, there is a development of chronic psychoses with periodic exacerbations.

Approximately 17% of patients have no neurological features.

[17]

Stages

The Lennox Gastaut syndrome can occur in the form of various stages:

• Atonic stage - characterized by a sharp drop in tone, 1-2 seconds. At the same time, consciousness may be disturbed. Since the stage lasts for a very short time, externally it can manifest itself with a sharp weakness in the neck, or with a nod of the head.
• Tonic stage - is characterized by an increase in muscle tone (muscles are sharply compressed, grouped, "rusty"). The stage lasts from a couple of seconds to several minutes - more often on waking from sleep.
• Absence stage - is a short "trip" of consciousness. The patient seems to freeze, fixing his eyes on one point. At the same time, no drop occurs.

In infants, seizures may be longer - up to half an hour, or frequent, with extremely short interruptions between attacks.

[18], [19], [20], [21]

Forms

Attacks in the Lennox Gastaut syndrome are not always the same and have their own specific differences:

• Generalized seizure:

1. convulsive seizure;
2. small attack (absense, myoclonia, akinesia);
3. status epilepticus.

• Focal attack:

1. motor attack (chewing, tonic, myoclonic cramps);
2. sensitive fit (visual, hearing, olfactory, taste, paroxysmal dizziness);
3. mental fit (paroxysmal psychosis, dysphoria);
4. automatism;
5. speech attack (paroxysmal loss of articulation);
6. reflex attack.

[22], [23], [24], [25], [26], [27]

Complications and consequences

The clinical symptoms of a pathology such as Lennox Gasto's syndrome require a prolonged and complex drug correction. Over time, the disease can be complicated by severe neurological and social consequences:

• relapse of seizures, even during treatment;
• resistance to treatment;
• intellectual inferiority, which remains throughout life;
• disturbed social and labor adaptation.

Tonic seizures occur against a background of a steady decrease in intellectual activity.

In addition, other frequent complications of Lennox Gastaut syndrome are possible:

• an attack can result in a fall and injury to the patient;
• epileptic status is a complication characterized by a series of repeated seizures accompanied by irreversible changes in the brain;
• an attack can end fatal - and this complication is not uncommon.

[28], [29], [30], [31], [32]

Diagnostics of the lennox Gastaut syndrome

The first stage in the diagnosis of Lennox Gastaut syndrome is an anamnesis. The doctor first of all will pay attention to the following questions:

• When did the first seizure begin?
• How often do seizures occur, how long do they last and what are they expressed in?
• Does the patient have other diseases? What medications is he treated for?
• If the disease is diagnosed in an infant, then it is necessary to specify how the mother's pregnancy and labor activity proceeded.
• Did the patient have a head injury?
• Have there been behavioral disorders or other oddities?

Analyzes are not a criterion for diagnosing Lennox Gastaut's syndrome, but often they can differentiate the disease from other pathologies:

• biochemistry of blood;
• determining the level of glucose in the blood;
• UAC;
• assessment of renal function (OAM);
• hepatic assays;
• spinal puncture;
• tests for the presence of infection in the body.

Instrumental diagnosis is the main and implies the use of such diagnostic techniques:

• Electroencephalography is a procedure that fixes and measures intracerebral waves.
• Videoelectroencephalography - a procedure similar to the previous one, which allows you to monitor the state of the brain in dynamics.
• Computer tomography is a computer scan of brain structures. High resolution allows qualitatively to consider, both bone formations, and soft fabrics.
• Magnetic resonance imaging is a safe and informative diagnostic method, which is most recommended for evaluating the brain condition in children.

In addition, the doctor can prescribe positron emission tomography, single-photon emission CT, which are performed only simultaneously with magnetic resonance imaging.

[33], [34], [35]

Differential diagnosis

Differential diagnosis is performed to exclude other diseases that could cause symptoms similar to Lennox Gastaut's syndrome. To such diseases, first of all, are:

• Short-term loss of consciousness associated with impaired cerebral circulation. Such moments, as a rule, are not accompanied by rhythmic convulsions.
• Migraine attacks accompanied by a strong (usually one-sided) headache.
• Panic attacks, which can be taken as partial seizures. Panic is usually accompanied by tachycardia, increased sweating, tremor, shortness of breath, weakness, phobia.
• Narcolepsy is a neurological disorder in which a person suddenly "disconnects", plunging into sleep. At the same time there is a sharp loss of muscle tone.

Treatment of the lennox Gastaut syndrome

For the treatment of Lennox's syndrome, Gasto is used, both medicamental and operational techniques.

Therapy with medicines has a positive effect in only 20% of patients. Operative treatment provides for the dissection of the calloused body - the operation of callosotomy. In addition, it is possible to carry out operations to stimulate the vagus nerve and resection of vascular tumor processes and malformations.

The use of drugs Carbamazepine and Phenytoin allows you to establish control over generalized seizures and reduce the frequency of seizures - but these medicines may in some cases have the opposite effect. Valproate preparations (valproate derivatives) stop or reduce any seizures, but their effect is too short.

A good effect is expected from Felbamate, which reduces the frequency and severity of seizures. But it is noticed, that reception of the given preparation is accompanied by a plenty of by-effects. Based on this, Felbamate is prescribed only in extreme cases, with regular monitoring of liver function and blood condition, for a period not exceeding 8 weeks.

Among other medications that are often used to treat Lennox Gastaut syndrome, there are drugs such as Nitrazepam and Vigabatrin.

Corticosteroid drugs, with a slow decrease in dosage, can help the patient at the initial stage of the syndrome, during epileptic status and during periods of exacerbation of the pathology. Additionally, Amatantadine, Imipramine, Tryptophan or Flumazenil are prescribed.

Medications for Lennox Gastaut syndrome

	Dosing and Administration	Side effects	Special instructions
Clobazam	Take 20-30 mg per day in two divided doses, or once a night. The course of treatment is 1 month.	Fatigue, trembling in the fingers, drowsiness, nausea, eating disorders.	Longer use of the drug should be agreed with the doctor.
Rufinamide	Take twice a day with food, starting with a daily dose of 200 mg, with the possible increase to 1000 mg per day.	Pain in the head, dizziness, drowsiness, vomiting.	Cancellation of the drug is carried out gradually, with a decrease in dosage by 25% every two days.
Convoolex	The drug is administered intravenously, in an average daily dose of 20-30 mg per kg of body weight.	Violations of the hepatic function, headache, ataxia, tinnitus, digestive disorders.	During treatment, it is necessary to monitor the patient's blood coagulability.
Lamotrigine	Tablets are taken with water, not crushing them, in an amount of 25 mg daily. Gradually, the dose can be increased to 50 mg per day.	Skin rashes, joint and muscle pain, nausea, diarrhea, impaired liver function.	The drug is not used to treat children under 3 years old.
Topiramate	Take the pill in its entirety, 25-50 mg per day (in 2 divided doses). Further individual correction of dosage in one or another direction is possible.	Anemia, changes in appetite, depression, drowsiness, speech disorders, memory impairment, tremor.	Cancellation of the drug is carried out gradually, so as not to cause resumption of seizures.

The use of immunoglobulins in the syndrome of Lennox Gasto

Over the past decade, clinical medicine has begun to actively use large intravenous doses of immunoglobulin. Such preparations are presented as a puffed, virus-inactivated, standard polyvalent human immunoglobulin. The basis of the drug is IgG immunoglobulin, as well as a small percentage of IgM and IgA.

Initially, immunoglobulin was introduced into use to eliminate violations of natural immunity in all possible conditions of primary or secondary immunodeficiency.

Immunoglobulin preparations are prepared on the basis of donor plasma. The mechanism of their action has not yet been fully elucidated: experts suggest a complex effect on the body.

Certain therapeutic schemes, which are guided by the treatment of Lennox Gastaut's syndrome, do not exist. The most commonly used dosages are from 400 to 2000 mg per kg of body weight per course, with a single dose of 100-1000 mg per kg for 1-5 injections. The frequency of injection may be different.

Vitamins

Vitamins are extremely necessary for the normal course of vital processes in the body of a patient with Lennox Gastaut syndrome. But the intake of vitamin preparations should be controlled, otherwise the patient's condition may be aggravated.

• Anxiety disorder of vitamin B ₆ may cause seizures in children.
• Prolonged use of anticonvulsant drugs can lower the level of certain vitamins in the body: tocopherol, vitamin D, ascorbic acid, vitamins B ₁₂, B ₆, B ₂, folic acid, β-carotene.
• The lack of the above vitamins in the body of a patient with Lennox Gasto's syndrome can lead to the appearance of various cognitive disorders.

Vitamins for the treatment of the syndrome should be taken only under the supervision of a doctor, because an unbalanced or chaotic intake of certain vitamin preparations can affect the effectiveness of other medicines. In addition, for example, prolonged or unreasonable intake of folic acid can lead to new convulsive seizures.

Physiotherapeutic treatment

Physiotherapy treatment with Lennox Gastaut syndrome is used with great care. Not all procedures are shown to patients. Very often even after one or two sessions, the patients experience an exacerbation.

The most loyal physicians refer to such physiotherapy methods:

• inductothermy (sometimes - in a complex with galvanic baths);
• hydrotherapy (massage shower, therapeutic baths);
• UHF;
• healing mud;
• solux;
• method of hyperbaric oxygenation;
• electrophoresis with iodine and novocaine, calcium chloride, lidase, etc.

With properly selected physiotherapy treatment, the frequency of attacks decreases, they become less pronounced. This effect can last up to six months.

Performing acupuncture and massage with Lennox Gastaut syndrome is not recommended.

Alternative treatment

With Lennox Gastaut's syndrome, you should follow a special diet that excludes sharp, salty, pickled foods, as well as coffee, chocolate, cocoa, carbonated drinks, alcohol. It is desirable to reduce the amount of fluid used, especially at night.

To prevent new seizures, it is useful to consume white onions, or to drink onion juice. The mechanism of action of onion juice is unknown, however it is noticed that it weakens and reduces the frequency of attacks.

Scientists have proved that daily regular consumption of omega-3 fatty acids, even in small amounts, can reduce the number of attacks in the Lennox Gastaut syndrome. Therefore, it is recommended to drink 1 tbsp every morning. L. Flaxseed oil or fish oil. In addition, it is possible to prepare marine fish of fatty species (this may be sardine, mackerel, tuna), or take special dietary supplements.

The people successfully apply Maryin root - it helps not only with the syndrome of Lennox Gasto, but also with neurasthenia, paralysis, epilepsy. Alcohol tincture of the petals of the plant is prepared from the calculation of 3 tbsp. L. Raw materials for 500 ml of vodka, it takes 1 month. Tincture take 1 tsp. Up to 3 times a day.

[36], [37], [38], [39]

Herbal Treatment

• Useful for the Lennox Gasto syndrome to drink infusion of succession. To make it, take 1 tbsp. L. Dry raw materials and pour 250 ml of boiling water. This drink should be drunk daily, in the morning and in the evening, half the glass.
• With prolonged and frequent attacks help infusion on the basis of seeds of cumin. You need to pour one cup of boiling water 1 tsp. Cumin, insist 8 hours in a thermos bottle. Drink infusion should be three times a day, 1 tbsp. Spoon.
• Three times a day you need to take a ready tincture of peony (you can buy at a pharmacy), in the amount of 40 drops per 100 ml of water, before eating. Peony can be replaced by an equally effective motherwort.
• It is useful to take inside the tincture of mistletoe - 1 tbsp. L. Three times a day, before meals. To prepare the tincture, you need to pour 100 g of raw 500 ml of vodka, leave in a dark closet for 3 weeks. After that, filter and use the medicine.

Homeopathy

Homeopathic remedies can be used both to prevent attacks and to eliminate an already existing attack. It is important to choose the right medicine based on the symptoms.

• Belladonna is used for sudden convulsions, which are accompanied by fever, red face, trembling in the body and limbs.
• Hamomilla is prescribed for convulsions, irritability, night cramps, angry attacks.
• Kuprum metallukum is used to eliminate seizures that occur against the background of dyspnea or croup.

When an attack occurs, the patient is given one dosage of 12C or 30C urgently. If this amount proved effective, then the procedure is suspended. If the seizures continue or are repeated, the dose is offered every quarter of an hour, until the patient's condition is relieved.

The development of side effects is virtually eliminated, even with the use of increased dosage of drugs.

Among the complex homeopathic remedies, specialists prefer the following means:

• Cerebrum compositum is an injectable solution intended for intramuscular or subcutaneous administration. The usual treatment lasts up to a month and a half. The drug is administered at 2.2 ml 1-3 times a week.
• Vertigoheel is a solution for injections, which is used intramuscularly for 1.1 ml 1-3 times a week, for 2-4 weeks.

Operative treatment

The specialists placed great hopes on the stimulation of the vagus nerve - this method of treatment of the Lennox Gasto syndrome still requires further development. It was assumed that the stimulation and operation of callosotomy had an approximately equivalent effect. However, a recent study showed that implantation of the stimulant may not be effective in all patients with Lennox Gasto's syndrome: kallosotomy proved to be more effective.

Callosotomy is an operative intervention, which consists in dissecting the corpus callosum - the basic structure that unites both hemispheres of the brain. This operation does not completely eliminate the disease, but prevents the rapid spread of convulsive activity from one hemisphere to another, which allows the patient to avoid falling during an attack.

The operation of callosotomy is not without side effects. Thus, in patients who underwent such an operation, there may be a violation of mental abilities, which is expressed in the difficulties of explanation or the impossibility of determining body parts. The reason for this is the broken connection between the hemispheres.

And another type of operation - focal cortical resection - is used relatively rarely, only in cases when a separate neoplasm in the form of a tumor or vascular cluster is diagnosed in the brain.

Prevention

There is no specific prevention of Lennox Gastaut's syndrome, since the true causes of the disease have not yet been elucidated. Moreover, many probable factors that can serve as a trigger mechanism in the development of pathology are unpredictable - among them birth trauma, inflammatory processes in the brain.

Patients who are prone to frequent attacks, it is recommended to comply with the milk-vegetable diet, to establish a rational mode of work and rest, to perform regular metered exercise. It is very important not to go on about negative emotions, dress in the weather (do not overcool and do not overheat), "forget" about the existence of cigarettes and spirits, do not drink coffee and strong tea.

If possible, it is desirable to visit nature more often, rest - this will reduce the frequency of seizures and make the life of the patient more qualitative.

[40], [41]

Forecast

The prognosis is considered unfavorable, but Lennox Gastaut's syndrome is not transformed into epilepsy. Relaxation of symptoms and the preservation of intellectual abilities are observed only in a certain number of patients - while tonic seizures continue to bother, but to a lesser extent.

The most unfavorable course of the syndrome in patients with impaired brain function, with early onset of the disease, with frequent seizures, with a repeated epileptic status.

The syndrome of Lennox Gasto at the moment is not completely cured.

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