What does infantile spasms mean in anamnesis. Prognostic criteria for the likelihood of termination of infantile spasms

ATTENTION!!! Once again I warn you that you do not need to watch these videos without the need! these videos are taken from the open source YOU-TUBE, the authors posting material there are warned that third parties will use it. I personally post this material because SO MANY PARENTS find it difficult to explain to doctors what is happening to their child. and also it is sometimes difficult for parents themselves to understand that their child does not "play like that", but he has convulsions.

pediatricians initially thought that the child had colic

infantile spasms, but without knowing the issue it is not easy to understand .

tonic-clonic seizure
Major epileptic seizures. The sudden onset of a generalized convulsive seizure indicates involvement in the pathological process of the cerebral cortex. Tonic-clonic seizures usually begin like this: the patient's eyes and mouth are open, the arms are bent and abducted, the legs are straightened. Before the onset of the tonic phase of the seizure, there is usually a contraction of the respiratory muscles, leading to vocalization. Then there is a compression of the jaws, often with biting the tongue, respiratory arrest with hypervolemia and cyanosis, urinary incontinence, less often feces. The tonic phase of the seizure, usually lasting 15-30 seconds, is immediately replaced by the clonic phase, characterized by violent rhythmic contractions of the muscles of all parts of the body, including the respiratory muscles. There are deviations of the eyeballs, grimacing, respiratory arrest. Clonic twitches gradually weaken, slow down, and the attack ends. The attack lasts, as a rule, 1-2 minutes. Then breathing normalizes and the patient falls asleep. After a few minutes, the patient may wake up, but he often feels drowsiness, severe fatigue and confusion within a few hours after the attack. In addition, there is often headache.

febrile convulsions



absence

Absences, unlike major epileptic seizures, are characterized by a shorter seizure duration and a more pronounced change in consciousness with minimal motor manifestations. They usually occur suddenly and become noticeable only with close observation and a noticeable change in the patient's usual behavior, may be accompanied by trembling of the eyelids and single convulsive twitching of the muscles of the face. Full recovery occurs within 5-10 seconds, and sometimes neither the patient himself nor his relatives may notice a seizure. Loss of postural tone (atonic or akinetic seizures) is uncommon, but when it does occur, differential diagnosis from fainting. In all cases, EEG is of diagnostic value, which reveals peak-wave changes with a frequency of 3 peaks per 1 s. To treat this condition, which indicates a special generalized disturbance of the electrical activity of the brain, specific medications are successfully used.

infantile spasms (Salaam's spasms))
Infantile spasms are manifested by a sudden contraction of the muscles of the neck, trunk, limbs, which, as a rule, are bilateral and symmetrical. The most typical flexor spasms ("Salaam's attacks") with flexion of the neck, torso, arms; flexion, adduction and elevation of the legs. Attacks are short, grouped in series; often occur immediately after waking up patients.

Infantile spasms (similar to the initial stage of the Moro reflex)


myoclonus

Adolescent myoclonic epilepsy begins between the onset of puberty (puberty) and 20 years of age. It is manifested by lightning-fast twitches (myoclonus), as a rule, of the hands while maintaining consciousness, sometimes accompanied by generalized tonic or tonic-clonic seizures. Most of these attacks occur in the interval of 1-2 hours before or after waking up from sleep. The electroencephalogram (EEG) often shows characteristic changes, and there may be an increased sensitivity to light flicker (photosensitivity). This form of epilepsy responds well to treatment.


tonic seizures


West syndrome belongs to the class of encephalopathic epilepsy, when seizures occur against the background of non-inflammatory diseases of the brain.

Epilepsy is understood progressive chronic illness , which is manifested by bouts of freezing or convulsions, paroxysmal (paroxysmal) disorders of consciousness, autonomic paroxysms (changes in vascular tone, pulse, respiration, etc.).

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

West syndrome usually occurs in infancy and has many reasons:

  • Asphyxia.
  • Metabolic disorders, gene mutations and genetic diseases.
  • Congenital brain disease (for example, tuberous sclerosis).
  • Brain hypoxia, birth intracranial bleeding (especially in premature babies).
  • brain infections.

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

Symptoms and signs of the disease

West syndrome occurs in 1.7-4.3 per 10 thousand infants and is 3-10% common cases epilepsy. Boys suffer the most (65%). In 80% of cases, additional diseases are established:

Thus, the most common form of the disease is symptomatic, that is, which accompanies another disease. Cryptogenic syndrome - the cause of the condition is not obvious or unknown - is determined in 10-14%. Cases of genetic predisposition are 2–5%.

Among the symptoms of West syndrome, the following should be highlighted:

  1. Explicit disturbances in psychomotor development.
  2. EEG changes characteristic of West's syndrome (hypsarrhythmia).
  3. Frequent epileptic seizures.

In almost all babies with West syndrome (95%), the first signs appear almost immediately after birth (within 3-7 months). Seizures are characterized short duration Therefore, it is not immediately possible to establish a correct diagnosis.

West syndrome is a serious disease that threatens the life of a child. This disease is also called infantile spasms. The attack passes all the time in the same way: the head is strongly bent, and the whole body of the child sharply leans forward.

These cramps usually appear during falling asleep or when the baby wakes up. Moreover, there can be more than 50 attacks per day.

Often during seizures baby loses consciousness. Because of them, the child begins to lag in the development of Psychomotor. Patients with West syndrome almost do not make contact with relatives, react poorly to the environment.

Primary symptoms of pathology

Strong crying of a child is the first symptom of an attack of West syndrome, so doctors often diagnose these children with colic. The typical symptoms of this disease are:

  1. Limbs involuntarily diverge to the sides.
  2. Generalized convulsions throughout the body, in the upper and lower extremities.
  3. Sharp tilts of the whole body forward.

As a rule, this attack lasts no more than a few seconds. There is a short pause and the attack is repeated. Sometimes spasms go away singly, but usually they happen in succession.

Babies with West syndrome often very irritable lag behind in mental development. Newborns with this disease most often behave like they are blind.

Epilepsy attacks


West syndrome is a type of catastrophic generalized epilepsy. It can manifest itself both symptomatically (most often) and cryptogenically (up to approximately 12% of the total number of cases).

The classic variant of this disease can be characterized by pronounced myoclonic or salaam spasms. In some cases, spasms take the form of serial short tilts of the head.

In West syndrome, epilepsy appears without certain disturbances in the functioning of the central nervous system or because of various neurological pathologies.

Infantile spasms lead to inhibited development of motor and mental functions in children, which in the future may be the cause of pronounced developmental delays in a child. In 85% of cases, a baby with West syndrome develops atactic and atonic disorders, microcephaly, cerebral palsy.

encephalopathy

As mentioned above, West syndrome is also called hypsarrhythmia with myoclonic encephalopathy. Hypsarrhythmia is a typical, but not pathognomonic, encephalogram pattern in children with this disease.

Typical hypsarrhythmia is characterized by constant high-amplitude and arrhythmic slow-wave activity, and also has multiple sharp waves and spikes. Moreover, there is no synchronization between the various departments of the hemispheres. In some cases, the patterns differ in amplitude asymmetry.


Most often, in 80% of cases, West syndrome begins in the 2nd or 3rd quarter of a child's life. At first, the development of the baby seems normal, and only then convulsions appear, which are the primary pathognomonic symptom.

In some cases, children have delayed psychomotor development. Not often, but you can observe changes in the EEG.

Muscle cramps or myoclonus affect almost the entire body. With these convulsions the limbs and body of the child bend. Contractions and spasms in the flexor muscles can be synchronous, bilateral, symmetrical, sudden, and last no more than 7–9 seconds.

In certain cases convulsive syndrome affects only one muscle group. During spasms, the upper and lower limbs scatter involuntarily to the sides, the head bends to chest. When the frequency of seizures is high, the baby may fall asleep.

Today, there are three separate varieties of West syndrome, which differ in the nature and degree of muscle damage:

  • Widespread spasms-spasms throughout the body. The limbs are bred to the sides, and the head "lies" on the chest.
  • Occipital spasms - head tilts back. Spasms last up to 10 seconds with a break of one second.
  • Nodding - multiple flexion spasms (especially on the head and upper limbs).

In babies with West syndrome, immediately after birth or within six months appears lag in mental and motor development. Frequent spasms only exacerbate the situation.

Cerebellar Syndrome

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

Consequences and complications

In almost all cases, the passage of West syndrome is very difficult, since it is manifested by significant brain disorders. Quite rarely, this disease goes away with the help of conservative treatment. But as a rule, even after effective treatment after a certain time, relapses appear.

Almost always, after the recovery of the child, he subsequently experiences rather severe and serious residual neurological phenomena: extrapyramidal manifestations, epilepsy and its equivalents. And also these patients develop mental disorders: mild dementia or idiocy. Only in 3% of cases (according to Gibbs) does an unexpected complete cure occur.

Diagnosis of the disease


West syndrome is diagnosed with the help of such doctors: an epileptologist, a neurosurgeon, a pediatrician, a neurologist, an endocrinologist, an endoscopist and an immunologist. Due to the use of modern devices, it is possible to establish the most accurate diagnosis.

Most often used: cerebroangiography, cranioscopy(in rather rare situations), computed tomography and radiomagnetic. And also to determine the pathological focus of epileptic seizures, neurophysiological studies are performed.

The most common ways to diagnose West syndrome are: gas encephalography and electroencephalography. Thanks to electroencephalography, hypsarrhythmia of biological curves can be detected:

  1. Low efficiency of light stimulation.
  2. The timing of the main curves is not determined. There may be "peaks of the curve" in the process of wakefulness or sleep.
  3. The amplitude of biological curves is irregular.

Sometimes with the help of gas encephalography, an increase in the ventricles of the brain can be observed. In the later stages of West syndrome, hydrocephalus is observed.

Differential diagnosis. This disease can be differentiated both with non-epileptic diseases that are common in newborns (motor restlessness, colic, respiratory attack, hyperexplexia, infantile masturbation), and with certain epileptic symptoms (for example, focal epilepsy). Electroencephalography plays an important role in differential diagnosis.

Treatment of West syndrome


In each specific case, the treatment is prescribed individually and depends on the cause that caused the pathological conditions of brain development and West syndrome. To date, the main treatment for West syndrome is steroid treatment with adrenocorticotropic hormone (ACTH) (vigabatrin, Sabril).

However, this treatment must be very careful and under the strict supervision of a doctor because vigabatrin and steroid drugs have a large number of serious side effects. And you also need to choose the appropriate anticonvulsant drugs, as well as medicines which will normalize blood circulation in the brain.

In some cases, the neurosurgeon is required to perform an operation, during which the adhesions of the meninges are cut, and the pathological focus is removed. This operation is done using stereotaxic surgery and various endoscopic methods.

A modern and rather expensive way to treat this disease is use of stem cells. This method is quite effective, however, it is not popular due to the high cost of the operation. The meaning of this method is that the damaged part of the brain is restored with the help of stem cells.

As a rule, the idiopathic variety of West syndrome is treated with special drugs:

  • Vitamins - for example, vitamin B6 (pyridoxine).
  • Hormonal steroid medicines- for example, Tetracosactide, Prednisolone, Hydrocortisone.
  • Anticonvulsants - for example, Nitrazepam, Epilim, Topamax (Topiramate), Depakote (Valproate), Klonopin (Clonazepam), Onfi (Clobazam), or Zonegran (Zonisamide).

Treatment is considered effective when reduced frequency and number of seizures. With well-chosen treatment, the child will continue to learn and develop normally. But you need to be aware that even modern medical drugs carry a large number of side effects:

  • fatigue;
  • decrease in concentration;
  • depression;
  • allergic skin reactions;
  • liver failure;
  • damage to the nervous system.

Therapeutic exercise and prevention


Therapeutic exercises for West syndrome must be carried out under the strict supervision of a sports medicine doctor and a rehabilitation therapist so that seizures do not worsen.

This type of treatment is quite common, however, does not show effective results without a combination of drugs.

With West syndrome, the absence of spasm for a long time cannot indicate that the disease has gone into remission. However, many doctors argue that if convulsions, spasms, EEG changes and hypsarrhythmia have not been noted for a month, then this is a recovery.

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

Main preventive measure West syndrome is timely diagnosis And competent treatment. Epileptic seizures, which are the main symptom of this disease, need to be stabilized.

Diagnostics

Diagnostic measures for West syndrome begin with the study clinical picture disease and examination of the patient. Infantile seizures are difficult to diagnose. Seizures are difficult to distinguish from erratic movements of the head and limbs. Specialists take into account the duration of spasms and their effect on sleep. Pediatricians, neuropathologists, geneticists, immunologists are engaged in diagnostics and treatment.

Instrumental methods of research of patients:


Classification of infantile spasms.

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

  • Flexor view - intense spasms of the flexor muscles of the body, arms and legs.
  • Extensor view - enhanced spasms of the muscle tissue responsible for the extensor function, characterized by simultaneous extension of the body, neck, upper and lower extremities.
  • Flexor-extensor type - a mixed type of spasm of muscle tissue with alternating extension and flexion of body parts.
  • Asymmetric muscle spasms on one side of the body. Characterized by the appearance in severe pathologies of the brain of children.

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

Symptomatic, these spasms with an established etiology, are characterized by a deviation in mental and nervous development after the manifestation of spasms, neurological disorders, the study clearly shows pathological abnormalities in the structure of the brain.

Cryptogenic, these spasms of unknown etiology, the child has a normal neuropsychiatric development before the onset of the disease, a certain type of convulsive processes is characteristic. Examination of the brain revealed no focal lesions.

idiopathic, are seizures that begin in the neonatal period and in childhood. They have a benign course, the rhythm of the transmission of nerve impulses is not disturbed, is characterized by the absence of neurological changes and normal mental development during the course of the disease. Sometimes they look focal, after a few seconds it seems that they come from many foci.

At times, spike discharges are generalized, but never look like a rhythmically repetitive and highly organized pater. These changes are almost permanent. Research into the etiology of infantile spasms began in the 1950s. As the facts accumulated, the polyetiology of the syndrome became apparent.

Infantile spasms have been described in intrauterine infections, various brain dysgenesis, a number of chromosomal abnormalities, and hereditary diseases. Among the latter, in particular, almost all neurocutaneous syndromes, many metabolic diseases from the common phenylketonuria to organic aciduria, including the extremely rare fumaric aciduria.

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

The expediency of dividing infantile spasms into cryptogenic and epileptic ones comes from a generalized experience in terms of features. clinical manifestations and the course of infantile spasms.

Cryptogenic infantile spasms are characterized by:

  • Lack of a clear etiological cause;
  • Normal neuropsychic development of the child until the development of the disease;
  • Absence of other types of seizures;
  • No signs of brain damage according to neuroradiological research methods (computed (CT) and nuclear magnetic resonance (NMR) tomography of the brain).

Symptomatic spasms are the result of various etiological factors. Symptomatic infantile spasms are characterized by:

  • Established etiology;
  • Delay in neuropsychic development until the development 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 are conditionally divided into three large groups according to the time of occurrence - prenatal, perinatal and postnatal. The cause of such infantile spasms can be any factor damaging the cerebral cortex.

Most children often have perinatal pathology of the central nervous system, brain injury, various chromosomal and gene anomalies. But it happens that there are also cryptogenic (in the absence of a clear etiological cause) infantile seizures.

Key points

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

Infantile spasms can be described as short and sometimes subtle attacks that occur in infants. These seizures are actually a rare form of epilepsy.

Only about 2,500 children each year will be diagnosed with the disease in the United States. These seizures or convulsions usually occur before the child is 1 year old, with most cases occurring in children around four months of age.

Symptoms

The cost of treatment in European clinics and Germany

A ten-day hospital stay in a German clinic and the treatment of West syndrome will cost an average of 5,000 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, the symptoms intensify over time, due to which the child may remain disabled for life or die. That is why it is important to treat West syndrome as soon as possible. This can be done with drugs such as adrenocorticotropic hormone and drugs based on valproic acid, for example, Valproates. If ACTH did not give desired effect, then glucocorticosteroids (GCS) are used, and Dexamethosone is most often prescribed from this group. Hormone treatment must be combined with anti-epilepsy drugs for the best effect.

According to experts, the following medications will also need to be included in the course of therapy:

  • benzodiazepine (clonazepam);
  • Vitamin B6;
  • Immunoglobulin (Octagam);
  • Vigabatrin.

The course of treatment should be carried out under the strict supervision of a doctor, and with hormonal therapy, the child is placed in a hospital. Periodically, you will need to do an EEG to monitor the effectiveness of the medications taken. Experts recommend checking that among the prescribed tablets there are no drugs that have a stimulating effect on the nervous system. It is desirable to introduce into the course of treatment drugs to improve metabolic processes, as they reduce the frequency of seizures and stimulate mental development.

If there is a neoplasm in the brain that provokes a worsening of the course of the disease, then surgical intervention may be required. This method is used in extreme cases, as it carries a great risk to the life of the child. Brain tissues at this age regenerate much better than in adults, but the operation itself is quite difficult.

Doctors urge parents to be like a doctor's advice about caring for sick children. After all, even if this disease is stopped, its consequences will still remain, and these include psychomotor delay, which is extremely difficult to eliminate.

Cause of infantile spasm

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

  • damage or immaturity of the cerebral cortex;
  • chromosomal and gene anomalies;
  • neurological and psychophysical disorders;
  • violations during fetal development (hypoxia, premature birth);
  • infectious diseases during pregnancy (bacterial or herpes viral meningitis);
  • Down syndrome;
  • tuberculous sclerotic syndrome;
  • complications during childbirth

In some rare cases, the cause may be a DPT vaccination.

Types of infantile spasms

Infantile spasms are of three types. Estensor, flexor-extensor or flexor. These are sudden contractions of the muscles of the head, limbs, trunk and neck. Flexor spasms are also called jackknife convulsions or Saalam convulsions. It looks like the person is hugging himself. Extensor spasms suddenly extend the neck, lower limbs, and trunk, combined with extension of the shoulders. As a result of flexor-extensor spasms, the actions are mixed. Cryptogenic infantile spasms occur in 9-15% of cases, the rest are symptomatic.

The main symptoms of the disorder

The manifestation of West's syndrome in children is clearly expressed 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 the pathology.

Epilepsy in West syndrome

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

  • convulsions;
  • hyperthermia;
  • lack of response to external stimuli;
  • fixed posture, motionless gaze.

Epilepsy can be short-term or long-term. A major seizure is preceded by sleep disturbance, loss of appetite. A generalized seizure is accompanied by:

  • partial stop respiratory function, muscle contraction;
  • coloring of the nasolabial triangle in cyanotic color;
  • convulsions, the duration of which is up to several minutes;
  • spontaneous defecation and urination;
  • vomiting, foam at the mouth.

During this period, it is necessary to protect the child from hitting hard objects, thus eliminating injury. Small manifestations are short in time, may occur several times a day, accompanied by intermittent convulsions, for example, flexion of the body.

encephalopathy

Pathology unites a group of diseases caused by disruption of the brain, which are based on non-inflammatory etiology. During the process, neurons die and the synapse (communication) between them is disrupted. Encephalopathy depresses the central nervous system, which leads to inhibition of the development and mental abilities of the child. The disease is characterized by the following symptoms:

  • anxiety, headache, irritability, tearfulness;
  • sleep disturbance, inversion is manifested by insomnia at night and lethargy during daylight hours;
  • deterioration or complete lack of appetite;
  • loss of short-term memory;
  • panic attacks (fear of insects, closed space, fear of being alone).

In children under 3 months of age, hydrocephalus may develop, an increase in the brain and head size due to poor blood outflow.

Symptomatic West syndrome

Spasms are the main symptom of myoclonic encephalopathy. They can be classified according to their nature:

  1. Propulsive - the cause of birth or intrauterine trauma. Occur unexpectedly several times a day. At the time of the attack, the child's torso begins to bend. This form is characterized by a delay in motor skills and mental development.
  2. Impulsive convulsions are manifested by contraction of all types of muscles, the child remains conscious, arms are spread apart, fists are clenched. Then the forelimbs are brought together around the body, like an embrace.
  3. Retropulsive - accompanied by fainting, the patient's eyeballs roll up, the body freezes in one position, the muscles are in the utmost tone. The head is thrown back, convulsions of the occiput are visually observed.
  4. Psychomotor - this rare form is characterized by the automatic performance of certain actions (laughing, crying, vomiting, the same movement, for example, tilting the head).

The disease is severe, life-threatening. It appears immediately after birth. Affects psychomotor development.

Cerebellar Syndrome

Pathology occurs against the background of a loss of communication between the parts of the brain. Accompanied by symptoms:

  1. Diadochokinesis, in which complex movements are impossible, their sequence is violated, actions are performed slowly or, conversely, chaotically (one is not completed, a quick transition to another).
  2. Finger trimmer.
  3. Muscles are sluggish and relaxed.
  4. Frequent bouts of dizziness.

The cerebellar syndrome in myoclonic encephalopathy is well characterized by the symptom of reverse shock.

Forms

Officially, SV is divided into symptomatic (up to 85%), as well as cryptogenic and idiopathic forms (together up to 20%). Nose clinical point of vision, the disease has only 2 forms, since there are practically no differences between the cryptogenic and idiopathic forms. The symptomatic form of West syndrome includes cases of the disease against the background of an already existing pathology of the brain or developmental disorders. Half of the children with a symptomatic form in history had a complicated course of the prenatal period: infections, metabolic disorders, genetic and chromosomal defects (Down's syndrome, etc.), as well as impaired intrauterine circulation in the mother. Rarely observed pathology of the birth period. This is hypoxic-ischemic brain damage, trauma and other complications in childbirth. Postnatal causes of WS include infections, trauma, hypoxic-ischemic strokes, and tumors.

The cryptogenic or idiopathic form of the disease is diagnosed in children with West syndrome epilepsy for no apparent reason, with normal psychomotor development and without brain damage prior to the onset of the disease. This is a more favorable form of ST.

The pathogenesis of West syndrome is currently unknown. Patients have a shortened phase of REM sleep (rapid eye movement phase), during which the EEG normalizes and the frequency of spasms decreases. In this regard, there is a version that in SW in the brain stem there is a dysfunction of serotonergic neurons involved in the formation of sleep cycles. There are other hypotheses that imply genetic and immune disorders in young patients.

Prognosis and possible complications

The disease is considered curable early stage development and timely detection. Proper therapy gives a stable result and does not affect the quality of life of the child in the future. The insidiousness of West syndrome lies in the fact that it is very difficult to diagnose in the first months after birth. Therefore, every 6 patient without proper treatment dies before 4 years of age.

In the majority of children, remission is supported by medications. They have more or less deviations in psychomotor development. Even if they managed to stop the convulsions, such babies are still intellectually behind their peers. Eliminating the symptoms of the disease does not eliminate it. In the future, the development of epilepsy is possible with the transition to the Lennox-Gastaut syndrome, which manifests itself throughout life.

The idiopathic type is completely treatable: 60% of children develop normally after therapy and are no different from their peers.

Clinical picture

Most often, the syndrome debuts in children aged 4–6 months, with earlier symptoms being an unfavorable prognostic factor. Infantile spasms of West syndrome can manifest themselves with high frequency and be extremely diverse - flexion of the body, vertical movements of the eyeballs, or eye movements similar to nystagmus, as well as “throwing up” handles like an oriental greeting, etc. One spasm lasts a fraction of a second, spasms grouped in series - up to 50 attacks in a series, the number of series per day - from one to several dozen. Often, seizures develop on waking and falling asleep, and may be accompanied by head or eye abduction to the side. Only half of the body may be involved in the spasm. The appearance of epileptic seizures means a stop in the psychomotor development of the baby, and often a regression of acquired skills. In 1-2% of cases, spontaneous self-healing is possible.

Diagnostic procedures for infantile spasms.

A clinical assessment of the development of the nervous system is carried out. Laboratory screening findings for electrolyte, metabolic, or other abnormalities are usually normal. In unclear cases, to identify the etiology, you can examine the cerebrospinal fluid, conduct neurometabolic tests, chromosomal analysis. CT scan(CT) and mainly magnetic resonance imaging (MRI) are mandatory before starting steroid therapy.

Interictal EEG:

The classic epileptiform pattern of hypsarrhythmia is recorded in 2/3 of patients. Asymmetric and modified hypsarrhythmia occur in 1/3 of cases.

Ictal EEG:

Up to 11 different ictal patterns can be recorded, ranging from 0.5 sec to 2 min. The most frequent pattern (72%) consists of a high-amplitude generalized slow wave, an episode of low-amplitude fast activity.

How to Diagnose Infantile Spasms

If a doctor suspects infantile spasms, they will order an electroencephalogram (EEG), which is easy to obtain and usually diagnosed. If this test is inconclusive, they may order a test called a video electroencephalogram (video-EEG). With this test, as with a conventional EEG, electrodes are placed on the child's skull to help doctors visualize brain waves. A video then captures the child's behavior. A doctor, usually a pediatric neurologist, will observe brain wave activity during and between spasms.

These tests usually last one to several hours and can be done in a doctor's office, laboratory, or hospital. They can also be repeated after a few days. Most children with infantile spasms will have disorganized brain wave activity. This is known as modified hysparhythmia. Very erratic brain wave activity for a milder response, known as hypsarrhythmia, occurs in about two-thirds of children with the disorder.

If your child is diagnosed with infantile spasms, their doctor may also order other tests to find out why the spasms are occurring. For example, an MRI can image the brain and show any abnormalities in its structure. Genetic testing can reveal genetic causes that contribute to seizures.

It is important that you apply immediately medical care if you think your child is having baby spasms. The disorder can have very serious developmental consequences, especially if left untreated. Your child has every chance to limit these negative consequences with early intervention.

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

Complications

Diagnosis of infantile spasms in children

  • Neuroimaging.
  • Video-ZEGsleep and wakefulness.
  • Laboratory studies according to clinical indications.

The diagnosis is established on the basis of clinical symptoms and a characteristic EEG pattern. Physical and neurological examinations are performed, but often no pathognomonic symptoms are detected, with the exception of tuberous sclerosis.

In the EEG, in the interictal period, as a rule, a picture of hypsarrhythmia (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal peak discharges) is revealed. Several options are possible (for example, modified - focal or asymmetric hypsarrhythmia). Ictal baseline EEG changes, interictal epileptiform activity is markedly weakened.

Tests to determine the cause of infantile spasms may include:

  • laboratory tests (for example, general analysis blood, determination of glucose in blood serum, electrolytes, urea, creatinine, Na, Ca, Mg, P, liver tests), if a metabolic disorder is suspected;
  • CSF analysis;
  • brain scan (MRI and CT).

Principle of treatment

The principles of treating West 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 German clinics. First of all, this is due to the diagnostic capabilities of large European clinics.

There is no universal treatment regimen for the syndrome in children. Regardless of the clinic in which the patient enters, therapy is selected taking into account the individual characteristics of the manifestation of the disease.

The prognosis will depend on how the patient responds to therapy. In the vast majority of cases, conservative treatment is practiced.

Medical therapy


Pyridoxine injections are prescribed to prevent vitamin B6 deficiency.

The main drugs in the treatment of West syndrome:

  • anticonvulsant drugs;
  • steroid hormones;
  • B vitamins.

With West syndrome, simultaneous therapy with ACTH (adrenocorticotropic hormone) and prednisolone is used. Such treatment has shown high efficiency, however, long-term hormone therapy can adversely affect the growth and physical development of the child, as a result of which it is important to correctly draw up a treatment regimen and select dosages. This can only be done by an experienced neurologist.

Anticonvulsants are an important part of symptomatic therapy. With West syndrome, the same drugs are used as for epilepsy in children. Clonazepam, Topiramate, Epilim are used. Dosages are selected individually.

B vitamins play an important role in therapy. They are necessary for the regulation of nervous activity. Pyridoxine injections are usually prescribed.

West syndrome is also treated with exercise therapy and massage. These methods help to reduce the severity of seizures, but are used only in conjunction with conservative therapy.

Surgery

In the presence of an organic lesion of the brain, for example, a tumor neoplasm, surgical intervention is performed. It carries risks, like any intervention in the brain, but in some cases it is the only way to cope with the disease. The operation is carried out with benign tumors or sclerotic changes in brain tissues (Bourneville's disease).

Homeopathy


Many prefer homeopathy to treat childhood illnesses, but West syndrome must be treated conservatively. The effectiveness of homeopathy is insufficient, moreover, it is impossible to be sure of the contents of such preparations. To date, there has not been a single confirmed case of a cure or improvement in the well-being of a child with West syndrome when taking homeopathic remedies. No need to self-medicate.

Causes

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

  • Prenatal grounds, which include infections and inflammation 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 activity;
  • Postnatal root causes - infectious diseases of the central nervous system, trauma to the spine and head of a different nature, oncological diseases brain, ischemic stroke.

Causes of the disease

In the vast majority of cases, West syndrome is symptomatic, and can be caused by a fairly large number of etiological factors, including hereditary ones.

The causes of West syndrome are as follows:

  • postnatal encephalitis;
  • intrauterine infections;
  • fetal hypoxia;
  • premature birth;
  • intracranial trauma to the fetus;
  • birth injury;
  • asphyxia of the newborn;
  • postnatal ischemia due to cord entanglement;
  • anomalies in the structure of the brain;
  • septal dysplasia;
  • point gene mutations.

Unfortunately, West syndrome is characterized by a high percentage of deaths, but if treatment is started in a timely manner, a long stage of remission can be achieved. At the same time, it should be noted that the development of such a pathology leads to a lag in psychomotor development, and it is not possible to completely eliminate this factor.

What to do?

If you think that you have West syndrome and symptoms characteristic of this disease, then doctors can help you: pediatrician, neonatologist, neurologist.

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

Description of the disease and ICD-10 code This disease has the following ICD-10 code: G40.2.

When a child begins to shake in 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 more chances the child has for a full recovery and a happy future.

What is West Syndrome

West syndrome is a severe form of epilepsy that has developed in young children due to brain damage and some other serious diseases. Characteristic features This pathology is considered mental retardation, as well as infantile spasms - nods or rapid tilts of the body, usually during falling asleep or waking up. At the same time, hypsarrhythmia is recorded on the encephalogram - abnormal high-amplitude activity of the brain.

West syndrome affects from 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 the young representatives of the stronger sex.

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

As a rule, newborns who are subsequently diagnosed with such a diagnosis are born outwardly healthy or with minor abnormalities. The debut of the disease falls on 3–7 months of life: during this period, pathology is diagnosed in 77% of patients. In children older than 1 year, the syndrome occurs only in 10% of cases.

Among patients with West syndrome, the mortality rate is high, and in surviving babies, by the age of 3, convulsions turn into another form of epilepsy, most often Lennox-Gastaut syndrome. With adequate treatment, complete remission can be achieved, but mental retardation usually occurs 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 pathological condition Key words: brain damage, genetic factors. With this form, children have an initial delay in psychomotor development, the patient is tormented by several types of seizures at once, 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 species is characterized by only type 1 seizures, structural changes in the brain are not visualized, and developmental delay occurs only after the onset of the disease, when its manifestations become apparent. The cryptogenic variety has a relatively favorable prognosis for life and a complete cure, proceeding in a milder form.

With West syndrome, cramps cover almost all muscles, including the neck, head and limbs. Contractions are usually symmetrical for the left and right sides bodies last 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 lesions, the following types of seizures are distinguished in West syndrome:

  • occipital - extensor convulsions of the neck with tilting of the head;
  • nodding (flexion) - contractions of the flexor muscles located on the arms and neck: nodding with the head, joining hands in front of the chest, etc .;
  • common (extensor) - convulsions covering the whole body: the arms and legs are spread out to the sides, resembling the manifestations of the Moro reflex.

If the seizures are repeated too often, the child may fall asleep immediately after they stop. Prolonged convulsions greatly affect development: the baby begins to lag behind his peers more and more in motor, mental and mental plans.

Causes

The infantile spasms that are characteristic of West syndrome occur due to improper interaction between the brain stem and its cortex. The immaturity of the central nervous system provokes violations of the 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 the muscle contractions typical of West's syndrome.

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

  • hypoxia during childbirth or prenatal period;
  • congenital malformations of the brain;
  • birth trauma;
  • 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 impetus for the onset of the disease is the intake of nootropics or vaccination, but these factors act as a catalyst, and not an independent cause. If the child's body is not functioning properly, West's syndrome will manifest itself sooner or later, but anything can serve as a trigger: from vaccinations to a stressful situation.

Symptoms and signs

The first sign of West syndrome may be the loud, inconsolable crying of a child. At the same time, district doctors attribute everything to intestinal colic. The correct diagnosis is made only when other alarming symptoms appear:

  • absence or slowdown in the pace of psychomotor development: the child does not roll over and does not 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. Often it seems to parents that he does not see anything at all;
  • muscle weakness (hypotension);
  • tearfulness and irritability;
  • the appearance of spasms.

Seizures are a typical manifestation of West syndrome. There are the following types of convulsions:

  • myoclonic - small symmetrical twitches 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, advancing 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 are noted during falling asleep and waking up, but provoking factors can be loud sounds, fright, 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 as sudden stops or falls of the child, respiratory failure, nystagmus, or twitching of the eyeballs. Before an attack, the baby may be frightened and scream, and after it - become lethargic and sleepy.

On the electroencephalogram (EEG), hypsarrhythmia is observed - 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 disorders:

  • trembling of the fingers;
  • sagging muscles;
  • dizziness;
  • inability to perform fast and complex movements;
  • a symptom of the absence of a "reverse shock".

Symptom of the absence of a "reverse push": the patient bends his arm with force at the elbow joint. The examiner tries to unbend it, which the patient resists by holding the arm in a bent position. Then the examiner suddenly stops the extension, and the patient's hand hits the chest with force.

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

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

  1. The first group includes children diagnosed with hypsarrhythmia, but there are no visible symptoms of the disease. Such babies should undergo an annual examination and do not need treatment.
  2. The second risk group includes children with the main signs of West syndrome and characteristic changes in 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 babies with West syndrome a chance to improve the quality of life, and sometimes to fully restore 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 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 determining the cause of West's syndrome;
  • PET of the brain - to determine the foci of hypometabolism in the brain tissues;
  • cerebral angiography - to detect cerebrovascular pathologies;
  • cranioscopy - to study defects in the structure of the skull.

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

  • infantile myoclonus;
  • infantile myoclonic epilepsy;
  • benign rolandic epilepsy;
  • Sandifer's syndrome;
  • various ticks.

Manifestations of West syndrome are often similar to a tick, however, in this disease, muscle spasms are provoked by emotional outbursts, and there are no abnormalities 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 carrying out all the necessary examinations.

Treatment of West syndrome

With early diagnosis and proper treatment stable remission of the disease can be achieved in more than 50% of cases. However, it happens that taking medication does not affect the number and intensity of seizures 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.

Medical therapy

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

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

Most often, vigabatrin is 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 high doses acts like an anticonvulsant in some patients.

Anticonvulsants prescribed for West syndrome - gallery

To improve the physical and psycho-emotional development, doctors usually prescribe drugs to children that normalize the metabolism and blood supply to the brain. Nootropics are recommended for babies 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 give strong side reactions. 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 medicines, usually prescribes drugs that increase 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 hormone therapy, treatment is carried out in a hospital setting.

If the doses of the drugs were chosen correctly and the patient has a positive trend, treatment should be continued for about 1.5-2 years from the moment of the last attack.

Surgical intervention

If infantile spasms are resistant to ongoing 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 dissects the adhesions of the meninges, removes tumors and vascular aneurysms, while trying to use the most sparing surgical methods.

If convulsions are manifested in the form of sudden falls, the patient may be shown a callosotomy - an operation to cut the corpus callosum. After surgery, children usually undergo neurorehabilitation in specialized centers.

Physiotherapy

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

Exercise therapy must necessarily be carried out under the guidance of an experienced rehabilitator and only after such classes are allowed by the attending physician. Otherwise, you can aggravate the intensity of seizures and worsen general state child.

In parallel with the development of motor skills, the baby needs to regularly engage with a defectologist, psychologist and speech therapist, who will develop the speech and fine motor skills of the child.

Non-traditional methods

Stem cell therapy can be singled out among effective non-traditional methods. 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.

To date, few decide on this measure, although such therapy has already managed to prove itself well. Stem cell therapy is not a panacea, but it helps some patients.

Nutrition Features

Many foreign doctors achieve good results in the treatment of West syndrome using a ketogenic diet. It is based on an increase in the diet of fats and a reduction in 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 seizures.

The ketogenic diet helps about 70% of patients, and seizures are reduced by more than 2 times, and in some children, convulsions disappear altogether. As a rule, the diet is prescribed for babies from 2 years old 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 fully recover. The remaining patients have certain deviations in physical and mental development.
  2. In the symptomatic form, the prognosis is much worse. The absence of consequences is observed only in 5–12% of cases, and mortality can reach 25%. Even with the onset of remission of the disease, children develop mental retardation, cerebral palsy, autism, mental retardation, many experience learning difficulties, have problems with memory, concentration and logical thinking. Approximately half of the patients present with movement disorders. Such a pessimistic prognosis is due to the negative impact on the body of the underlying disease. It is on its course that the life expectancy of the patient depends.

The prognosis will be more favorable with timely treatment. If the medications are chosen correctly from the first weeks of the disease, 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 later or later after the onset of seizures, the chances of recovery will be minimal.

The condition of the child can worsen the uncontrolled intake of nootropics and vaccination.

There is no cure 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 development of the child as close as possible to the age norm.

Dr. Komarovsky about seizures in children - video

West syndrome is an insidious and dangerous disease. The percentage of infant mortality with it is quite high, therefore, at the first alarming symptoms, it is necessary to consult a doctor and diagnose as quickly as possible. The correct selection of drugs, together with competent rehabilitation with the participation of psychologists, defectologists, speech therapists and exercise therapy specialists, increases the child's chances of a full recovery.

This type of seizure is usually combined with hypsarrhythmia on the EEG.

Seizures may go away on their own around age 5, but may morph into other types of seizures.

The pathophysiology of infantile spasms is not fully understood, but these seizures may reflect a disruption in the interaction between the cortex and the brainstem. Infantile spasms may be caused by CNS immaturity, brain malformations, and brain damage in the first months of life. A common cause 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 trunk and limbs, sometimes within seconds. Spasms range from slight nodding of the head to shuddering of the whole body. They are accompanied by flexion (flexion), extension (extension) or, most often, both flexion and extension in the muscles of the limbs (mixed spasms). Spasms usually recur throughout the day in clusters, often several dozen, mostly shortly after waking up, and sometimes during sleep.

As a rule, infantile spasms are accompanied by impaired motor and mental development. IN initial stages developmental regression is possible (for example, children may lose the ability to sit or roll over).

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

Diagnosis of infantile spasms in children

  • Neuroimaging.
  • Video-ZEGsleep and wakefulness.
  • Laboratory studies according to clinical indications.

The diagnosis is established on the basis of clinical symptoms and a characteristic EEG pattern. Physical and neurological examinations are performed, but often no pathognomonic symptoms are detected, with the exception of tuberous sclerosis.

In the EEG, in the interictal period, as a rule, a picture of hypsarrhythmia (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal peak discharges) is revealed. Several options are possible (for example, modified - focal or asymmetric hypsarrhythmia). Ictal baseline EEG changes, interictal epileptiform activity is markedly weakened.

Tests to determine the cause of infantile spasms may include:

  • laboratory tests (for example, complete blood count, determination of glucose in blood serum, electrolytes, urea, creatinine, Na, Ca, Mg, P, liver tests), if a metabolic disorder is suspected;
  • CSF analysis;
  • brain scan (MRI and CT).

Treatment of infantile spasms in children

Infantile spasms are difficult to treat, and the optimal treatment regimen is debatable. Apply ACTH 20-60 units intramuscularly once a day. Many anticonvulsants are ineffective; valproate is preferred, the second choice is clonazepam. The effect of the use of nitrazepam, topiramate, zonisamide or vigabatrin has also been noted.

The ketogenic diet can also be effective, but difficult to maintain.

In some cases, surgical treatment may be successful.


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 neuropsychic development of the child is disturbed. 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 the normal functioning of the brain, characterized by sharp contractions of the muscle tissue of the upper body. Such spasms are referred to as epileptic encephalopathy. Their peculiarity is that they are extremely short in time, 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, cooing stops. A spasm develops, affecting either one side of the body, or the whole body, the head deviates, the eyes are diverted to the side.

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

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

  • Flexor view - intense spasm of the flexor muscles of the body, arms and legs;
  • Extensor view - increased spasm of the muscle tissue responsible for the extensor function, characterized by simultaneous, upper and lower extremities.
  • Flexor-extensor type - a mixed type of spasm of muscle tissue with alternating extension and flexion of body parts;
  • Asymmetric spasms - spasm of the muscle tissue of one side of the body. Characterized by the appearance of severe pathologies.

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

  • Symptomatic - these are spasms with an established etiology, characterized by a deviation in mental and nervous development after the manifestation of spasms, neurological disorders, the study clearly shows pathological abnormalities in the structure of the brain;
  • Cryptogenic - these are spasms of unknown etiology, the child has a normal neuropsychiatric development before the onset of the disease, a certain type of convulsive processes is characteristic. With focal lesions is not detected;
  • Idiopathic - these are convulsive seizures that begin in the neonatal period and in childhood. They have a benign course, the rhythm of the transmission of nerve impulses is not disturbed, 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 in which infantile spasms occur, they have:

  • Prenatal grounds, which include infections and inflammation 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 activity;
  • Postnatal root causes - infectious diseases of the central nervous system, trauma to the spine and head of a different nature, oncological diseases of the brain, ischemic strokes.

Infantile spasms have a major difference from others epileptic seizures. The spasm is sudden and short in time, begins with unreasonable children's crying or a complete lack of vigorous activity: movements, speech. Before the onset of an attack, children are irritable.

Video