West syndrome: Baby in trouble. Symptomatic epilepsy with infantile spasms epilepsy infantile spasms
Observation of each kid movement usually brings many joy to parents. But when the child appears the symptoms of serious neurological disease, such as infantile spasms, this joy can quickly turn alarm.
Infantile spasms are small seizures with great consequences. Early recognition of this disease leads to best results treatment; Conversely - late diagnosis and delay with the beginning of therapy increases the risk of developing resistant brain damage.
What is infantile spasms?
Infantile spasms (also known as Vesta syndrome) are one of the forms of epilepsy, and are encountered with a frequency of 1 for 2000 children. Usually they begin in the period from 2 to 12 months, most often between 4m and 8m month of life.
Although the "spasm" lasts lasts only 1-2 seconds, they usually occur with the "attack-break-bout" series, and breaks usually make up 5-10 seconds. During the spasm, the whole body of the child is suddenly strained, the hands describe the arc, legs and heads can bend forward. Despite this, infantile spasms are sometimes very difficult to notice, as they can manifest themselves alone with a surveillance of the eyes up and a slight reduction in abdominal muscles. Most often, infantile spasms appear immediately after the awakening of the baby; The most rarely they happen during sleep.
Shortly after the appearance of seizures (in the coming weeks), parents may notice a number of changes in their child:
- - loss of previously learned skills (for example, the child stops turning over, sit, crawl, poverty - although there has already mastered these stages of development)
- - Loss of social interaction and smiles skills
- - Increased conversion, or vice versa, unusual silence.
How exactly do infantile spasms look like?
Parents and all other people who care for the baby are recommended to watch video No. 1 from attached to the post of video. On it we see a series of typical infantile spasms in the infant. Each spasm lasts less than 1 second, during each spasm the child on the face can be seen surprise, the look freezes, the hands rise and slightly scatter on the sides. In the intervals between each spasm, the child looks good - it is very typical for infantile spasms.
Early diagnosis is extremely important!
It is very important to diagnose infantile spasms as soon as possible. If you suspect your child infantile spasms - contact your pediatrician immediately. It will be very good if you get to remove the "attack" video from your child so that the doctor can see it with his own eyes. Many states, conscious or unconscious movements may be mistakenly regarded by parents as infantile spasms: infant masturbation, "redistribution" of a child after urination, chills with fever, conscious movements for playing and so on - in this case, a pediatrician will just calm you. If the pediatrician deems your description or your video is truly similar to infantile spasms - he will send a child to a consultation to the children's neurologist.
In order for a neurologist to better figure out the features of your child's disease, he can designate a study called electroencephalogram video monitoring (video EEG monitoring). This research method allows you to register specific brain electrical impulses characteristic of infantile spasms. Deciphering the recording received during the study, the neurologist will look for a special type of EEG pattern, called plastering. Usually for the neurologist it is not difficult to confirm the diagnosis of infantile spasms, but the identification of the causes of these seizures can be a very difficult task for the doctor.
Causes of infantile spasms:
There are many reasons for infantile spasms; Almost any abnormal brain or brain injury can lead to them. More than 50 genetic / metabolic diseases are associated with infantile spasms, and therefore many patients have other disorders leading to developmental delay (for example, children's cerebral palsy, Down syndrome, tuberous sclerosis, etc.) before the start of the seizures.
Determination of the cause of infantile spasms is very important because it affects treatment and forecast.
Treatment options:
The American Academy of Neurology and Children's Neurological Community recommend http://www.neurology.org/content/62/10/1668.long Adrenocorticotropic hormone (ACTH) as the first line of therapy of infantile spasms. This medicine is introduced in the form of injection. The first doses of the child will be introduced in the hospital and will leave it there to observe side effects on the drug. Then, if everything is in order, you will be discharged home, pre-instructing you about the rules for the injection of your child. Since the child should receive a 6-week medicine, you will enter it at home yourself. The purpose of such therapy is:
- - complete termination of infantile spasms.
- - Reduction of abnormal changes in the electroencephalogram.
What is the prognosis in children with infantile spasms?
Even if infantile spasms managed to completely eliminate with the help of treatment, many such children develop other types of epilepsy, as well as intellectual or other deviations in development, at a later age.
The faster the treatment started, the more favorable forecast. In children who had normal development before the start of infantile spasms, with timely therapy, perhaps even complete recovery without any consequences in the future.
PPS More detailed and scientific about Vesta syndrome can be read here.
Infantile spasms - cramps characterized by sudden bending of the body forward, bending or extension of hands, pulling or flexing legs.
This type of attacks, as a rule, is combined with Hypsarithmia on EEG.
Causes can pass on their own ages about 5 years, but can transform into other types of attacks.
The pathophysiology of infantile spasms is unknown to the end, but these attacks can reflect the violation of the interaction between the bark and the brain barrel. The cause of infantile spasms may be the immaturity of the CNS, the defects of brain development and brain damage in the first months of life. A common cause of the development of infantile spasms is tuberous sclerosis. The nature of the seizures may also be idiopathic.
Symptoms and signs of infantile spasms in children
Infantile spasms begin with sudden rapid tonic contractions of the body and limbs, sometimes within a few seconds. Spasms range from light nodes head until shuddering the whole body. They are accompanied by flexion (flexia), extension (extension) or, most often, and bending, and extension in the muscles of the limbs (mixed spasms). Spasms are usually repeated during the day clusters, often several dozen, mostly shortly after awakening, and sometimes during sleep.
As a rule, infantile spasms are accompanied by a violation of motor and mental Development. In the initial stages of the disease, a development regression is possible (for example, children can lose the ability to sit or turn over).
Premature mortality rate infantile spasms I ran away from 5 to 31%, death occurs under the age of 10 years and depends on the etiology of the latter.
Diagnosis of infantile spasms in children
- Neurovalization.
- Video Zagsna and wakefulness.
- Laboratory studies on clinical indications.
The diagnosis is established on the basis of clinical symptoms and the characteristic EEG picture. Forecological and neurological examination is carried out, but often pathognomonic symptoms are not detected, the exception is tuberous sclerosis.
In EEG, in the intercreant period, as a rule, a picture of plastering (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal peak discharges is detected. Perhaps several variants (for example, modified - focal or asymmetric plastering). Ictal background EEG is changing, intercotal epileptiform activity is noticeably weakened.
Tests to determine the cause of infantile spasms may include:
- laboratory studies (for example, a general blood test, the determination in blood serum glucose, electrolytes, urea, creatinine, Na, Ca, Mg, P, liver samples), if the metabolic disorder is suspected;
- analysis of SMG;
- brain scanning (MRI and CT).
Treatment of infantile spasms in children
Infantile spasms with difficulty are treatable, and the optimal pattern of therapy is discount. ACTG is used for 20-60 units intramuscularly once a day. Many anticonvulsant drugs are ineffective; Preferably, the use of hollows, the preparation of the second selection queue is clonezepam. The effect of the use of nitrazempama, topiramate, zonizamide or vigabolin is also noted.
A ketogenic diet can also be effective, but it is difficult to maintain it.
In some cases, successful may be surgery.
Infantile spa
Infantile spasm or wester syndrome is called epileptic syndrome. Available spasms in the form of a series. They can occur with different intensity, intensifying or on the contrary declining. The number of attacks can reach up to thirty and count to twenty episodes during the day. It happens more often at night. The study of the etiology of this state began in the fifties of the last century.
Infantile spasms in children
Infantile spasms are more common in children under the age of three. For one hundred born kids accounts for 0.4% of the fallen. The first attack can happen between three months to one year. The baby lying on his back suddenly raises and begins to bend his hands, raises his head, top The bodies and at the same time sharply straightens the legs. The attack can last a few seconds and be multiple and accompanied by crying and irritability. Before starting the seizure, the child stops active activity, not gulit, looks at one point, may not move. In the future, it develops other types of convulsive manifestations. The kid also has a violation of psychomotor development and a delay of intelligence that can be maintained in adulthood. Such attacks are difficult to treat.
Cause of infantile spasm
Depending on the etiology of spasms there are symptomatic and cryptogenic. The reason for their occurrence may be the following:
- damage or immaturity of the cortex of the brain;
- chromosomal and gene anomalies;
- neurological and psychophysical disorders;
- disorders during the period of intrauterine development (hypoxia, premature delivery);
- infectious diseases during pregnancy (bacterial or herpes viral meningitis);
- down syndrome;
- tuberculous sclerotic syndrome;
- complications during childbirth
In some rare cases, the reason may be in vaccination of the vaccination of ADC.
Types of infantile spasms
Infantile spasms are three species. Estenorous, flexor-extensor or flexor. These are sudden contractions of the muscles of the head, limbs, body and neck. Flexor spasms are called still convulsions by the type of folding knife or salyam convulsions. It looks like a person hugs himself. Extensor spasms suddenly extend the neck, lower limbs and a torso in combination with the extension of the shoulders. As a result of flexor-extensor spasms, mixed action. Cryptogenic infantile spasms are found in 9-15% of cases, the rest are symptomatic.
Infantile Spasms: Diagnostics
To determine the causes of convulsion, a complete medical diagnostic examination is carried out. To eliminate metabolic disorders, a number are carried out laboratory studies: on the content of the level of glucose, calcium, sodium, magnesium, amino acids. To eliminate hypoxia, blood gases are investigated, sowing of the existing fluid in the body is taken. The spinal fluid (liquor) is also investigated. An immunological and virological examination is carried out to eliminate the intrauterine infection. It is also necessary to make magnetic resonance tomography, computer tomography, Crantio and Spondylography. Electricencephalography fixes the activity of sharp wave attacks. For a complete picture of the disease, video monitoring of spasms is carried out. It is necessary to consult a neurologist, genetics, speech therapist, oculist, psychologist and psychiatrist. In young children, cognitive functions according to the procedure of Bazhenova are estimated before the year. The older children define the level of IQ. In most cases, infantile spasms are transformed in epileptic after a while. Treatment in this case by anticonvulsants is not always effective.
Discussions
Epilepsy. Infantile spasms.
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Infantile spasms - epileptic syndrome observed in children up to a year and characterized by flexor, extensor or flexor-extensor spasms, neuro-psychic development delay and the presence of hydrochyrithmia on EEG.
Three options for infantile spasms are possible - flexor, extensor, flexor-extensor.
Flexor spasms consist of sudden bending of the head, neck and all limbs. During a bending paroxism of infantile spasms, the child impresses "hugging himself." Extensor spasms are characterized by a sudden extension of the neck and trunk, the lower extremities, combined with the extension and abduction of the shoulders. A similar extensional version of infantile spasms simulates the reflex moro. Flexor-extensorial spasms include mixed paroxysms, manifested with a flexy of neck, torso, upper limbs and the extension of the lower extremities or, more rarely, bending the hands and extension of the legs.
Depending on the etiology, all infantile spasms are divided into cryptogenic and symptomatic. The expediency of the separation of infantile spasms to cryptogenic and epileptic proceeds from the generalized experience by the characteristics of clinical manifestations and the flow of infantile spasms.
For cryptogenic infantile spasms are characteristic:
Lack of a clear etiological cause;
Normal neuropsychic development of the child until the disease is developed;
Lack of other types of seizures;
Lack of signs of brain damage according to the data of neuroradiological research methods (computer (CT) and nuclear magnetic resonance (NMR) tomography of the brain).
Symptomatic spasms are a consequence of various etiological factors. For symptomatic infantile spasms are characteristic:
Delayed neuropsychic development until the cause of the disease;
Often - pathological changes in CT-and NMR studies of the brain.
Infantile spasms are sometimes accompanied by a cry, a smile grimace, a frightened expression of the face, turning out the eyes, nystagm, the expansion of pupils, eyelids, limbs, pallorium or redness of the face, stopping the breath. After convulsion, drowsiness is observed, especially if the series of attacks was long. In the interconception period, children are irritable, plaxive, disturbed sleep. Causes occur most often before falling asleep or after waking up. Factors provoking paroxysms include fright, various manipulations, feeding.
Infantile spasms begin at heart age and disappear in early childhood. Under the age of 6 months they make up 67% of the total number of paroxysmal episodes; from 6 months to 1 year - 86%; After 2 years - 6%.
Infantile spasms can be the first manifestation convulsive syndrome The child has. The first seizures are an abortive character and can be taken by parents for the fright reaction, the manifestation of pain in the stomach, etc. First, they are single, then the frequency is growing. At this stage can be remission and exacerbations that are difficult to foresee. As the child grows and the development of the child, the frequency of convulsion decreases. The average duration of infantile spasms - from 4 to 30-35 months. After 3 years, they are rare. According to P. Jeavons, etc. (1973), in 25% of children, infantile spasms are terminated under the age of 1 year, in 50% - up to 2 years, in the rest - up to 3-4, sometimes 5 years.
Changes to EEG (gypsumithmia) do not always correlate with the start of convulsion, sometimes they appear somewhat later. Gypsumithmia is characteristic of developing brain and is observed only with infantile spasms in young children.
The universal concomitant feature of infantile spasms is the delay in mental development, which is observed in 75-93% of patients, the formation of motor skills is also violated. Therefore, in early age children, it is more correct to talk about the delay in psychomotor development, which is celebrated in initial stage Diseases. It becomes more distinct when a series of seizures appear. The degree of delay depends on the start of the start of convulsion and from the premorbid characteristics of the child. Normal psychomotor development before the appearance of convulsion is observed in 10-16% of children.
This epilepsy begins in infancy with a sudden shudder of the entire body, a quick nodium, similar to the Eastern greeting - both shores are lifted up and to the side, the head leans on the chest, the look is directed up. Very often before the first convulsions of this type, in all likelihood, brain damage has already come. About 20% of babies do not detect any changes before the first attack.
Epilepsy for such children is much better. Treatment is extremely difficult. A quick accurate diagnosis and proper treatment. Currents currently used, for example, hormones (Synacten-Depot, ACTH), Valproat (Dec.bin, Convoilelex, Apilexin) and benzodiazepines (Anteltexin, clonazepam) have a side effect and, unfortunately, not always able to influence malignant flow Diseases, even if the attacks stop. Attacks indicate only the disease of the brain, and not about the course of this disease. Some improvement periods should not be revalued so that there is no disappointment. Most patients must constantly observe the epileptologist's doctor.
Another name for this form of epilepsy Vesta syndrome (this is the name of the doctor who at the end of last century first described this disease from his son).
West syndrome (infantile spasms)
Clinical and electroencephalographic characteristics epileptic attacks Children vary depending on age. A visual example of such eye-dependent phenomena is infantile spasms - a unique type of attacks associated with early childhood. Infantile spasms are a specific age phenomenon that occurs in children only in the first two years of life is most common - in the interval of 4 to 6 months, and approximately 90% of patients - up to 12 months. The incidence of Vesta syndrome is estimated as 0.4 per 1000 children born alive.
Definition
TO characteristic features This syndrome includes myoclonic attacks, Hypsarithmia for EEG and retreat of psychomotor development. This triad is sometimes referred to as Vesta syndrome. However, infantile spasms do not clearly comply with this definition. Other names of the disease are found in the literature: massive spasms, salaamos convulsions, flexor spasms, attacks by the type of "folding knife", massive myoclonic seizures, infantile myoclonic convulsions.
Typically, infantile spasms have a stereotypical nature of one child. In addition, the occurrence of infantile spasms in the form of a series is characterized.
Despite the fact that these attacks resemble myoclonic or tonic convulsions, infantile spasms are a separate type of attacks. Mioclonic twitching is fast lightning reduction limited duration, while tonic convulsions are a long muscular reduction of increasing intensity. True spasm includes a characteristic muscular reduction, continuing 1-2 C and a maximum of a peak slower than myoclonies, but faster than tonic convulsions.
Infantile spasms are divided into three types: flexor, extension and mixed flexor-extensor. Flexor spasms are short-term contraction of the muscle flexor torso, neck, upper and lower extremities. Spasms in the muscles of the upper extremities cause hands to bring, "as if the child hugs himself with his hands" or, on the contrary, breeding hands bent in the elbow joints, in different directions. Extensor spasms include, predominantly extensitive muscle contraction, causing a rapid sharp extension of the neck and torso in combination with extension and assignment or leading hands, yoga or upper and lower limbs at the same time. Mixed flexor-extensor spasms include flexion of the neck, body and upper limbs and extension of the lower extremities or bending the lower extremities and the extension of the hands in combination with a varying degree of flexion of the neck and the torso. Sometimes asymmetric spasms resembling a "fencer posture" are developing. Infantile spasms are often combined with the deviation of the eyes or the appearance of Nistagma.
Asymmetric spasms may occur if there is no simultaneous bilateral reduction in the muscles of the limbs. This type of spasms is usually found in the symptomatic form of infantile spasms in babies with severe brain damage, the agent of the corn body or a combination of these violations. Such local neurological symptoms, like the deviation of the eyes or turn the head, can be combined with both symmetric and asymmetric spasms. Asymmetric spasms usually occur isolated, but they can also develop after a focal attack or precede it; In some cases, infantile spasms may occur simultaneously with a generalized or focal attack.
Infantile spasms often occur in the form of a series ("cluster spasms"). The intensity and frequency of spasms in each series can enhance, reaching the peak, and then it is progressively decreased. The number of attacks in the series varies significantly, and may exceed 30 spasms. The number of episodes per day also varies; Some patients have up to 20 per day. A series of infantile spasms can develop at night, although they are rarely observed during sleep. During or after a series of infantile spasms, as a rule, there is a crying or irritability of the child.
Gypsarithmia in a child with infantile spasms. High-amplitude disorganized activity in the background entry, intermitted with multifocal spikes and sharp waves
The chaotic character of the EEG creates the impression of the complete disorganization of the cortical rhythm. During sleep, there are discharges of polyspars and slow waves. It is surprisingly saving in some patients with sleepy spindles in combination with expressed anomalies of the EEG background entry. In the phase of fast sleep, it is possible to reduce the severity of plastering or its complete disappearance. Infantile spasms are associated with a decrease in the overall duration of sleep and the duration of fast sleep. Different varieties of dryritemia are described, including patterns with inter impassary synchronization, resistant focus of abnormal discharges, amplitude reduction episodes, and high-amplitude slow-wave activity in combination with separate sharp waves and spikes. Various plaster pattern patterns are often not correlated with the forecast of the disease.
Although hypsitemy or modified drying pattern is the most common types of intercreant pathological activity on EEG, in some patients with infantile spasms, these EEG patterns may be absent. In some cases, there may be lack of plastering at the beginning of the disease and the appearance of this pattern later, in the process of developing the disease. Although the plaster is associated mainly with infantile spasms, this pattern occurs in other diseases.
Like an intercotal pattern, ICTAL changes on EEG with infantile spasms are also variable. The most characteristic EEG pattern during an attack consists of positive waves in the Vertex-Central region; There may also be a low-amplitude fast (with a frequency of 14-16 Hz) activity or diffuse cladding of the curve, called "Electro-Decremental Event".
The presence of focal anomalies is one of the variants of the main pattern of plaster, which can be associated with focal attacks, focal attacks may precede a series of infantile spasms, accompany it or develop in combination with it. This observation suggests that the cortical "pacemeters" can play an important role in the development of infantile spasms.
The electroencephalographic picture in this disease is inconsistent and can undergo evolution in time. Some patients with infantile spasms in the debut of diseases may be absent. In other patients, there may be a slowdown in bioelectric activity in combination with rare epileptiform activity, in the future there is a transformation into a pattern of plastering. The re-conducting of the electroencephalographic study in the process of further observation may be necessary in order to demonstrate a pattern of plaster (in those children who have no plastering at the beginning of the disease).
Infantile spasm or wester syndrome is called epileptic syndrome. Available spasms in the form of a series. They can occur with different intensity, intensifying or on the contrary declining. The number of attacks can reach up to thirty and count to twenty episodes during the day. It happens more often at night. The study of the etiology of this state began in the fifties of the last century.
Infantile spasms are more common in children under the age of three. For one hundred born kids accounts for 0.4% of the fallen. The first attack can happen between three months to one year. The baby lying on his back suddenly raises and begins to bend his arms, raises his head, the top of the body and at the same time sharply straightens his legs. The attack can last a few seconds and be multiple and accompanied by crying and irritability. Before starting the seizure, the child stops active activity, not gulit, looks at one point, may not move. In the future, it develops other types of convulsive manifestations. The kid also has a violation of psychomotor development and a delay of intelligence that can be maintained in adulthood. Such attacks are difficult to treat.
Cause of infantile spasm
Depending on the etiology of spasms there are symptomatic and cryptogenic. The reason for their occurrence may be the following:
- damage or immaturity of the cortex of the brain;
- chromosomal and gene anomalies;
- neurological and psychophysical disorders;
- disorders during the period of intrauterine development (hypoxia, premature delivery);
- infectious diseases during pregnancy (bacterial or herpes viral meningitis);
- down syndrome;
- tuberculous sclerotic syndrome;
- complications during childbirth
In some rare cases, the reason may be in vaccination of the vaccination of ADC.
Types of infantile spasms
Infantile spasms are three species. Estenorous, flexor-extensor or flexor. These are sudden contractions of the muscles of the head, limbs, body and neck. Flexor spasms are called still convulsions by the type of folding knife or salyam convulsions. It looks like a person hugs himself. Extensorial spasms suddenly extend the neck, lower limbs and torso in combination with the extension of the shoulders. As a result of flexor-extensor spasms, mixed action. Cryptogenic infantile spasms are found in 9-15% of cases, the rest are symptomatic.
Infantile Spasms: Diagnostics
To determine the causes of convulsion, a complete medical diagnostic examination is carried out. To eliminate metabolic disorders, a number of laboratory studies are carried out: on the content of the level of glucose, calcium, sodium, magnesium, amino acids. To eliminate hypoxia, blood gases are investigated, sowing of the existing fluid in the body is taken. The spinal fluid (liquor) is also investigated. An immunological and virological examination is carried out to eliminate the intrauterine infection. It is also necessary to make magnetic resonance tomography, computed tomography, crane and spondylography. Electricencephalography fixes the activity of sharp wave attacks. For a complete picture of the disease, video monitoring of spasms is carried out. It is necessary to consult a neurologist, genetics, speech therapist, oculist, psychologist and psychiatrist. In young children, cognitive functions according to the procedure of Bazhenova are estimated before the year. The older children define the level of IQ. In most cases, infantile spasms are transformed in epileptic after a while. Treatment in this case by anticonvulsants is not always effective.
Savoruds for hypoxia and intracranial labor injury. Most often they are found in newborns. Hypoxia, accompanied, as a rule, with a violation of cerebral hemo- and liquorodynamics, leads to a common or local brain echo, acidosis, diapell hemorrhage.
Convulsions in such children Appear immediately after birth or on the 2-3rd day, with subarachnoid hemorrhage, they occur most often after applying to the chest. Cauls are developing against the background of neurological violations: anxiety, sleep disorders, an increase in muscle tone and tendon reflexes, oppression of unconditional reflexes, difficulties of sucking and swallowing, pares of cranknot nerves. They most often wear clonic character, begin with the muscles of the face and then distributed to the limbs. The current seizure is different. They can fully relieve in the maternity hospital or again occur in a few months. Sometimes, starting in the maternity hospital, they are periodically repeated.
Cramps for developmental anomalies nervous system . Microcephalus, hydrocephalus, porencephalia, atrophy of the cerebral cortex, cerebellum hypoplasia can be accompanied during the newborn period by convulsions. Development defects are often combined with intrauterine hypoxia, asphyxia in childbirth and intracranial generic injury. Convulsions wear tonic-clonic character and arise against the background of pronounced focal changes nervous system (paresa, paralysis, sharp depression of unconditional reflexes, dysfunction). Neurorentgenic studies confirm the diagnosis.
Suggesogues in infectious diseases. During the newborn, the convulsions are most often observed during sepsis. They also take place in 30-50% of newborns with meningitis and usually arise in pronounced changes in spinal fluid. Cramps begin with a twitching of the eyes, the muscles of the face, and then, as the gravity increases, the states are moving to generalized. Facial attacks in meningitis are accompanied by an increase in temperature, inflammatory blood changes and liquor.
Early children's cramps
After period newborn Motor component The seizure becomes more pronounced. However, in infants, the classical sequence of convulsive seizures as well as newborns is rarely observed. Psychomotor automates are observed less often than other forms of seizures and at an early age, their diagnosis is difficult.
Mioclonic type of convulsive seizures (Small passive seizures or infantile spasms) are observed mainly in infants. The frequency of permanent seizures 1: 4000-6000 newborns, among children of the 1st year of life with convulsive syndrome, they make up 30.8%. For this type of convulsion characteristic: lightning fuses; impaired mental function; Specific changes to EEG. The classic picture of infantile spasms is characterized by a bilateral symmetric muscle contraction. Spasms are a flexor, extensor or mixed type.
With flexor spasme There is a sudden flexion of the neck, torso and limbs with simultaneous assignment or bringing the latter. The mixed type is characterized by a bending or extension of the body, hands and legs are inflicted. Flexor spasm meets most often, more rarely observed mixed and even less often extensive. At the same child at the same time can be various forms spasms. The infantile spasms also include partial fragmentary forms - rocks, shuddering, bending and extension of hands and legs, turning the head. At the same time, lateralization is possible - preferential contraction of the muscles of one side of the body. Rodes look like a quick tilt head forward. They are often combined with shudding and precede a flexor or extensor spasm or replace it. The most characteristic feature of infantile spasms is a tendency to seriality. Some spasms are less likely. The duration of convulsion from a split second to several seconds. The duration of the seizures series can be from a few seconds to 20 minutes and more. During the day, the number of paroxysms ranges from one to several hundred and even thousands. Shutting down consciousness in this form of seizure short-term.
Infantile spa Sometimes they are accompanied by a cry, a smile grimace, a frightened facial expression, surgery, nystagm, the expansion of pupils, eyelid shake, limbs, pallorium or redness of the face, stopping the breath. After convulsion, drowsiness is observed, especially if the series of attacks was long. In the interconception period, children are irritable, plaxive, disturbed sleep. Causes occur most often before falling asleep or after waking up. Factors provoking paroxysms include fright, various manipulations, feeding.
Infantile spa Begin at heart age and disappear in early childhood. Under the age of 6 months they make up 67% of the total number of paroxysmal episodes; from 6 months to 1 year - 86%; After 2 years - 6%.
Infantile spa There may be the first manifestation of convulsive syndrome in a child. The first seizures are an abortive character and can be taken by parents for the fright reaction, the manifestation of pain in the stomach, etc. First, they are single, then the frequency is growing. At this stage can be remission and exacerbations that are difficult to foresee. As the child grows and the development of the child, the frequency of convulsion decreases. The average duration of infantile spasms - from 4 to 30-35 months. After 3 years, they are rare. According to P. Jeavons, etc. (1973), in 25% of children, infantile spasms are terminated under the age of 1 year, in 50% - up to 2 years, in the rest - up to 3-4, sometimes 5 years.
Changes on EEG (Hypsarithmia) do not always correlate with the start of convulsion, sometimes they appear somewhat later. Hypsarithmia is characteristic of the developing brain and is observed only with infantile spasms in young children.
Universal accompanying sign of infantile spasms There is a delay in mental development, which is observed in 75-93% of patients, the formation of motor skills is also violated. Therefore, children in early age it is more correct to talk about the delay in psychomotor development, which is already noted in the initial stage of the disease. It becomes more distinct when a series of seizures appear. The degree of delay depends on the start of the start of convulsion and from the premorbid characteristics of the child. Normal psychomotor development before the appearance of convulsion is observed in 10-16% of children.
Focal neurological violations (Pares, paralysis, squint, nistagm) are found in 34-70% of cases. As a rule, they are observed in children with cerebral paralysis, microcephalus, anomalies for the development of the central nervous system.
Forecast with infantile spasms Favorable in children with normal psychomotor development, with short-term cramps that are uncomplicated by other forms of seizures. In early early, seriality and duration, combination with other types of convulsions, the presence of neurological and mental violations there is a deep delay in psychomotor development.
Absans - The form of small seizures, which are also observed in young children and are characterized by a short-term stopping of the gaze. Sometimes at this time the child produces sucking, chewing movements, combustion, licking by the language. The attack may be accompanied by redness or pale face, easy assignment eye apples. They are observed less frequently than passive seizures.
Big convulsive attacks in children An early age is more often an abortive character. In the structure, the seal predominates the tonic component. When turning the head towards children breast-age Frequently fixed in an asymmetric pose. The attacks may be accompanied by a temperature lift, vomiting, stomach pains and other vegetative symptoms. Incoming urination is observed less frequently than in older children. After the seizure, the child is sluggish, stunned, falls asleep or on the contrary is excited, muscular hypotension is expressed.
Partial cramps in children An early age is manifested by clonic twitching of mimic muscles, muscles of the language, distal limbs. The seizure, starting locally, can go to generalized. Often at this age, adverscent convulsive seizures are observed, accompanied by turning the head and eyes, and sometimes the torso, aside. The seizure is often accompanied by a tonic tension of his hand and legs on the side of the head of the head.
Causes of the first year of life.
Cramps are chaotic, mostly painful, reduction of various muscle groups.
The reasons that lead to the appearance of convulsion in children are quite diverse. The main ones are the following:
1. Infectious diseases. Meningitis, encephalitis, brain abscesses lead to the affix of the brain and violation of the nervous impulse.
2. Mother's drug addiction during pregnancy. Narcotic substances The process of the intrauterine formation of the brain is disturbed, so the children born from mothers-drug addicts may have a seizure.
3. Endocrine diseases. Diabetes, diseases of the thyroid gland, adrenal glands can cause the appearance of a convulsion in a child at any age.
4. Humidated heredity. Some genetic diseases They lead to a violation of the development of the brain, as a result of which the development of convulsive syndrome in a child may be observed.
5. Tumor lesions of the brain cause a violation of a nervous impulse by nerve fibersWhat is why children appear in children.
6. Lack of calcium.
7. Improper use of drugs. Some drugs, such as diuretical, cause a decrease in the content of calcium in the blood, which causes the development of convulsion. Also the appearance of convulsion is observed in the overdose of vitamin D3 and the development of such a state as spasofilia.
8. A seizure may appear when hypothermia (for example, a convulsion limb will be reduced cold water). But if it repeats often, you need to consult a doctor.
For convulsions, you can take an attack of epilepsy, so in diagnosis it is necessary to keep in mind this disease.
In children over 1 months, the following varieties of seizures are more common:
1. Primary-generalized (tonic crylonic, according to Grand Mal type). For them, the tonic phase is characterized by a duration of less than 1 minute, with eye swing up. At the same time, gas exchange decreases (due to the tonic reduction of the respiratory muscles), which is accompanied by cyanosis. The clonic phase of seizures follows the tonic, expressing in clonic twitching of the limbs (usually 1-5 minutes); Gas exchange at the same time improving. It may be observed: hypersivia, tachycardia, metabolic / respiratory acidosis. The permanent state is more often on less than 1 hour.
2. Focal motor cramps (partial, with simple symptoms). They are characterized by the emergence in one of the upper limbs or in the face of the face. Such convulsions lead to the deviation of the head and the assignment of the eye towards the hemisphere, the opposite localization of a convulsive focus. Focal cramps can begin in a limited area, without losing consciousness or, on the contrary, to generalize and resemble second-Russian generalized tonic block convulsions. Paralysis of Todd or the head and eye leads in the direction of the affected hemisphere serve as directions to the hearth. They appear after the attack of the specified convulsion.
3. temporal or psychomotor cramps (partial, with complex symptoms). At about 50% of cases, they are preceded by aura. There can be imitate other types of cramps, be focal, motor, according to the type of Grand Mal or frozen the gaze. Sometimes they look more complex: with stereotypical automatisms (running - have begun walking, laughter, licking lips, unusual hands, face muscles, etc.).
4. Primary-generalized anxissue attacks (by Petit Mal type). Rarely develop in the first year of life (more typical of children over 3 years).
5. Infantile spasms (with dryritemium - according to EEG). More often appear on the 1st year of life, characterized by pronounced myoclonic (salaam) spasms. Infantile spasms (Vesta syndrome) may develop due to the presence of various neurological pathology or without any obvious preceding violations. With infantile spasms, psychomotor development slows down, in the future, the likelihood of a pronounced lag in development is high.
6. Mixed generalized cramps (small motor or atypical Petit Mal). This group of convulsive disorders is typical for Lennox - Gastor's syndrome, which is characterized by frequent, poorly sustainable convulsions, including atonic, myoclonic, tonic and clonic, which accompanies EEG pattern with atypical spikes (from English. Spike - peak) and waves (less than three Spike-waves in 1 s), multifocal spikes and polyspacks. The age of patients more often exceeds 18 months, but this syndrome can develop on the 1st year of life following infantile spasms (Vesta syndrome transformation). Children often have a pronounced lag in development.
7. Fabry convulsions (FS). They are noted in children, starting from 3 months of age, with increasing body temperature (\u003e 38.0 ° C). As a rule, there are primary-generalized tonic crylonic, although they can be tonic, atonic or clonic.
Febral convulsions are considered simple if they were observed once, no more than 15 minutes lasted and there was no focal symptoms. Complicated febrile convulsions are characterized by a repeated occurrence, duration and presence of pronounced foci. To all patients under the age of 12 months, it is necessary to carry out lumba puncture and metabolic screening in order to clarify the causes of convulsion.
Risk factors for the development of epilepsy with FS include:
- guidelines for neurological disorders or psychomotor disorders
development;
- the presence in the family history of Afrybral seizures;
- complex character of febrile cramps.
In the absence or availability of only one risk factor, the likelihood of the development of Africral seizures is only 2%. In the presence of two or more risk factors, the likelihood of epilepsy increases to 6-10%.
Treatment.
The treatment of convulsive syndrome in children must be started with the first prefigure. General principles This aid is given below.
First assistance to a child with convulsion / convulsions
If the child's convulsion appears, it is necessary to put it on a flat surface, try to protect it from foreign objects, since, making chaotic movements with hands and legs, the child can cause injury. You must open the window. The child needs to provide access to oxygen, so it is impossible to "bile" and "hang" over the child, making it difficult to access fresh air. If a child has a tight collar on a shirt, the upper buttons need to unbuckle. In no case can you try to insert foreign objects into your mouth, especially sharp, as it can lead to serious injury. It is necessary to take measures for the reflex recovery of breathing, namely, patted the child on the cheeks, sprinkle with cold water, give rise to the ammonia from a distance of 10-15 cm. After these activities, it is necessary to urgently consult a doctor who can differentiate convulsive syndrome and develop concrete recommendations. For its treatment, based on the type of convulsion and the reasons for their occurrence.
An important role in establishing the causes of the occurrence of the child is belonging to a child survey.
Diagnosis of convulsive syndrome includes:
Common blood test, overall urine analysis, for children up to 3 years old Sulkovich urine analysis to exclude spasmophilia.
Determination of the electrolyte composition of blood. Special attention is paid to the decrease in the content of calcium and magnesium in the blood.
Determination of blood glucose.
Determination of blood gas composition. Pay attention to the oxygen content and carbon dioxide.
Conducting lumbal puncture with a study of liquor with the determination of the content of sugar, protein, electrolytes, cellular composition to eliminate the infectious brain damage.
Ultrasonic studies of the brain for children with unclosed large spring, tomography brain for older children.
Electroencephalography to determine the work of the brain and the detection of vascular disorders.
Only on the basis of these studies can verify the diagnosis.
Drug therapy Special cases of convulsive syndrome are based on the following principles:
Primary generalized convulsions (Grand Mal). Tenobarbital, phenytoin (Epdantoin, Epanutin), carbamazepine are used. As an alternative, Valproids (depailage, depair) or acetazolamide can be used in some cases.
Partial simple cramps (focal).
Fenobarbital, phenytoin (Epdantoin, Epanutin), carbamazepine, prison is used. As other therapeutic agents, if necessary, can be used (preparations of valproic acid (depair, depailage), Wigabria (Sabril, Sabrilex, Sabrilan), Keppr, etc.)
Partial complex convulsions ( temple epilepsy)
. The priority appointment of carbamazepine, phenytoin and prison is envisaged. Alternatives to them are the preparations of phenobarbital, hollows and acetasolamide (as well as Metorsximid, Etosucemid, Petynimide, Zaront).
Primary generalized convulsions (Petit Mal, Absans)
. The main anti-epileptic drugs of AEP in the described clinical situation - ethosuximide, hollows, METSUSCIMID. Other funds: acetazolamide, clonazepam, hlobazam, phenobarbital.
Infantile spa
. Most effective drugs For the treatment of infantile are seized: the Cintic analogue of the Aktg-Synacten depot, Vigabatrin (Sabril, Sabrilex, Sabrilan), Valproaty (Depailage, Depakin), Keppra, Etosucemid (Zarontin, Pennis, Petynimide), hlobazam (frizium). Other types of therapies include the use of phenytoin (Epdantoin, Epanutin), Tooks, phenobarbital, acetasolamide. If possible, a ketogenic diet (CD) can be applied.
Febrile cramps. The feasibility of appointing children anticonvulsants with the FS for many years continues to remain extremely discountable. Nevertheless, when making a decision in favor of conducting preventive therapy using AEP, phenobarbital preparations are most often used, less often hipproat.
Mixed generalized cramps.
The main AEP: Phenobarbital, Valproat, clonazepam, hlobazam (frizium). As alternatives, acetazolamide, diazepams, ethosuximide, phenytoin, METSUSCIMID, carbamazepine, and transplan, etc. can be used.
Dosing the main anticonvulsants (on the 1st year of life)
- diazepam - 0.1-0.3 mg / kg to 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);
- Prison - 5-25 mg / kg / day (1-2 times);
- carbamazepine - 15-30 mg / kg / day (2-3 times, per os);
- Etosuximide - 20-30 mg / kg / day (2 times);
- METSUSCIMID - initial dose of 5-10 mg / kg, supporting - 20 mg / kg (2-fold, per os);
- Valproaty - 25-60 mg / kg / day (2-3 times, per os);
- clonazepam - 0.02-0.2 mg / kg / day (2-3 times, per os);
- paraulates - 300 mg (0.3 ml / kg, rectally);
- Acetazolamide (diakarb) - the initial dose of 5 mg / kg, supporting - 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 considered the fact that Phenitoin (Epdantoin, Epanutin) in the neonatal period is absorbed with low efficiency, although subsequently its disposal is gradually improving.
Valproic acid preparations with simultaneous purpose interact with phenythine and phenobarbital, leading to an increase in the level of their blood content. With long-term assignment of the hollows, you must control the indicators general analysis Blood, as well as explore 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 (for 3 months), and subsequently 1 time in 3-6 months.
Almost all the anticonvulsants known to date largely or less have a so-called richitogenic action, leading to the appearance or aggravation of the manifestations of vitamin D-deficient Rahita. In this regard, children of the first year of life, receiving treatment with anticonants, should ensure adequate admission of vitamin D (D2 - Ergocalciferol, or D3 - cholecalciferol), as well as calcium preparations.
Cramps in young children.
Clinical manifestations Vesta and Lennox syndromes - Gasto are described quite widely (see separate articles on our website). As already mentioned, they can be celebrated both in the first 12 months of life, and later, although more characteristic of 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 attacks, passing into complex partial, followed by secondary generalization.
Frebelous concessions in young children are found with no less frequency than on 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.
Cramps in children older than 3 years
Primary-generalized anxissue attacks - type of attacks, mainly occurring in children of this age group. Their detection and conduct of adequate treatment is fully in the competence of children's neurologists and epileptologists. Pediatricians and representatives of other pediatric specialties should not be ignored by the identified episodes of short-term "disconnection" of children (with the lack of reaction to appeal) or complaints about the peculiar episodes of "thoughtfulness".
Juvenile Mioclone-Epilepsy - This is a subtype of an idiopathic generalized disease with the manifestations of attacks by the type of impulsive Petit Mal. The appearance of convulsion is characteristic after 8 years. Distinctive feature It is the presence of myoclonium, the severity of which varies from the minimum (regarded as "clumsy") to periodic falls. There is no violations of consciousness. Nevertheless, most such patients have sporadic tonic clonic convulsions, while the absursions take place about one third of children with this type of epilepsy.
Catrimaging epilepsy.
Chronic group paroxysmal statesassociated with menstrual cycle. They can be brought as one of the examples of the age-dependent convulsive states in relation to female patients who have reached the period of puberty.
Febral convulsions can be celebrated in children over 3 years (in preschool age), although during this period of life they meet with a significantly lower frequency. The presence of episodes of Africral seizures (without increasing body temperature) indicates the development of symptomatic epilepsy, which must be treated in accordance with the principles formulated by the International Anti-Epileptic League (ILAE).
Lennox-Gasto Syndrome. Diagnostic criteria. Treatment.
Lennox-Gasto-epileptic encephalopathy syndrome children's agecharacterized by polymorphism of seizures, cognitive impairment, specific changes in the electroencephalogram and resistance to therapy.
types of attacks: paroxysms of falls, Tonic attacks and atypical absansions. Consciousness can be saved or turned off briefly. After the fall, seizures are not observed, and the child immediately gets up. Frequent seizures of drops often lead to injuries. The attacks include sudden bending of the neck and torso, the rise of the hands in the state of the semicircle or extension, the extension of the legs, the reduction of facial muscles, rotational movements of the eyeballs, apnea, hyperemia. They can occur both during the daytime and, especially often, at night.
With Lennox Syndrome, Tonic, tonic-clonic, atonic, myoclonic attacks and absansions occur until hundreds of times a day. Atonic seizures lead to numerous drops.
Even before the appearance of the first attacks, children, as a rule, suffer from the lag in psychomotor development, which is aggravated with the beginning of the disease.
Debut 2-12 years old with 2 peaks at 3 and 9 years