West syndrome (infantile spasms). What is this West Syndrome: Causes of Diseases, Symptoms and Signs, Methods for Treating Disease Infantile Spasms in Children Treatment

06.06.2020

Clinical and electroencephalographic characteristics epileptic attacks Children vary depending on age. Visual example Such eye-dependent phenomena are infantile spasms - a unique type of attacks associated with early childhood. Infantile spasms are a specific age phenomenon that occurs in children only in the first two years of life is most common - in the interval of 4 to 6 months, and approximately 90% of patients - up to 12 months. The incidence of Vesta syndrome is estimated as 0.4 per 1000 children born alive.

Definition

The characteristic features of this syndrome include myoclonic attacks, drying gypsum on EEG and a retreat of psychomotor development. This triad is sometimes referred to as Vesta syndrome. However, infantile spasms do not clearly comply with this definition. Other names of the disease are found in the literature: massive spasms, salaamos convulsions, flexor spasms, attacks by the type of "folding knife", massive myoclonic seizures, infantile myoclonic convulsions.

Typically, infantile spasms have a stereotypical nature of one child. In addition, the occurrence of infantile spasms in the form of a series is characterized.

Despite the fact that these attacks resemble myoclonic or tonic convulsions, infantile spasms are a separate type of attacks. Mioclonic twitching is fast lightning reduction limited duration, while tonic convulsions are a long muscular reduction of increasing intensity. True spasm includes a characteristic muscular reduction, continuing 1-2 C and a maximum of a peak slower than myoclonies, but faster than tonic convulsions.

Infantile spasms are divided into three types: flexor, extension and mixed flexor-extensor. Flexor spasms are short-term contraction of the muscle flexor torso, neck, upper and lower extremities. Spasms in muscles upper limbs They cause bringing hands, "as if the child hugs himself with his hands" or, on the contrary, breeding hands bent in the elbow joints, in different directions. Extensor spasms include, predominantly extensitive muscle contraction, causing a rapid sharp extension of the neck and torso in combination with extension and assignment or leading hands, yoga or upper and lower limbs at the same time. Mixed flexor-extensor spasms include flexion of the neck, body and upper limbs and extension of the lower extremities or bending the lower extremities and the extension of the hands in combination with a varying degree of flexion of the neck and the torso. Sometimes asymmetric spasms resembling a "fencer posture" are developing. Infantile spasms are often combined with the deviation of the eyes or the appearance of Nistagma.

Asymmetric spasms may occur if there is no simultaneous bilateral reduction in the muscles of the limbs. This type of spasms is usually found in the symptomatic form of infantile spasms in babies with severe brain damage, the agent of the corn body or a combination of these violations. Such local neurological symptomsAs the deviation of the eyes or turn the head, can be combined with both symmetrical and asymmetric spasms. Asymmetric spasms usually occur isolated, but they can also develop after a focal attack or precede it; In some cases, infantile spasms may occur simultaneously with a generalized or focal attack.

Infantile spasms often occur in the form of a series ("cluster spasms"). The intensity and frequency of spasms in each series can enhance, reaching the peak, and then it is progressively decreased. The number of attacks in the series varies significantly, and may exceed 30 spasms. The number of episodes per day also varies; Some patients have up to 20 per day. A series of infantile spasms can develop at night, although they are rarely observed during sleep. During or after a series of infantile spasms, as a rule, there is a crying or irritability of the child.

Gypsarithmia in a child with infantile spasms. High-amplitude disorganized activity in the background entry, intermitted with multifocal spikes and sharp waves

The chaotic character of the EEG creates the impression of the complete disorganization of the cortical rhythm. During sleep, there are discharges of polyspars and slow waves. It is surprisingly saving in some patients with sleepy spindles in combination with expressed anomalies of the EEG background entry. In the phase of fast sleep, it is possible to reduce the severity of plastering or its complete disappearance. Infantile spasms are associated with a decrease in the overall duration of sleep and the duration of fast sleep. Different varieties of dryritemia are described, including patterns with inter impassary synchronization, resistant focus of abnormal discharges, amplitude reduction episodes, and high-amplitude slow-wave activity in combination with separate sharp waves and spikes. Various plaster pattern patterns are often not correlated with the forecast of the disease.

Although hypsitemy or modified drying pattern is the most common types of intercreant pathological activity on EEG, in some patients with infantile spasms, these EEG patterns may be absent. In some cases, there may be lack of plastering at the beginning of the disease and the appearance of this pattern later, in the process of developing the disease. Although the plaster is associated mainly with infantile spasms, this pattern occurs in other diseases.

Like an intercotal pattern, ICTAL changes on EEG with infantile spasms are also variable. The most characteristic EEG pattern during an attack consists of positive waves in the Vertex-Central region; There may also be a low-amplitude fast (with a frequency of 14-16 Hz) activity or diffuse cladding of the curve, called "Electro-Decremental Event".

The presence of focal anomalies is one of the variants of the main pattern of plaster, which can be associated with focal attacks, focal attacks may precede a series of infantile spasms, accompany it or develop in combination with it. This observation suggests that the cortical "pacemeters" can play an important role in the development of infantile spasms.

The electroencephalographic picture in this disease is inconsistent and can undergo evolution in time. Some patients with infantile spasms in the debut of diseases may be absent. In other patients, there may be a slowdown in bioelectric activity in combination with rare epileptiform activity, in the future there is a transformation into a pattern of plastering. The re-conducting of the electroencephalographic study in the process of further observation may be necessary in order to demonstrate a pattern of plaster (in those children who have no plastering at the beginning of the disease).

Savoruds for hypoxia and intracranial labor injury. Most often they are found in newborns. Hypoxia, accompanied, as a rule, with a violation of cerebral hemo- and liquorodynamics, leads to a common or local brain echo, acidosis, diapell hemorrhage.

Convulsions in such children Appear immediately after birth or on the 2-3rd day, with subarachnoid hemorrhage, they occur most often after applying to the chest. Cauls are developing against the background of neurological violations: anxiety, sleep disorders, an increase in muscle tone and tendon reflexes, oppression of unconditional reflexes, difficulties of sucking and swallowing, pares of cranknot nerves. They most often wear clonic character, begin with the muscles of the face and then distributed to the limbs. The current seizure is different. They can fully relieve in the maternity hospital or again occur in a few months. Sometimes, starting in the maternity hospital, they are periodically repeated.

Cramps for developmental anomalies nervous system . Microcephalus, hydrocephalus, porencephalia, atrophy of the cerebral cortex, cerebellum hypoplasia can be accompanied during the newborn period by convulsions. Development defects are often combined with intrauterine hypoxia, asphyxia in childbirth and intracranial generic injury. The convulsions are tonic-clonic character and arise against the background of severe focal changes in the nervous system (paresa, paralysis, sharp depression of unconditional reflexes, dysfunction). Neurorentgenic studies confirm the diagnosis.

Suggesogues in infectious diseases. During the newborn, the convulsions are most often observed during sepsis. They also take place in 30-50% of newborns with meningitis and usually arise in pronounced changes in the spinal fluid. Cramps begin with a twitching of the eyes, the muscles of the face, and then, as the gravity increases, the states are moving to generalized. Facial attacks in meningitis are accompanied by an increase in temperature, inflammatory blood changes and liquor.

Early children's cramps

After period newborn Motor component The seizure becomes more pronounced. However, in infants, the classical sequence of convulsive seizures as well as newborns is rarely observed. Psychomotor automates are observed less often than other forms of seizures and at an early age, their diagnosis is difficult.

Mioclonic type of convulsive seizures (Small passive seizures or infantile spasms) are observed mainly in infants. The frequency of permanent seizures 1: 4000-6000 newborns, among children of the 1st year of life with convulsive syndrome, they make up 30.8%. For this type of convulsion characteristic: lightning fuses; delay mental Development; Specific changes to EEG. The classic picture of infantile spasms is characterized by a bilateral symmetric muscle contraction. Spasms are a flexor, extensor or mixed type.

With flexor spasme There is a sudden flexion of the neck, torso and limbs with simultaneous assignment or bringing the latter. The mixed type is characterized by a bending or extension of the body, hands and legs are inflicted. Flexor spasm meets most often, more rarely observed mixed and even less often extensive. At the same child at the same time can be various forms spasms. The infantile spasms also include partial fragmentary forms - rocks, shuddering, bending and extension of hands and legs, turning the head. At the same time, lateralization is possible - preferential contraction of the muscles of one side of the body. Rodes look like a quick tilt head forward. They are often combined with shudding and precede a flexor or extensor spasm or replace it. The most characteristic feature of infantile spasms is a tendency to seriality. Some spasms are less likely. The duration of convulsion from a split second to several seconds. The duration of the seizures series can be from a few seconds to 20 minutes and more. During the day, the number of paroxysms ranges from one to several hundred and even thousands. Shutting down consciousness in this form of seizure short-term.

Infantile spa Sometimes they are accompanied by a cry, a smile grimace, a frightened facial expression, surgery, nystagm, the expansion of pupils, eyelid shake, limbs, pallorium or redness of the face, stopping the breath. After convulsion, drowsiness is observed, especially if the series of attacks was long. In the interconception period, children are irritable, plaxive, disturbed sleep. Causes occur most often before falling asleep or after waking up. Factors provoking paroxysms include fright, various manipulations, feeding.

Infantile spa Begin at heart age and disappear in early childhood. Under the age of 6 months they make up 67% of the total number of paroxysmal episodes; from 6 months to 1 year - 86%; After 2 years - 6%.

Infantile spa There may be the first manifestation of convulsive syndrome in a child. The first seizures are an abortive character and can be taken by parents for the fright reaction, the manifestation of pain in the stomach, etc. First, they are single, then the frequency is growing. At this stage can be remission and exacerbations that are difficult to foresee. As the child grows and the development of the child, the frequency of convulsion decreases. The average duration of infantile spasms - from 4 to 30-35 months. After 3 years, they are rare. According to P. Jeavons, etc. (1973), in 25% of children, infantile spasms are terminated under the age of 1 year, in 50% - up to 2 years, in the rest - up to 3-4, sometimes 5 years.

Changes on EEG (Hypsarithmia) do not always correlate with the start of convulsion, sometimes they appear somewhat later. Gypsumithmia is characteristic of developing brain and is observed only with infantile spasms in children early age.

Universal accompanying sign of infantile spasms There is a delay in mental development, which is observed in 75-93% of patients, the formation of motor skills is also violated. Therefore, in early age children, it is more correct to talk about the delay in psychomotor development, which is celebrated in initial stage Diseases. It becomes more distinct when a series of seizures appear. The degree of delay depends on the start of the start of convulsion and from the premorbid characteristics of the child. Normal psychomotor development before the appearance of convulsion is observed in 10-16% of children.

Focal neurological violations (Pares, paralysis, squint, nistagm) are found in 34-70% of cases. As a rule, they are observed in children with cerebral paralysis, microcephalus, anomalies for the development of the central nervous system.

Forecast with infantile spasms Favorable in children with normal psychomotor development, with short-term cramps that are uncomplicated by other forms of seizures. With early start, seriality and duration, combination with other types of seizures, the presence of neurological and mental violations There is a deep delay in psychomotor development.

Absans - The form of small seizures, which are also observed in young children and are characterized by a short-term stopping of the gaze. Sometimes at this time the child produces sucking, chewing movements, combustion, licking by the language. The attack may be accompanied by redness or pale face, easy assignment eye apples. They are observed less frequently than passive seizures.

Big convulsive attacks in children An early age is more often an abortive character. In the structure, the seal predominates the tonic component. When turning the head to the side of the children of the breast is often fixed in an asymmetric pose. The attacks may be accompanied by a temperature lift, vomiting, stomach pains and other vegetative symptoms. Incoming urination is observed less frequently than in older children. After the seizure, the child is sluggish, stunned, falls asleep or on the contrary is excited, muscular hypotension is expressed.

Partial cramps in children An early age is manifested by clonic twitching of mimic muscles, muscles of the language, distal limbs. The seizure, starting locally, can go to generalized. Often at this age, adverscent convulsive seizures are observed, accompanied by turning the head and eyes, and sometimes the torso, aside. The seizure is often accompanied by a tonic tension of his hand and legs on the side of the head of the head.

Vesta syndrome refers to the class of encephalopathic epileps, when attacks appear against the background of non-inflammatory brain diseases.

Under epilepsy understand progressive chronic diseasewhich is manifested by the attacks of frozen or convulsion, paroxism (paroxysmal) disorders of consciousness, vegetative paroxysms (changes in the tone of vessels, pulse, respiratory, etc.).

As well as increasing changes in the mental and emotional sphere, noted in the intergovernmental period. During epilepsy, certain changes appear on the electroencephalogram (EEG) - the graphic indicator of the electrical work of the brain.

West syndrome, as a rule, meets in infant age and has many reasons:

Asphyxia.
Metabolism disorders, gene mutations and genetic diseases.
Congenital brain disease (for example, tuberous sclerosis).
Hypoxia of the brain, generic intracranial bleeding (especially in premature children).
Brain infection.

Wherein asphyxia fruit It is the most common in the development of this disease. As a rule, it appears as a result of complicated labor.

Symptoms and signs of the disease

West syndrome is observed in 1,7-4.3 per 10 thousand infants and is 3-10% of the general cases of epilepsy. Most of all the boys suffer (65%). In 80% of cases, additional diseases are established:

Thus, the most frequent form of the disease is symptomatic, that is, which accompanies another disease. Cryptogenic syndrome - the cause of the state is not obvious or unknown - is determined at 10-14%. Cases of genetic susceptibility are 2-5%.

Among the symptoms of West syndrome, you need to highlight the following:

Explicitly pronounced violations in psychomotor development.
Characteristic for Vesta Syndrome Changes in EEG (Hypsarithmia).
Frequent epileptic seizures.

Almost all kids with Vesta syndrome (95%) first signs appear almost immediately after birth (within 3-7 months). The seizures are characterized small durationTherefore, it is not necessary to determine the diagnosis correctly.

West syndrome is a heavy disease that threatens the life of a child. And also this disease is called infaltile spasms. The attack goes all the time equally: the head is very bent, and the whole body of the child sharply leans forward.

These cramps, as a rule, appear during falling asleep or at the time of the awakening of the baby. And the attacks may be over the day more than 50.

Often in the process of attacks the kid loses consciousness. Because of them, the child begins the lag in the development of psychomotoric. Patients with Vest Syndrome almost do not come to contact with relatives, react poorly to the environment.

Primary symptoms of pathology

A strong crying of a child is the first symptom of the attack of Vesta syndrome, because doctors often put these children diagnosed colic. Standard symptoms of this disease are:

The limbs involuntarily diverge on the parties.
Generalized cramps in the whole torso, in the upper and lower limbs.
Sharp slopes of the whole body forward.

As a rule, this attack lasts no more than a few seconds. There comes a small pause, and the attack is re-repeated. Sometimes spasms are part of one, but usually they happen a turn.

Kids with Vest Syndrome often very irritable, lag in mental development. Newborn with such a disease most often behave like blind.

Epilepsy attacks

West syndrome is one of the varieties of catastrophic generalized epilepsy. It can manifest as symptomatic (most often) and cryptogenically (approximately 12% of the total number of cases).

The classic version of this disease can be described pronounced myoclonic or salaam spasms. In some cases, spasms have the form of serial short head slopes.

In the wizest syndrome, epilepsy appears without certain disorders in the functioning of the central nervous system or due to different neurological pathologies.

Infantile spasms lead to the inhibited development of motor and mental functions in children, which may later be a pronounced pronounced lag at the child in development. In 85% of cases, the baby with Vestin syndrome develops attactic and atonic violations, microcephaly, children's cerebral paralysis.

Encephalopathy

As already mentioned above, Vesta syndrome is also also referred to as Mioclonic encephalopathy. Gypsarithmia is a typical, but non-pathognomonic pathne encephalogram in children with this disease.

Typical hypsarithmia is distinguished by a constant high amplitude and arrhythmic slow-wave activity, and also has multiple sharp waves and spikes. And between different departments, the gemichepher does not have some kind of synchronization. In some cases, patterns are distinguished by amplitude asymmetry.

Most often, in 80% of cases, Vesta syndrome begins on 2-3 quarters of the child's life. At first, the development of the baby seems normal, and only then convulsions are manifested, which are a primary pathogenous symptom.

In some cases, children are celebrated psychomotor Development Delay. Not often, but you can observe changes in EEG.

Muscular cramps or myoclonies affect almost the whole body. With these convulsions limbs and body baby bend. Abbreviations and spasms in flexing muscles can be synchronous, bilateral, symmetrical, sudden, and no more than 7-9 seconds continue.

In certain cases convulsional syndrome It affects only some one muscular group. The upper and lower limbs during spasms scatches involuntarily to the side, the head bends into the chest. When the frequency of cramps is high, then the baby can fall into sleep.

Today there are three separate types of welf syndrome, characterized by the character and the degree of muscle damage:

Common spasms-spasms throughout the body. The limbs are bred to the parties, and the head "falls" to the chest.
Growing spasms - head tilting back. Spasms continue up to 10 seconds with a break in one second.
Quay - multiple bending spasms (especially on the head and upper limbs).

Kids with Vest's syndrome immediately after birth or for six months appear mental and Motor Development Loge. Frequent spasms only aggravate the position.

Cerebellum syndrome

Sometimes, with Vest syndrome, cerebellar syndrome appears. This is the pathology of the cerebellum or a violation of its connection with the rest of the brain departments. Distinctive symptoms of ceremony syndrome:

Consequences and complications

Almost in all cases, the passage of Vesta syndrome is very heavy, since it is manifested by significant brain disorders. Pretty rarely this disease passes with conservative treatment. But usually, even after effective treatment After a certain time, recurrences are manifested.

Almost always after recovering the child, he happened in the subsequently difficult and serious residual neurological phenomena: Extrapyramidal manifestations, epilepsy and its equivalents. Also, these patients appear in psyche violations: light lungs or idiocy. Only in 3% of the case (according to GibbS) there is an unexpected complete cure.

Diagnosis of the disease

Vesta syndrome diagnosis is performed using such doctors: epileptologist, neurosurgeon, pediatrician, neurologist, endocrinologist, endoscopist and immunologist. Due to the use of modern devices, you can set the most accurate diagnosis.

Most often applies: cerebriangiography, Craneoscopy. (In fairly rare situations), computed tomography and radio magnetic. And also to determine the pathological focus of epilepsy seizures, neurophysiological studies are performed.

The most common ways to diagnose Vesta syndrome are considered: gas encephalography and electroencephalography. Thanks to the electroencephalography, it is possible to identify biological curves:

Low efficiency light stimulation.
The synchronization of the main curves is not defined. "Curve peaks" may appear in the process of wakefulness or sleep.
The amplitude of biological curves is irregular.

Sometimes with the help of gas encephalography, it is possible to observe an increase in brain ventricles. In the last stages of West syndrome, hydrocephalus is observed.

Differential diagnosis. Differentiate this disease can be both with non-epileptic diseases that are frequent in newborns (motor anxiety, colic, respiratory attack, hyper-exploitation, infantile masturbation) and with defined epileptic symptoms (for example, focal epilepsy). An important role in differential diagnostics is played by electroencephalography.

Treatment of Vesta syndrome

In each case, the treatment is prescribed individual and depends on the cause that caused the pathological states of brain development and Vesta syndrome. To date, the main method of treatment of West syndrome is steroid treatment with adrenocorticotropic hormone (ACTH) (Vigabatrin, Sabril).

However, this treatment is obliged to be very careful and passing under clear control of the doctorbecause vigabtrin and steroid drugs have a large number of serious side phenomena. And also need to choose suitable anticonvulsants, as well as medicinal productswhich will allow normalize blood circulation in the brain.

In some cases, the neurosurgeon is obliged to perform the operation in the process of which spikes are cut brain shellAnd the pathological center is cleaned. Such an operation occurs with stereotactic surgery and various endoscopic methods.

The modern and pretty expensive way to treat this disease is use of stem cells. This method is quite effective, however, it is unpopulated due to the high cost of the operation. The meaning of this method is that the damaged section of the brain is restored by stem cells.

As a rule, idiopathic variety of Vesta syndrome is treated with special preparations:

Vitamins - For example, Vitamin B6 (pyridoxin).
Hormonal steroids medicinal preparations - For example, tetracoactide, prednisone, hydrocortisone.
Anticonvulsants - For example, Nitrazempam, Epilim, Topamax (Topiramat), Depakote (Valproat), Klonopin (clonazepam), Onfi (hlobazam) or ZoneGran (Zonisamide).

Treatment is considered effective in the case when the frequency and number of seizures is reduced.. With a competently chosen treatment, the child will continue to be normally learning and developed. But it is necessary to realize that even modern medical medicines carry a large number of adverse events:

fatigue;
reduced concentration;
depression;
allergic skin reactions;
liver failure;
defeat nervous system.

Medical Physical Culture and Prevention

Therapeutic gymnastics in Vesta syndrome is obliged to be carried out under the clear control of the doctor of sports medicine and rehabilitol, so that the seizures are not aggravated.

This method of treatment is pretty commonHowever, does not show effective results without a complex of drugs.

With Vesta Syndrome, the lack of spasm has no time to indicate that the disease has moved to the step of remission. However, many doctors argue that if convulsions, spasms, changes in EEG and gypsumithmia were not marked for a month, then this is recovery.

Alas, these cases are very rare. According to certain data, only 9% of all people are fully cured, this amount is only 3% of Gibbs.

The main preventive measure of Vesta syndrome is timely diagnosis and competent treatment. Epileptic attacks that are the main symptom of this disease must be stabilized.

Diagnostics

Diagnostic measures for Vesta syndrome begin with learning clinical picture Diseases and examination of the patient. Infant attacks are difficult to diagnose. Cramps are difficult to distinguish from random movements with heads and limbs. Experts take into account the duration of spasms and their effect on sleep state. Pediatricians, neuropathologists, genetics, immunologists are engaged in diagnosis and treatment.

Tool methods for the study of patients:

Classification of infantile spasms.

Like any typical species epileptic seizuresSpasms are divided into types:

Flexo species - intensive spasms of bending muscles of the body, hands and feet.
Extensor view - enhanced muscle tissue spasms, which is responsible for the extension function, characterized by simultaneous extension of the body, neck, upper and lower extremities.
Flexor-extensional view - a mixed type of muscle spasm with alternating extension and bending of body departments.
Asymmetric muscle spasms of one side of the body. Characterized by the appearance of children's brain patologies in severe pathologies.

Depending on the reasons resulting in the appearance and type of attack, spasms are grouped on:

SymptomaticThese spasms with installed etiology are characterized by the deviation of mental and nervous development after the manifestation of spasms, disorders of a neurological nature, the study clearly visible pathological anomalies in the structure of the brain.

CryptogenicThese spasms are not established by etiology, the child has normal psycho-neurological development before the disease, a certain type of convulsive processes is characterized. When examining the brain of focal lesions is not detected.

Idiopathicare convulsive attackswhich begin in the neonatal period and in childhood. Have a benign flow, the rhythm of the transfer of nerve impulses is not violated, is characterized by the absence of neurological changes and normal mental development in the course of the disease. Sometimes they look focal, after a few seconds it seems that they proceed from many foci.

At times, the discharges in the form of spikes are generalized, but never look like a rhythmically repeated and highly organized pater. These changes are almost constant. Studies of the etiology of infantile spasms were started in the 50s of our century. As the facts accumulated, the polyethology of the syndrome has become apparent.

Infantile spasms were described with intrauterine infections, various brain digesions, under a number of chromosomal anomalies and hereditary diseases. Among the latter, in particular, almost all neuro skin syndromes, many metabolic diseases from frequently occurring phenylketonurium to organic acudurium, including extremely rare fumaric aciduria.

Serious perinatal brain damage can be given to the development of such symptomatic infantile spasms. It may be anoxia-ischemia, intravenricular and subarachnoid hemorrhages, neonatal hypoglycemia. Depending on the etiology, all infantile spasms are divided into cryptogenic and symptomatic.

The expediency of the separation of infantile spasms to cryptogenic and epileptic proceeds from the generalized experience by the characteristics of clinical manifestations and the flow of infantile spasms.

For cryptogenic infantile spasms are characteristic:

Lack of a clear etiological cause;
Normal neuropsychic development of the child until the disease is developed;
Lack of other types of seizures;
Lack of signs of brain damage according to the data of neuroradiological research methods (computer (CT) and nuclear magnetic resonance (NMR) tomography of the brain).

Symptomatic spasms are a consequence of various etiological factors. For symptomatic infantile spasms are characteristic:

Installed etiology;
Delayed neuropsychic development until the cause of the disease; neurological disorders;
Often - pathological changes in CT-and NMR studies of the brain.

All these etiological factors and, accordingly, symptomatic infantile spasms in terms of occurrence are conditionally divided into three large groups - prenatal, perinatal and postnatal. The cause of such infantile spasms may be any factor brain bark.

Most children often detect perinatal pathology of the CNS, brain injury, various chromosomal and gene anomalies. But it happens that both cryptogenic (in the absence of a clear etiological cause) are infantile seizures.

Key points

Infantile spasms are usually found in children under 1 year.
Infantile spasms are a rare form of epilepsy.
Early treatment can reduce the risk of complications.

Infantile spasms can be described as short, and sometimes fine attacks that arise from babies. These seizures are actually a rare form of epilepsy.

Only about 2,500 children will be diagnosed with a disease in the United States each year. These seizures or convulsions usually arise before the child turned 1 year old, most of the cases fall on children aged for about four months.

Symptoms

Cost of treatment in European clinics and Germany

A ten-day stay in the hospital German clinic and the treatment of Vesta syndrome will cost an average of 5 thousand euros. A two-week hospital in Israel and Germany costs about the same - about 7 thousand euros. The exact cost of treatment depends on the pricing policy of the clinic and can vary from 5 to 15 thousand euros.

Course of therapy

In such a pathological process, symptoms in time increases because of which the child can remain disabled for a lifetime or die. That is why in the syndrome of Vesta treatment it is important to spend as soon as possible. This can be done by medicines according to the type of adrenocorticotropic hormone and drugs based on valproic acid, for example, hipproat. If the ATG did not give the right effect, glucocorticosteroids (GKS) are used and dexametoson is most often applied from this group. Hormone treatment is necessarily combined with epilepsy drugs for better effect.

According to specialists, the course of therapy will also need to include such medicines:

Benzodiazepine (clonazepam);
Vitamin B6;
Immunoglobulin (octagam);
Vigabatrin.

The course of treatment should be carried out under the strict control of the doctor, and in hormonal therapy, the child is put on the hospital. Periodically, it will be necessary to do EEG to monitor the effectiveness of the medications taken. Experts recommend to check that there are no drugs with drugs with an exciting effect on the nervous system. It is advisable to introduce drugs to the course of treatment to improve metabolic processes, as they reduce the frequency of attacks and stimulate mental development.

If there is a neoplasm in the brain, provoking the deterioration of the disease of the disease, then a surgical intervention may be required. This method uses in extreme cases, as it carries a big risk to the child's life. Brain fabrics at such age regenerate significantly better than adult people, but the operation itself is quite heavy.

Doctors strongly recommend parents to resemble the doctor's advice about the care of patients with children. After all, even if you stop this disease, then your consequences will still be left and there is a delay in psychomotoric, which is essential is extremely difficult.

Cause of infantile spasm

Depending on the etiology of spasms there are symptomatic and cryptogenic. The reason for their occurrence may be the following:

damage or immaturity of the cortex of the brain;
chromosomal and gene anomalies;
neurological and psychophysical disorders;
disorders during the period of intrauterine development (hypoxia, premature delivery);
infectious diseases during pregnancy (bacterial or herpes viral meningitis);
down syndrome;
tuberculous sclerotic syndrome;
complications during childbirth

In some rare cases, the reason may be in vaccination of the vaccination of ADC.

Types of infantile spasms

Infantile spasms are three species. Estenorous, flexor-extensor or flexor. These are sudden contractions of the muscles of the head, limbs, body and neck. Flexor spasms are called still convulsions by the type of folding knife or salyam convulsions. It looks like a person hugs himself. Extensorial spasms suddenly extend the neck, lower limbs and torso in combination with the extension of the shoulders. As a result of flexor-extensor spasms, mixed action. Cryptogenic infantile spasms are found in 9-15% of cases, the rest are symptomatic.

The main symptoms of violations

The manifestation of West syndrome in children are clearly pronounced myoclonic or salaam (flexor) convulsions. Sometimes the disease is accompanied by spasms in the form of monotonous nods of the head. Signs differ in the nature of the manifestation of pathology.

Epilepsy in West Syndrome

This is one of the main satellites of the disease, which is in infancy manifests itself:

convulsions;
hyperthermia;
lack of reaction to external stimuli;
fixed pose, a fixed look.

Epilepsy can be short-lived or long. A large seizure is preceded by a sleep disorder, loss of appetite. The generalized attack is accompanied by:

partial stopping of the respiratory function, cutting muscle;
the color of the nasosal triangle in the blue color;
convulsions whose duration is up to several minutes;
spontaneous defecation and urination;
vomot, foam at the mouth.

During this period, it is necessary to protect the child from strikes on solid items, thus eliminating injury. Small manifestations of short time, can occur several times a day, accompanied by intermittent convulsions, for example, body bending.

Encephalopathy

Pathology unites a group of diseases caused by a violation of the brain's work, which is based on unrealistic etiology. During the process, neurons die and synaps (communication) between them is disturbed. Encephalopathy depresses the central nervous system, which leads to the braking of the development and mental abilities of the child. The disease is characterized by the following symptomatics:

anxiety, headache, irritability, plastic;
sleep impairment, inversion is manifested by insomnia at night and lethargy in a bright time of day;
deterioration or complete absence of appetite;
loss of short-term memory;
attairs of panic (fear of insects, closed space, fear remain alone).

In children, under 3 months of life, the development of hydrocephalus, an increase in the brain and the size of the head due to poor blood outflows is possible.

Symptomatic syndrome Vesta

The main feature of myoclonic encephalopathy is spasms. They can be classified by nature:

Passionable - the reason for the appearance of generic or intrauterine injury. There are unforeseen several times a day. At the time of the attack, the child's body begins to bend. This form is characterized by the delay in motility and mental development.
Impulsive convulsions are manifested by a reduction in all types of muscles, the child remains in consciousness, the hands are divorced to the side, the cams are compressed. Then the forelimbs are reduced around the body, as if an embrace.
Retropulus - accompanied by fainting, the eyeballs of the patient rolled out, the body freezes in one position, the muscles are in the limit tone. The head throws back, the shutter cramps visually observed.
Psychomotor - this rare form is characterized by automatic performing certain actions (laughter, crying, vomiting, the same movement, for example, head slope).

The disease belongs to heavy, rushes a threat to life. Manifests immediate after birth. Affects psychomotor development.

Cerebellum syndrome

Pathology arises against the background of the loss of communication between the brain departments. Accompanied by symptoms:

Diatochokinesis, in which complex movements are impossible, their sequence is broken, the actions are performed slowed down or, on the contrary, chaotically (one, quick transition to another).
Hand trimmer.
Muscles are sluggish, relaxed.
Often repeated bouts of dizziness.

The cerebellar syndrome at myoclonic encephalopathy is well characterized by the symptom of the opposite push.

Forms

Officially sv are divided into symptomatic (up to 85%), as well as cryptogenic and idiopathic forms (together to 20%). But from a clinical point of view, there are only 2 forms in a disease, since the differences between cryptogenic and idiopathic forms are practically absent. The symptomatic form of Vest's syndrome includes cases of the disease against the background of the already existing brain pathology or developmental disorders. Half children with a symptomatic form in history had a complicated course of the intrauterine period: infections, metabolic disorders, genetic and chromosomal defects (Down syndrome, etc.), as well as a violation of intrauterine blood circulation from the mother. Less often observes the pathology of the generic period. This is a hypoxic-ischemic brain lesion, injury and other complications in childbirth. For postnatal reasons, sv are infections, injuries, hypoxic-ischemic strokes and tumors.

Cryptogenic, or idiopathic, the form of the disease is diagnosed in children with epilepsy Vesta syndrome without visible reasons, with normal psychomotor development and without damage to the brain before the disease occurs. This is a more favorable form of St.

The pathogenesis of Vesta syndrome is currently unknown. Patients are shortened by the REM-sleep phase (the phase of the rapid movement of the eyes), during which the EEG is normalized and reduced the frequency of spasms. In this regard, there is a version that when the brain trunk has dysfunction of serotonergic neurons involved in the formation of sleep cycles. There are other hypotheses that imply genetic and immune disorders in small patients.

Forecast and possible complications

The disease is considered healing at an early stage of development and timely detection. Proper therapy gives sustainable result And does not affect the quality of the child's life. The cunningness of Vesta syndrome is that it is very difficult to diagnose in the first months after birth. Therefore, each 6 patient is dying up to 4 years without proper treatment.

At the main part of children, remission is maintained by medicine preparations. They have a greater or lesser extent, there are deviations in psychomotor development. Even if you managed to stop convulsions, such babies still intelligently lagging behind their peers. Elimination of symptoms of the disease does not eliminate it. In the future, the development of epilepsy is possible with the transition in Lennox-Gasto syndrome, which manifests itself all his life.

Idiopathic form is completely treatment: 60% of children after therapy are normally developing and no different from their peers.

Clinical picture

Most often, the syndrome debuts in children aged 4-6 months, and an earlier symptomic prognostic factor. Vestin Syndrome Infantyl Spasms can manifest themselves with high frequency and be extremely varied - flexion torso, vertical movements Eye apples, or eye movement, similar to Nistagm, as well as "Walking" the handles in the type of Eastern greeting, etc. One spasm lasts a split second, spasms are grouped in the series - up to 50 attacks in the series, the number of series in days - from one to several dozens. Often attacks develop when waking up and falling asleep, may be accompanied by a head or eye assignment. Only half of the body can be involved in spasm. The appearance of epilepsy attacks means stopping the psychomotor development of the baby, and often the regression of the acquired skills. In 1-2% of cases, a spontaneous self-appearance is possible.

Diagnostic procedures of infantile spasms.

Held clinical Evaluation development of the nervous system. Laboratory screening data for electrolyte, metabolic or other disorders is usually normal. In obscure cases to identify etiology, the liquor can be explored, carry out neurometabolic tests, chromosomal analysis. Computed tomography (CT) and mainly magnetic resonance tomography (MRI) are mandatory before the start of steroids.

Interictal EEG:

The classic epileptiform pattern of plaster is recorded in 2/3 patients. Asymmetric and modified gypsumithmia are found in 1/3 cases.

Ictal EEG:

Up to 11 different Ictal patterns can be registered with a duration of 0.5 seconds to 2 minutes. The most frequent pattern (72%) consists of a high-amplitude generalized slow wave, the episode of low-amplitude rapid activity.

How to diagnose infantile spasms

If the doctor suspects infantile spasms, it will order the electroencephalogram (EEG), which is easy to obtain and usually diagnose. If this test is inconclusive, they can order a test called video electo-dealephalogram (video EEG). With this test, like with ordinary EEG, the electrodes are placed on the skull of the child to help doctors visualize brain waves. The video records the child's behavior. Doctor, usually pediatric neurologistwill observe the activity of brain waves during and between spasms.

These tests usually last from one to several hours, and it can be done in the doctor's office, a laboratory or hospital. They can also be repeated in a few days. Most children with infantile spasms will have disorganized activity of the brainwave. This is known as modified hyperships. A very chaotic brainwave activity for a softer response known as plastering is observed in about two thirds of children with disorder.

If your child is diagnosed with infantile spasms, their doctor can also order other tests to find out why spasms arise. For example, MRI can display the brain and show any anomalies in its structure. Genetic testing can reveal genetic causes that contribute to the sequels.

It is important that you immediately applied for medical care if you think that your child has children's spasms. Disorder can have very serious consequences for development, especially if it is not treated. Your child has every chance to limit these negative consequences with early interference.

In a recent study, presented at the annual meeting of the American Society of Epilepsy, almost half of children with disorder were not properly diagnosed during the month or more, and some were not undiagnosed for many years. It is important to be aggressive in finding answers.

Complications

Diagnosis of infantile spasms in children

Neurovalization.
Video Zagsna and wakefulness.
Laboratory studies on clinical indications.

The diagnosis is established on the basis of clinical symptoms and the characteristic EEG picture. Forecological and neurological examination is carried out, but often pathognomonic symptoms are not detected, the exception is tuberous sclerosis.

In EEG, in the intercreant period, as a rule, a picture of plastering (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal peak discharges is detected. Perhaps several variants (for example, modified - focal or asymmetric plastering). Ictal background EEG is changing, intercotal epileptiform activity is noticeably weakened.

Tests to determine the cause of infantile spasms may include:

laboratory studies (for example, a general blood test, the determination in blood serum glucose, electrolytes, urea, creatinine, Na, Ca, Mg, P, liver samples), if the metabolic disorder is suspected;
analysis of SMG;
brain scanning (MRI and CT).

Principle of treatment

The principles of treatment of Vesta syndrome differ in different clinics, but in general, the approach of German and Russian medicine is the same, so infantile spasms are often treated in Germany clinics. First of all, this is due to the diagnostic capabilities of the major European clinics.

The universal treatment scheme of syndrome in children does not exist. Regardless of the clinic in which the patient comes, therapy is selected taking into account the individual characteristics of the disease.

The forecast will depend on how the patient reacts to therapy. In the overwhelming majority of cases, conservative treatment is practiced.

Medical therapy

Pyridoxine injections are prescribed for prevention of vitamin B6 deficit

Major drugs in West Syndrome:

anticonvulsant drugs;
steroid hormones;
vitamins of group V.

In Vesta syndrome, simultaneous ACTH therapy (adrenocorticotropic hormone) and prednoxone is used. Such treatment has shown high efficiency, but long-term hormone therapy may adversely affect the growth and physical development of the child, as a result of which it is important to correctly compile a treatment regimen and choose dosage. This can only be done by an experienced neurologist.

Anticonvulsant medications are an important part of symptomatic therapy. In the extension syndrome, the same drugs are used as for epilepsy in children. It is used clonazepam, topiramate, epilim. Dosages are selected individually.

An important role in therapy is played by vitamins of group V. They are necessary for regulation nervous activity. Usually prescribed pyridoxine injections.

Vesta syndrome is also treated with the help of LFK and massage. These methods help reduce the severity of attacks, but apply only together with conservative therapy.

Surgery

In the presence of organic lesions of the brain, for example, tumor neoplasm, surgical intervention is carried out. It is associated with risks, as well as any interference with the work of the brain, but in some cases it is the only way to cope with the disease. The operation is carried out at benign tumors or sclerotic changes in brain tissues (Burneville's disease).

Homeopathy

Many prefer homeopathy for the treatment of childhood diseases, but Vesta syndrome must be treated conservative methods. The efficiency of homeopathy is insufficient, moreover, it is impossible to be exactly confident in the contents of such drugs. To date, there is no confirmed case of cure or improving the child's well-being with Vesta syndrome when taking homeopathic remedies. No need to deal with self-medication.

Causes of occurrence

Depending on whether infantile spasms appear at what period of time, they have:

Prenatal bases to which infections and inflammation are infected with intrauterine, congenital pathologies and defects of the central nervous system, genetic and chromosomal deviations;
Perinatal etiologies - the hypoxia of the brain under the lesions of ischemic nature, complicated generic activity;
Postnatal root causes - infectious diseases of the central nervous system, injury to the spine and head various character, oncological diseases of the brain, ischemic strokes.

Causes of disease

In the overwhelming majority of cases, West syndrome is symptomatic, and may be due to a rather large number of etiological factors, including hereditary.

The reasons for Vesta Syndrome The following:

postnatal encephalitis;
intrauterine infections;
hypoxia fetal;
premature childbirth;
intracranial injury of the fetus;
birth injury;
asphyxia of the newborn;
postnatal ischemia due to a crucial cord;
abnormalities of the brain structure;
septal dysplasia;
point genes.

Unfortunately, Vesta syndrome is characterized by a high percentage of fatal outcomes, but if you begin treatment in a timely manner, you can achieve a long-term stage of remission. At the same time, it should be noted that the development of such pathology leads to retribution in psychomotor development, and eliminate this factor is not fully submitted possible.

What to do?

If you think you have West Syndrome And characteristic of this disease symptoms, you can help doctors: pediatrician, neonatologist, neurologist.

Etiology and pathogenesis Vestus syndrome occurs with the following pathologies: intrauterine infection with cytomegalovirus or herpes virus,

The description of the ailment and the code on the ICD-10 This disease has the following code on the ICD-10: G40.2.

When a child begins to shake in convulsions, most parents are experiencing a real shock. They do not know where to run, and lose precious time. In Vesta syndrome, every week is important: the earlier the diagnosis will be carried out and the treatment has begun, the more the child has a chance of full recovery And a happy future.

What is West Syndrome

Vesta syndrome is a heavy form of epilepsy, developing in small children due to brain lesions and some other heavy diseases. The characteristic signs of this pathology are considered to be a lag in mental development, as well as infantile spasms - nodes or fast tons of body, as a rule, during a fallback or awakening. At the enencephalogram, the hypsarithmia is recorded - anomalous high-amplitude activity of the brain.

West syndrome suffer from 1 to 4 people out of every 10 thousand kids. This disease accounts for up to 9% of all epileptic seizures in children and 25% of infant epilepsy cases. The incidence is higher among boys: the cunning syndrome is affecting about 60% of the young representatives of the strong sex.

Disease has received its name in honor of the British Vesta doctor, which described the symptoms of pathology in 1841 on the basis of observations of his own son. Later, a lot of synonyms appeared in the disease: Weste's syndrome, Poklonny convulsions, spasm (TIK) of Salaama, Gibbs Hyppsure, Mioclonic encephalopathy with dryritemium, convulsive epilepsy with drying syndrome, flexosis syndrome. Initially, the syndrome was ranked by the varieties of generalized epilepsy, but later the doctors moved it into the category of epileptic encephalopalopaths, in which the attacks are provoked by non-inflammatory brain diseases.

As a rule, newborns, which subsequently raises such a diagnosis, are born outwardly healthy or with minor deviations. Disease debut accounts for 3-7 months of life: during this period, pathology is diagnosed in 77% of patients. In children over 1 year, the syndrome takes place only in 10% of cases.

Among the sick-sick wester syndrome is high, the percentage of mortality, and the surviving kids to the 3-year age convulsions are moving into another form of epilepsy, most often in Lennox-Gasto's syndrome. With adequate treatment, it is possible to achieve complete remission, but mental development delay is usually observed in patients for a long time.

Forms of the disease

There are 2 main forms of West syndrome.

Symptomatic - characterized by the presence of an explicit cause of pathological condition: brain damage, genetic factors. In this form, children have an initial delay in psychomotor development, the patient is tormented by several types of seizures, and structural changes are noted in its brain.
Symptomatic wizest syndrome is considered the most severe type of disease, and the forecast of treatment and life is often disappointing.
Cryptogenic (idiopathic) - differs from the symptomatic form visible reasons Sailor and diagnosed approximately 12% of patients. This species is characterized by only 1 type of convulsion, structural changes of the brain are not visualized, and development delay occurs only after the debit of the disease, when its manifestations become obvious. Cryptogenic variety has a relatively favorable forecast for life and full cure, flowing in a more easy form.

In veste syndrome, almost all muscles are covered with convulsions, including neck, head and limb. Reducing is usually symmetrical for the left and right side of the body, lasts up to 10 seconds and can be repeated many times during the day.

Sometimes spasms affect only one muscle group. Depending on the localization of their lesion, the following varieties of convulsion are distinguished during Vesta syndrome:

zatilicchny - extensible neck convulsions with heading of the head;
quay (bending) - Reducing the muscle-flexor muscles located on hand and neck: nodders, the connection of the hands in front of the breast, etc.;
common (extensible) - convulsions covering the whole body: arms and legs are scattered at the sides, resembling the manifestation of moro reflex.

If convulsions are repeated too often, the child can fall asleep immediately after their termination. Long-term convulsions strongly affect the development: the baby begins to lag behind the peers in the engine, mental and mental plans.

Causes of occurrence

Sophisticated infantile spasms characteristic of West syndrome arise due to improper interaction of the brain barrel and its bark. The immaturity of the central nervous system provokes disorders between the brain and adrenal glands, as a result of which the hypothalamus synthesizes too much corticoliberin. An excess of this hormone and causes muscle contractions typical of weta syndrome.

In 85-88% of cases, doctors manage to establish the cause of this disease. The following factors can provoke syndrome:

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

Often the impetus for the beginning of the disease is the reception of nootropics or vaccination, but these factors act as a catalyst, and not an independent reason. If the child's body functions incorrectly, Vesta syndrome will sooner or later manifest itself, anything can serve as a descent mechanism: from vaccinations to a stressful situation.

Symptoms and signs

The first sign of Vest's syndrome can be a loud loose-free crying of a child. Sectional doctors are written off all on intestinal colic. The correct diagnosis is made only when other alarming symptoms appear:

the lack of or slowing the pace of psychomotor development: the child does not turn over and does not sit down, does not stretch to toys, he disappears a grabbing reflex;
vision problems: the baby does not look into the eyes, does not fix the view on the items. Often parents seem to see not see anything at all;
muscle lethargy (hypotension);
floodiness and irritability;
appearance of spasms.

Causes are a typical manifestation of Vesta syndrome. Distinguish the following types of convulsions:

mioclonic - small symmetrical twist of the muscles of the body, limbs and persons taking place by short-term series;
tonic - continuous cuts of individual muscle groups: nodding, shrug shrug, mixing and breeding limbs, folding the body in half.

As a rule, the manifestations of Vesta syndrome begin with myoclonic convulsions, and over time they are transformed into tonic attacks. Most often, the seizures are noted during a fallback and awakening, but provoking factors can be loud sounds, fright, as well as light and tactile stimulation.

Cramps are characterized by some scholarship, following each other with an interval that does not exceed 1 minute. Sometimes spasms are manifested in the form of sudden stops or drops of a child, respiratory disorders, nystagma or chopping eyeballs. Before the attack, the baby can be afraid and shout, and after it - becomes sluggish and sleepy.

At the same time, plastering is observed on the electroencephalogram (EEG) - the absence of a normal rhythm of the electrical activity of the brain. In some cases, cerebellar syndrome may develop, which is characterized by the following violations:

trembling fingers;
muscle flabbiness;
dizziness;
inability to perform quick and complex movements;
the symptom of the absence of a "reverse push."

The symptom of the lack of "reverse push": the patient with force bends his hand in the elbow joint. The explore is trying to bleed it, which the patient has resistance, holding the hand in the bent position. Then the investigating suddenly stops the extension, and the patient's hand with power hits in the chest.

The sequence of the appearance of symptoms of West syndrome depends on its shape. Thus, with idiopathic species, psychomotor development delay is observed after a convulsive debut, and the symptomatic type of disease is characterized by the initial developmental delay, while infantile spasms and changes to EEG are registered later.

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

The first group includes children who are diagnosed with dryritemia, but there are no visible symptoms of the disease. Such kids must undergo an annual survey and do not need treatment.
The second risk group belongs to children with the main signs of West syndrome and characteristic changes to the EEG. They are appointed special treatmentAnd once every six months they pass a detailed examination.
The third risk group includes patients with bright symptoms for which the lack of treatment is equivalent to death.

Timely diagnostics and adequate treatment give kids with Vesta syndrome a chance for improving the quality of life, and sometimes on full health restoration.

Infantile Spasms by the child - video

Diagnostics

At the very first alarming symptoms, it is necessary to show the child to a neurologist, which can designate additional consultations in genetics, epileptologist, immunologist, endocrinologist, as well as neurosurgeon. After the inspection of the baby, the doctor sends a small patient for additional surveys:

EEG - to identify the disorganized brain activity during sleep and wakefulness;
CT brain - to determine changes in cerebral structures;
MRI brain - for accurate diagnosis of structural disorders and determining the cause of the Vesta syndrome;
PET brain - to determine the foci of hypoetabolism in brain tissues;
cerebral angiography - to identify vascular cerebral pathologies;
craneoscopy - to study the defects of the structure of the skull.

Conducting these studies allows you to distinguish wester syndrome from diseases with similar symptoms:

infant myocloss;
infant myoclonic epilepsy;
benign Rolandic epilepsy;
sander syndrome;
a variety of ticks.

West syndrome manifestations are often similar to TIK, but at the same disease muscle spasms We are provoked by emotional splashes, and there are no violations on EEG.

Sometimes after the electroencephalogram, it turns out that spasms were actually colic or respiratory attacks. In any case, to diagnose the doctor can only after all the necessary surveys.

Treatment of Vesta syndrome

With timely diagnosis and proper treatment of the resistance of the disease, it is possible to achieve more than 50% of cases. However, it happens that the reception of medicines does not affect the number and intensity of attacks and does not contribute to the development of the child. The success of therapy depends on the causes of the disease, the degree of brain damage and the form of Vesta syndrome.

Medical therapy

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

In the early 1990s, another breakthrough occurred in medicine: a positive effect on Vigabatrina (Sabrile) was discovered. This drug gives not so much side EffectsAs ACTH, it is better tolerated by children, as a result of its reception in patients there are fewer recurrences after the end of treatment.

Most often, Vigabatrin is used if the cause of Vesta syndrome has become tuberous sclerosis. In other cases, this tool may not be as effective as steroids.

To reduce the number and intensity of seizures, the following anti-wurals can be applied:

valproeva Acid;
vitamin B6, which in large doses acts on some patients is similar to anticonvulsants.

Anticonvulsants appointed with West Syndrome - Gallery

To improve physical and psycho-emotional development, doctors are usually prescribed to children drugs normalizing metabolism and blood supply to the brain. Nootropics are recommended to babies with extreme caution, since brain stimulation can provoke a strengthening of seizures even against the background of anticonvulsant reception.

It should be borne in mind that hormonal and steroid drugs give strong adverse reactions. Patients may arise:

fatigue;
depression;
concentration inability;
allergic reactions;
endocrine disorders;
diseases of the peripheral nervous system;
liver lesions.

A competent doctor along with major medicines usually prescribes drugs that increase immunity and supporting the liver. Throughout the course, the specialist must constantly monitor the patient's condition on the basis of blood tests and EEG testimony. In the case of hormonal therapy, treatment is carried out in the hospital.

If the doses of drugs were chosen correctly and the patient has a positive trend, treatment needs to continue about 1.5-2 years since the last attack.

Surgical intervention

If infantile spasms proved to be resistant to medical therapyAnd the MRI is clearly visible by the pathological center, the neurosurgeon can recommend to commit excision of the affected brain. During the operation, a specialist dishes the spike of brain shells, removes tumors and vascular aneurysms, while trying to apply the most gentle surgical methods.

If convulsions are manifested in the form of sudden falls, the patient can be shown by Callosotomy - an operation on the dissemination of the corpore body. After surgical intervention Children usually pass neurorerabaty in specialized centers.

Physiotherapy

Regular LFC classes for children with Vesta syndrome are simply necessary to restore the physical form and the development of new motor skills. As a rule, after the cessation of attacks, kids are quite quickly starting to sit, crawl, walk and even run, but without the correct selection of medicines, therapeutic physical culture will not bring due results.

The LFC must be carried out under the guidance of an experimental rehabilitologist and only after such classes will allow the attending physician. Otherwise, the intensity of convulsion can aggravate and worsen the overall state of the child.

In parallel with the development of motor skills, the baby needs to be regularly engaged in a defectologist, a psychologist and a speech therapist who will develop the speech and a small motorcycle of the child.

Unconventional methods

Among the effective unconventional methods, the treatment with stem cells can be distinguished. This method is very expensive and not recognized as official medicine. It is based on the fact that damaged areas of the brain are restored due to the administration of donor stem cells into the body.

To date, a few are solved for this measure, although such therapy has already managed to establish themselves well. Treatment with stem cells can not be called panacea, but it helps some patients.

Features of nutrition

Many foreign doctors seek good results In the treatment of wester syndrome using a ketogenic diet. It is based on an increase in fat and reduction of carbohydrates and proteins. In this case, the metabolism changes, and the body begins to produce a large amount of ketones, which reduce the frequency and intensity of convulsion.

A ketogenic diet helps approximately 70% of patients, and the attacks are reduced by more than 2 times, and in some children convulsions disappear at all. As a rule, the diet is prescribed to the kids from 2 years and is not particularly effective in the treatment of cramps in adolescents and adults.

Food Rich - Gallery

Media forecast, mortality rate, lifespan and prevention

In very rare cases, Vesta syndrome passes spontaneously, without any treatment. More often the attacks go under the influence of medicines and operational interventionSometimes the disease is transformed into other forms of epilepsy.

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

With idiopathic varieties are completely restored from 37 to 44% of children. The remaining patients have certain deviations in physical and mental development.
With symptomatic form, the forecast is much worse. The absence of consequences is observed only in 5-12% of cases, and mortality can reach 25%. Even with the occurrence of remission of the disease in children, the CRR develops (mental delay), cerebral palsy, autism, mental backwardness, many have difficulty learning, have problems with memory, concentration and logical thinking. Approximately half of the patients, there are motor disorders. Such a pessimistic forecast is due to the negative impact on the body of the underlying disease. It is from his flow that the life expectancy of the patient depends.

The forecast will be more favorable with the beginning of treatment. If medicines are chosen from the first weeks of the disease, the chances of full recovery increase several times. After 1-2 months, the percentage of a favorable outcome decreases twice.

If the treatment has begun in six months and later after the debut of seizures, the chances of recovery will be minimal.

The condition of the child can worsen the uncontrolled reception of nootrops and vaccination.

Preventing Vest Syndrome does not exist. It is important to diagnose this disease in time and correctly choose treatment - then even with symptomatic form it is possible to achieve significant improvements and bring the development of the child to the age norm as much as possible.

Dr. Komarovsky about children's cramps - video

Vesta syndrome is a cunning and dangerous disease. The percentage of child mortality with it is quite high, so at the very first alarming symptoms it is necessary to contact the doctors and maximally perform diagnostics. The correct selection of drugs in conjunction with competent rehabilitation with the participation of psychologists, defectologists, speech therapists and specialists of the FFC increase the chances of the child to complete recovery.

Infantile spasms - cramps characterized by sudden bending of the body forward, bending or extension of hands, pulling or flexing legs.

This type of attacks, as a rule, is combined with Hypsarithmia on EEG.

Causes can pass on their own ages about 5 years, but can transform into other types of attacks.

The pathophysiology of infantile spasms is unknown to the end, but these attacks can reflect the violation of the interaction between the bark and the brain barrel. The cause of infantile spasms may be the immaturity of the CNS, the defects of brain development and brain damage in the first months of life. A common cause of the development of infantile spasms is tuberous sclerosis. The nature of the seizures may also be idiopathic.

Symptoms and signs of infantile spasms in children

Infantile spasms begin with sudden rapid tonic contractions of the body and limbs, sometimes within a few seconds. Spasms range from light nodes head until shuddering the whole body. They are accompanied by flexion (flexia), extension (extension) or, most often, and bending, and extension in the muscles of the limbs (mixed spasms). Spasms are usually repeated during the day clusters, often several dozen, mostly shortly after awakening, and sometimes during sleep.

As a rule, infantile spasms are accompanied by a violation of motor and mental development. In the initial stages of the disease, a development regression is possible (for example, children can lose the ability to sit or turn over).

An indicator of premature mortality in infantile spasms ranges from 5 to 31%, death occurs under the age of 10 years and depends on the etiology of the latter.

Diagnosis of infantile spasms in children

Neurovalization.
Video Zagsna and wakefulness.
Laboratory studies on clinical indications.

Tests to determine the cause of infantile spasms may include:

laboratory studies (for example, a general blood test, the determination in blood serum glucose, electrolytes, urea, creatinine, Na, Ca, Mg, P, liver samples), if the metabolic disorder is suspected;
analysis of SMG;
brain scanning (MRI and CT).

Treatment of infantile spasms in children

Infantile spasms with difficulty are treatable, and the optimal pattern of therapy is discount. ACTG is used for 20-60 units intramuscularly once a day. Many anticonvulsant drugs are ineffective; Preferably, the use of hollows, the preparation of the second selection queue is clonezepam. The effect of the use of nitrazempama, topiramate, zonizamide or vigabolin is also noted.

A ketogenic diet can also be effective, but it is difficult to maintain it.

In some cases, surgical treatment can be successful.

Infantile spa

Infantile spasm or wester syndrome is called epileptic syndrome. Available spasms in the form of a series. They can occur with different intensity, intensifying or on the contrary declining. The number of attacks can reach up to thirty and count to twenty episodes during the day. It happens more often at night. The study of the etiology of this state began in the fifties of the last century.

Infantile spasms in children

Infantile spasms are more common in children under the age of three. For one hundred born kids accounts for 0.4% of the fallen. The first attack can happen between three months to one year. The baby lying on his back suddenly raises and begins to bend his hands, raises his head, top The bodies and at the same time sharply straightens the legs. The attack can last a few seconds and be multiple and accompanied by crying and irritability. Before starting the seizure, the child stops active activity, not gulit, looks at one point, may not move. In the future, it develops other types of convulsive manifestations. The kid also has a violation of psychomotor development and a delay of intelligence that can be maintained in adulthood. Such attacks are difficult to treat.

Cause of infantile spasm

Depending on the etiology of spasms there are symptomatic and cryptogenic. The reason for their occurrence may be the following:

damage or immaturity of the cortex of the brain;
chromosomal and gene anomalies;
neurological and psychophysical disorders;
disorders during the period of intrauterine development (hypoxia, premature delivery);
infectious diseases during pregnancy (bacterial or herpes viral meningitis);
down syndrome;
tuberculous sclerotic syndrome;
complications during childbirth

In some rare cases, the reason may be in vaccination of the vaccination of ADC.

Types of infantile spasms

Infantile Spasms: Diagnostics

To determine the causes of convulsion, a complete medical diagnostic examination is carried out. To eliminate metabolic disorders, a number of laboratory studies are carried out: on the content of the level of glucose, calcium, sodium, magnesium, amino acids. To eliminate hypoxia, blood gases are investigated, sowing of the existing fluid in the body is taken. The spinal fluid (liquor) is also investigated. An immunological and virological examination is carried out to eliminate the intrauterine infection. It is also necessary to make magnetic resonance tomography, computer tomography, Crantio and Spondylography. Electricencephalography fixes the activity of sharp wave attacks. For a complete picture of the disease, video monitoring of spasms is carried out. It is necessary to consult a neurologist, genetics, speech therapist, oculist, psychologist and psychiatrist. In young children, cognitive functions according to the procedure of Bazhenova are estimated before the year. The older children define the level of IQ. In most cases, infantile spasms are transformed in epileptic after a while. Treatment in this case by anticonvulsants is not always effective.

Discussions

Epilepsy. Infantile spasms.

4 messages

Infantile spasms - epileptic syndrome observed in children up to a year and characterized by flexor, extensor or flexor-extensor spasms, neuro-psychic development delay and the presence of hydrochyrithmia on EEG.

Three options for infantile spasms are possible - flexor, extensor, flexor-extensor.

Flexor spasms consist of sudden bending of the head, neck and all limbs. During a bending paroxism of infantile spasms, the child impresses "hugging himself." Extensor spasms are characterized by a sudden extension of the neck and trunk, the lower extremities, combined with the extension and abduction of the shoulders. A similar extensional version of infantile spasms simulates the reflex moro. Flexor-extensorial spasms include mixed paroxysms, manifesting the flexy of the neck, torso, the upper limbs and the extension of the lower extremities or, more rarely, bending the hands and the extension of the legs.

Depending on the etiology, all infantile spasms are divided into cryptogenic and symptomatic. The expediency of the separation of infantile spasms to cryptogenic and epileptic proceeds from the generalized experience by the characteristics of clinical manifestations and the flow of infantile spasms.

For cryptogenic infantile spasms are characteristic:

Lack of a clear etiological cause;

Normal neuropsychic development of the child until the disease is developed;

Lack of other types of seizures;

Lack of signs of brain damage according to the data of neuroradiological research methods (computer (CT) and nuclear magnetic resonance (NMR) tomography of the brain).

Symptomatic spasms are a consequence of various etiological factors. For symptomatic infantile spasms are characteristic:

Delayed neuropsychic development until the cause of the disease;

Often - pathological changes in CT-and NMR studies of the brain.

Infantile spasms are sometimes accompanied by a cry, a smile grimace, a frightened expression of the face, turning out the eyes, nystagm, the expansion of pupils, eyelids, limbs, pallorium or redness of the face, stopping the breath. After convulsion, drowsiness is observed, especially if the series of attacks was long. In the interconception period, children are irritable, plaxive, disturbed sleep. Causes occur most often before falling asleep or after waking up. Factors provoking paroxysms include fright, various manipulations, feeding.

Infantile spasms begin at heart age and disappear in early childhood. Under the age of 6 months they make up 67% of the total number of paroxysmal episodes; from 6 months to 1 year - 86%; After 2 years - 6%.

Infantile spasms can be the first manifestation of a convulsive syndrome in a child. The first seizures are an abortive character and can be taken by parents for the fright reaction, the manifestation of pain in the stomach, etc. First, they are single, then the frequency is growing. At this stage can be remission and exacerbations that are difficult to foresee. As the child grows and the development of the child, the frequency of convulsion decreases. The average duration of infantile spasms - from 4 to 30-35 months. After 3 years, they are rare. According to P. Jeavons, etc. (1973), in 25% of children, infantile spasms are terminated under the age of 1 year, in 50% - up to 2 years, in the rest - up to 3-4, sometimes 5 years.

Changes to EEG (gypsumithmia) do not always correlate with the start of convulsion, sometimes they appear somewhat later. Hypsarithmia is characteristic of the developing brain and is observed only with infantile spasms in young children.

The universal concomitant feature of infantile spasms is the delay in mental development, which is observed in 75-93% of patients, the formation of motor skills is also violated. Therefore, children in early age it is more correct to talk about the delay in psychomotor development, which is already noted in the initial stage of the disease. It becomes more distinct when a series of seizures appear. The degree of delay depends on the start of the start of convulsion and from the premorbid characteristics of the child. Normal psychomotor development before the appearance of convulsion is observed in 10-16% of children.

This epilepsy begins in infancy with a sudden shudder of the entire body, a quick nodium, similar to the Eastern greeting - both shores are lifted up and to the side, the head leans on the chest, the look is directed up. Very often before the first convulsions of this type, in all likelihood, brain damage has already come. About 20% of babies do not detect any changes before the first attack.
Epilepsy for such children is much better. Treatment is extremely difficult. A quick accurate diagnosis and proper treatment are crucial. Currents currently used, for example, hormones (synacten-depot, ACTH), Valproat (Depaakin, Convoilelex, Apilexin) and benzodiazepines (Anteltexin, clonazepam) have an side effect and, unfortunately, not always able to affect the malignant course of the disease, Even if the attacks stop. Attacks indicate only the disease of the brain, and not about the course of this disease. Some improvement periods should not be revalued so that there is no disappointment. Most patients must constantly observe the epileptologist's doctor.
Another name for this form of epilepsy Vesta syndrome (this is the name of the doctor who at the end of last century first described this disease from his son).

West syndrome (infantile spasms)

The clinical and electroencephalographic characteristics of epileptic attacks in children vary depending on age. A visual example of such eye-dependent phenomena is infantile spasms - a unique type of attacks associated with early childhood. Infantile spasms are a specific age phenomenon that occurs in children only in the first two years of life is most common - in the interval of 4 to 6 months, and approximately 90% of patients - up to 12 months. The incidence of Vesta syndrome is estimated as 0.4 per 1000 children born alive.

Definition

Typically, infantile spasms have a stereotypical nature of one child. In addition, the occurrence of infantile spasms in the form of a series is characterized.

Infantile spasms are divided into three types: flexor, extension and mixed flexor-extensor. Flexor spasms are short-term contraction of the muscle flexor torso, neck, upper and lower extremities. Spasms in the muscles of the upper extremities cause hands to bring, "as if the child hugs himself with his hands" or, on the contrary, breeding hands bent in the elbow joints, in different directions. Extensor spasms include, predominantly extensitive muscle contraction, causing a rapid sharp extension of the neck and torso in combination with extension and assignment or leading hands, yoga or upper and lower limbs at the same time. Mixed flexor-extensor spasms include flexion of the neck, body and upper limbs and extension of the lower extremities or bending the lower extremities and the extension of the hands in combination with a varying degree of flexion of the neck and the torso. Sometimes asymmetric spasms resembling a "fencer posture" are developing. Infantile spasms are often combined with the deviation of the eyes or the appearance of Nistagma.

Asymmetric spasms may occur if there is no simultaneous bilateral reduction in the muscles of the limbs. This type of spasms is usually found in the symptomatic form of infantile spasms in babies with severe brain damage, the agent of the corn body or a combination of these violations. Such local neurological symptoms, like the deviation of the eyes or turn the head, can be combined with both symmetric and asymmetric spasms. Asymmetric spasms usually occur isolated, but they can also develop after a focal attack or precede it; In some cases, infantile spasms may occur simultaneously with a generalized or focal attack.

Gypsarithmia in a child with infantile spasms. High-amplitude disorganized activity in the background entry, intermitted with multifocal spikes and sharp waves

Definition

Early children's cramps

Symptoms and signs of the disease

Primary symptoms of pathology

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Encephalopathy

Cerebellum syndrome

Consequences and complications

Diagnosis of the disease

Treatment of Vesta syndrome

Medical Physical Culture and Prevention

Diagnostics

Classification of infantile spasms.

Key points

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Course of therapy

Cause of infantile spasm

Types of infantile spasms

The main symptoms of violations

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Encephalopathy

Symptomatic syndrome Vesta

Cerebellum syndrome

Forms

Forecast and possible complications

Clinical picture

Diagnostic procedures of infantile spasms.

How to diagnose infantile spasms

Diagnosis of infantile spasms in children

Principle of treatment

Medical therapy

Surgery

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Causes of occurrence

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Epilepsy. Infantile spasms.

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West syndrome (infantile spasms)

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