Advertising on the portal

Grimacing smiles are signs of infantile spasms. Main epileptic syndromes in children: neonatal seizures, infantile spasms, febrile seizures, Lennox-Gastaut syndrome

Clinical and electroencephalographic characteristics of epileptic seizures in children vary depending on age. A clear example of such age-dependent phenomena is infantile spasms, a unique type of seizure associated with early childhood. Infantile spasms are a specific age-related phenomenon that occurs in children only in the first two years of life, most often between 4 and 6 months, and in approximately 90% of patients before 12 months. The incidence of West syndrome is estimated to be 0.4 per 1000 live births.

Definition

Characteristic features of this syndrome include myoclonic seizures, hypsarrhythmia on the EEG, and psychomotor retardation. This triad is sometimes called West syndrome. However, infantile spasms do not clearly fit this definition in all cases. There are other names for the disease in the literature: massive spasms, Salaam spasms, flexor spasms, jackknife attacks, massive myoclonic seizures, infantile myoclonic spasms.

Typically, infantile spasms are stereotypical in one child. In addition, the occurrence of infantile spasms in the form of series is characteristic.

Although these seizures resemble myoclonic or tonic seizures, infantile spasms are a separate type of seizure. Myoclonic jerks are rapid, lightning-fast contractions of limited duration, while tonic spasms are prolonged muscle contractions of increasing intensity. A true spasm involves a characteristic muscle contraction that lasts 1-2 seconds and reaches a maximum (peak) slower than myoclonus, but faster than tonic spasms.

Infantile spasms are divided into three types: flexor, extensor and mixed flexor-extensor. Flexor spasms are short-term contractions of the flexor muscles of the trunk, neck, upper and lower extremities. Spasms in the muscles of the upper extremities cause the abduction of the arms, “as if a child were hugging himself with his arms,” or, on the contrary, the spreading of the arms, bent at the elbow joints, in different directions. Extensor spasms involve predominantly extensor muscle contraction, causing rapid, sharp extension of the neck and torso in combination with extension and abduction or adduction of the arms, legs, or upper and lower extremities simultaneously. Mixed flexor-extensor spasms include flexion of the neck, trunk, and upper extremities and extension of the lower extremities, or flexion of the lower extremities and extension of the arms in combination with varying degrees of flexion of the neck and trunk. Sometimes asymmetric spasms develop, reminiscent of the “fencing position.” Infantile spasms are often combined with eye deviation or the appearance of nystagmus.

Asymmetrical spasms can occur when simultaneous bilateral contraction of the limb muscles is not observed. This type of spasm usually occurs in symptomatic infantile spasms in infants with severe brain damage, agenesis of the corpus callosum, or a combination of these disorders. Local neurological symptoms such as eye deviation or head rotation can be combined with both symmetrical and asymmetrical spasms. Asymmetrical spasms usually occur in isolation, but they may also occur after or precede a focal seizure; In some cases, infantile spasms may occur simultaneously with a generalized or focal seizure.

Infantile spasms often occur in series (“cluster spasms”). The intensity and frequency of spasms in each series can increase, reaching a peak, and then progressively decrease. The number of attacks in a series varies significantly, and can exceed 30 spasms. The number of episodes per day also varies; some patients have up to 20 per day. A series of infantile spasms may occur at night, although they are rarely observed during sleep. During or after a series of infantile spasms, the child usually cries or becomes irritable.

Hypsarrhythmia in a child with infantile spasms. Noteworthy is the high-amplitude disorganized activity in the background recording, alternating with multifocal spikes and sharp waves

The chaotic nature of the EEG creates the impression of complete disorganization of the cortical rhythm. During sleep, discharges of polyspikes and slow waves occur. It is surprising that sleep spindles persist in some patients in combination with significant abnormalities in background EEG recordings. During the REM sleep phase, the severity of hypsarrhythmia may decrease or completely disappear. Infantile spasms are associated with a decrease in total sleep duration and REM sleep duration. Various types of hypsarrhythmia have been described, including patterns with interhemispheric synchronization, persistent focus of abnormal discharges, episodes of decreased amplitude, and high-amplitude slow-wave activity in combination with isolated sharp waves and spikes. Various patterns of hypsarrhythmia are common and do not correlate with disease prognosis.

Although hypsarrhythmia or a modified hypsarrhythmic pattern are the most common types of interictal abnormal EEG activity, some patients with infantile spasms may not have these EEG patterns. In some cases, there may be no hypsarrhythmia at the onset of the disease and the appearance of this pattern later, during the development of the disease. Although hypsarrhythmia is associated primarily with infantile spasms, this pattern occurs in other diseases.

Like the interictal pattern, ictal EEG changes during infantile spasms are also variable. The most characteristic EEG pattern during an attack consists of positive waves in the vertex-central region; Low-amplitude fast (14-16 Hz) activity or diffuse flattening of the curve, called an “electro-decremental event,” may also be observed.

The presence of focal abnormalities is one variant of the underlying hypsarrhythmia pattern that may be associated with focal seizures; focal seizures may precede, accompany, or develop in conjunction with a series of infantile spasms. This observation suggests that cortical pacemakers may play an important role in the development of infantile spasms.

The electroencephalographic picture in this disease is not constant and may evolve over time. In some patients with infantile spasms, hypsarrhythmia may be absent at the onset of the disease. In other patients, there may be a slowdown in bioelectrical activity in combination with rare epileptiform activity, which subsequently transforms into a hypsarrhythmia pattern. Repeated electroencephalographic studies during follow-up may be necessary to demonstrate the pattern of hypsarrhythmia (in those children in whom hypsarrhythmia was not detected at the onset of the disease).

When a child begins to shake with convulsions, most parents experience a real shock. They don't know where to run and waste precious time. With West syndrome, every week is important: the sooner the diagnosis is made and treatment is started, the greater the child’s chances for a full recovery and a happy future.

What is West syndrome

West syndrome is a severe form of epilepsy that develops in young children due to brain damage and some other serious diseases. Characteristic signs of this pathology are mental retardation, as well as infantile spasms - nodding or rapid tilting of the body, usually when falling asleep or waking up. In this case, the encephalogram records hypsarrhythmia - abnormal high-amplitude brain activity.

West syndrome affects 1 to 4 people out of every 10 thousand babies. This disease accounts for up to 9% of all epileptic seizures in children and 25% of cases of infantile epilepsy. The incidence is higher among boys: the insidious syndrome affects about 60% of young representatives of the stronger sex.

The disease received its name in honor of the British doctor West, who described the symptoms of the pathology in 1841 based on observations of his own son. Later, the disease acquired many synonyms: West syndrome, bowing convulsions, Salaam spasm (tic), Gibbs hypsarrhythmia, myoclonic encephalopathy with hypsarrhythmia, convulsive epilepsy with hypsarrhythmia, flexion seizure syndrome. Initially, the syndrome was classified as a type of generalized epilepsy, but later doctors transferred it to the category of epileptic encephalopathies, in which attacks are provoked by non-inflammatory diseases of the brain.

As a rule, newborns who are subsequently diagnosed with this condition are born apparently healthy or with minor abnormalities. The onset of the disease occurs at 3–7 months of life: during this period, pathology is diagnosed in 77% of patients. In children over 1 year of age, the syndrome occurs in only 10% of cases.

Among those with West syndrome, the mortality rate is high, and in surviving children, by the age of 3, seizures develop into another form of epilepsy, most often Lennox-Gastaut syndrome. With adequate treatment, complete remission can be achieved, but mental retardation is usually observed in patients for a long time.

Forms of the disease

There are 2 main forms of West syndrome.

  1. Symptomatic - characterized by the presence of a clear cause of the pathological condition: brain damage, genetic factors. With this form, children experience an initial delay in psychomotor development, the patient suffers from several types of seizures, and structural changes are noted in his brain.

    Symptomatic West syndrome is considered the most severe type of the disease, and the prognosis for treatment and life is often disappointing.

  2. Cryptogenic (idiopathic) - differs from the symptomatic form in the absence of a visible cause of seizures and is diagnosed in approximately 12% of patients. This type is characterized by only 1 type of seizures, structural changes in the brain are not visualized, and developmental delay occurs only after the onset of the disease, when its manifestations become obvious. The cryptogenic variety has a relatively favorable prognosis for life and complete recovery, occurring in a milder form.

With West syndrome, cramps affect almost all muscles, including the neck, head and limbs. Contractions are usually symmetrical on the left and right sides of the body, last up to 10 seconds and can be repeated many times throughout the day.

Sometimes spasms affect only one muscle group. Depending on the location of their lesion, the following types of seizures in West syndrome are distinguished:

  • occipital - extension convulsions of the neck with throwing back of the head;
  • sternocleidomastoid (flexion) - contractions of the flexor muscles located on the arms and neck: nodding the head, joining the arms in front of the chest, etc.;
  • widespread (extensor) - convulsions that cover the entire body: the arms and legs are thrown to the sides, reminiscent of manifestations of the Moro reflex.

If seizures occur too frequently, the child may fall asleep immediately after they stop. Prolonged convulsions greatly affect development: the baby begins to lag further and further behind his peers in motor, mental and psychological terms.

Causes

The infantile spasms characteristic of West syndrome occur due to improper interaction between the brain stem and its cortex. Immaturity of the central nervous system provokes disruption of connections between the brain and the adrenal glands, as a result of which the hypothalamus synthesizes too much corticoliberin. An excess of this hormone causes muscle contractions typical of West syndrome.

In 85–88% of cases, doctors are able to determine the cause of this disease. The following factors can provoke the syndrome:

  • hypoxia during childbirth or the intrauterine period;
  • congenital malformations of the brain;
  • birth injuries;
  • intrauterine infections;
  • genetic and chromosomal abnormalities;
  • asphyxia;
  • intracranial hemorrhages;
  • postnatal ischemia;
  • encephalopathy;
  • meningitis;
  • tuberous sclerosis;
  • neurofibromatosis;
  • pigment incontinence syndrome;
  • phenylketonuria;
  • metabolic disorders;
  • tumors;
  • hereditary predisposition.

Often the trigger for the onset of the disease is the use of nootropics or vaccination, but these factors act as a catalyst and not an independent cause. If the child’s body does not function correctly, West syndrome will manifest itself sooner or later, but anything can serve as a trigger: from vaccination to a stressful situation.

Symptoms and signs

The first sign of West syndrome may be a child's loud, inconsolable crying. Local doctors attribute everything to intestinal colic. A correct diagnosis is made only when other warning symptoms appear:

  • absence or slowing of the pace of psychomotor development: the child does not roll over or sit down, does not reach for toys, his grasping reflex disappears;
  • vision problems: the baby does not look into the eyes, does not fix his gaze on objects. It often seems to parents that he sees nothing at all;
  • muscle flaccidity (hypotonia);
  • tearfulness and irritability;
  • the appearance of spasms.

Seizures are a typical manifestation of West syndrome. The following types of convulsions are distinguished:

  • myoclonic - small symmetrical twitching of the muscles of the trunk, limbs and face, occurring in short series;
  • tonic - prolonged contractions of individual muscle groups: nodding the head, shrugging the shoulders, bringing and spreading the limbs, folding the body in half.

As a rule, manifestations of West syndrome begin with myoclonic seizures, and over time they transform into tonic seizures. Most often, seizures occur during falling asleep and waking up, but provoking factors can be loud sounds, fear, as well as light and tactile stimulation.

Convulsions are characterized by a certain seriality, following each other with an interval not exceeding 1 minute. Sometimes spasms manifest themselves in the form of sudden stops or falls of the child, breathing problems, nystagmus or twitching of the eyeballs. Before an attack, the baby may get scared and scream, and after it, become lethargic and sleepy.

In this case, the electroencephalogram (EEG) shows hypsarrhythmia - the absence of a normal rhythm of electrical activity in the brain. In some cases, cerebellar syndrome may develop, which is characterized by the following disorders:

  • trembling of fingers;
  • muscle laxity;
  • dizziness;
  • inability to perform fast and complex movements;
  • a symptom of the absence of a “reverse push”.

A symptom of the absence of a “reverse push”: the patient bends his arm with force at the elbow joint. The examiner tries to straighten it, to which the patient resists, keeping his arm in a bent position. Then the examiner suddenly stops extension, and the patient’s hand hits the chest with force.

The sequence of appearance of symptoms of West syndrome depends on its form. Thus, with the idiopathic type, a delay in psychomotor development is observed after the convulsive onset, and the symptomatic type of the disease is characterized by an initial developmental delay, while infantile spasms and changes in the EEG are recorded later.

Depending on clinical manifestations and electroencephalogram results, patients are divided into 3 risk groups.

  1. The first group includes children who have been diagnosed with hypsarrhythmia, but have no visible symptoms of the disease. Such babies should undergo annual examination and do not need treatment.
  2. The second risk group includes children with the main signs of West syndrome and characteristic changes on the EEG. They are prescribed special treatment, and every six months they undergo a detailed examination.
  3. The third risk group includes patients with severe symptoms, for whom the lack of treatment is tantamount to death.

Timely diagnosis and adequate treatment give children with West syndrome a chance to improve their quality of life, and sometimes even to fully restore their health.

Infantile spasms in a child - video

Diagnostics

At the first alarming symptoms, it is necessary to show the child to a neurologist, who can prescribe additional consultations with a geneticist, epileptologist, immunologist, endocrinologist, and also a neurosurgeon. After examining the baby, the doctor sends the little patient for additional examinations:

  • EEG - to detect disorganized brain activity during sleep and wakefulness;
  • CT scan of the brain - to determine changes in cerebral structures;
  • MRI of the brain - for accurate diagnosis of structural disorders and determination of the cause of West syndrome;
  • PET of the brain - to determine foci of hypometabolism in brain tissue;
  • cerebral angiography - to identify cerebral vascular pathologies;
  • cranioscopy - to study structural defects of the skull.

Carrying out these studies makes it possible to distinguish West syndrome from diseases with similar symptoms:

  • infantile myoclonus;
  • infantile myoclonic epilepsy;
  • benign rolandic epilepsy;
  • Sandifer syndrome;
  • various tics.

Manifestations of West syndrome are often similar to tics, but in this disease muscle spasms are provoked by emotional outbursts, and no abnormalities are observed on the EEG.

Sometimes, after an electroencephalogram, it turns out that the spasms were actually colic or respiratory attacks. In any case, the doctor can make a diagnosis only after conducting all the necessary examinations.

Treatment of West syndrome

With timely diagnosis and proper treatment, stable remission of the disease can be achieved in more than 50% of cases. However, it happens that taking medications does not affect the number and intensity of attacks and does not contribute to the development of the child. The success of therapy also depends on the causes of the disease, the degree of brain damage and the form of West syndrome.

Drug therapy

Until 1958, West syndrome was considered an incurable disease, so a real revolution in this area was the discovery of the positive effect of adrenocorticotropic hormone (ACTH drugs) and Prednisolone on patients. The dose and duration of steroid therapy are selected individually, but in most cases, attacks decrease or disappear during the use of significant dosages of ACTH for 1-2 months. The EEG shows visible improvements with this treatment: hypsarrhythmia disappears, and a normal rhythm of brain activity appears.

In the early 90s of the last century, another breakthrough occurred in medicine: a positive effect of vigabatrin (Sabril) on patients was discovered. This drug does not have as many side effects as ACTH, is better tolerated by children, and as a result of taking it, patients experience fewer relapses after treatment.

Vigabatrin is most often used if the cause of West syndrome is tuberous sclerosis. In other cases, this remedy may not be as effective as steroids.

The following anticonvulsants may be used to reduce the number and intensity of seizures:

  • valproic acid;
  • vitamin B6, which in large doses acts like anticonvulsants in some patients.

Anticonvulsants prescribed for West syndrome - gallery

To improve physical and psycho-emotional development, doctors usually prescribe drugs to children that normalize metabolism and blood supply to the brain. Nootropics are recommended for children with extreme caution, since brain stimulation can provoke increased seizures even while taking anticonvulsants.

It should be borne in mind that hormonal and steroid drugs have strong side effects. Patients may experience:

  • fatigue;
  • depression;
  • inability to concentrate;
  • allergic reactions;
  • endocrine disorders;
  • diseases of the peripheral nervous system;
  • liver damage.

A competent doctor, along with basic medications, usually prescribes drugs that enhance immunity and support liver function. Throughout the course, the specialist must constantly monitor the patient’s condition based on blood tests and EEG readings. In the case of hormonal therapy, treatment is carried out in a hospital setting.

If the doses of the drugs were selected correctly and the patient shows positive dynamics, treatment should be continued for about 1.5–2 years starting from the moment of the last attack.

Surgical intervention

If infantile spasms turn out to be resistant to drug therapy, and the pathological focus is clearly visible on MRI, the neurosurgeon may recommend excision of the affected area of ​​the brain. During the operation, the specialist cuts through the adhesions of the meninges, removes tumors and vascular aneurysms, while trying to use the most gentle surgical methods.

If seizures manifest themselves in the form of sudden falls, the patient may be indicated for callosotomy, an operation to cut open the corpus callosum. After surgery, children usually undergo neurorehabilitation in specialized centers.

Therapeutic exercise

Regular physical therapy classes for children with West syndrome are simply necessary to restore physical fitness and practice new motor skills. As a rule, after the attacks stop, babies quickly begin to sit, crawl, walk and even run, but without the correct selection of medications, physical therapy will not bring the desired result.

Exercise therapy must be carried out under the guidance of an experienced rehabilitation therapist and only after such exercises are authorized by the attending physician. Otherwise, you can aggravate the intensity of the seizures and worsen the general condition of the child.

In parallel with the development of motor skills, the child needs to regularly study with a speech pathologist, psychologist and speech therapist, who will develop the child’s speech and fine motor skills.

Unconventional methods

Among the effective non-traditional methods are stem cell treatment. This method is very expensive and is not recognized by official medicine. It is based on the fact that damaged areas of the brain are restored due to the introduction of donor stem cells into the patient’s body.

Today, few people decide to take this measure, although such therapy has already proven itself well. Stem cell treatment is not a panacea, but it helps some patients.

Nutritional Features

Many foreign doctors achieve good results when treating West syndrome using a ketogenic diet. It is based on increasing fat in the diet and reducing carbohydrates and proteins. At the same time, the metabolism changes, and the body begins to produce large amounts of ketones, which reduce the frequency and intensity of seizures.

The ketogenic diet helps about 70% of patients, and seizures are reduced by more than 2 times, and in some children, convulsions disappear completely. As a rule, the diet is prescribed to children from 2 years of age and is not particularly effective in treating seizures in adolescents and adults.

Foods rich in fats - gallery

Cure prognosis, mortality rate, life expectancy and prevention

In very rare cases, West syndrome resolves spontaneously, without any treatment. More often, seizures go away under the influence of medications and surgery; sometimes the disease transforms into other forms of epilepsy.

The prognosis of treatment directly depends on the form of West syndrome.

  1. With the idiopathic variety, 37 to 44% of children recover completely. The remaining patients have certain deviations in physical and mental development.
  2. With the symptomatic form, the prognosis is much worse. No consequences are observed in only 5–12% of cases, and mortality can reach 25%. Even with the onset of remission of the disease, children develop mental retardation (mental development delay), cerebral palsy, autism, mental retardation, many experience learning difficulties, have problems with memory, concentration and logical thinking. Approximately half of the patients experience movement disorders. Such a pessimistic prognosis is due to the negative impact of the underlying disease on the body. The patient’s life expectancy depends on its course.

The prognosis will be more favorable if treatment is started on time. If medications are selected correctly from the first weeks of illness, the chances of a full recovery increase several times. After 1–2 months, the percentage of a favorable outcome is halved.

If treatment is started six months or later after the onset of seizures, the chances of recovery will be minimal.

The child’s condition can be worsened by uncontrolled use of nootropics and vaccination.

There is no prevention for West syndrome. It is important to diagnose this disease in time and choose the right treatment - then, even with a symptomatic form, it is possible to achieve significant improvements and bring the child’s development as close as possible to the age norm.

Doctor Komarovsky about seizures in children - video

West syndrome is an insidious and dangerous disease. The percentage of infant mortality in this case is quite high, so at the first alarming symptoms it is necessary to contact a doctor and carry out a diagnosis as quickly as possible. The correct selection of medications together with competent rehabilitation with the participation of psychologists, speech pathologists, speech therapists and exercise therapy specialists increases the child’s chances of a full recovery.

Convulsions in children of the first year of life.
Cramps are chaotic, mostly painful, contractions of various muscle groups.
The reasons that lead to seizures in children are quite varied. The main ones are the following:
1. Infectious diseases. Meningitis, encephalitis, and brain abscesses lead to brain damage and disruption of nerve impulse transmission.
2. Maternal drug abuse during pregnancy. Drugs disrupt the process of intrauterine brain formation, so children born to drug-addicted mothers may experience seizures.
3. Endocrine diseases. Diabetes mellitus, diseases of the thyroid gland, and adrenal glands can cause seizures in a child at any age.
4. Burdened heredity. Some genetic diseases lead to impaired brain development, which may result in the development of seizures in a child.
5. Tumor lesions of the brain cause a disruption in the conduction of nerve impulses along nerve fibers, which is why children experience seizures.
6. Lack of calcium.
7. Incorrect use of medications. Some drugs, such as diuretics, cause a decrease in calcium in the blood, which causes seizures. The appearance of seizures is also observed with an overdose of vitamin D3 and the development of a condition such as spasmophilia.
8. A cramp may occur during hypothermia (for example, a limb will cramp in cold water). But if this happens frequently, you need to see a doctor.
Seizures can be mistaken for an epileptic attack, so when diagnosing, it is necessary to keep this disease in mind.

In children older than 1 month, the following types of seizures are more common:
1. Primary generalized (tonic-clonic, grand mal type). They are characterized by a tonic phase lasting less than 1 minute, with the eyes rolling upward. At the same time, gas exchange decreases (due to tonic contraction of the respiratory muscles), which is accompanied by cyanosis. The clonic phase of seizures follows the tonic phase, expressed in clonic twitching of the limbs (usually 1-5 minutes); This improves gas exchange. May be observed: hypersalivation, tachycardia, metabolic/respiratory acidosis. The postictal state often lasts less than 1 hour.
2. Focal motor seizures (partial, with simple symptoms). They are characterized by their occurrence in one of the upper limbs or in the facial area. Such convulsions lead to deviation of the head and diversion of the eyes towards the hemisphere opposite to the localization of the convulsive focus. Focal seizures can begin in a limited area, without loss of consciousness, or, conversely, generalize and resemble secondary generalized tonic-clonic seizures. Indications of the focus are Todd's palsy or abduction of the head and eyes towards the affected hemisphere. They appear after an attack of these seizures.
3. Temporal or psychomotor seizures (partial, with complex symptoms). In approximately 50% of cases they are preceded by an aura. They can imitate other types of seizures, be focal, motor, grand mal, or with frozen gaze. Sometimes they look more complex: with stereotypical automatisms (running - in those who have begun to walk, laughter, licking lips, unusual movements of the hands, facial muscles, etc.).
4. Primary generalized absence seizures (petit mal type). Rarely develop in the first year of life (more typical for children over 3 years of age).
5. Infantile spasms (with hypsarrhythmia - according to EEG data). They most often appear in the 1st year of life and are characterized by pronounced myoclonic (salaam) spasms. Infantile spasms (West syndrome) can develop due to the presence of various neurological pathologies or without any obvious previous disorders. With infantile spasms, psychomotor development slows down, and in the future there is a high probability of severe developmental delay.
6. Mixed generalized seizures (small motor or atypical petit mal). This group of seizure disorders is typical of Lennox-Gastaut syndrome, which is characterized by frequent, poorly controlled seizures, including atonic, myoclonic, tonic and clonic, which are accompanied by an EEG pattern with atypical spikes (from the English Spike - peak) and waves (less than three spike-waves in 1 s), multifocal spikes and polyspikes. The age of patients often exceeds 18 months, but this syndrome can develop in the 1st year of life following infantile spasms (transformation from West syndrome). Children often have severe developmental delays.
7. Febrile seizures (FS). They are observed in children starting from 3 months of age, with an increase in body temperature (>38.0 o C). As a rule, they are primarily generalized tonic-clonic, although they can be tonic, atonic or clonic.
Febrile seizures are considered simple if they occurred once, lasted no more than 15 minutes and did not have focal symptoms. Complex febrile seizures are characterized by repeated occurrence, duration and the presence of pronounced focality. All patients under 12 months of age require a lumbar puncture and metabolic screening to determine the cause of seizures.
Risk factors for the development of epilepsy in FS include:
- indications of the presence of neurological disorders or psychomotor impairments
development;
- a family history of afebrile seizures;
- complex nature of febrile seizures.
In the absence or presence of only one risk factor, the likelihood of developing afebrile seizures is only 2%. If two or more risk factors are present, the likelihood of epilepsy increases to 6-10%.



Treatment.

Treatment of convulsive syndrome in children must begin with first aid. The general principles of this assistance are given below.

First aid for a child with a seizure/convulsions
When convulsions appear, the child must be laid on a flat surface and try to protect him from foreign objects, since by making chaotic movements with his arms and legs, the child can injure himself. You need to open the window. The child needs to be provided with access to oxygen, so you cannot “huddle” and “hang” over the child, making it difficult for fresh air to reach. If the child has a tight collar on his shirt, the top buttons must be undone. Under no circumstances should you try to insert foreign objects, especially sharp ones, into your child's mouth, as this can lead to serious injury. Then it is necessary to take measures to reflexively restore breathing, namely, pat the child on the cheeks, splash the face with cold water, let ammonia breathe in from a distance of 10-15 cm. After these measures, you must urgently consult a doctor who can differentiate the convulsive syndrome and develop specific recommendations for its treatment based on the type of seizures and the causes of their occurrence.
An important role in establishing the causes of seizures belongs to the examination of the child.
Diagnosis of seizure syndrome includes:
General blood test, general urinalysis, for children under 3 years of age, urine analysis according to Sulkovich to exclude spasmophilia.
Determination of blood electrolyte composition. Particular attention is paid to reducing calcium and magnesium levels in the blood.
Determination of blood glucose.
Determination of blood gas composition. Pay attention to the oxygen and carbon dioxide content.
Carrying out a lumbar puncture with examination of the cerebrospinal fluid to determine the content of sugar, protein, electrolytes, and cellular composition to exclude infectious damage to the brain.
Ultrasound examination of the brain for children with an open fontanel, tomography of the brain for older children.
Electroencephalography to determine brain function and detect vascular disorders.
Only on the basis of these studies can the diagnosis be verified.

Drug therapy for special cases of convulsive syndrome is based on the following principles:
Primary generalized seizures (grand mal). Usually phenobarbital, phenytoin (epdantoin, epanutin), carbamazepine are used. As an alternative, in some cases, valproates (Depalept, Depakine) or acetazolamide can be used.
Partial simple seizures (focal).
Phenobarbital, phenytoin (epdantoin, epanutin), carbamazepine, primidone are used. If necessary, other therapeutic agents can be used (valproic acid preparations (Depakine, Depalept), vigabatrin (Sabril, Sabrilex, Sabrilan), Keppra, etc.)
Partial complex seizures (temporal lobe epilepsy) . Priority administration of the drugs carbamazepine, phenytoin and primidone is provided. An alternative to them are phenobarbital drugs, valproate and acetazolamide (as well as methsuximide, ethosuxemide, petinimide, zarontin).
Primary generalized seizures (petit mal, absence seizures) . The main antiepileptic drugs AEDs in the described clinical situation are ethosuximide, valproates, methsuximide. Other agents: acetazolamide, clonazepam, clobazam, phenobarbital.
Infantile spasms . The most effective drugs for the treatment of infantile spasms are: synthetic analogue of ACTH - synacthen depot, vigabatrin (Sabril, Sabrilex, Sabrilan), valproates (Depalept, Depakine), Keppra, ethosuxemide (Zarontin, Petnidan, Petinimide), clobazam (Frisium). Other types of therapy include the use of phenytoin (epdantoin, epanutin), talox, phenobarbital, acetazolamide. If possible, a ketogenic diet (KD) can be used.
Febrile seizures. The advisability of prescribing anticonvulsants to children for FS has continued to be extremely controversial for many years. However, when deciding in favor of preventive therapy using AEDs, phenobarbital drugs are most often used, and valproates are used less often.
Mixed generalized seizures.
The main AEDs: phenobarbital, valproate, clonazepam, clobazam (Frisium). As alternatives, acetazolamide, diazepam, ethosuximide, phenytoin, methsuximide, carbamazepine, as well as tranxene, etc. can be used.
Dosing of main anticonvulsants (in the 1st year of life)
- diazepam - 0.1-0.3 mg/kg up to a maximum dose of 5 mg intravenously slowly;
- phenytoin - 5 mg/kg/day (2 times, per os);
- phenobarbital - 3-5 mg/kg/day (2-3 times, per os);
- primidone - 5-25 mg/kg/day (1-2 times);
- carbamazepine - 15-30 mg/kg/day (2-3 times, per os);
- ethosuximide - 20-30 mg/kg/day (2 times);
- methsuximide - initial dose 5-10 mg/kg, maintenance dose - 20 mg/kg (2 times, per os);
- valproate - 25-60 mg/kg/day (2-3 times, per os);
- clonazepam - 0.02-0.2 mg/kg/day (2-3 times, per os);
- paraldehyde - 300 mg (0.3 ml/kg, rectally);
- acetazolamide (diacarb) - initial dose 5 mg/kg, maintenance dose - 10-20 mg/kg (per os).

Features of the treatment of seizures in children of the first year of life (including newborns).
You should always take into account the fact that phenytoin (epdantoin, epanutin) is absorbed with low efficiency in the neonatal period, although subsequently its utilization gradually improves.
Valproic acid preparations, when administered simultaneously, interact with phenytoin and phenobarbital, leading to an increase in their levels in the blood. With long-term administration of valproate, it is necessary to monitor the indicators of a general blood test, as well as examine the level of liver enzymes (ALT, AST) initially (in the first months of therapy) with a frequency of once every 2 weeks, then monthly (for 3 months), and subsequently - 1 once every 3-6 months.
Almost all currently known anticonvulsants, to a greater or lesser extent, have a so-called rickets effect, leading to the appearance or aggravation of manifestations of vitamin D deficiency rickets. In this regard, children of the first year of life receiving treatment with anticonvulsants should be provided with an adequate supply of vitamin D (D2 - ergocalciferol, or D3 - cholecalciferol), as well as calcium supplements.

Convulsions in young children.
The clinical manifestations of West and Lennox-Gastaut syndromes are described quite widely (see separate articles on our website). As already indicated, they can be observed both in the first 12 months of life and later, although they are more typical for young children.
Secondary generalized seizures. These include epilepsy with manifestations in the form of simple and/or complex partial seizures with secondary generalization, as well as simple partial seizures turning into complex partial seizures with subsequent secondary generalization.
Febrile seizures in young children occur with no less frequency than in the 1st year of life. The principles of approaches to their diagnosis and therapeutic tactics do not differ from those in children of the first year of life.

Seizures in children over 3 years of age
Primary generalized absence seizures– a type of seizure mainly found in children of this age group. Their identification and provision of adequate treatment is entirely within the competence of pediatric neurologists and epileptologists. Pediatricians and representatives of other pediatric specialties should not ignore identified episodes of short-term “switching off” of children (with a lack of response to treatment) or complaints about peculiar episodes of “thoughtfulness.”
Juvenile myoclonus epilepsy is a subtype of idiopathic generalized disease with manifestations of impulsive petit mal attacks. The appearance of seizures is typical after 8 years of age. A distinctive feature is the presence of myoclonus, the severity of which varies from minimal (regarded as “clumsiness”) to periodic falls. No disturbances of consciousness are noted. However, most of these patients have sporadic tonic-clonic seizures, with absence seizures occurring in approximately one third of children with this type of epilepsy.
Catamenial epilepsies.
A group of chronic paroxysmal conditions associated with the menstrual cycle. They can be cited as one example of age-dependent convulsive conditions in relation to female patients who have reached puberty.
Febrile seizures can also occur in children over 3 years of age (in preschool age), although during this period of life they occur with significantly less frequency. The presence of episodes of afebrile seizures (without fever) in this pathology indicates the development of symptomatic epilepsy, which must be treated in accordance with the principles formulated by the International League Against Epilepsy (ILAE).

Lennox-Gastaut syndrome. Diagnostic criteria. Treatment.

Lennox-Gasta syndrome is an epileptic encephalopathy of childhood, characterized by polymorphism of seizures, cognitive impairment, specific changes in the electroencephalogram and resistance to therapy.

types of seizures: paroxysms of falls, tonic seizures and atypical absence seizures. Consciousness can be maintained or switched off briefly. After the fall, no convulsions are observed, and the child gets up immediately. Frequent falls often lead to injuries. Attacks include sudden flexion of the neck and torso, raising the arms in a state of semiflexion or extension, straightening the legs, contraction of the facial muscles, rotational movements of the eyeballs, apnea, and facial flushing. They can occur both during the daytime and, especially often, at night.

In Lennox Gastaut syndrome, tonic, tonic-clonic, atonic, myoclonic seizures and absence seizures occur up to hundreds of times a day. Atonic attacks lead to numerous falls.

Even before the onset of the first attacks, children, as a rule, suffer from a lag in psychomotor development, which worsens with the onset of the disease.

Debut 2-12 years with 2 peaks at 3 and 9 years


Infantile spasms are a type of convulsive processes in the muscle tissue of the upper and lower extremities, in the body and in the neck. Spasmodic convulsions occur in infancy when the child’s neuropsychological development is impaired. Any abnormal processes or pathologies of the brain can lead to infantile spasms in children born to healthy parents.

Infantile spasms are a type of non-inflammatory disorder of normal brain function characterized by sudden contractions of muscle tissue in the upper body. Such spasms are classified as epileptic encephalopathy. Their peculiarity is that they are extremely short in duration, but the number of involuntary muscle contractions varies from ten to two hundred spasms for one seizure. The number of seizures can reach up to ten per day.

Such spasms in children begin with changes in behavior: the activity of movements decreases, the humming stops. A spasm develops, affecting either one side of the torso or the entire body, the head deviates, the eyes move to the side.

There are no age criteria for the disease. Seizures can begin immediately after the birth of a child and can appear in adulthood.

Like any typical type of epileptic seizure, spasms are divided into types:

  • Flexor type - intense spasm of the flexor muscles of the body, arms and legs;
  • Extensor type - increased spasm of muscle tissue responsible for the extensor function, characterized by simultaneous movement of the upper and lower extremities.
  • Flexor-extensor type - a mixed type of spasm of muscle tissue with alternating extension and flexion of body parts;
  • Asymmetrical spasms - spasms of muscle tissue on one side of the body. It typically appears in severe pathologies.

Depending on the reasons that led to the occurrence and the type of attack, spasms are grouped into:

  • Symptomatic - these are spasms with an established etiology, characterized by deviations in mental and nervous development after the manifestation of spasms, neurological disorders, when examined, pathological abnormalities in the structure of the brain are clearly visible;
  • Cryptogenic are spasms of unknown etiology; the child has normal psychoneurological development before the onset of the disease; a certain type of convulsive processes is characteristic. No focal lesions are detected;
  • Idiopathic are seizures that begin in the neonatal period and in childhood. They have a benign course, the rhythm of transmission of nerve impulses is not impaired, is characterized by the absence of neurological changes and normal mental development during the course of the disease.

Causes

Depending on the period of time during which infantile spasms occur, they have:

  • Prenatal causes, which include infections and inflammations occurring in utero, congenital pathologies and defects of the central nervous system, genetic and chromosomal abnormalities;
  • Perinatal etiologies - cerebral hypoxia with ischemic lesions, complicated labor;
  • Postnatal root causes are infectious diseases of the central nervous system, injuries of the spine and head of various types, oncological diseases of the brain, ischemic strokes.

Infantile spasms have a major difference from other epileptic seizures. The spasm is sudden and short in time, begins with unreasonable child crying or a complete lack of active activity: movements, speech. Before an attack occurs, children are irritable.

Video