home→Diseases→ Benign neonatal seizures. Modern approach to understanding and treatment of neonatal seizures

Benign neonatal seizures. Modern approach to understanding and treatment of neonatal seizures

Morozova T.M., Evtushenko S.K., Omelyanenko A.A., Balakhonova O.N., Donetsk National Medical University named after I.I. M. Gorky

Summary

Neonatal seizures are often the first sign of neurological dysfunction and an important indicator of further cognitive impairment and developmental delay. This article presents the identification, clinical manifestation, diagnosis, specific (standard and alternative) treatment of neonatal seizures.

Keywords

convulsions, newborns, treatment.

Neonatal seizures (NS)- this is a polyetiological clinical syndrome of the neonatal period, which indicates primarily cerebral disorders.

NS occurs in the first 4 weeks of life of a full-term newborn (from the 1st to the 28th day). For preterm infants, this corresponds to a post-conception age of 44 weeks (post-conception age is equal to the sum of the gestational period before birth and the duration of the postnatal period).

The frequency of NS ranges from 0.7 to 16 (L. Nirupama, 2000; M. Levene, 2002) per 1000 live births, which is explained by the complexity of identification. NS is an ambiguous age-dependent phenomenon, seizures are often non-expanded, with no secondary generalization, and, as a rule, go unnoticed, not always distinguishable from normal activity. In the process of myelination and synaptogenesis, seizures "evolve". In addition, so-called "hidden" seizures are often recorded, that is, seizures without clinical manifestations, which are diagnosed only by electroencephalography (L. Nirupama, 2000; M.S. Scher, 2002; G.B. Boylan, 2002).

In most cases (over 90%), NS are symptomatic, and only about 10% are hereditary (idiopathic NS). According to the observations of Mizrahi and Kellaway, hypoxic-ischemic encephalopathy (HIE) has the largest share (32%), intracranial hemorrhages (ICH) account for 17%, intrauterine infections (IUI) with lesions of the central nervous system- 14%, cerebral malformations - 7%, metabolic disorders - 6%, congenital metabolic disorders - 3%, phakomatoses - 2% and unknown causes - 10%.

Since the immature brain is highly epileptogenic, the presence of NS is often the first sign of neurological dysfunction. Seizures usually indicate the severity of the pathology and are the main symptom predicting cognitive and motor deficits in further development child. The prognosis of NS is unfavorable in most cases, mortality is from 15 to 40%. In 11-90% of surviving children, dramatic and long-term consequences are manifested by epileptic encephalopathy with cognitive impairment, learning and communication difficulties, deviant behavior, motor delay.

It has been proven that the resistance of the brain to the damaging effects of seizures in newborns is high during the first week of life, and then decreases (O. Cataltepe, 1995). An inverse relationship has been established between the degree of maturity of newborns and the incidence of seizures. Why are seizures dangerous for the developing brain?

The attack reduces the levels of ATP, phosphocreatine, glucose and increases the level of ADP, pyruvate, lactate => transition to glycolysis and anaerobic metabolism => glucose deficiency, hypoxia.

An increase in lactate => local vasodilation and impaired autoregulation of cerebral blood flow => increased risk of bleeding (from the germinal matrix and into the penumbra) of ischemic infarcts).

Despite the increased blood flow, the high metabolic demand due to the seizure is not compensated, there is a decrease in protein metabolism / DNA synthesis, PKN and irreparable damage during cell division => slowing down neuronal differentiation and myelination, disruption of synaptic connections and apoptosis.

Thus, a seizure occurs when a large group of neurons undergo excessive synchronized depolarization. Violation of the level of glutamate, aspartate, calcium influx, energy deficiency, the development of hypoxia and the loss of cerebral autoregulation of blood flow with its increase cause a secondary damaging effect. In this regard, there is a need for adequate diagnosis and treatment of NS from the first days of a baby's life.

In the International Classification of Epilepsy (1989), NS are categorized as age-dependent convulsive syndromes, however, idiopathic NS are verified as forms of hereditary (familial) epilepsy that debut in the neonatal period.

As a polyetiological syndrome, NS has a fairly wide range of clinical manifestations and time of manifestation, which should be taken into account when making a diagnosis. The nosological diagnosis, the specific manifestation of which is NS, is determined by a whole range of criteria: gestational age, anamnestic family data, primarily prenatal history of development, intranatal situation and the nature of the course of early neonatal adaptation. The anamnesis and the clinical syndrome complex accompanying neonatal convulsions are important keys to revealing the etiology of NS:

A family history of convulsions in the neonatal period suggests that the infant has genetic syndrome. Some of these syndromes are considered benign and often disappear within the neonatal period.

A detailed pregnancy history, looking for signs that suggest the possibility of TORCH infection, fetal distress, preeclampsia, or maternal infection may also facilitate the search for etiology.

The history of childbirth is equally important: the type of delivery and the documented traumatic factor. The Apgar score also suggests an etiological factor. However, a low score without the need for resuscitation and subsequent intensive care is unlikely to be associated with NS.

The postpartum history is no less significant: NS in infants with an unprecedented prenatal history and delivery may be the result of postnatal causes. The presence of tremor may suggest delivery with analgesia or neonatal hypocalcemia. Instability of blood pressure, fever suggest infection or sepsis.

Taking into account the features of the morphofunctional maturity of the CNS of a newborn, the following types of NS are distinguished:

Fragmentary NS:

ophthalmic (ocular);
oroalimentary;
motor;
vegetative.

Clonic NS:

focal;
multifocal;
generalized (bilateral).

Myoclonic NS:

focal;
multifocal;
generalized.

Tonic NS:

focal;
generalized.

Phenomenological classification of NS (J. Volpe, 2001) and their semiotics:

1. Fragmentary NS (soft, atypical, erased, abortive - subtle).

1.1. Ocular phenomena: a) tonic deviation; b) rhythmic nystagmoid twitching of the eyeballs);

c) blinking, opening eyes, fading gaze. These attacks must be differentiated from manifestations of CSF distension, paresis of the oculomotor nerves, metabolic encephalopathy (Leigch's syndrome), "dancing eyes" syndrome with latent neuroblastoma.

1.2. Oroalimentary (oral-buccal-lingual-facial) automatisms: a) chewing; b) swallowing movements; c) sucking movements; d) smacking; e) paroxysmal movements of the tongue; e) unusual grimaces, paroxysmal smile.

1.3. Motor phenomena: a) "pedaling", "boxing" or raking movements in the limbs with a short-term change in muscle tone; b) adversive neck attacks; c) chaotic movements of the upper and lower extremities.

1.4. Seizures: a) limpness; b) fading; c) loss of consciousness; d) diffuse decrease in muscle tone; e) cessation of motor activity.

1.5. Vegetative reactions: short-term changes: a) heart rate and blood pressure; b) skin color (cyanosis); c) salivation; d) hiccups.

1.6. "Convulsive" apnea.

Genesis: HIE, brain anomalies, hereditary metabolic disorders, toxic-metabolic disorders, cerebral hemorrhages (infratentorial, parenchymal), IUI. Ictal EEG - slow waves and changes in the type of "peak - wave" complexes.

2. Clonic NS (CNS): rhythmic muscle twitching of individual parts of the trunk, face and limbs, usually with a frequency of 1-3 per second. They occur in children over 36 weeks of gestation.

2.1. Focal CNS: rhythmic clonic twitches of the face and limbs with a clear laterization, combined with adversion of the head and eyes. In some cases, focal status epilepticus is formed. After an attack, transient mono- or hemiparesis of the extremities may develop.

Genesis: cerebral infarction, hematoma, bacterial meningitis, arteriovenous malformation, tumor, etc. On the EEG - focal foci of epileptic activity according to the type of "peak - wave" complexes.

2.2. Multifocal CNS: seizures affecting individual muscle groups, unstable, fragmentary, migrating from one limb to another and from one side of the body to the opposite. Often associated with sleep apnea.

Genesis: electrolyte disturbances (hypomagnesemia, hypocalcemia), low pyridoxine levels, impaired cortical differentiation and migration. Rarely occur in the recovery phase after acute asphyxia.

2.3. Generalized KNS. Generalized clonic seizures in 95% of cases in newborns are focal in nature with secondary generalization. With the formation of a generalized clonic seizure, there is a loss of consciousness, there may be disturbances in the rhythm of breathing with cyanosis, hypersalivation. Such attacks indicate the maturity of the brain of the newborn and occur mainly in full-term children.

Genesis: HIE, birth trauma, metabolic disorders.

3. Myoclonic NS (MNS).

3.1/3.2. Focal / multifocal MHC: a) axial MHC - lightning-fast flexion of the head, neck such as "pecks", "nods" with a frequency of 1-8 per second or less, can be combined with vegetative-visceral disorders, dilated pupils; b) MHC of the limbs - rhythmic symmetrical flexion of the limbs, more often of the arms, with a frequency of once per second or 1-2 per 10 seconds. Often mimic the spontaneous Moro reflex.

3.3. Generalized MHC - a combination of "pecks" with flexor flexion or extension of the limbs, nodding of the head. Relatively symmetrical, synchronous myoclonic jerks.

Genesis: severe diffuse brain damage, terminal phase of asphyxia, hereditary metabolic diseases (NBO), hereditary degenerative diseases, cerebral malformations. In the future, myoclonic seizures can become integral part syndromes West, Otahara, Ajkardi, Lennox - Gastaut.

4. Tonic NS (TNS): indicate damage to the structures of the forebrain.

4.1. Focal TNS: a) stereotypical, often short-term tonic changes in position and muscle tone in one limb, tonic tension in the neck muscles, flexion or extension of one limb (imitation of an asymmetric tonic neck reflex); b) adversion of the head. Accompanied by apnea, tonic deviation of the eyeballs or gaze fixation.

4.2. Generalized TNS: a) attacks of the type of decerebrate rigidity lasting less than one minute, consisting in retraction of the neck muscles and extension of the arms and legs; b) flexion of the arms and extension of the legs according to the type of decortication posture. They are combined with upward deviation of the eyeballs and paroxysmal respiratory failure, which resembles a prolonged breath.

Genesis: more common in the first day of life in small children with neonatal HIE, intraventricular hemorrhages. Ictal EEG: specific slow wave activity originating from stem structures and basal ganglia.

Idiopathic NS, or nosologically independent epileptic syndromes, are divided into benign idiopathic (among which there are two forms - benign familial NS and benign idiopathic NS) and malignant idiopathic NS (early myoclonic encephalopathy, early infantile epileptic encephalopathy, migratory partial epilepsy in young children).

Benign familial neonatal seizures (P. Plouin, 1985) are inherited in an autosomal dominant manner, the genetic marker is localized on the 8th or long arm of the 20th chromosome. Debut - 2-3rd day of life against the background of relative well-being. Seizures occur mainly during sleep with a frequency of 35 times a day. The duration of the attack is 1-3 minutes. Short multifocal clonic seizures are combined with apnea, ocular and autonomic phenomena, and oral automatisms. The duration of convulsions is from 5-7 days to 6 weeks, regardless of the appointment of anticonvulsants. Ictal EEG: amplitude suppression, generalized spike waves. Interictal EEG corresponds to the age norm.

Benign idiopathic neonatal seizures ("fifth day seizures"). The main reason is an acute zinc deficiency. Occur in full-term children against the background of complete well-being. Apgar score at the 5th minute - not less than 9 points. Manifestation on the 5-7th day of life. The frequency of seizures is up to 15-20 per day or in the form of neonatal epileptic status of generalized multifocal, less often focal clonic convulsions lasting up to 20 hours. Seizures are resistant to therapy, accompanied by apnea up to 1 minute, cyanosis. Ictal EEG: θ - sharp waves or alternating bursts of θ-waves. Prospectively - normal development.

Early myoclonic encephalopathy. Debut - the first 28 days. There is a series of seizures up to continuous status epilepticus. Convulsions in the form of fragmentary chaotic myoclonic twitches in the muscles of the face and limbs. Characterized by the addition of focal phenomena (eye aversion, apnea, redness of the face) and tonic extensional extension of the limbs. EEG pattern: "flare - depression" with modification to hypsarrhythmia and focal spikes after 3-5 months.

Early infantile epileptic encephalopathy (Otahara syndrome). Debut in the first 20 days of life. Generalized tonic or focal motor seizures, serial (10-20 episodes), frequency 100-300 times a day. They occur both during sleep and while awake. Clonic convulsions and myoclonus are also observed. EEG pattern: "flash - depression". Severe neurological deficit.

Migrating partial epilepsy in young children. Debut - from 13 days to 7 months. Motor seizures with a vegetative component (apnea, cyanosis, flushing of the face), secondary polymorphism with generalization, eye symptoms. Frequency - from 5 to 30 attacks several times a day or periods of 2-5 days. EEG pattern: multifocal activity predominantly of temporal localization, slowing down of background activity. Progressive neurological deficit.

Consider the main etiological factors of symptomatic NS.

Hypoxic-ischemic encephalopathy (35-56%). Pathological condition due to severe hypoxemia and ischemia: Apgar score below 4 points, resuscitation from the first minutes of life. There is a decrease in the level of PO2 below 40 mm of water column, an excess of CO2, metabolic acidosis - blood pH below 7.2. Clinical manifestations: cerebral depression to coma, symptoms of increased intracranial pressure, cerebral edema. Convulsions in the 1st-3rd day of life, often recurring, generalized clonic, tonic, multifocal, atypical with a status course. Insufficient susceptibility to anticonvulsants.

ICH is traumatic (10%). Documented precedent in childbirth. A catastrophic worsening of the condition is observed on the 1st day, more often at the 3-8th hour of life. Attention is drawn to the change in the nature of the cry and the loss of sociability, a decrease in muscle tone and motor activity, signs of progressive intracranial hypertension or acute hydrocephalus. Eye symptoms are important (ptosis, anisocoria, strabismus, fixed gaze, constant vertical and horizontal nystagmus, impaired oculocephalic reflex and decreased pupillary response to light, a symptom of closed eyelids). Natal trauma is accompanied by: metabolic acidosis, hypoxemia, progressive post-hemorrhagic anemia, decrease in hematocrit or lack of its increase during infusion therapy. Convulsions: focal, clonic, generalized, tonic, apnea, tonic postures, respiratory disorders.

Intrauterine infections (5-10%). Symptoms debut in the early neonatal period: intrauterine growth retardation, hepatosplenomegaly, jaundice, exanthema, fever, respiratory disorders, cardiovascular failure, focal neurological disorders, convulsions. thrombocytopenia. Microcephaly, calcifications in the brain. IUI diagnostic standard:

Direct detection of the causative agent of the disease, its genome or antigens, direct methods - virological, bacteriological, PCR, DNA hybridization. Cerebrospinal fluid is used to diagnose lesions of the nervous system.

Detection of specific immune response markers (indirect diagnostic methods). Detection in the child's blood serum of specific antibodies to the antigens of the pathogen. Requirements: a) serological examination should be carried out before the introduction of blood products (plasma, immunoglobulins, etc.); b) serological examination of newborns and children should be carried out with simultaneous serological examination of mothers; c) serological examination should be carried out by the method of "paired sera" with an interval of 2-3 weeks. In this case, the study must be performed using the same technique in the same laboratory. Serological markers of the acute phase of the infectious process are IgM and low-avid IgG. As the severity of the process subsides, the avidity of IgG antibodies increases, highly avid immunoglobulins are formed, which almost completely replace the synthesis of IgM.

Cerebral malformations (9-16%). NS occurs with structural disorders of neuroontogenesis in the first 20 weeks of pregnancy. The nature of the seizures: short duration (no more than 1 min), high frequency of automatisms in the initial phase, secondary generalization of seizures. Often demonstrative and unusual motor phenomena (pedaling, gestural automatisms), pronounced motor manifestation, including atypical postures like bilateral or unilateral tonic postures and/or atonic episodes. Complex partial seizures with minimal impairment of consciousness. Between seizures, the EEG sometimes shows unusual and extremely active focal epileptic discharges in the form of repetitive spike waves.

The standard for diagnosing cerebral malformations: 1) the absence of a clear precedent, including severe hypoxia during childbirth; 2) convulsive syndrome resistant to therapy; 3) muscular hypotension during the neonatal period; 4) focal neurological deficit; 5) a delay in the pace of psychomotor development and a violation of the formation of postural reflexes; 6) radiological methods of examination of the brain, confirming the malformation (MRI, PET); 7) specific immunological studies cerebrospinal fluid for verification of intrauterine encephalitis.

Hereditary metabolic diseases account for 3%. The NBO clinic is characterized by polymorphism, diagnosis is difficult, and treatment is often ineffective. However, this figure may be higher, given the 3% of newborns in whom convulsions are combined with hypoglycemia, metabolic acidosis, jaundice, malnutrition, diarrhea, vomiting, hepato- and splenomegaly, nystagmus, cataracts, shortness of breath. A delay in the diagnosis of NBO is dangerous for the development of ICH, a septic process. Among the NBOs debuting NS are:

arginine succinate lyase deficiency;

deficiency of carbamoyl phosphate synthetase;

nonketotic hyperglycinemia;

sickness with the smell of maple syrup urine;

isovaleric acidemia;

propionic acidemia;

methylmalonic acidemia;

deficiency of acyl-CoA fatty acid dehydrogenase;

Zellweger's syndrome;

biotinidase deficiency;

ornithinecarbamoyltransferase deficiency;

tyrosinemia type I;

tryptophanuria;

Hyperornithemia - Hyperammonemia - Homocitrullinuria (HHH) syndrome.

The diagnostic standard includes features of the anamnesis and clinical manifestations characteristic of the group of congenital metabolic defects:

Leading signs of NBO: autosomal recessive type of transmission of a genetic defect, systemic nature of the lesion, steady progression.

Anamnestic data: genealogical history with the study of the pedigree - consanguineous marriage, neurological signs in one of the parents, no indication of pathology during pregnancy and childbirth, the presence of a well-being interval between the birthday and the first signs of the disease. Of particular note are: fetal death, spontaneous abortion, fetal hyperactivity in utero, death of children in early childhood, sudden infant death syndrome, Reye's syndrome.

Neurodistress syndrome (syndrome of acute neurological disorders): increased excitability or depression of the functions of the nervous system, anorexia, vomiting, weight loss, oculomotor disorders, unusual movements, muscle hypotension, impaired consciousness (lethargy, coma), hypothermia, pyramidal syndrome, multiple organ changes , psychomotor retardation.

Convulsions are polymorphic, multifocal, myoclonic, resistant to therapy, prone to a status course.

Respiratory distress: violation of the rhythm of breathing (hyperpnea, apnea, shortness of breath or acidotic breathing), which are caused by a toxic effect on the respiratory center, in the absence of pathology of the heart and lungs.

Extraneural anomalies are combined with neurological symptoms. The polysystemic lesion is manifested by facial dysmorphias, skin and hair anomalies, skeletal disorders, cardiomyopathies, conduction disorders, arrhythmias, fibroelastosis, pulmonary anomalies, hepato- and splenomegaly, pancreatic, kidney, polycystic, hearing impairment. The pathology of the visual analyzer (cataract, glaucoma, optic nerve hypoplasia, retinal degeneration) and specific changes in the smell and color of urine are also characteristic.

The combination of two of the above symptoms should direct clinical thinking towards innate errors of exchange.

Phakomatosis (1.5-2%). The manifestation of NS in this case is variable, the clinic is polymorphic. The prognosis depends on the nature of the pathology. Tuberous sclerosis (TS) and encephalotrigeminal angiomatosis occupy the main place among the phakomatoses manifested by NS. The therapy is ineffective.

Seizures in TS are more often in the form of generalized or focal clonic, less often myoclonic atypical seizures. Skin manifestations are represented by depigmented oval spots of the "ash-leaf" type. Neuroradiological features are characterized by calcified subependymal and intracerebral tubers, which are usually identified in the second year of life.

NS in the form of focal clonic, less often tonic or atypical seizures, which are combined with a characteristic cavernous angioma on the head, according to the branches trigeminal nerve, glaucoma, and sometimes contralateral hemiparesis, are characteristic features Sturge-Weber syndrome. Treatment and prognosis depend on the nature of morphological changes in the brain.

Metabolic and toxic-metabolic disorders are the cause of NS in 5-10% of cases. The leading role among them belongs to hypocalcemia, hypomagnesemia, hypoglycemia.

Hypocalcemia - a condition in which serum calcium levels fall below normal limits: total calcium is reduced< 2,2 ммоль/л, ионизированный < 1,18 ммоль/л. Гипокальциемия встречается с момента рождения ребенка, но поскольку расходование кальция в организме новорожденного очень экономное, клинические симптомы гипокальциемии в виде судорог, тетании появляются при снижении уровня общего кальция у недоношенных ≤ 1,5 ммоль/л, у доношенных - ≤ 1,75-1,5 ммоль/л. По времени возникновения гипокальциемии подразделяют на ранние - в первые 24-48-72 часа жизни и поздние, как правило, они возникают на 6-7-й день после рождения.

Iatrogenic causes of hypocalcemia: prescription of barbiturates, steroid hormones, aminoglycosides, vincristine, amphotericin B, long-term use furosemide, the introduction of soda, citrate, heparin. It must be emphasized that one of the main mechanisms of iatrogenic hypocalcemia is a decrease in the level of magnesium, which leads to a decrease in the content of PTH.

The reasons for the decrease in ionized calcium with normal levels of total calcium: the introduction of citrate with replacement blood transfusions, heparin, intravenous fat emulsions, alkalosis against the background of hyperventilation or with the introduction of alkaline solutions. According to the duration of hypocalcemia are divided into transient and persistent.

Treatment of hypocalcemia in newborns

intravenous calcium gluconate 10% solution 1 ml/kg very slowly. In the case of hypocalcemia in small children, its correction is carried out at a rate of 1-1.5 mg / kg of elemental calcium per hour (only through a lineomat);

intramuscularly magnesium sulfate 25% solution 0.2 ml / kg 2 r / day.

If there is no effect, although, as a rule, it develops already "on the needle", then after 15-60 minutes you can repeat the introduction of calcium gluconate at the same dose. It should be noted that most often the failure of calcium administration is due to the fact that they forget to introduce magnesium sulfate, but, unfortunately, they introduce Relanium or Seduxen, which have no effect. Their introduction is inappropriate! To further maintain normal calcium levels, calcium supplements are administered orally at each feeding.

It must be remembered that 1 ml of 10% calcium gluconate solution is equal to 9 mg of elemental calcium. The presence of persistent hypocalcemia in a newborn is an absolute indication for consulting an endocrinologist and prescribing appropriate therapy.

Hypomagnesemia: a decrease in magnesium levels below 0.62 mmol / l (normal 0.62-0.91 mmol / l). Causes: persistent diarrhea, taking diuretic drugs, the introduction of hyperosmolar glucose solutions, an excess amount of chloride and calcium gluconate, a violation of the intake of magnesium from food, a malabsorption defect in the intestine. Clinical symptoms: generalized and focal convulsions, hyperexcitability, tremor, muscle trembling, unusual cry, muscle hypotension, edema, bradycardia, respiratory rhythm disturbance.

Hypoglycemia: Decreased glucose levels below 2.8 mmol/L in a term infant and 1.1 mmol/L in a premature infant.

Causes of hypoglycemia: pathology of pregnancy (anomaly of the placenta, multiple pregnancy), prematurity and malnutrition, asphyxia, birth trauma, sepsis, meningitis, hyaline membrane disease, treatment of the mother with sulfonamides, administration of more than 6 g of glucose per hour to the mother during childbirth, sudden cessation of glucose administration newborn, late breastfeeding, adrenogenital syndrome, hemorrhage in the adrenal glands. Hyperinsulinism (adenoma and hyperplasia of the pancreas, diabetes at mother). NBO - organic aciduria (propionic, methylmalonic, isovaleric, leucinosis, tyrosinemia), mitochondrial encephalomyopathy, glycogenosis. Beckwith-Wiedemann syndrome (exophthalmos, macroglossia, gigantism and pancreatic hyperplasia).

Clinic: convulsions, breast rejection, shrill crying, cyanosis, tachypnea and apnea, tachycardia, tremor, muscle hypotension.

Treatment of hypoglycemia: intravenous bolus 10% glucose solution 2 ml/kg for 5-10 minutes, followed by a drip of 6-8 mg/kg/min. Monitor blood glucose levels after 30 minutes. When subnormal levels are reached, switch to 5% glucose solution.

Pyridoxine-dependent NS occur at low levels of pyridoxine and its coenzyme, pyridoxal-5-phosphate, in the blood. Pyridoxine and its coenzymes are involved in the synthesis of antiepileptic substrates and inhibitory mediators in the CNS. Pyridoxine deficiency is observed with alimentary insufficiency, aminoacidopathy. Pyridoxine-dependent convulsions can occur in utero (in this case, the mother notes rhythmic clonic twitches) and in the first 72 hours of life. Clinically, pyridoxine-dependent NS are manifested by generalized clonic, myoclonic contractions of the "peck" type and generalized shudders. This type of NS is often associated with developmental delay. The EEG shows specific slow-wave activity. For the relief of seizures, pyridoxine is prescribed - at least 100 mg per day.

Among the toxic-metabolic disorders leading to NS, hyperbilirubinemia is distinguished. The clinic of bilirubin encephalopathy consists of a classic symptom complex: lethargy, rigidity, opisthotonus, high-pitched cry, fever, and convulsions. NS caused by bilirubin damage to the brain (kernicterus) occurs on the 5th-7th day of life and usually manifests itself as generalized tonic or fragmentary convulsions with the development of apnea and cyanosis.

NS due to the toxic effects of anesthetics and medications. Local anesthetics used in parturient women during epidural anesthesia, paracervical blockade (lidocaine) or locally during episiotomy, can penetrate the placental barrier. At the same time, clinical manifestations resemble conditions caused by asphyxia: bradycardia, hypotension, apnea, impaired reflex activity, oculocephalic reflex and pupillary reactions, dilated pupils. Seizures develop in the first 6 hours of life and proceed in the form of a generalized tonic seizure. Often combined with apnea and pulmonary hypoventilation. Unlike infants with HIE, these newborns spontaneously improve after 24-48 hours. Therapy is aimed at eliminating the drug by forced diuresis. The use of anticonvulsants is inappropriate.

The state of cerebral excitability (tremor, hypersensitivity to sensory stimuli, child's excitability and motor restlessness, reduced sleep duration, increased muscle tone, autonomic disorders), turning into a seizure, can be observed with the so-called withdrawal syndrome. Most often, these disorders are recorded in children of mothers who took narcotic and medications during pregnancy. Substances that most often cause passive dependence in the fetus include: narcotic analgesics, alcohol, barbiturates, tricyclic antidepressants. Convulsions are accompanied by bouts of cyanosis, areflexia and can last up to 3-7 days. On the 4-6th day, gastrointestinal disorders (sluggish sucking, regurgitation, vomiting and diarrhea) join. Therapeutic effect achieved with the appointment of phenobarbital or diazepam (for gastrointestinal disorders).

To identify the main etiological factor and properly differentiate convulsions related to the neonatal period from non-epileptic events in this period is another task of differential diagnosis. Paroxysmal conditions of non-epileptic origin include: jitteriness, apnea of respiratory and cardiac origins, ophthalmic non-convulsive phenomena, hyperexplexia, benign nocturnal neonatal myoclonus, tonic postures, Sandifer's syndrome.

Jitteriness (hyperexcitability) - rapid generalized trembling of the whole body. Tremor may occur spontaneously or be provoked by tactile or auditory stimulation and is not associated with ophthalmic and autonomic phenomena. Consciousness is preserved. Trembling decreases with passive flexion or a change in the position of the limbs. Seizures, unlike tremors, are clonic, often associated with ophthalmic and autonomic phenomena, and do not respond to passive motor and sensory stimuli.

Other motor non-epileptic phenomena are: large-scale tremor, which appears when an asymmetric neck tonic reflex is evoked and is due to the reaction of the red nucleus, extension paroxysms with dorsiflexion thumbs arms, extension with leg tremor, spontaneous Babinski reflex (stretches), cyclic movements, grimaces. All these phenomena are causally determined, induced by external stimuli and, unlike myoclonic, tonic and clonic seizures, are stopped by a change in the position of the child or passive flexion of the limbs.

Apnea of respiratory and cardiac genesis should be distinguished from epileptic apnea, in which the heart rate is stable, and apnea is combined with autonomic phenomena, paroxysms of transient muscular hypotension and changes in the EEG.

Ophthalmic non-convulsive phenomena: nystagmus, fixed gaze, deviation of the eyeballs, Graefe's and Willy's symptoms, opsoclonus. All these phenomena are usually causally determined and occur with vestibular loads. They are not accompanied by a violation of the rhythm of breathing and motor stereotyped reactions. Ophthalmic convulsions are spontaneous, involuntary, occur at rest, are accompanied by apnea attacks, an autonomic reaction, and motor stereotypes.

Opsoclonus. Rapid, conjugating, multidirectional movements of the eyeballs, aggravated by sound stimulation. In some cases, it is accompanied by myoclonic twitches of various muscle groups. Consciousness is not disturbed. Opsoclonus is commonly seen in neonatal forms of degenerative disease. In the future, when it is combined with myoclonus and ataxia, it is necessary to carry out differential diagnostics with an intracranial volumetric process.

Hyperexplexy. hereditary disease, occurs only in response to provocation, even minor stimuli. It is based on a pathological strengthening of the quadrigeminal "start reflex" of the midbrain. In severe cases, the child, taken in his arms, stretches, there is a diffuse increase in muscle tone, sometimes apnea and bradycardia. The tonic episode is stopped by forcible flexion of the neck or hips. The EEG is characterized by normal basic rhythms.

Benign nocturnal neonatal myoclonus. Rapid myoclonic twitches of various muscle groups. Myoclonus bilateral, asynchronous, asymmetric, often migrate from one part of the body to another and are observed during sleep. They debut in the first week of life. Unlike myoclonus of epileptic origin, the duration of paroxysms of benign myoclonus is shorter (several minutes). Video control and EEG do not show pathological epileptic patterns.

Tonic postures are accompanied by increased intracranial pressure, kernicterus, ICH, irritation of the meninges, decerebrate rigidity due to compression of midbrain structures. This kind of muscle tension is also causally determined. An indirect distinguishing feature is the strength of muscle tension - rigidity during tonic convulsions is pronounced, does not decrease in response to external influences, while tonic tension of non-epileptic origin decreases or increases with a change in the position of the child's body.

Sandifer syndrome. With hiatal hernia and gastroesophageal reflux, infants develop "dystonic" postures (torso twist, head tilt, development of torticollis) that are associated with food intake and facilitate its passage from the esophagus to the stomach.

Thus, when diagnosing NS, it is necessary to take into account the presence in infants of certain conditions that are not related to convulsive phenomena and do not require specific treatment.

NS require close attention and strict monitoring in order to establish the true genesis of the convulsive state in the newborn as quickly as possible. The identification of metabolic disorders and infections of the central nervous system is urgent. However, it should be noted that even with the full use of the entire modern arsenal of diagnostic tools, the causes of 10% of seizures remain unknown.

When determining the tactics of therapy, a number of fundamental questions arise: what is the genesis of NS, when should anticonvulsants be prescribed, the choice of the first drug and its dose, the need to change the antiepileptic drug, the use of polytherapy, determining the time to cancel treatment.

Therapy for convulsive conditions of the neonatal period is divided into standard (starting, traditional) and alternative. Alternative therapy is prescribed for resistant NS, when there are risk factors for severe neurological deficit. In addition to combining various anticonvulsants, an alternative approach in the treatment of NS also includes a specific diet, enpits, vitamins, or cofactors specific to inborn errors of metabolism.

With the status, it is necessary to be able to conduct mechanical ventilation, to administer drugs intravenously:

Phenobarbital: 10 mg/kg, then 1 mg/kg/h to 40 mg/kg/day;

tonic and myoclonic nature of NS;

high frequency, polymorphism of seizures, status and serial course;

Apgar score below 4 points, neonatal resuscitation;

IVL for more than 7 days;

structural changes in the brain during neuroimaging;

resistance to ongoing starting anticonvulsant therapy;

cerebral malformations, NBO, phakomatoses.

It must be remembered that valproic acid is contraindicated in hyperammonemia and non-ketotic hyperglycinemia.

The answer to the question about the timing of therapy (several days before EEG normalization or within 4-6 months) requires taking into account the entire spectrum of causes of NS and the probability of recurrence, which is 4-20%. When the convulsions are stopped, J.J. Volpe recommends a step-by-step approach to stopping anticonvulsants. And completely cancel them if the results of neurological studies are normal (interictal EEG corresponds to age, there are no neurological symptoms, gross structural anomalies). If the results are abnormal, the cause should be considered and the anticonvulsant should be changed taking into account the semiotics and phenomenology of the seizures. If the neurological status remains normal at follow-up examinations for 1 month, the anticonvulsant can be discontinued within 2 weeks. If neurological symptoms persist and there are no epileptic patterns on the EEG, treatment should be continued. If there is abnormal activity on the EEG, anticonvulsants are prescribed for a long time. It is recommended to repeat the examination every 3 months.

When predicting the outcome of NS, it is necessary to take into account several factors: the genesis of NS (NBO, phakomatosis, brain anomalies), the age of the infant at their manifestation, the features of structural changes in the brain (the most unfavorable cerebral malformations), the nature of NS (tonic and myoclonic), the presence of a family history of epilepsy.

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There are many forms of epilepsy that occur exclusively in childhood or adolescence. It is the age dependence of many varieties of epilepsy that is the main distinguishing feature of childhood epileptology.

Epilepsy and convulsive syndromes of the neonatal period

Although the duration of the neonatal period is short, a number of epileptic syndromes are characteristic of newborns.

Benign familial seizures (convulsions) in newborns

Benign neonatal epilepsy (with an autosomal dominant type of inheritance) of three types, manifested in the first 7 days of life (starting from three days). The family history necessarily includes indications of the presence of seizures in the past in the patient's family members (in the neonatal period). The connection of seizures with specified congenital metabolic disorders has not been established. Benign familial neonatal seizures manifest as focal and multifocal or generalized tonic-clonic (convulsive) seizures. These seizures are characterized by a short duration (1-2 minutes) and a significant frequency (20-30 episodes per day). Subsequently, after 1 to 3 weeks, the seizures stop spontaneously spontaneously.

Benign non-familial convulsions (seizures) of the newborn ("fifth-day seizures")

This epilepsy with a debut in the early neonatal period has also another name (benign idiopathic neonatal seizures). The disease was first described in the late 1970s. Convulsive seizures develop in full-term newborns who have not previously had signs of pathology from the central nervous system. The debut of seizures occurs by the end of the 1st week of life (in 80-90% of cases - between the 4th and 6th days), and their peak occurs on the 5th day of life (hence the name). The seizures described usually take the form of multifocal clonic convulsions, which are often accompanied by apnea. In most cases, benign idiopathic neonatal seizures last no more than 24 hours (they always stop after 15 days after onset). In 80% of cases, during the convulsive period in newborns, the development of status epilepticus is noted.

Early infantile epileptic encephalopathy with EEG suppression/flare pattern (Otahara syndrome)

Early infantile epileptic encephalopathy is a rare disease related to malignant forms of childhood epilepsy. It usually debuts in the neonatal period (or at the age of 1-3 months). The disease is characterized by tonic seizures, the frequency of which varies greatly (10-300 episodes per day). In children, there is a rapid formation of a neurological deficit and mental retardation. A specific burst/suppression pattern in electroencephalography (EEG) is present in children with Otahara syndrome both in sleep and wakefulness. With magnetic resonance imaging (MRI) of the brain, patients have gross anomalies in the development of the central nervous system. Among children with early infantile epileptic encephalopathy with a pattern of "flare/suppression" on the EEG, mortality by the age of 1 year reaches 40-50%. At 4-6 months of age, Otahara syndrome can transform into West syndrome.

Early myoclonic (epileptic) encephalopathy

Described by J. Aicardi and F. Goutières (1978); debuts mainly in the neonatal period (sometimes up to 3 months of age). In the genesis of the disease, the role of genetic factors and some "congenital errors of metabolism" (propionic aciduria, methylmalonic acidemia, urine disease with the smell of maple syrup, etc.) is assumed. Clinically manifested by frequent myoclonic seizures. The latter are usually not associated with EEG changes during an attack, but in some cases epileptiform discharges "depression/flare" are recorded simultaneously with myoclonus. Myoclonus is more often fragmentary (slight twitching of the distal extremities, eyelids, or corners of the mouth); at the same time, focal (partial) seizures, massive myoclonus and tonic spasms can be noted (isolated or serial - occur by 3-4 months). The appearance of tonic spasms in a child suggests the presence of atypical West syndrome, but soon the main manifestations of the disease resume and persist for a long time. Focal seizures (complex partial seizures - with eyes opening or autonomic symptoms: apnea, facial flushing; clonic convulsions in different parts of the body, etc.) become the main type of seizures in early myoclonic epileptic encephalopathy. An interictal EEG study in children registers a depression/flash pattern, consisting of discharges lasting 1–5 sec, alternating with almost isoelectric periods (lasting 3–10 sec). The described EEG pattern becomes more distinct during sleep (especially in the deep sleep phase). The initial depression/flare pattern after reaching the age of 3-5 months is replaced by atypical hypsarrhythmia or multifocal paroxysms, but in most cases this is only a transient phenomenon. The disease is accompanied by high mortality or progressive decay of psychomotor functions (up to vegetative status), although as age increases, the frequency and severity of focal seizures and myoclonus gradually decrease.

Vitamin B 6 dependent epilepsy

A relatively rare hereditary disease characterized by drug-resistant seizures. Belongs to the group of metabolically caused epilepsy. It develops in newborns whose mothers received pyridoxine for a long time during pregnancy, as well as in a specific hereditary metabolic defect (with an increased need for vitamin B6). There are cases of the onset of pyridoxine-dependent seizures in children older than 1 month and even in the second year of life. Between seizures, children remain restless, react with muscle twitches to external stimuli. The disease is not amenable to conventional anticonvulsant treatment, but the appointment of vitamin B 6 in high doses (25 mg / kg / day) quickly leads to the normalization of the condition.

Malignant migratory partial seizures (seizures) of infancy

An extremely rare epileptic syndrome described by G. Coppola et al. (1995). To date, only about 50 cases of the disease have been reported in various countries of the world. Malignant migrating partial convulsions in 50% of cases are observed in the first days of life; the remaining 50% fall on the age of 1-3 months. At the onset, seizures are focal clonic in nature, and after a few weeks they become multifocal, moreover, they are extremely frequent and pharmacoresistant to antiepileptic therapy. An EEG study in children reveals pronounced multifocal epileptic activity; metabolic disorders are not detected, and MRI signs pathological changes missing. Pathological anatomical examination revealed signs of neuronal loss in the hippocampus.

Epilepsy in children of the first year of life (1-12 months)

Upon reaching 1 month of age, the number of varieties of epileptic syndromes specific to the first year of a child's life is practically not inferior to that characteristic of the neonatal period.

Infantile spasms (West syndrome)

This variant of catastrophic epilepsy (generalized) is symptomatic (the vast majority of cases) or cryptogenic (10-20%). It manifests itself in children in the first year of life (more often between the 3rd and 8th months). In the classical version, West syndrome is characterized at the time of the attack by a combination of flexion and extensor movements, that is, severe myoclonic (Salaam) spasms, sometimes serial short flexion movements of the head (“nods”). Infantile spasms can develop both due to the presence of various neurological pathologies, and without any obvious previous disorders of the central nervous system. With infantile spasms, psychomotor development slows down, and in the future there is a high probability of a pronounced developmental delay. In 80% of cases with West syndrome, microcephaly, signs of infantile cerebral palsy, atonic-atactic disorders, etc. are found. ) background. The prognosis of West syndrome is determined by the effectiveness of the therapy, but is generally unfavorable.

Severe myoclonus epilepsy of infancy (Dravet's syndrome)

The disease described by C. Dravet (1978, 1992) debuts in the first year of life (between the 2nd and 9th months), which often occurs after the development of a febrile episode, shortly after vaccination or infection. Dravet syndrome is characterized by the appearance of generalized or unilateral clonic convulsions (usually against the background of hyperthermia or fever), which occurs against the background of the previous normal psychomotor development of the child during the first year of life. Gradually (over several weeks or months), the child develops afebrile myoclonic and partial (focal) seizures. A progressive increase in the frequency of myoclonus (isolated or serial) precedes the onset of generalized seizures in patients. Children have moderate cerebellar and pyramidal signs associated with gross motor deficits and gait ataxia. Disorders of psychomotor development are subsequently noted in children up to about 4 years of age. Often with Dravet syndrome, children develop status epilepticus (convulsive or myoclonic). EEG data during the first year of life usually remain within the normal range, although spontaneous photoinduced spike-wave discharges occur in some patients. Subsequently, ictal EEG studies in Dravet syndrome are characterized by the presence of myoclonic or clonic seizures (generalized peak-wave or polypeak-wave activity). Generalized discharges increase in a state of relaxation; focal and multifocal peaks and sharp waves are observed simultaneously. Traditional and new antiepileptic drugs usually do not prevent the recurrence of seizures in Dravet syndrome. The prognosis for intellectual development in Drave's syndrome is always unfavorable.

Idiopathic benign partial epilepsies of infancy

Usually debut in children aged 3-20 months (more often between the 5th and 8th months). First described by K. Watanabe et al. (1987), as a result of which they initially received the general name "Watanabe's syndrome". They are characterized by manifestations in the form of complex partial (focal) seizures and a favorable prognosis (elimination of epileptic seizures within 3 months after the onset). The average number of attacks is about 7; some patients have exceptionally complex partial seizures, others have only secondary generalized seizures, and in about half of the cases there is a combination of them. During an attack, patients are characterized by decreased response to stimuli, cessation of motor activity, moderate convulsive twitches, lateral eye opening, and cyanosis. Main clinical signs This group of epilepsies is characterized by a high incidence of cluster seizures, a short duration of seizures, as well as initially normal parameters of the interictal EEG study (subsequently, paroxysmal discharges may be detected in some children).

Similar to idiopathic benign partial epilepsy of infancy, but exclusively familial paroxysmal conditions with debut in the first year of life are called "benign infantile familial convulsions". In 1997, similar cases of familial epilepsy were described with the subsequent formation of choreoathetosis - family convulsions with choreathetosis.

Epilepsy in young children (1-3 years)

For young children (from 12 to 36 months), in the first place, Doose syndrome, Lennox-Gastaut syndrome, benign myoclonus-epilepsy of infancy, hemiconvulsion-hemiplegia syndrome, idiopathic partial epilepsy of infancy, absence epilepsy of early childhood, electrical status epilepticus slow-wave sleep, early and late childhood neuronal lipofuscinosis (types I and II).

Myoclonic astatic epilepsy of early childhood (Doose syndrome)

Represents epilepsy with myoclonic-astatic seizures (of varying duration). Attacks debut at the age of 1-5 years. More often the disease affects boys. Astatic and myoclonic seizures can be combined, with myoclonus occurring both before, during, and after an aseptic seizure. Attacks come on suddenly and are almost always accompanied by falls. Myoclonus is noted in the form of different severity of symmetrical twitches in the arms and muscles of the shoulders of the belt, which is combined with a tilt of the head (“nods”). There are no signs of loss of consciousness in children at the time of the attack. Before the onset of the disease, the psychomotor development of children usually corresponds to the norm. In some children, the disease is complicated by the risk of developing dementia (presumably due to the development of the status epilepticus of absences). EEG records generalized bilateral-synchronous complexes of peak waves (3 or more per 1 sec, 2-4 Hz). The prognosis for myoclonic-astatic epilepsy in early childhood is not very favorable.

Lennox-Gastaut syndrome, or early childhood myokinetic epilepsy with slow peak waves

A group of heterogeneous pathology with epileptic seizures (atonic, tonic, atypical absences), intellectual deficiency and a characteristic EEG pattern. As with West syndrome, symptomatic and cryptogenic variants of the disease are distinguished in Lennox-Gastaut syndrome. Early forms debut at about 2 years of age. Up to 30% of cases are the result of transformation from West syndrome. Clinically, the Lennox-Gastaut syndrome is characterized by myoclonic-astatic seizures, Salaam convulsions (lightning nodding), atypical absences, tonic seizures (often during sleep). Generalized tonic-clonic, myoclonic, and focal (partial) seizures may occur. For children, a series of seizures with changes in consciousness (stupor) and a gradual transition to status epilepticus are typical. Apart from epileptic seizures, in the neurological status, cerebral paresis / paralysis, as well as atonic-astatic disorders (up to 40% of patients) may be noted. In children, there is a decrease in intelligence (of varying degrees), pronounced impairments in cognitive functions are observed. According to EEG data, changes in background activity in the form of slow peak waves are typical.< 3 Гц, ночные серии пиков (гипсаритмия с наличием «острых» феноменов). Часто присутствуют мультифокальные изменения. Методы нейровизуализации позволяют выявить фокальные и диффузные структурные нарушения. До 75-80% случаев болезни резистентны к проводимой терапии. Прогноз вариабелен, но в целом считается малоблагоприятным .

Benign myoclonus epilepsy of infancy

It debuts in children under 3 years of age (usually at 1-2 years), although it can sometimes manifest from the age of 4-11 months. Before the onset of the disease, there are usually no previous signs of psychomotor developmental disorders. Refers to generalized / idiopathic or symptomatic epilepsy. It differs from Dravet's syndrome in lesser severity and a more favorable prognosis. Benign myoclonus epilepsy of infancy is characterized by the appearance of short-term myoclonic seizures involving the head and upper limbs. Other types of seizures do not occur in this form of epilepsy, and the frequency and intensity of existing paroxysms is variable. A moderate delay in intellectual development is possible. The diagnosis is established on the basis of the clinical features of seizures, as well as according to EEG data. An ictal EEG study determines generalized epileptic activity with irregular peaks, peak waves, sharp waves (more often asymmetric than bilaterally synchronous); interictal EEG data are either unchanged or moderately disturbed (sharp waves, peaks, peak-wave complexes, acute/slow wave - with a predominance of early stages sleep).

Hemiconvulsion-hemiplegia syndrome (HHS)

The disease often debuts at an early age (1-3 years), although the onset of the disease is possible in the period from the 1st to the 4th year of life (starting from about 5 months). The first manifestations are suddenly developed prolonged hemiconvulsions in the form of clonic convulsions (status), which are sometimes (not in all cases) associated with hyperthermia (febrile convulsions). After a convulsive episode in children, hemiplegia is noted. After 12-36 months, patients develop focal (partial) epilepsy. When EEG in children, there is a peak- and polypeak-slow-wave activity with a frequency of 10-12 Hz (mainly in the occipital leads). In some cases, hemiconvulsion-hemiplegia syndrome is considered as an atypical consequence of prolonged febrile convulsions in infancy and / or early childhood.

Benign partial epilepsy of infancy and early childhood with vertex peaks and waves during sleep

Highlighted by Italian epileptologists G. Capovilla and F. Beccaria (2000) in contrast to benign partial epilepsy of infancy, described by K. Watanabe et al. (1987). It debuts at 13-30 months, EEG changes (characteristic peaks and waves during non-REM sleep with localization in the vertex regions of the brain) are observed only during sleep (EEG on awakening always does not show epileptiform changes), the clinical picture of seizures also has some differences . The prognosis for benign partial epilepsy of infancy and early childhood with vertex peaks and waves during sleep is favorable.

Absence epilepsy of early childhood

This epileptic syndrome, whose name was proposed by H. Doose et al. (1965), includes a heterogeneous group of patients. Absence epilepsy of early childhood is clinically characterized by generalized tonic-clonic convulsions and/or myoclonic-astatic seizures, the presence of irregular peak-wave discharges in the EEG (2-3 Hz), and often an unfavorable prognosis. Absence epilepsy of early childhood sometimes debuts at a later age - up to 5 years.

Eyelid myoclonus with absences (Jivons syndrome)

Age-dependent epileptic syndrome, which is a form of photosensitivity epilepsy, was described by P. M. Jeavons (1977). It may debut earlier than childhood absence epilepsy (at 2-5 years), although it most often occurs at the age of 6-8 years. Slightly more common in girls. Most patients have a family history of idiopathic generalized epilepsy. J. Guiwer et al. (2003) emphasize that Jevons syndrome is a myoclonic rather than an absence syndrome. Although Jevons syndrome refers to idiopathic generalized epilepsy, it is possible that this concept includes a whole group of photosensitivity conditions. The main symptom of Jevons syndrome is myoclonus of the eyelids, and the main trigger factor for the disease is the closure of the eyes in the presence of light. Absences in Jevons syndrome are not always observed; they have a short duration (3-6 seconds), appear after closing the eyes and are accompanied by a clear rhythmic pattern of eyelid myoclonus, as well as retropulsion of the eyeballs in association with the tonic component of the muscles involved in the pathological process. Absences in Jevons syndrome do not appear without eyelid myoclonus. In most cases, generalized tonic-clonic seizures are observed in children, but the frequency of these seizures is relatively low. EEG in patients recorded electrophysiological paroxysms associated with eye closure and photosensitivity. Disturbances of consciousness in Jevons syndrome are not as pronounced as in childhood absence epilepsy or juvenile absence epilepsy. Intellectual development in myoclonus of the eyelids with absences is practically not affected, although mild or moderate intellectual deficits have been reported in some cases. The diagnosis is easily confirmed by video-EEG, since it reveals the association of generalized paroxysmal activity with eye closure. In general, the prognosis for Jevons syndrome is favorable, although this form of epilepsy is more often lifelong. Cases of the formation of drug resistance of the disease are known.

Early childhood (infantile) neuronal ceroid lipofuscinosis type I (classic)

Representative of the group of progressive myoclonus epilepsy. It is associated with a deficiency of the enzyme α-neuraminidase (sialidase), also known as "myoclonus cherry stone syndrome" or "Santavuori-Haltia disease". May appear from 6 to 24 months of age. The main clinical manifestations of the disease are myoclonus and generalized tonic-clonic seizures, which are subsequently joined by ataxia with gait disorder. The disease is characterized by the presence of a “cherry stone” symptom in the fundus and a progressive decrease in vision (up to blindness). In some cases, the intellectual development of children does not suffer, but some patients develop dementia. With the help of neuroimaging methods, diffuse atrophy of the hemispheres of the cerebral cortex and cerebellum can be detected. Therapy consists in the use of antimyoclonic drugs, nootropics, levocarnitine, vitamins (E, A, group B).

Late childhood (infantile) neuronal ceroid lipofuscinosis type II

Like early childhood neuronal lipofuscinosis, the late form of the disease refers to progressive myoclonus epilepsy. This type of pathology was previously also known under the name "Jansky-Bilshovsky disease", or "late childhood amaurotic idiocy". Late childhood neuronal ceroid lipofuscinosis type II usually appears later than early (type I), from the end of the 1st year of life to 2-3 years of age, although congenital forms of the disease have also been described. The disease is characterized by myoclonic seizures and ataxia. In addition to the described damage to the central nervous system, children also suffer from other internal organs and the musculoskeletal system (sensorineural hearing loss, hernias - inguinal, scrotal, umbilical; hepatosplenomegaly, pathological joint mobility, gradually changing to limitedness, etc.). An ophthalmological examination revealed a symptom of "cherry pit", as well as clouding of the cornea. Characterized by profound mental disorders. The diagnosis is confirmed by the study of neuraminidase activity in fresh fibroblasts and leukocytes. The EEG shows fast low-voltage activity in patients, although it slows down in dementia. Generalized peak-wave outbreaks are absent or rare. The principles of treatment used in late childhood neuronal lipofuscinosis type II are consistent with those in the early form of type I disease.

Continue reading the article in the next issue.

V. M. Studenikin, doctor of medical sciences, professor, academician of the Russian Academy of Natural Sciences

FSBI "NTsZD" RAMS, Moscow

To determine the further prognosis, the etiological factors of seizures play the greatest role. For example, children whose seizures develop as a result of congenital brain anomalies, hypoxia-ischemia, or postnatal hypocalcemia have a poorer prognosis compared with children with small subarachnoid hemorrhages or transient hypocalcemia.

EEG is also valuable predictive criterion in newborns with seizures. Moreover, the main background of bioelectrical activity is more important for the prognosis than the nature of epileptiform changes. Children with frequent and prolonged seizures usually have a worse prognosis than children with infrequent seizures. However, there are exceptions: children with benign familial neonatal seizures have frequent seizures and an excellent prognosis. Finally, children with normal neurological status during seizures have a better prognosis than children with neurological impairment.

Benign familial neonatal seizures

Unlike older children, not many epileptic syndromes have been described in newborns, since not all neonatal seizures are a symptom. More often, neonatal seizures develop in response to an acute disorder. cerebral circulation. However, five epileptic syndromes are known in newborns and infants, three of which have a favorable prognosis and two of which have an unfavorable prognosis: benign familial neonatal seizures (also called familial neonatal seizures), benign neonatal seizures, benign partial epilepsy of infancy, early infantile epileptic encephalopathy of infancy (CEEM). ), early myoclonic epileptic encephalopathy (EMEE).

The diagnosis of benign familial neonatal seizures in newborns with seizures is based on five criteria:

normal neurological status;
no other cause for seizures;
normal further development and normal intelligence;
- positive family anamnesis in relation to seizures in newborns or infants;
onset of seizures during neonatal or infancy.

In many children, seizures will debut in the first week of life, and only in a small number of cases later. This condition is one of several hereditary epileptic syndromes of the newborn. Linkage analysis in large families of patients with benign neonatal seizures revealed two disease loci located on chromosomes 20ql3.3 and 8q24. These genes code for voltage-dependent potassium channels expressed in the brain (KCNQ2 and KCNQ3). Attacks, usually frequent in the first days of life, then stop. In the period between attacks, children, as a rule, are completely healthy. The most common type of seizures is clonic seizures, focal or multifocal, but generalized seizures also occur. Generalized seizures are short, lasting no more than 1-2 minutes, but can develop frequently, up to 20-30 times a day.

The interictal EEG is of little help in the diagnosis of benign familial neonatal seizures because it can be either normal or abnormal. No specific diagnostic changes were found on the EEG. If any disturbances are detected on the EEG, then they are usually transient. Ictal EEG is characterized by flattening of the main rhythm, and then there are bilateral changes in the form of spikes and sharp waves. These changes may correlate with a generalized seizure.

Morozova T.M., Evtushenko S.K., Omelyanenko A.A., Balakhonova O.N., Donetsk National Medical University named after I.I. M. Gorky

Summary

Keywords

convulsions, newborns, treatment.

Neonatal seizures (NS)- this is a polyetiological clinical syndrome of the neonatal period, which indicates primarily cerebral disorders.

In most cases (over 90%), NS are symptomatic, and only about 10% are hereditary (idiopathic NS). According to the observations of Mizrahi and Kellaway, hypoxic-ischemic encephalopathy (HIE) has the largest share (32%), intracranial hemorrhages (ICH) account for 17%, intrauterine infections (IUI) with damage to the central nervous system - 14%, cerebral malformations - 7% , metabolic disorders - 6%, congenital metabolic disorders - 3%, phakomatoses - 2% and unknown causes - 10%.

Since the immature brain is highly epileptogenic, the presence of NS is often the first sign of neurological dysfunction. Seizures usually indicate the severity of the pathology and are the main symptom that predicts cognitive and motor deficits in the further development of the child. The prognosis of NS is unfavorable in most cases, mortality is from 15 to 40%. In 11-90% of surviving children, dramatic and long-term consequences are manifested by epileptic encephalopathy with cognitive impairment, learning and communication difficulties, deviant behavior, motor delay.

The attack reduces the levels of ATP, phosphocreatine, glucose and increases the level of ADP, pyruvate, lactate => transition to glycolysis and anaerobic metabolism => glucose deficiency, hypoxia.

An increase in lactate => local vasodilation and impaired autoregulation of cerebral blood flow => increased risk of bleeding (from the germinal matrix and into the penumbra) of ischemic infarcts).

A family history of convulsions in the neonatal period suggests that the infant has a genetic syndrome. Some of these syndromes are considered benign and often disappear within the neonatal period.

A detailed pregnancy history, looking for signs that suggest the possibility of TORCH infection, fetal distress, preeclampsia, or maternal infection may also facilitate the search for etiology.

Taking into account the features of the morphofunctional maturity of the CNS of a newborn, the following types of NS are distinguished:

Fragmentary NS:

ophthalmic (ocular);
oroalimentary;
motor;
vegetative.

Clonic NS:

focal;
multifocal;
generalized (bilateral).

Myoclonic NS:

focal;
multifocal;
generalized.

Tonic NS:

focal;
generalized.

Phenomenological classification of NS (J. Volpe, 2001) and their semiotics:

1. Fragmentary NS (soft, atypical, erased, abortive - subtle).

1.1. Ocular phenomena: a) tonic deviation; b) rhythmic nystagmoid twitching of the eyeballs);

1.4. Seizures: a) limpness; b) fading; c) loss of consciousness; d) diffuse decrease in muscle tone; e) cessation of motor activity.

1.5. Vegetative reactions: short-term changes: a) heart rate and blood pressure; b) skin color (cyanosis); c) salivation; d) hiccups.

1.6. "Convulsive" apnea.

2. Clonic NS (CNS): rhythmic muscle twitching of individual parts of the trunk, face and limbs, usually with a frequency of 1-3 per second. They occur in children over 36 weeks of gestation.

Genesis: cerebral infarction, hematoma, bacterial meningitis, arteriovenous malformation, tumor, etc. On the EEG - focal foci of epileptic activity according to the type of "peak - wave" complexes.

Genesis: electrolyte disturbances (hypomagnesemia, hypocalcemia), low pyridoxine levels, impaired cortical differentiation and migration. Rarely occur in the recovery phase after acute asphyxia.

Genesis: HIE, birth trauma, metabolic disorders.

3. Myoclonic NS (MNS).

3.3. Generalized MHC - a combination of "pecks" with flexor flexion or extension of the limbs, nodding of the head. Relatively symmetrical, synchronous myoclonic jerks.

Genesis: severe diffuse brain damage, terminal phase of asphyxia, hereditary metabolic diseases (NBO), hereditary degenerative diseases, cerebral malformations. In the future, myoclonic seizures can become an integral part of the West, Otahara, Ajkardi, Lennox-Gastaut syndromes.

4. Tonic NS (TNS): indicate damage to the structures of the forebrain.

Consider the main etiological factors of symptomatic NS.

Hypoxic-ischemic encephalopathy (35-56%). Pathological condition caused by severe hypoxemia and ischemia: Apgar score below 4 points, resuscitation from the first minutes of life. There is a decrease in the level of PO2 below 40 mm of water column, an excess of CO2, metabolic acidosis - blood pH below 7.2. Clinical manifestations: cerebral depression to coma, symptoms of increased intracranial pressure, cerebral edema. Convulsions in the 1st-3rd day of life, often recurring, generalized clonic, tonic, multifocal, atypical with a status course. Insufficient susceptibility to anticonvulsants.

The standard for diagnosing cerebral malformations: 1) the absence of a clear precedent, including severe hypoxia during childbirth; 2) convulsive syndrome resistant to therapy; 3) muscular hypotension during the neonatal period; 4) focal neurological deficit; 5) a delay in the pace of psychomotor development and a violation of the formation of postural reflexes; 6) radiological methods of examination of the brain, confirming the malformation (MRI, PET); 7) specific immunological studies of cerebrospinal fluid for verification of intrauterine encephalitis.

arginine succinate lyase deficiency;

deficiency of carbamoyl phosphate synthetase;

nonketotic hyperglycinemia;

sickness with the smell of maple syrup urine;

isovaleric acidemia;

propionic acidemia;

methylmalonic acidemia;

deficiency of acyl-CoA fatty acid dehydrogenase;

Zellweger's syndrome;

biotinidase deficiency;

ornithinecarbamoyltransferase deficiency;

tyrosinemia type I;

tryptophanuria;

Hyperornithemia - Hyperammonemia - Homocitrullinuria (HHH) syndrome.

The diagnostic standard includes the features of the anamnesis and clinical manifestations characteristic of the group of congenital metabolic defects:

Leading signs of NBO: autosomal recessive type of transmission of a genetic defect, systemic nature of the lesion, steady progression.

Convulsions are polymorphic, multifocal, myoclonic, resistant to therapy, prone to a status course.

The combination of two of the above symptoms should direct clinical thinking towards innate errors of exchange.

NS in the form of focal clonic, less often tonic or atypical seizures, which are combined with a characteristic cavernous angioma on the head, respectively, branches of the trigeminal nerve, glaucoma, and sometimes contralateral hemiparesis, are characteristic features of the Sturge-Weber syndrome. Treatment and prognosis depend on the nature of morphological changes in the brain.

Metabolic and toxic-metabolic disorders are the cause of NS in 5-10% of cases. The leading role among them belongs to hypocalcemia, hypomagnesemia, hypoglycemia.

Iatrogenic causes of hypocalcemia: the appointment of barbiturates, steroid hormones, aminoglycosides, vincristine, amphotericin B, long-term use of furosemide, administration of soda, citrate, heparin. It must be emphasized that one of the main mechanisms of iatrogenic hypocalcemia is a decrease in the level of magnesium, which leads to a decrease in the content of PTH.

Treatment of hypocalcemia in newborns

intramuscularly magnesium sulfate 25% solution 0.2 ml / kg 2 r / day.

Hypoglycemia: Decreased glucose levels below 2.8 mmol/L in a term infant and 1.1 mmol/L in a premature infant.

Causes of hypoglycemia: pathology of pregnancy (anomaly of the placenta, multiple pregnancy), prematurity and malnutrition, asphyxia, birth trauma, sepsis, meningitis, hyaline membrane disease, treatment of the mother with sulfonamides, administration of more than 6 g of glucose per hour to the mother during childbirth, sudden cessation of glucose administration newborn, late breastfeeding, adrenogenital syndrome, hemorrhage in the adrenal glands. Hyperinsulinism (adenoma and hyperplasia of the pancreas, maternal diabetes). NBO - organic aciduria (propionic, methylmalonic, isovaleric, leucinosis, tyrosinemia), mitochondrial encephalomyopathy, glycogenosis. Beckwith-Wiedemann syndrome (exophthalmos, macroglossia, gigantism and pancreatic hyperplasia).

Clinic: convulsions, breast rejection, shrill crying, cyanosis, tachypnea and apnea, tachycardia, tremor, muscle hypotension.

Treatment of hypoglycemia: intravenous bolus of 10% glucose solution 2 ml/kg for 5-10 minutes, followed by drip injection of 6-8 mg/kg/min. Monitor blood glucose levels after 30 minutes. When subnormal levels are reached, switch to 5% glucose solution.

NS caused by the toxic effect of anesthetics and medications. Local anesthetics used in parturient women for epidural anesthesia, paracervical blockade (lidocaine), or topical episiotomy may cross the placental barrier. At the same time, clinical manifestations resemble conditions caused by asphyxia: bradycardia, hypotension, apnea, impaired reflex activity, oculocephalic reflex and pupillary reactions, dilated pupils. Seizures develop in the first 6 hours of life and proceed in the form of a generalized tonic seizure. Often combined with apnea and pulmonary hypoventilation. Unlike infants with HIE, these newborns spontaneously improve after 24-48 hours. Therapy is aimed at eliminating the drug by forced diuresis. The use of anticonvulsants is inappropriate.

The state of cerebral excitability (tremor, hypersensitivity to sensory stimuli, child's excitability and motor restlessness, reduced sleep duration, increased muscle tone, autonomic disorders), turning into a seizure, can be observed with the so-called withdrawal syndrome. Most often, these disorders are recorded in children of mothers who took drugs and medications during pregnancy. Substances that most often cause passive dependence in the fetus include: narcotic analgesics, alcohol, barbiturates, tricyclic antidepressants. Convulsions are accompanied by bouts of cyanosis, areflexia and can last up to 3-7 days. On the 4-6th day, gastrointestinal disorders (sluggish sucking, regurgitation, vomiting and diarrhea) join. The therapeutic effect is achieved by prescribing phenobarbital or diazepam (for gastrointestinal disorders).

Other motor non-epileptic phenomena are: large-scale tremor, which appears when an asymmetric neck tonic reflex is evoked and is due to the reaction of the red nucleus, extension paroxysms with dorsiflexion of the thumbs, extension with leg tremor, spontaneous Babinski reflex (stretches), cyclic movements, grimaces. All these phenomena are causally determined, induced by external stimuli and, unlike myoclonic, tonic and clonic seizures, are stopped by a change in the position of the child or passive flexion of the limbs.

Hyperexplexy. Hereditary disease, occurs only in response to provocation, even minor stimuli. It is based on a pathological strengthening of the quadrigeminal "start reflex" of the midbrain. In severe cases, the child, taken in his arms, stretches, there is a diffuse increase in muscle tone, sometimes apnea and bradycardia. The tonic episode is stopped by forcible flexion of the neck or hips. The EEG is characterized by normal basic rhythms.

Thus, when diagnosing NS, it is necessary to take into account the presence in infants of certain conditions that are not related to convulsive phenomena and do not require specific treatment.

With the status, it is necessary to be able to conduct mechanical ventilation, to administer drugs intravenously:

Phenobarbital: 10 mg/kg, then 1 mg/kg/h to 40 mg/kg/day;

tonic and myoclonic nature of NS;

high frequency, polymorphism of seizures, status and serial course;

Apgar score below 4 points, neonatal resuscitation;

IVL for more than 7 days;

structural changes in the brain during neuroimaging;

resistance to ongoing starting anticonvulsant therapy;

cerebral malformations, NBO, phakomatoses.

It must be remembered that valproic acid is contraindicated in hyperammonemia and non-ketotic hyperglycinemia.

Literature
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Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_1.jpg" alt="(!LANG:>Neonatal Seizures G.N.">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_2.jpg" alt="(!LANG:> Lecture Objectives To clarify the role of seizures in brain damage in a newborn To teach to distinguish between convulsive and non-convulsive"> Задачи лекции Выяснить роль судорог в повреждении мозга новорожденного Научить отличать судорожные и несудорожные пароксизмы Научить диагностировать судорожный синдром и проводить неотложную терапию Обосновать прогноз при судорожном синдроме!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_3.jpg" alt="(!LANG:>Neonatal seizures are paroxysmal conditions in newborns, manifested by generalized or local muscle contractions , vegetative-visceral"> Неонатальные судороги – пароксизмальные состояния у новорожденных, проявляющиеся генерализованными или локальными мышечными сокращениями, вегетативно-висцеральными нарушениями, а также – имитацией безусловных двигательных автоматизмов, сопровождающиеся специфическими изменениями на электроэнцефалограмме (ЭЭГ) в приступный период по типу пик-волны или медленно-волновой активности.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_4.jpg" alt="(!LANG:>Neonatal seizures are a polyetiological clinical syndrome reflecting early cerebral disorders. More than 90 % - symptomatic"> Неонатальные судороги – полиэтиологический клинический синдром, отражающий ранние церебральные нарушения. Более 90% - симптоматические Менее 10% случаев - наследственно детерминированные (идиопатические) судороги.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_5.jpg" alt="(!LANG:>Incidence 1.1-16 per 1000 newborns. In premature babies ( 32–36 weeks) frequency of neonatal seizures 1.6–8%,"> Частота 1,1–16 на 1000 новорожденных. У недоношенных детей (32–36 нед) частота неонатальных судорог 1,6–8%, При экстремально низкой массе тела (меньше 31 нед) – около 20%. Неонатальные судороги в 1,5–2 раза чаще наблюдаются у мальчиков!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_6.jpg" alt="(!LANG:>Impaired activation and depolarization synchronization of neurons Convulsive bioelectric discharge Neurophysiology">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_7.jpg" alt="(!LANG:>Inhibitory postsynaptic potentials No differentiated dendritic system Poor axonal myelination Immature brain Spread restriction"> Ингибирующие постсинаптические потенциалы Нет дифференцированной дендритной системы Слабая миелинизация аксонов Незрелый мозг Ограничение распространения фокальной электрической активности!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_8.jpg" alt="(!LANG:>REPEATED CRAMPS Hypoventilation or Apnea BP ATP ADP Release of VKA PO2 PCO2 Cerebral CSC"> ПОВТОРНЫЕ СУДОРОГИ Гиповентиляция или апноэ АД АТФ АДФ Освобождения АВК РО2 РСО2 КСК Церебрального кровотока ПОВРЕЖДЕНИЕ МОЗГА Лактат Мозговой кровоток кровотечение Глюкоза мозга Захвата АКВ Глюта- мата гликолиза КСК – кардио- сосудистый колапс АКВ – аминокислоты возбуждения!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_9.jpg" alt="(!LANG:>International Classification of Neonatal Seizures (2002) (by clinical presentation) 2. Clonic convulsions 3. Fragmentary"> Международная классификация неонатальных судорог (2002) (по клинической картине) 2. Клонические судороги 3. Фрагментарные судороги (атипичные, абортивные, стертые) 4. Миоклонические судороги 5. ЭЭГ-позитивные неонатальные судороги 1. Тонические судороги!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_10.jpg" alt="(!LANG:> Variants of seizures in newborns: (by localization of manifestations) Focal Generalized Multifocal">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_11.jpg" alt="(!LANG:>International classification of neonatal seizures (2002) 1. Tonic seizures: a) focal b) generalized 2. clonic"> Международная классификация неонатальных судорог (2002) 1. Тонические судороги: а) фокальные; б) генерализованные; 2. Клонические судороги: а) фокальные; б) мультифокальные; в) генерализованные (билатеральные);!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_12.jpg" alt="(!LANG:>3. Fragmentary (atypical, abortive, erased) seizures (convulsive equivalents a) motor b) ophthalmic c) apnea"> 3. Фрагментарные (атипичные, абортивные, стертые) судороги (судорожные эквиваленты): а) моторные; б) офтальмические; в) апноэ; 4. Миоклонические судороги: а) фокальные; б) генерализованные; Международная классификация неонатальных судорог (2002)!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_13.jpg" alt="(!LANG:>5. EEG-positive neonatal seizures (no clinical manifestations, EEG specific convulsive activity is detected)."> 5. ЭЭГ-позитивные неонатальные судороги (клинические проявления отсутствуют, на ЭЭГ выявляется специфическая судорожная активность). Международная классификация неонатальных судорог (2002)!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_14.jpg" alt="(!LANG:>1. Tonic - generalized, focal decerebration posture, m.b. respiratory rhythm disorder, eye movements, diffuse"> 1. Тонические – генерализованные, фокальные поза децеребрации, м.б. нарушение ритма дыхания, глазные движения, диффузный цианоз или гиперемия кожи возникают: в остром периоде гипоксически-ишемической энцефалопатии, при массивных церебральных кровоизлияниях, при врожденных аномалиях мозга, при билирубиновой интоксикации. на ЭЭГ во время приступа специфическая (типа медленно-волновой) активность!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_15.jpg" alt="(!LANG:>2. Clonic - rhythmic muscle twitches of individual parts of the trunk, face and limbs with a frequency of 1–8"> 2.Клонические – ритмичные мышечные подергивания отдельных частей туловища, лица и конечностей с частотой 1–8 сокращений в секунду. Имеют корковый генез и в момент приступа сопровождаются специфическими !} diffuse changes on the EEG, by the type of peak-wave. In the interictal period, the EEG may be intact. Causes determine the clinical manifestations of clonic seizures

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_16.jpg" alt="(!LANG:>Focal clonic neonatal seizures are more likely to occur with structural changes within one hemisphere: focal heart attacks"> Фокальные клонические неонатальные судороги чаще возникают при структурных изменениях в пределах одногополушария: фокальные инфаркты мозга, церебральные очаговые кровоизлияния, врожденные артериовенозные мальформации, опухоли, абсцессы!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_17.jpg" alt="(!LANG:>Multifocal clonic neonatal seizures occur in neonates with diffuse cerebral cortical seizure activity"> Мультифокальные клонические неонатальные судороги возникают у новорожденных с диффузной судорожной активностью коры головного мозга и чаще наблюдаются: при метаболических нарушениях (гипокальциемия, гипогликемия, низкий уровень пиридоксина в плазме крови и др.) при диффузных корковых дисплазиях (микрополигирия, поликистоз и др.), в восстановительном периоде гипо- ксически-ишемической энцефалопатии.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_18.jpg" alt="(!LANG:>Generalized (bilateral) clonic neonatal convulsions - symmetrical limb convulsions occurring with loss of consciousness, rhythm disturbance"> Генерализованные (билатеральные) клонические неонатальные судороги- симметричные судороги конечностей, протекающие с потерей сознания, нарушением ритма дыхания, цианозом, гиперсаливацией при ЭЭГ-мониторинге установлено, что в 95% случаев фокальные клонические неонатальные судороги трансформируются в билатеральные. обычно наблюдаются у доношенных детей как с диффузными, так и очаговыми поражениями мозга.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_19.jpg" alt="(!LANG:>3. Slender (early hippocampal maturation) typical motor neonatal seizures - mimic asymmetric cervico-tonic"> 3. Субтильные (раннее созревание гипокампа) типичные моторные неонатальные судороги - имитируют асимметричный шейно-тонический рефлекс (АШТР), «плавающие» движения рук, «педалирование» стоп, сосательные движения губ, стереотипные высовывания и сосание языка (оперкулярные пароксизмы). Могут протекать с потерей сознания, апноэ или диффузным цианозом. На ЭЭГ во время приступа выявляются специфические изменения по типу пик-волна.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_20.jpg" alt="(!LANG:> ophthalmic (oculomotor) neonatal convulsions Horizontal nystagmus attacks, stereotypic paroxysms of tonic deviation eyeballs"> офтальмические (глазодвигательные) неонатальные судороги Приступы горизонтального нистагма, стереотипные пароксизмы тонической девиации глазных яблок и их движения по типу симптомов «заходящего или восходящего солнца»; могут сочетаться с потерей сознания, апноэ или цианозом. При проведении ЭЭГ во время приступа выявляется патологическая судорожная активность. 3. Субтильные (раннее созревание гипокампа)!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_21.jpg" alt="(!LANG:> atypical neonatal seizures, manifested by a short-term loss of consciousness (neonatal absences), especially difficult to diagnose."> атипичные неонатальные судороги, проявляются кратковременной потерей сознания (неонатальные абсансы), особенно трудны для диагностики. Обычно эти приступы (абсансы, petit mal) свойственны детям 3–5 лет при абсанс-эпилепсии (пикнолепсии), у новорожденнных диагностируются только при проведении ЭЭГ-мониторинга в течение суток. 3. Субтильные (раннее созревание гипокампа)!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_22.jpg" alt="(!LANG:> epileptic neonatal apnea – Clinical picture manifested by short-term episodes of breath holding without phenomena "> epileptic neonatal apnea - The clinical picture is manifested by short-term episodes of breath holding without bradycardia. EEG during an attack is a specific slow-wave activity or peak-waves emanating from deep (sometimes stem) brain structures. Cause : hypoxic-ischemic encephalopathy, brain anomalies, metabolic and toxic-metabolic disorders, less often - cerebral hemorrhages and neuroinfections 3. Slender (early maturation of the hippocampus)

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_23.jpg" alt="(!LANG:>4. Myoclonic - Rapid, short muscle spasms, jerks. Harbinger of infantile spasms.On the EEG - specific"> 4.Миоклонические – Быстрые, короткие мышечные спазмы, вздрагивания. Предвестник инфантильных спазмов. На ЭЭГ - специфические изменения по типу гиперсинхронизированной медленно-волновой высокоамплитудной активности (гипсаритмии), как во время приступов, так и между ними.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_24.jpg" alt="(!LANG:>in severe cases of hypoxic-ischemic encephalopathy, in cerebral anomalies - agenesis of the corpus callosum body and transparent"> в тяжелых случаях гипоксически-ишемической энцефалопатии, при церебральных аномалиях – агенезии мозолистого тела и прозрачной перегородки (синдром Айкарди), микрополигирии и др., при наследственных болезнях обмена веществ при наследственно-дегенеративных заболеваниях ЦНС. Это – клиническая манифестация эпилептических младенческих синдромов – West, Lennox–Gaustaut’s, миоклонической эпилептической энцефалопатии. 4.Миоклонические –!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_25.jpg" alt="(!LANG:> axial myoclonic neonatal spasms - lightning-fast flexion of the head and neck like "peck" , "nods""> аксиальные миоклонические неонатальные судороги – молниеносное сгибание головы и шеи типа «клевков», «кивков» с частотой 1–8 приступов в секунду, иногда с вегетативно-висцеральными нарушениями, расширением зрачков; 4.Миоклонические – фокальные, мультифокальные!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_26.jpg" alt="(!LANG:> myoclonic neonatal limb cramps - rhythmic rapid symmetrical flexion of the limbs, often arms, from"> миоклонические неонатальные судороги конечностей - ритмичные быстрые симметричные сгибания конечностей, чаще рук, с частотой 1 приступ в сек или 1–2 приступа в 10 сек. Часто имитируют спонтанный рефлекс Моро, обычно – только его первую фазу. Однако рефлекс Моро всегда имеет провоцирующий фактор (звуковой, тактильный и др.), миоклонические судороги возникают спонтанно; 4.Миоклонические – фокальные, мультифокальные!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_27.jpg" alt="(!LANG:> Mixed myoclonic neonatal spasms - a combination of pecks with flexor flexion or extension limbs."> смешанные миоклонические неонатальные судороги – сочетание «клевков» с флексорным сгибанием или разгибанием конечностей. 4.Миоклонические – фокальные, мультифокальные!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_28.jpg" alt="(!LANG:>Key questions in the management of children with motor paroxysms 1. Are seizures convulsions? .2."> Основные вопросы при ведении детей с двигательными пароксизмами 1. Являются ли пароксизмы судорогами. 2. Какова причина судорог? 3. Как лечить? 4. Определить прогноз.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_29.jpg" alt=">">

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_30.jpg" alt="(!LANG:>Non-convulsive paroxysms "convulsive readiness" - manifestations of high neuroreflex excitability (restless behavior , irritated cry, tremor"> Несудорожные пароксизмы «судорожная готовность» - проявления высокой нервнорефлекторной возбудимости (беспокойное поведение, раздраженный крик, тремор различной амплитуды, вздрагивание при действии внешних раздражителей). Тремор носит рефлекторный функциональный характер и обусловлен реакцией красных ядер на вестибулярную нагрузку («рубральный тремор»). При изменении положения ребенка или пассивном сгибании конечностей, рубральный тремор сразу прекращается.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_31.jpg" alt="(!LANG:>Decerebrate postural setting - activation of the reticulospinal tract. Decerebrate stiffness most often occurs when compression"> Децеребрационная позотоническая установка - активация ретикулоспинальных путей. Наиболее часто децеребрационная ригидность возникает при сдавлении среднего мозга при повышении внутричерепного давления и обычно сочетается с глазодвигательными симптомами (Грефе, «заходящего солнца»), а также с нарушением ритма дыхания в виде бради- и тахипноэ. При изменении положения ребенка (реакция вестибулярного аппарата) тоническое напряжение мышц усиливается или уменьшается Несудорожные пароксизмы!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_32.jpg" alt="(!LANG:>Ophthalmic non-convulsive phenomena - nystagmus, fixed gaze, deviation of the eyeballs, different kinds strabismus, symptoms"\u003e Ophthalmic non-convulsive phenomena - nystagmus, fixed gaze, deviation of the eyeballs, various types of strabismus, symptoms of Graefe and "setting sun", opsoclonus. If not accompanied by a violation of the rhythm of breathing, cardiac activity, discoloration of the skin and specific motor reactions (freezing, startling, etc.) Opsoclonus, the phenomenon of "dancing eyes" - a rapid twitching of the eyeballs in different directions, aggravated by sound stimulation. Opsoclonus is usually observed in neonatal forms of degenerative diseases of the central nervous system. Non-convulsive paroxysms

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_33.jpg" alt="(!LANG:>Apnea with immaturity of respiratory center regulation (in preterm infants) in a full-term baby, Mb broncho-obstructive conditions,"> Апноэ при незрелости регуляции дыхательного центра (у недоношенных) у доношенного ребенка, м.б. бронхообструктивные состояния, инфекции, сердечно-сосудистые расстройства, надпочечниковая недостаточность, гипогликемия и электролитный дисбаланс. При несудорожном апноэ более 60 с м.б. цианоз и брадикардия Несудорожные пароксизмы!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_34.jpg" alt="(!LANG:>Hyperexplexy Syndrome - is manifested by high motor reactivity that occurs only in response to provocation. On auditory, tactile or"> Синдром гиперэксплексии –проявляется высокой двигательной реактивностью, возникающей только на провокацию. На слуховой, тактильный или световой раздражитель возникает резкое тоническое напряжение мышц-разгибателей, переразгибание головы, застывание взора длительностью несколько секунд. Причина – наследственно обусловленная высокая реактивность подкорковых образований (бугры четверохолмия) головного мозга на любой сенсорный стимул. Несудорожные пароксизмы!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_35.jpg" alt="(!LANG:>“Benign sleep myoclonus” Occurs during sleep, in the form of muscle contractions hands (two-way, synchronous)"> «Доброкачественный миоклонус сна» Возникает во время сна, в виде мышечных сокращений рук (двусторонних, синхронных) по типу «взмаха крыльев». !} General state the child is satisfactory, the behavior during periods of wakefulness corresponds to age. EEG-conducted during natural sleep has no deviations from age norms. Specific treatment for "benign sleep myoclonus" in newborns is not required, the prognosis for further psychomotor development is favorable. Nonconvulsive paroxysms

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_36.jpg" alt="(!LANG:>Groups of neonatal seizures: symptomatic neonatal seizures; idiopathic neonatal seizures;"> Группы судорожных состояний неонатального периода: симптоматические неонатальные судороги; идиопатические неонатальные судороги; эпилепсия и эпилептические синдромы детского возраста, дебютирующие в неонатальном периоде.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_37.jpg" alt="(!LANG:>Neonatal seizures - mostly symptomatic!">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_38.jpg" alt="(!LANG:>Main causes of symptomatic seizures in newborns: 1. Hypoxic-traumatic brain injury 2. Metabolic disorders 3."> Основные причины симптоматических судорог у новорожденных: 1. Гипоксически-травматическое поражение мозга 2. Метаболические нарушения 3. Внутричерепные инфекции 4. Дизгенезия мозга 5. Синдром абстиненции!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_39.jpg" alt="(!LANG:>Implementation of risk factors in the first 48 hours of life">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_40.jpg" alt="(!LANG:>Differential diagnosis of neonatal symptomatic seizures 1. Seizures due to intrauterine and perinatal infection : TORCH infection; Sepsis,"> Дифференциальный диагноз неонатальных симптоматических судорог 1. Судороги, обусловленные внутриутробной и перинатальной инфекцией: TORCH-инфекции; Сепсис, бактериальный менингит; Простой герпес.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_41.jpg" alt="(!LANG:>Hypoxic-traumatic cerebral disorders: - Cerebral ischemia grade 2-3 Intracranial hemorrhage thrombosis of cerebral vessels">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_42.jpg" alt="(!LANG:>3. Metabolic disorders: Neonatal hypoglycemia Neonatal hypocalcemia Neonatal hypomagnesemia Withdrawal syndrome">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_43.jpg" alt="(!LANG:>Congenital metabolic diseases: - fructose dysmetabolism; - ganglioside disorders; - glycine encephalopathy; -"> Врожденные болезни обмена веществ: - фруктозный дизметаболизм; - нарушения ганглиозидов; - глициновая энцефалопатия; - дефицит гликоген-синтетазы; - болезнь мочи «кленового сиропа»; - кетотическая гиперглицинемия; - нарушения мочевого цикла.!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_44.jpg" alt="(!LANG:>4. Genetic and birth defects: Pigment loss Pyridoxine dependent conditions Anomalies brain development">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_45.jpg" alt="(!LANG:>Suggested causes of neonatal seizures by time of occurrence. First days of life. Infection (sepsis, bacterial"> Предполагаемые причины неонатальных судорог по времени их возникновения. Первые сутки жизни. Инфекция (сепсис, бактериальный менингит, TORCH-инфекция) Гипоксия или травма (субарахноидальные кровоизлияния, гипоксически-ишемическая энцефалопатия, внутрижелудочковые кровоизлияния, разрыв мозжечкового намета) Метаболические нарушения (пиридоксин-зависимые и гипогликемические состояния) Прием !} medicines(usually in the first 12 hours)

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_46.jpg" alt="(!LANG:>Second - third day of life. Infection (sepsis, bacterial meningitis) Brain hemorrhages (subdural and subarachnoid"> Вторые - третьи сутки жизни. Инфекция (сепсис, бактериальный менингит) Мозговые кровоизлияния (субдуральные и субарахноидальные кровоизлияния, церебральные инфаркты, внутримозговые кровоизлияния, перивентрикулярные-внутрижелудочковые кровоизлияния) Наследственные болезни обмена веществ (глициновая энцефалопатия, недостаточность гликоген-синтетазы, гипопаратиреоидизм, потеря пигмента, церебральный дисгенез, нарушения мочевого цикла) Синдром отмены лекарств!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_47.jpg" alt="(!LANG:>Third day - first week of life. Metabolic disorders (hypoparathyroidism, nutritional hypocalcemia , urinary disorders)"> Третьи сутки - первая неделя жизни. Метаболические нарушения (гипопаратиреоидизм, пищевая гипокальциемия, нарушения мочевого цикла) Мозговые кровоизлияния (внутримозговые кровоизлияния, церебральные инфаркты) Наследственная патология (церебральный дисгенез, семейная склонность к неонатальным судорогам) Билирубиновая энцефалопатия!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_48.jpg" alt="(!LANG:>After the first week of life. Metabolic disorders (hypoparathyroidism, fructose dysmetabolic disorders, ketotic hyperglycinemia, maple disease"> После первой недели жизни. Метаболические нарушения (гипопаратиреоидизм, дисметаболические нарушения фруктозы, кетотическая гиперглицинемия, болезнь «кленового сиропа» мочи, нарушения мочевого цикла) Герпетический энцефалит Церебральный дисгенез!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_49.jpg" alt="(!LANG:>Diagnostic Routine Special">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_50.jpg" alt=">">

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_51.jpg" alt=">">

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_52.jpg" alt="(!LANG:>TACTICS OF TREATMENT Federal Guidelines for the Use of Medicines, Edition X, Moscow, 2009 Anticonvulsants"> ТАКТИКА ЛЕЧЕНИЯ Федеральное руководство по использованию лекарственных средств, выпуск Х, Москва, 2009 Противосудорожные препараты рекомендуется назначать при наличии > 3 эпизодов кратковременных судорог в течении часа или одного эпизода > 3 мин.(?)!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_53.jpg" alt="(!LANG:> TREATMENT Anticonvulsants (Federal Guidelines for the Use of Medicines, Issue X , Moscow, 2009)"> ТАКТИКА ЛЕЧЕНИЯ Противосудорожные препараты (Федеральное руководство по использованию лекарственных средств, выпуск Х, Москва, 2009) 1) седуксен (диазепам, сибазон) - в/в 0,04 - 0,1 мл/ кг 0,5 % раствора!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_54.jpg" alt="(!LANG:>In case of inefficiency (1) 2) sodium thiopental 5mg/kg i.v. in a jet, then 2.5 - 1 mg / kg / hour."> При неэффективности (1) 2) тиопентал натрия 5мг/кг в/в струйно, затем 2,5 – 1 мг/кг/час. !} Side effects: drop in blood pressure, impaired intracardiac conduction, hypothermia, competitive inhibition of bilirubin metabolism TACTICS OF TREATMENT Anticonvulsants:

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_55.jpg" alt="(!LANG:>TREATMENT Anticonvulsants: 3) Maintenance therapy with anticonvulsants - phenobarbital per os 3-5 mg/kg/day"> ТАКТИКА ЛЕЧЕНИЯ Противосудорожные препараты: 3) Поддерживающая терапия антиконвульсантами - фенобарбитал per os 3-5 мг/кг/сутки – в два приема на 2 – 3 недели (?)!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_56.jpg" alt="(!LANG:>What determines the duration of anticonvulsant therapy? Neurological examination Etiology of convulsive paroxysms EEG data ">

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_57.jpg" alt="(!LANG:>Neonatal seizures caused by metabolic disorders are corrected: 1. Hypocalcemia ( total calcium level">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_58.jpg" alt="(!LANG:>2. Hypomagnesemia (often combined with hypocalcemia). Critical magnesium level - 0.5 mmol/l For intravenous administration"> 2. Гипомагниемия (часто сочетается с гипокальциемией). Критический уровень магния -0,5 ммоль/л. Для в/в введения 25 % р-р магния сульфата разводят до 1 % концентрации 10 % глюкозой и вводят медленно (1 мл/мин) 6-10 мл 1 % раствора. Осложнения от в/в введения - угнетение дыхания, брадикардия. При неонатальных судорогах обусловленных метаболическими нарушениями проводится их коррекция:!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_59.jpg" alt="(!LANG:>3. Hypoglycemia .6 mmol/l."> 3. Гипогликемия Диагноз устанавливают при уровне глюкозы в крови доношенного ребенка менее 2,6 ммоль/л. Для снятия судорожного синдрома вводят внутривенно: в/в 10% р-р глюкозы 2 мл/кг в течение 1 минуты, затем 1 мл/мин, затем переходят на в/в капельную инфузию 10% раствора глюкозы со скоростью 5 мл/кг в час. При неонатальных судорогах обусловленных метаболическими нарушениями проводится их коррекция:!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_60.jpg" alt="(!LANG:>4. Pyridoxine-dependent seizures: IV or IM 50-100 mg of vitamin B6, i.e. 1-2 ml 5%"> 4. Пиридоксин-зависимые судороги: в/в или в/м 50-100 мг витамина В6, т.е. 1-2 мл 5% раствора пиридоксина гидрохлорида. При неонатальных судорогах обусловленных метаболическими нарушениями проводится их коррекция:!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_61.jpg" alt="(!LANG:>Frequency of favorable prognosis of neonatal seizures of various etiologies Prognosis is favorable for 80-90- 100% of newborns with: subarachnoid"> Частота благоприятного прогноза неонатальных судорог разной этиологии Прогноз благоприятен для 80-90-100% новорожденных при: субарахноидальных кровоизлияниях гипокальциемии с поздним началом лекарственных воздействиях семейных доброкачественных судорогах!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_62.jpg" alt="(!LANG:>Prognosis is favorable for 50-60% of newborns with: hypoxic-ischemic encephalopathy cerebral infarction hypoglycemia hypocalcemia with early"> Прогноз благоприятен для 50-60% новорожденных при: гипоксически-ишемической энцефалопатии инфаркте мозга гипогликемии гипокальциемии с ранним началом гипомагнезиемии гипонатриемии гипернатриемии!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_63.jpg" alt="(!LANG:>The prognosis is favorable for 10-20% of children with: congenital metabolic disorders, intracranial infections.">!}

Src="https://present5.com/customparser/17612707_54165407%20---%20cramp.ppt/slide_64.jpg" alt="(!LANG:>Prognosis for various types of seizures Focal and multifocal clonic seizures are more favorable than mild ,"> Прогноз при различных вариантах судорог Фокальные и мультифокальные клонические судороги более благоприятные чем субтильные, миоклонические и тонические!}