home→Diagnostics→ As they understood that the child had infantile spasms. Types of epileptic seizures (video)

As they understood that the child had infantile spasms. Types of epileptic seizures (video)

Convulsions in children of the first year of life.
Cramps are chaotic, mostly painful, contractions of various muscle groups.
The reasons that lead to the appearance of seizures in children are quite diverse. The main ones are the following:
1. Infectious diseases. Meningitis, encephalitis, brain abscesses lead to brain damage and disruption of the nerve impulse.
2. Mother's drug addiction during pregnancy. Narcotic substances disrupt the process of intrauterine brain formation, therefore, children born to drug addict mothers may experience seizures.
3. Endocrine diseases. Diabetes, diseases thyroid gland, adrenal glands can cause seizures in a child at any age.
4. Burdened heredity. Some genetic diseases lead to a violation of the development of the brain, as a result of which the development of a convulsive syndrome in a child can be observed.
5. Tumor lesions of the brain cause a violation of the conduction of a nerve impulse along the nerve fibers, which causes convulsions in children.
6. Lack of calcium.
7. Misapplication medicines. Some drugs, such as diuretics, cause a decrease in calcium in the blood, which causes seizures. Also, the appearance of seizures is observed with an overdose of vitamin D3 and the development of a condition such as spasmophilia.
8. A cramp may appear during hypothermia (for example, it will cramp a limb into cold water). But if this happens often, you need to see a doctor.
For convulsions, you can take an attack of epilepsy, therefore, when diagnosing, this disease must also be taken into account.

In children older than 1 month, the following types of seizures are more common:
1. Primary generalized (tonic-clonic, like grand mal). They are characterized by a tonic phase lasting less than 1 minute, with the eyes rolling up. At the same time, gas exchange decreases (due to tonic contraction of the respiratory muscles), which is accompanied by cyanosis. The clonic phase of seizures follows the tonic phase, resulting in clonic twitching of the limbs (usually 1-5 minutes); gas exchange is improved. May be noted: hypersalivation, tachycardia, metabolic/respiratory acidosis. The postictal state often lasts less than 1 hour.
2. Focal motor convulsions (partial, with simple symptoms). They are characterized by the occurrence in one of upper limbs or on the face. Such convulsions lead to deviation of the head and abduction of the eyes in the direction of the hemisphere opposite to the localization of the convulsive focus. Focal seizures can begin in a limited area, without loss of consciousness, or, conversely, generalize and resemble secondarily generalized tonic-clonic seizures. Indications for the focus are Todd's paralysis or abduction of the head and eyes towards the affected hemisphere. They appear after an attack of these convulsions.
3. Temporal or psychomotor cramps (partial, with complex symptoms). In about 50% of cases, they are preceded by an aura. They may mimic other types of seizures, be focal, motor, grand mal, or stare. Sometimes they look more complex: with stereotypical automatisms (running - for those who have begun to walk, laughter, lip licking, unusual movements of the hands, facial muscles, etc.).
4. Primary generalized absence seizures (like petit mal). Rarely develop in the first year of life (more typical in children older than 3 years).
5. Infantile spasms (with hypsarrhythmia - according to EEG data). More often appear in the 1st year of life, characterized by severe myoclonic (Salaam) spasms. Infantile spasms (West syndrome) may develop due to the presence of various neurological pathologies or without any obvious previous disorders. With infantile spasms, psychomotor development slows down, and in the future there is a high probability of a pronounced developmental delay.
6. Mixed generalized convulsions (small motor or atypical petit mal). This group of seizure disorders is typical of Lennox-Gastaut syndrome, which is characterized by frequent, poorly controlled seizures, including atonic, myoclonic, tonic, and clonic, accompanied by an EEG pattern with atypical spikes (from English Spike - peak) and waves (less than three spike-waves in 1 s), multifocal spikes and polyspikes. The age of patients more often exceeds 18 months, but this syndrome can develop in the 1st year of life after infantile spasms (transformation from West syndrome). Children often have a marked developmental delay.
7. Febrile convulsions (FS). Observed in children, starting from 3 months of age, with an increase in body temperature (> 38.0 ° C). As a rule, they are primary generalized tonic-clonic, although they can be tonic, atonic or clonic.
Febrile convulsions are considered simple if they occurred once, lasted no more than 15 minutes, and there were no focal symptoms. Complex febrile convulsions are characterized by repeated occurrence, duration and presence of pronounced foci. All patients under 12 months of age should undergo lumbar puncture and metabolic screening to determine the cause of seizures.
Risk factors for developing epilepsy in FS include:
- indications of the presence of neurological disorders or disorders of psychomotor
development;
- a family history of afebrile seizures;
- the complex nature of febrile convulsions.
In the absence or presence of only one risk factor, the likelihood of developing afebrile seizures is only 2%. In the presence of two or more risk factors, the likelihood of epilepsy increases to 6-10%.

Treatment.

Treatment of convulsive syndrome in children should begin with the provision of first aid. General principles this help is given below.

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

Drug therapy of particular cases of convulsive syndrome is based on the following principles:
Primary generalized convulsions (grand mal). Phenobarbital, phenytoin (epdantoin, epanutin), carbamazepine are usually used. As an alternative, in some cases, valproates (depalept, depakine) or acetazolamide can be used.
Partial simple convulsions (focal).
Phenobarbital, phenytoin (epdantoin, epanutin), carbamazepine, primidone are used. As other therapeutic agents, if necessary, can be used (valproic acid preparations (depakin, depalept), vigabatrin (sabril, sabrilex, sabrilan), keppra, etc.)
Partial complex convulsions (temporal lobe epilepsy) . The primary appointment of carbamazepine, phenytoin and primidone is envisaged. Alternative drugs are phenobarbital, valproate and acetazolamide (as well as metsuximide, ethosuxemide, petinimide, zarontin).
Primary generalized convulsions (petit mal, absence seizures) . The main antiepileptic drugs AED in the described clinical situation are ethosuximide, valproates, metsuximid. Other agents: acetazolamide, clonazepam, clobazam, phenobarbital.
Infantile spasms . Most effective drugs for the treatment of infantile spasms are: a synthetic analogue of ACTH - synacthen depot, vigabatrin (sabril, sabrilex, sabrilan), valproates (depalept, depakine), keppra, ethosuxemide (zarontin, petnidan, petinimide), clobazam (frizium). Other therapies include the use of phenytoin (epdantoin, epanutin), talox, phenobarbital, acetazolamide. If available, a ketogenic diet (KD) may be used.
Febrile convulsions. The advisability of prescribing anticonvulsants to children with FS has been extremely debatable for many years. Nevertheless, when making a decision in favor of conducting preventive therapy using AEDs, phenobarbital preparations are most often used, less often valproates.
Mixed generalized convulsions.
The main AEDs: phenobarbital, valproate, clonazepam, clobazam (frizium). As alternatives, acetazolamide, diazepam, ethosuximide, phenytoin, metsuximide, carbamazepine, as well as tranxen and others can be used.
Dosing of the main anticonvulsants (in the 1st year of life)
- diazepam - 0.1-0.3 mg / kg up to maximum dose 5 mg intravenously slowly;
- phenytoin - 5 mg / kg / day (2 times, per os);
- phenobarbital - 3-5 mg / kg / day (2-3 times, per os);
- primidone - 5-25 mg/kg/day (1-2 times);
- carbamazepine - 15-30 mg / kg / day (2-3 times, per os);
- ethosuximide - 20-30 mg / kg / day (2 times);
- metsuximide - initial dose 5-10 mg/kg, maintenance - 20 mg/kg (2 times, per os);
- valproates - 25-60 mg / kg / day (2-3 times, per os);
- clonazepam - 0.02-0.2 mg / kg / day (2-3 times, per os);
- paraldehyde - 300 mg (0.3 ml/kg, rectally);
- acetazolamide (diacarb) - initial dose 5 mg/kg, maintenance - 10-20 mg/kg (per os).

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

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

Seizures in children older than 3 years
Primary generalized absence seizures- a type of seizure, mainly found in children of this age group. Their identification and adequate treatment is entirely within the competence of pediatric neurologists and epileptologists. Pediatricians and representatives of other pediatric specialties should not disregard the identified episodes of short-term "disconnection" of children (with no response to treatment) or complaints about peculiar episodes of "thoughtfulness".
Juvenile myoclonus epilepsy is a subtype of idiopathic generalized disease with impulsive petit mal seizures. The appearance of seizures is typical after 8 years of age. A distinctive feature is the presence of myoclonus, the severity of which varies from minimal (regarded as "clumsiness") to periodic falls. No impairment of consciousness is noted. However, most of these patients have sporadic tonic-clonic seizures, with absences occurring in about one third of children with this type of epilepsy.
catamenial epilepsy.
A group of chronic paroxysmal conditions associated with menstrual cycle. They can be given as one example of age-dependent convulsive conditions in relation to female patients who have reached puberty.
Febrile seizures can occur in children older than 3 years (in preschool age), although during this period of life they occur with a much lower frequency. The presence of episodes of afebrile convulsions in this pathology (without an increase in body temperature) indicates the development of symptomatic epilepsy, which must be treated in accordance with the principles formulated by the International League Against Epileptic (ILAE).

Lennox-Gastaut syndrome. Diagnostic criteria. Treatment.

Lennox-Gasto-epileptic encephalopathy of childhood, characterized by seizure polymorphism, cognitive impairment, specific changes in the electroencephalogram, and resistance to therapy.

types of seizures: paroxysms of falls, tonic seizures and atypical absences. Consciousness can be preserved or switched off briefly. After the fall, there are no convulsions, and the child immediately gets up. Frequent bouts of falls often lead to injury. Attacks include sudden flexion of the neck and trunk, raising the arms in a state of semi-flexion or extension, extension of the legs, contraction of the facial muscles, rotational movements of the eyeballs, apnea, flushing of the face. They can occur both during the daytime and, especially often, at night.

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

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

Debut 2-12 years old with 2 picks at 3 and 9 years old

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:

Explicit disturbances in psychomotor development.
EEG changes characteristic of West's syndrome (hypsarrhythmia).
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:

Limbs involuntarily diverge to the sides.
Generalized convulsions throughout the body, in the upper and lower extremities.
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. Upper and lower limbs during spasms, it scatters 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, as it manifests itself in 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

The diagnosis of West syndrome is made 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:

Low efficiency of light stimulation.
The timing of the main curves is not determined. There may be "peaks of the curve" in the process of wakefulness or sleep.
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 pathological conditions 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 drugs - 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

Physiotherapy with West syndrome, it 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. All depending on the etiology infantile spasms 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

Infantile spasms usually occur in children under 1 year of age.
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 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 medications 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 violation

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:

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.
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.
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.
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:

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).
Finger trimmer.
Muscles are sluggish and relaxed.
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 occur 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 is revealed (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal peak discharges). 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).

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 organic damage 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.

Convulsions with hypoxia and intracranial birth trauma. They are most common in newborns. Hypoxia, usually accompanied by impaired cerebral hemo- and liquorodynamics, leads to general or local cerebral edema, acidosis, and diapedetic hemorrhages.

Seizures in these children appear immediately after birth or on the 2-3rd day, with subarachnoid hemorrhage, they occur most often after being applied to the chest. Convulsions develop against the background of neurological disorders: anxiety, sleep disorders, increased muscle tone and tendon reflexes, inhibition of unconditioned reflexes, difficulty in sucking and swallowing, cranial nerve paresis. They are most often clonic in nature, starting in the muscles of the face and then spreading to the extremities. The course of seizures is different. They can completely stop in the maternity hospital or reappear after a few months. Sometimes, starting in the maternity hospital, they are periodically repeated.

Convulsions with anomalies in the development of the nervous system. Microcephaly, hydrocephalus, porencephaly, atrophy of the cerebral cortex, cerebellar hypoplasia may be accompanied by convulsions during the neonatal period. Malformations are often combined with intrauterine hypoxia, birth asphyxia and intracranial birth trauma. Convulsions are tonic-clonic in nature and occur against the background of pronounced focal changes in the nervous system (paresis, paralysis, a sharp inhibition of unconditioned reflexes, malnutrition). Neuroradiological studies confirm the diagnosis.

Convulsions in infectious diseases. In the neonatal period, convulsions are most often observed with sepsis. They also occur in 30% to 50% of neonates with meningitis and usually occur when there is marked change in the cerebrospinal fluid. Convulsions begin with twitching of the eyes, facial muscles, and then, as the severity of the condition increases, they become generalized. Convulsive attacks in meningitis are accompanied by fever, inflammatory changes in the blood and cerebrospinal fluid.

Seizures in young children

After a period newborns the motor component of the seizure becomes more pronounced. However, in infants, the classic sequence of convulsive seizures, as well as in newborns, is rarely observed. Psychomotor automatisms are less common than other forms of seizures and are difficult to diagnose at an early age.

Myoclonic type of seizures(minor propulsive seizures or infantile spasms) occur mainly in infants. The frequency of propulsive seizures is 1:4000-6000 newborns, among children of the 1st year of life with convulsive syndrome, they make up 30.8%. This type of convulsions is characterized by: lightning-fast convulsive paroxysms; impaired mental function; specific EEG changes. The classic picture of infantile spasms is characterized by bilateral symmetrical muscle contraction. Spasms are flexor, extensor or mixed type.

With flexor spasm there is a sudden flexion of the neck, trunk and limbs with their simultaneous abduction or adduction of the latter. The mixed type is characterized by flexion or extension of the torso, arms and legs are unbent. Flexor spasm is the most common, more rarely mixed and even more rarely extensor. The same child may have different forms of spasms at the same time. Infantile spasms also include partial fragmentary forms - nodding, shuddering, flexion and extension of the arms and legs, turning the head. In this case, lateralization is possible - a predominant contraction of the muscles of one side of the body. Nods look like a quick tilt of the head forward. They are often combined with tremors and precede or replace flexor or extensor spasm. The most characteristic feature of infantile spasms is the tendency to seriality. Single spasms are less often observed. The duration of convulsions is from a fraction of a second to several seconds. The duration of a series of attacks can be from a few seconds to 20 minutes or more. During the day, the number of paroxysms ranges from single to several hundred and even thousands. Turning off consciousness in this form of convulsions is short-term.

Infantile spasms sometimes accompanied by a scream, a grimace of a smile, a frightened expression, rolling of the eyes, nystagmus, dilated pupils, trembling of the eyelids, limbs, pallor or redness of the face, respiratory arrest. After convulsions, drowsiness is observed, especially if the series of attacks was prolonged. In the interictal period, children are irritable, tearful, sleep is disturbed. Seizures occur most often before falling asleep or after waking up. The factors provoking paroxysms include fear, various manipulations, feeding.

Infantile spasms begin in infancy and disappear in early childhood. Before the age of 6 months, they account for 67% of the total number of paroxysmal episodes; from 6 months to 1 year - 86%; after 2 years - 6%.

Infantile spasms may be the first manifestation of a convulsive syndrome in a child. The first attacks are abortive in nature and can be mistaken by parents for a fright reaction, a manifestation of abdominal pain, etc. At first they are single, then their frequency increases. At this stage, there may be remissions and exacerbations that are difficult to foresee. As the child grows and develops, the frequency of seizures decreases. The average duration of infantile spasms is from 4 to 30-35 months. After 3 years they are rare. According to P. Jeavons et al. (1973), in 25% of children, infantile spasms stop before the age of 1 year, in 50% - up to 2 years, in the rest - up to 3-4, sometimes 5 years.

Changes to EEG(hypsarrhythmia) do not always correlate with the onset of seizures, sometimes they appear a little later. Hypsarrhythmia is characteristic of the developing brain and is observed only in infantile spasms in young children.

Universal accompanying symptom of infantile spasms is a mental retardation, which is observed in 75-93% of patients, the formation of motor skills is also impaired. Therefore, in young children, it is more correct to speak of a delay in psychomotor development, which is noted already in the initial stage of the disease. It becomes more distinct when a series of seizures appear. The degree of delay depends both on the time of onset of seizures and on the premorbid characteristics of the child. Normal psychomotor development before the onset of seizures is observed in 10-16% of children.

Focal neurological disorders(paresis, paralysis, strabismus, nystagmus) occur in 34-70% of cases. As a rule, they are observed in children with cerebral palsy, microcephaly, anomalies in the development of the central nervous system.

Prognosis for infantile spasms favorable in children with normal psychomotor development, with short-term convulsions uncomplicated by other forms of seizures. With an early onset, seriality and duration, combined with other types of seizures, the presence of neurological and mental disorders, a deep delay in psychomotor development is observed.

Absences- a form of small seizures, which are also observed in young children and are characterized by a short stop of the gaze. Sometimes at this time the child produces sucking, chewing movements, smacking, licking with the tongue. The attack may be accompanied by redness or blanching of the face, slight abduction of the eyeballs. They are less common than propulsive seizures.

Major seizures in children early age are more often abortive in nature. The structure of the seizure is dominated by the tonic component. When turning the head to the side, infants are often fixed in an asymmetrical posture. Attacks may be accompanied by fever, vomiting, abdominal pain and other autonomic symptoms. Involuntary urination is less common than in older children. After the seizure, the child is lethargic, stunned, falls asleep or, on the contrary, excited, muscle hypotension is pronounced.

Partial convulsions in children early age are manifested by clonic twitches of facial muscles, muscles of the tongue, distal extremities. A seizure, starting locally, can turn into a generalized one. Often at this age, adversive convulsive seizures are observed, accompanied by a turn of the head and eyes, and sometimes the body, to the side. The seizure is often accompanied by tonic tension of the arm and leg on the side of the turning of the head.

Infantile spasms are convulsions characterized by sudden flexion of the torso forward, flexion or extension of the arms, extension or flexion of the legs.

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.

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; clonazepam is the second-line drug of choice. 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.

Attention, only TODAY!

What's happened infantile spasms we learn from this article...

The clinical and electroencephalographic characteristics of epileptic seizures in children may vary with age. Infantile spasms are a good example of such age-dependent phenomena. This is, so to speak, a unique type of seizure associated with early childhood. Criteria that characterize West's syndrome.

West's syndrome belongs to epileptic syndromes, but at the same time it is singled out as a separate disease according to a number of criteria, the main of which is age dependence. In its vast majority of cases, this disease occurs in infancy (up to a year). True, later Clinical signs diseases, under the age of five years, and isolated cases in adults.

Infantile spasms are a specific age-related phenomenon that occurs in children only in the first two years of life, most often between 4 and 6 months, and in approximately 90% of patients before 12 months. The incidence of West syndrome is estimated at 0.4 per 1,000 live births. And although the spasm itself usually lasts only 1-2 seconds, they usually occur in a series of “attack-break-attack”, and such breaks are usually 5-10 seconds.

During such spasms, the child's whole body suddenly becomes very tense, the arms describe an arc, the legs and head may bend forward. But despite this, infantile spasms can sometimes be very difficult to notice due to the fact that they can be manifested only by rolling the eyes up and a slight contraction of the abdominal muscles. More often, infantile spasms appear immediately after the baby wakes up, and much less often they occur during sleep.

Shortly after the onset of seizures, parents may notice a number of specific changes in their child:

Previously learned skills may be lost (the child stops rolling over, sitting, crawling, babbling, although he has already mastered these stages of development well).
Loss of social interaction skills and smiles.
Increased tearfulness or vice versa, silence unusual for a child.
Note: Talk to your pediatrician if your child is not currently at the appropriate developmental milestones. Trust your instincts, you know your child best!

What do infantile spasms look like?

As a rule, each spasm lasts less than 1 second, during each spasm the child shows surprise on his face, his eyes freeze, his arms rise and spread slightly to the sides. Between each spasm, the baby will look good, which is very typical of infantile spasms. A typical interictal EEG pattern in infantile spasms is hypsarrhythmia.

The term "hypsarrhythmia" was first introduced by F. Gibbs and E. Gibbs in 1952 to describe the interictal EEG pattern characteristic of infantile spasms. They described hypsarrhythmia as follows:

Rare slow waves and high voltage spikes. Such spikes change from time to time both in duration and localization.

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:

idiopathic, 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. Sometimes they look focal, after a few seconds it seems that they come from many foci.

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 on the characteristics of the clinical manifestations and 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.

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

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. Computed tomography (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:

Treatment of infantile spasms.

Therapeutic therapy of infantile spasms is carried out with the help of adrenocorticotropic hormone preparations - a synthetic version of the biological substance produced by the glands of the endocrine system, stimulates the performance of vital body functions. As a result of the application, the frequency of seizures is noticeably reduced, and as a rule, hypsoarrhythmic processes improve.

Treatment is also with steroid medications to relieve childhood seizures.
Medicines to reduce the frequency and strength of seizures - anticonvulsants: valproic acid, nitrazepam.
Complex vitamin preparations group B to improve brain activity.

In cases of detection of a focal lesion, a decision can be made to promptly remove the pathology or a corpus callosum is transected. True, at this stage in the development of medicine, it is impossible to cure infantile spasms in children, since their progress smoothly turns into another type of epileptic seizures. The development of the central nervous system of the child is difficult, pathological changes in brain tissue occur. But it is possible to improve the quality of life with the help of qualified assistance and treatment without delay.

Prognosis of infantile spasms.

If infantile spasms are completely eliminated with treatment, many of these children may develop other types of epilepsy, as well as intellectual or other developmental disabilities later in life. The sooner treatment is taken, the better the prognosis.

In children who had normal development before the onset of infantile spasms, with timely therapy, even a complete recovery is possible without any consequences in the future.