What can be confused with infantile spasms. What is West syndrome: causes of the disease, symptoms and signs, methods of treating the disease

Antipyretics for children are prescribed by a pediatrician. But there are emergency situations for fever when the child needs to be given medicine immediately. Then the parents take responsibility and use antipyretic drugs. What is allowed to give to infants? How can you bring down the temperature in older children? What medicines are the safest?

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. Seizures 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 developmental anomalies 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; delay mental development; 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 start at 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 when infantile spasms in children early age.

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 already noted in initial stage diseases. 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 early onset, seriality and duration, combination with other types of seizures, the presence of neurological and mental disorders there is a deep delay in psychomotor development.

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 eyeballs. They are less common than propulsive seizures.

Major seizures in children early age are more often abortive in nature. The tonic component predominates in the structure of the seizure. 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 departments limbs. 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.

Serial spastic contractions in individual muscle groups or of a generalized nature, occurring against the background of a delay in neuropsychic development and accompanied by a hypsarrhythmic EEG pattern. Manifests at the age of 4 years, mainly in the 1st year of life. In most cases, it is symptomatic. Diagnosis of the syndrome is based on clinical findings and EEG results. To identify the underlying pathology, CT or MRI, PET of the brain, consultation of a geneticist, neurosurgeon are required. Treatment is possible with antiepileptic drugs, steroids (ACTH, prednisolone), vigabatrin. According to the indications, the issue of surgical treatment (callosotomy, removal of the pathological focus) is resolved.

General information

West's syndrome is named after the doctor who observed its manifestations in his child and first described it in 1841. In connection with the manifestation of the syndrome at an early age and the course of convulsions as a series of individual spasms, the paroxysms characterizing West's syndrome were called infantile spasms. Initially, the disease was classified as generalized epilepsy. In 1952, a specific hypsarrhythmic EEG pattern was studied, which is pathognomonic for this form of epilepsy and is characterized by slow-wave asynchronous activity with random spikes of high amplitude. In 1964, specialists in the field of neurology identified West's syndrome as a separate nosology.

The introduction of neuroimaging into neurological practice made it possible to determine the presence of focal lesions of the brain substance in patients. This forced neurologists to reconsider their views on West's syndrome as generalized epilepsy and classify it in a number of epileptic encephalopathies. In 1984, the evolution of the epileptic form of encephalopathy from its early variant to West syndrome, and over time to Lennox-Gastaut syndrome, was revealed.

Currently, West syndrome accounts for about 2% of all cases of epilepsy in children and about a quarter of infantile epilepsy. The prevalence is, according to various sources, from 2 to 4.5 cases per 10 thousand newborns. Boys get sick somewhat more often (60%). 90% of cases of manifestation of the syndrome occur in the 1st year of life, with a peak at the age of 4 to 6 months. As a rule, by the age of 3 years, muscle spasms disappear or transform into other forms of epilepsy.

Causes of West Syndrome

In the vast majority of cases, West syndrome is symptomatic. It can occur due to intrauterine infections (cytomegaly, herpes infection), postnatal encephalitis, fetal hypoxia, premature birth, intracranial birth trauma, newborn asphyxia, postnatal ischemia due to late clamping of the umbilical cord. West syndrome may be the result of anomalies in the structure of the brain: septal dysplasia, hemimegaloencephaly, agenesis corpus callosum etc. In some cases, infantile spasms are a symptom of phakomatoses (pigment incontinence syndrome, tuberous sclerosis, neurofibromatosis), point gene mutations or chromosomal aberrations (including Down syndrome). Cases of phenylketonuria with infantile spasms are mentioned in the literature.

In 9-15%, West syndrome is idiopathic or cryptogenic, i.e. its underlying cause is not established or obvious. Often, the presence of cases of fibril convulsions or epileptic seizures in the family history of a sick child is traced, that is, there is a hereditary predisposition. A number of researchers indicate that vaccination, in particular the introduction of DPT, may be a factor provoking West's syndrome. This may be due to the coincidence of the timing of vaccination and the age of the typical onset of the syndrome. However, reliable data confirming the provocative role of vaccines have not yet been obtained.

Pathogenetic mechanisms of the occurrence of infantile spasms are the subject of study. There are several hypotheses. One of them links West's syndrome to a disorder in the functioning of serotonergic neurons. Indeed, patients have a decrease in the level of serotonin and its metabolites. But it is not yet known whether it is primary or secondary. An immunological theory linking West's syndrome to an increase in the number of activated B cells has also been discussed. The positive therapeutic effect of ACTH formed the basis of the hypothesis of malfunctions in the "brain-adrenal" system. Some researchers suggest that the syndrome is based on an excessive amount (hyperexpression) of excitatory synapses and conductive collaterals, which form an increased excitability of the cortex. They associate the asynchrony of the EEG pattern with the lack of myelin, which is physiological for this age period. As the brain matures, its excitability decreases and myelination increases, which explains the further disappearance of paroxysms or their transformation into the Lennox-Gastaut syndrome.

Symptoms of West Syndrome

As a rule, the symptom of West debuts in the first year of life. In some cases, its manifestation occurs at an older age, but not later than 4 years. The basis of the clinic is serial muscle spasms and impaired psychomotor development. The first paroxysms often appear against the background of an already existing delay in psychomotor development (ZPR), but in 1/3 of cases they occur in initially healthy children. Deviations in neuropsychological development are most often manifested by a decrease and loss of the grasping reflex, axonal hypotension. There may be a lack of eye tracking of objects and a disorder of gaze fixation, which is a prognostically unfavorable criterion.

Muscle spasms are sudden, symmetrical and short-term. Their seriality is typical, while the interval between consecutive spasms lasts at least 1 minute. Usually there is an increase in the intensity of spasms at the beginning of the paroxysm and its decline at the end. The number of spasms that occur per day varies from units to hundreds. The most common occurrence of infantile spasms occurs during falling asleep or immediately after sleep. Sharp loud sounds and tactile stimulation are capable of provoking paroxysm.

The semiotics of paroxysms that accompany West's syndrome depends on which muscle group is contracting - extensor (extensor) or flexor (flexor). On this basis, spasms are classified into extensor, flexor and mixed. Most often, mixed spasms are observed, then flexion, most rarely extensor. In most cases, spasms of several types are observed in one child, and which spasm will prevail depends on the position of the body at the time of the onset of the paroxysm.

There may be a generalized contraction of all muscle groups. But more often local spasms are observed. So, cramps in the neck flexors are accompanied by nods of the head, spasms in the muscles of the shoulder girdle resemble a shrug. Typical is a paroxysm of the "jackknife" type, due to contraction of the abdominal flexor muscles. In this case, the body seems to be folded in half. Infantile spasms of the upper extremities are manifested by abduction and adduction of the arms to the body; from the side it seems that the child hugs himself. The combination of such spasms with paroxysm of the "jackknife" type is associated with the greeting "salaam" adopted in the East, therefore it was called the "salaam attack". In children who can walk, spasms can proceed according to the type of drop attacks - unexpected falls with the preservation of consciousness.

Along with serial spasms, West syndrome may be accompanied by non-convulsive seizures, manifested sudden stop motor activity. Sometimes there are paroxysms limited to twitching of the eyeballs. Possible respiratory failure due to spasm of the respiratory muscles. In some cases, asymmetric spasms occur, manifested by the abduction of the head and eyes to the side. There may be other types of epileptic seizures: focal and clonic. They are combined with spasms or have an independent character.

Diagnosis of West syndrome

West syndrome is diagnosed by the main triad of signs: attacks of cluster muscle spasms, delayed psychomotor development and hypsarrhythmic EEG pattern. The age of onset of spasms and their association with sleep are important. Difficulties in diagnosis arise with a late onset of the syndrome. During the diagnosis, the child is consulted by a pediatrician, pediatric neurologist, epileptologist, geneticist. West syndrome should be differentiated from benign infantile myoclonus, benign rolandic epilepsy, infantile myoclonic epilepsy, Sandifer's syndrome (head tilt like torticollis, gastroesophageal reflux, episodes of opisthotonus that can be mistaken for spasms).

Interictal (interictal) EEG is characterized by the presence of disorganized, chaotic, dynamically changing spike-wave activity, both during wakefulness and during sleep. Polysomnography reveals the absence of spike activity during the deep stages of sleep. Hypsarrhythmia is recorded in 66% of cases, usually in the early stages. Later, some organization of the chaotic EEG pattern is observed, and at the age of 2-4 years, its transition to the “sharp-slow wave” complexes. The most common ictal EEG pattern (i.e., the EEG rhythm during spasms) is generalized high-amplitude slow-wave complexes followed by inhibition of activity for at least 1 second. When registering focal changes on the EEG, one should think about the focal nature of the brain lesion or the presence of anomalies in its structure.

Treatment of West syndrome

West's syndrome was considered resistant to ongoing therapy until the discovery in 1958 of the effect of ACTH drugs on seizures. Therapy with ACTH and prednisolone leads to a significant improvement or complete cessation of infantile spasms, which is accompanied by the disappearance of the hypsarrhythmic EEG pattern. Until now, there are no unambiguous decisions among neurologists regarding the doses and duration of steroid therapy. Studies have shown that in 90% of cases, therapeutic success was achieved with the use of large doses of ACTH. The terms of therapy can vary within 2-6 weeks.

A new stage in the treatment of infantile spasms began in 1990-1992. after finding a positive therapeutic effect vigabatrin. However, the benefit of treatment with vigabatrin has so far been proven only in patients with tuberous sclerosis. In other cases, studies have shown greater effectiveness of steroids. On the other hand, steroid therapy is less well tolerated than vigabatrin and has a higher relapse rate.

Of the anticonvulsants, only nitrazepam and valproic acid have been shown to be effective. At individual patients described the therapeutic effect of large doses of vitamin B6, which was noted in the first weeks of therapy. In case of infantile spasms resistant to ongoing therapy, with the presence of a pathological focus confirmed by tomography, a consultation with a neurosurgeon is indicated to resolve the issue of resection of the focus. If such an operation is not possible, then in the presence of drop attacks, a total callosotomy (crossing of the corpus callosum) is performed.

Prognosis of West syndrome

Usually, by the age of 3, there is a regression and disappearance of infantile spasms. But in about 55-60% of cases, they transform into another form of epilepsy, most often Lennox-Gastaut syndrome. Pharmacoresistance is often noted in infantile spasms that accompany Down's syndrome. Even with successful relief of paroxysms, West syndrome has an unsatisfactory prognosis in terms of the psychomotor development of the child. Possible cognitive and behavioral disorders, cerebral palsy, autism, learning difficulties. Residual psychomotor deficit is not observed only in 5-12% of cases. ZPR is noted in 70-78% of children, movement disorders - in 50%. A serious prognosis has West syndrome, caused by anomalies or degenerative changes in the brain. In this case, mortality can reach 25%.

A more favorable prognosis is for cryptogenic and idiopathic West's syndrome in the absence of ZPR before the onset of spasms. In this group of patients, residual intellectual or neurological deficit is absent in 37-44% of children. Delaying the start of treatment adversely affects the prognosis of the disease. Prognostic assessment is hampered by the fact that long-term consequences also depend on the underlying pathology, against which the symptomatic West syndrome occurs.

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 impaired conduction 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 may 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 the upper limbs or in 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 restoration of breathing, namely, pat the child on the cheeks, splash the face cold water, let the ammonia breathe from a distance of 10-15 cm. After these measures, it is urgent to consult a doctor who can differentiate the convulsive syndrome and develop specific recommendations for its treatment based on the type of seizures and their causes.
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. Special attention is given to reduce 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.
Conducting a lumbar puncture with a study of cerebrospinal fluid with the determination of the content of sugar, protein, electrolytes, cellular composition to exclude infection brain.
Ultrasonography 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 deciding 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 to a maximum dose of 5 mg intravenously slowly;
- phenytoin - 5 mg / kg / day (2 times, per os);
- phenobarbital - 3-5 mg / kg / day (2-3 times, per os);
- primidone - 5-25 mg/kg/day (1-2 times);
- carbamazepine - 15-30 mg / kg / day (2-3 times, per os);
- ethosuximide - 20-30 mg / kg / day (2 times);
- 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 parameters of the 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 the menstrual cycle. They can be cited as one example of age-dependent convulsive conditions in relation to female patients who have reached puberty.
Febrile seizures can 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 seizures 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-Gastaut syndrome - epileptic encephalopathy 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 may appear as 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

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 the initial stages of the disease, 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 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 serum glucose, 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 it may be successful surgery.

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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.

Symptoms and signs of infantile spasms in children

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 serum glucose, 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

Infantile spasm or West syndrome is called epileptic syndrome. Spasms occur in series. They can occur with different intensity, increasing or vice versa decreasing. The number of attacks can reach up to thirty and include up to twenty episodes during the day. More often this happens at night. The study of the etiology of this condition began in the fifties of the last century.

Infantile spasms in children

Infantile spasms are more common in children under the age of three. One hundred babies born account for 0.4% of cases. The first attack can occur within three months to one year. Lying on his back, the baby suddenly raises and begins to bend his arms, raises his head, upper body and at the same time sharply straightens his legs. The attack can last several seconds and be repeated and be accompanied by crying and irritability. Before the onset of a seizure, the child stops active activity, does not coo, looks at one point, and may not move. In the future, he develops other types of convulsive manifestations. The baby also has a violation of psychomotor development and intellectual retardation, which can persist into adulthood. Such seizures are difficult to treat.

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.

Infantile Spasms: Diagnosis

To determine the causes of seizures, a complete medical diagnostic examination is performed. To rule out metabolic disorders, a number of laboratory research: on the content of glucose, calcium, sodium, magnesium, amino acids. To exclude hypoxia, blood gases are examined, the sowing of the existing fluid in the body is taken. The cerebrospinal fluid (CSF) is also examined. To exclude intrauterine infection, an immunological and virological examination is performed. It is also necessary to do magnetic resonance imaging, computed tomography, cranio and spondylography. Electroencephalography captures the activity of acute wave attacks. For a complete picture of the disease, video monitoring of spasms is carried out. It is necessary to consult a neurologist, geneticist, speech therapist, optometrist, psychologist and psychiatrist. In young children under one year old, cognitive functions are evaluated according to the Bazhenova method. In older children, the IQ level is determined. In most cases, infantile spasms eventually transform into epileptic ones. Treatment in this case with anticonvulsant drugs is not always effective.

Discussions

Epilepsy. Infantile spasms.

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Infantile spasms is an epileptic syndrome observed in children under one year old and characterized by flexor, extensor or flexor-extensor spasms, delayed neuropsychic development and the presence of hypsarrhythmia on the EEG.

Three variants of infantile spasms are possible - flexor, extensor, flexor-extensor.

Flexor spasms consist of sudden flexion of the head, neck, and all limbs. During the flexion paroxysm of infantile spasms, the child gives the impression of "embracing himself." Extensor spasms are characterized by sudden extension of the neck and trunk, lower extremities combined with extension and abduction of the shoulders. A similar extensor variant of infantile spasms simulates the Moro reflex. Flexor-extensor spasms include mixed paroxysms manifested by flexion of the neck, trunk, upper extremities and extension of the lower extremities or, more rarely, flexion of the arms and extension of the legs.

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:

Delay in neuropsychic development until the development of the disease;

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

Infantile spasms are sometimes accompanied by screaming, 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 in the 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.

A universal concomitant symptom of infantile spasms is mental retardation, which is observed in 75-93% of patients, and 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.

This epilepsy begins in infancy with a sudden shudder of the whole body, a quick nod, similar to an oriental greeting - both shoulders are lifted up and to the side, the head is bowed to the chest, the gaze is directed upward. Very often, already before the first convulsions of this type, in all likelihood, damage to the brain has already occurred. About 20% of babies do not find any changes before the first attack.
The course of epilepsy in such children is much better. Treatment is extremely difficult. Rapid, accurate diagnosis and proper treatment. Drugs currently in use, such as hormones (Synacthen Depot, ACTH), valproate (Depakine, Convulex, Apilepsin) and benzodiazepines (antelepsin, clonazepam) have side effect and, unfortunately, are not always able to influence the malignant course of the disease, even if the attacks stop. Attacks indicate only a disease of the brain, and not about the course of this disease. Some periods of improvement should not be overestimated to avoid disappointment. Most patients should be constantly monitored by an epileptologist.
Another name for this form of epilepsy is West syndrome (this is the name of the doctor who at the end of the last century first described this disease in his son).

West syndrome (infantile spasms)

Clinical and electroencephalographic characteristics of epileptic seizures in children vary with age. A good example of such age-related phenomena is infantile spasms, a unique type of seizure associated with early childhood. Infantile spasms are an age-specific 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.

Definition

TO characteristics This syndrome includes myoclonic seizures, hypsarrhythmia on the EEG, and psychomotor retardation. This triad is sometimes referred to as West syndrome. However, infantile spasms do not clearly fit this definition in all cases. There are other names of the disease in the literature: massive spasms, Salaam convulsions, flexor spasms, jackknife seizures, massive myoclonic seizures, infantile myoclonic convulsions.

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

Although these seizures resemble myoclonic or tonic seizures, infantile spasms are a separate type of seizure. Myoclonic twitches are fast, lightning-fast contractions of limited duration, while tonic convulsions are prolonged muscle contractions of increasing intensity. True spasm includes a characteristic muscle contraction lasting 1-2 s and reaching a maximum (peak) slower than myoclonus, but faster than tonic convulsions.

Infantile spasms are divided into three types: flexor, extensor, and mixed flexor-extensor. Flexor spasms are short-term contractions of the flexor muscles of the trunk, neck, upper and lower extremities. Spasms in the muscles of the upper extremities cause adduction of the arms, "as if a child hugs himself with his arms" or, on the contrary, the spread of the arms bent in elbow joints, in different directions. Extensor spasms involve predominantly extensor muscle contraction, causing rapid abrupt extension of the neck and trunk, combined with extension and abduction or adduction of the arms, yoga, or upper and lower extremities simultaneously. Mixed flexor-extensor spasms include flexion of the neck, trunk, and upper extremities and extension of the lower extremities, or flexion of the lower extremities and extension of the arms, in combination with pronounced varying degrees flexion of the neck and torso. Sometimes asymmetric spasms develop, resembling the "swordsman's pose". Infantile spasms are often associated with eye deviation or nystagmus.

Asymmetric spasms can occur when there is no simultaneous bilateral contraction of the muscles of the limbs. This type of spasm usually occurs in the symptomatic form of infantile spasms in infants with severe brain damage, agenesis of the corpus callosum, or a combination of these disorders. Such local neurological symptoms as deviation of the eyes or head rotation can be combined with both symmetrical and asymmetric spasms. Asymmetric spasms usually occur in isolation, but they may also develop after or precede a focal seizure; in some cases, infantile spasms may occur simultaneously with a generalized or focal seizure.

Infantile spasms often occur in series ("cluster spasms"). The intensity and frequency of spasms in each series may increase, reaching a peak, and then progressively decrease. The number of seizures in a series varies considerably, and may exceed 30 spasms. The number of episodes per day also varies; some patients have up to 20 per day. A series of infantile spasms may develop at night, although they rarely occur during sleep. During or after a series of infantile spasms, as a rule, there is a crying or irritability of the child.

Hypsarrhythmia in a child with infantile spasms. Attention is drawn to the high-amplitude disorganized activity in the background recording, interspersed with multifocal spikes and sharp waves.

The chaotic nature of the EEG gives the impression of a complete disorganization of the cortical rhythm. During sleep, discharges of polyspike and slow waves occur. Surprising is the persistence of sleep spindles in some patients, in combination with marked abnormalities in the background EEG recording. In the phase of REM sleep, a decrease in the severity of hypsarrhythmia or its complete disappearance is possible. Infantile spasms are associated with reduced total sleep and REM sleep. Various varieties of hypsarrhythmia have been described, including patterns with hemispheric synchronization, a persistent focus of abnormal discharges, episodes of decreased amplitude, and high-amplitude slow-wave activity in combination with isolated sharp waves and spikes. Various patterns of hypsarrhythmia are common and do not correlate with disease prognosis.

Although hypsarrhythmia or a modified hypsarrhythmic pattern is the most common type of interictal abnormal EEG activity, these EEG patterns may be absent in some patients with infantile spasms. In some cases, there may be no hypsarrhythmia at the onset of the disease and the appearance of this pattern later, in the course of the disease. Although hypsarrhythmia is associated mainly with infantile spasms, this pattern also occurs in other diseases.

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

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

The electroencephalographic picture in this disease is unstable and may evolve over time. In some patients with infantile spasms, hypsarrhythmia may be absent at the onset of the disease. In other patients, slowing down of bioelectrical activity is possible in combination with rare epileptiform activity, further transformation into a hypsarrhythmia pattern is observed. Re-conducting an electroencephalographic study during follow-up may be necessary in order to demonstrate a pattern of hypsarrhythmia (in those children in whom hypsarrhythmia was not detected at the onset of the disease).