[Clinical and functional disturbances in epilepsy patients with schizencephaly]. / Kliniko-funktsional'nye narusheniya u patsientov s epilepsiei pri shizentsefalii.

OBJECTIVE: To study the features of diagnosing and predicting structural epilepsy in children with schizencephaly (SE) based on the analysis of clinical, electrophysiological and neuroimaging results. MATERIAL AND METHODS: Fifteen patients with epilepsy and SE (seven boys and eight girls), aged from 3 months to 14 years, were examined. RESULTS: Unilateral SE was detected in ten patients (closed - in four, open - in six), bilateral open SE was detected in five patients. The predominant localization of the anomaly is in the frontal region. In 100% of cases, cognitive and motor impairments of varying severity were detected. In the study group, 11 patients (73.3%) were diagnosed with epilepsy before the age of 6 years. The clinical presentations of epilepsy in children with SE included focal seizures in ten patients (73.3%), epileptic spasms in three patients (20.0%), focal seizures with secondary generalization in five (33.3%), atonic - in one child (6.7%). Refractory epilepsy was noted in 26.7% children with SE, and the absence of positive electroencephalographic changes in 40% of children. CONCLUSIONS: The extent of structural brain damage in SE in patients with epilepsy correlates with the degree of cognitive and motor deficits. The form of epilepsy, the semiotics of epileptic seizures, and the effectiveness of antiepileptic therapy do not depend on the type of SE, but correlate with the extent of cortical disorders.

[Diagnostic and prognostic significance of the level of IgM antibodies to myelin basic protein in children with traumatic brain injury]. / Diagnosticheskoe i prognosticheskoe znachenie urovnya antitel k osnovnomu belku mielina v krovi detei s cherepno-mozgovoi travmoi.

OBJECTIVE: To study the content of antibodies to the basic protein of myelin IgM in the blood of children with traumatic brain injury for a comprehensive assessment of the severity and prediction of outcomes of TBI. MATERIAL AND METHODS: 81 children with traumatic brain injury (TBI) of varying severity and 29 children of the comparison group aged 2 months to 18 years were examined, in whom the content of IgM antibodies in blood plasma was determined. RESULTS: In groups of children with different severity of TBI, the average content of IgM antibodies in the blood is higher than in children of the comparison group, and the values of IgM antibodies correlate with the severity of TBI. In children with moderate TBI, the average level of IgM antibodies in the blood is significantly higher than in children with mild TBI, and in children with severe TBI, the average level of IgM antibodies in the blood is significantly higher than in children with moderate TBI. A repeated study found a significant decrease in the average content of IgM antibodies in severe TBI in children without post-traumatic epilepsy compared to their average content in children with post-traumatic epilepsy. CONCLUSION: Normalization (elimination) of IgM antibodies in children in the late period of TBI is associated with a prognostically favorable course of the autoimmune process in the brain; its absence indicates a prolonged autoimmune process. After TBI, it is recommended to include in the examination of children an immunological study of the content of antibodies to the basic protein of myelin IgM in the blood, the indicators of which, in combination with other data, make it possible to predict the outcomes of TBI.

[Pharmacotherapy of psychomotor developmental delay in 6-12 months preterm infants with hypoxic-ischemic encephalopathy (the double-blind comparative multicenter placebo-controlled study)]. / Farmakoterapiia zaderzhki psikhomotornogo razvitiia u detei 6-12 mes, rozhdennykh nedonoshennymi i perenesshikh gipoksicheski-ishemicheskoe porazhenie golovnogo mozga (dvoinoe slepoe sravnitel'noe mnogotsentrovoe platsebo-kontroliruemoe issledovanie).

AIM: To evaluate the efficacy and safety of hopantenic acid (Pantogam) in the complex treatment of prematurely born infants, aged 6-12 months, with psychomotor developmental delay due to hypoxic-ischemic encephalopathy. MATERIAL AND METHODS: Eighty-seven patients were randomized into two groups: 44 received standardized treatment and pantogam for two months, 43 standardized treatment and placebo. Pantogam (syrup 100 mg/ml) or placebo were prescribed orally 15-30 minutes after feeding, twice a day, in a daily dosage of 30-50 mg/kg body weight. The assessment of psychomotor development from birth to two years was performed with the Griffiths Mental Development Scales (GMDS-ER) twice (before and after completion of therapy). RESULTS: The response to two month therapy determined as the reduction of developmental delay for more than 6% of the initial GMDS-ER general quotient (GQ) score was significantly better in the group I after pantogam treatment (63.6% of patients) compared to group II (36.4%, p=0.021). Group I demonstrated the significant decrease of the developmental delay in two domains ('Personal-Social' and 'Performance') and a trend to overcome the delay in three other domains: 'Locomotor', 'Hearing and Speech', 'Eye and Hand Coordination'. The improvement after pantogam treatment was more obvious in the subgroup of infants born late preterm (gestational age 34-36 weeks) compared to infants born moderate preterm (gestational age 32-33 weeks). The favorable safety profile of pantogam was confirmed, comparable to that of placebo. CONCLUSION: Pantogam is efficient and safe medication in the complex treatment of psychomotor developmental delay in preterm infants, aged 6-12 months.

[Phenotypic and neuroimaging differentiation of polymicrogiry in children]. / Fenotipicheskaia i neirovizualizatsionnaia differentsiatsiia polimikrogirii u detei.

AIM: To study the results of the examination of children and to assess the degree of correlation of cortical disorders with phenotypic and neuroimaging characteristics of polymicrogyria (PMG). MATERIAL AND METHODS: An analysis of disease history, neurological data, semiotics of epileptic seizures, neuroimaging and electrophysiological results was performed in 27 patients with PMG. RESULTS AND CONCLUSION: Correlations between the prevalence of cortical malformation and the increased frequency of abnormalities in internal organs, stigma of dysembryogenesis, cognitive and motor disorders are shown.

[New opportunities in the optimization of epilepsy treatment in adolescents]. / Novye vozmozhnosti optimizatsii terapii épilepsii u podrostkov.

In the pubertal period, the most severe forms of childhood epilepsy persist and are modified; genetically determined syndromes, atypical for early and late age periods, make their debut. Hereditary predisposition, instability of homeostatic mechanisms, neuroendocrine restructuring at the age of puberty and the influence of factors contributing to the realization of a genetic defect lead to a long-term transformation of mediator systems and formation of epileptic activity in adolescents. The authors present common approaches in the treatment and characterization of the modern antiepileptic drug perampanel, which is highly effective in treatment of patients with resistant forms of epilepsy. The article presents a summarized overview of the clinical forms of adolescent epilepsy observed in a psychoneurological department and an analysis of treatment results. A clinical case of the successful use of the antiepileptic drug perampanel in a female patient with focal seizures with secondary generalization of epilepsy, type I neurofibromatosis is presented.

[Algorithms of mono- and polytherapy in clinical epileptology. Part 1. General principles of drug choice]. / Algoritmy mono- i politerapii v klinicheskoi épileptologii. Chast' 1. Obshchie printsipy vybora farmakoterapii.

The large number of antiepileptic drugs (AEDs) at the physician's disposal provides not only a broad therapeutic potential in the treatment of epilepsy (EP), but creates difficulties in the adequate choice of AED. The sufficient experience in the management of patients with epilepsy has been gained so far in the world, based on which the International League Against Epilepsy (ILAE), updated classification, adopted the basic definition of efficiency, remission, resistance, evidence of research on the effectiveness of AED therapy, and introduced the concept of "resolved" epilepsy. In this article, a group of Russian experts suggest recommendations on the main steps in the choice of therapy in epilepsy. Possible drug interactions between different AEDs and other drugs as well as main characteristics of mono- and polytherapy of epilepsy are described. Some features of the use of AEDs in the elderly, characteristics of the "female" epilepsy related to the reproductive function and basic requirements for the therapy of epilepsy in children are presented.

[Algorithms of mono- and polytherapy in clinical epileptology]. / Algoritmy mono- i politerapii v klinicheskoi épileptologii. Chast' 2. Osobennosti lecheniia otdel'nykh grupp patsientov.

The large number of antiepileptic drugs (AEDs) at the physician's disposal provides not only a broad therapeutic potential in the treatment of epilepsy (EP), but creates difficulties in the adequate choice of AED. The sufficient experience in the management of patients with epilepsy has been gained so far in the world, based on which the International League Against Epilepsy (ILAE), updated classification, adopted the basic definition of efficiency, remission, resistance, evidence of research on the effectiveness of AED therapy, and introduced the concept of "resolved" epilepsy. In this article, a group of Russian experts suggest recommendations on the main steps in the choice of therapy in epilepsy. Possible drug interactions between different AEDs and other drugs as well as main characteristics of mono- and polytherapy of epilepsy are described. Some features of the use of AEDs in the elderly, characteristics of the "female" epilepsy related to the reproductive function and basic requirements for the therapy of epilepsy in children are presented.

[Diagnosis and correction of cognitive impairment in preschool age children with epilepsy].

OBJECTIVE: To study cognitive impairment and the efficacy of hopantenic acid (pantogam) in children, aged from 4 to 7 years, with epilepsy and cognitive impairment. MATERIAL AND METHODS: The study included 40 children with epilepsy treated with pantogam (the main group) and 23 children with epilepsy who did not receive pantogam (the comparison group). All children were diagnosed with cryptogenic epilepsy and symptomatic epilepsy. We presented the main results of the clinical examination including routine EEG, video-EEG monitoring and brain MRI. An analysis of cognitive impairment in children of the comparison group and in the main group before and after treatment with pantogam was performed using a set of neurocognitive tests (the Luscher Color Test, «Piano playing test¼, «Three word test¼ and «Four is redundant test¼). RESULTS AND CONCLUSION: There were a significant decrease in cognitive impairment in children of the main group compared to the comparison group as well as age normalization and improvement of EEG after 2 months of treatment in 15% of the patients. The good tolerability of the drug and no increase in the frequency of seizures were observed.

[The effect of antiepileptic therapy on the level of hormones in the blood serum of girls with epilepsy]. / Vliianie osobennostei protivoépilepticheskoi terapii na uroven' gormonov v syvorotke krovi u bol'nykh épilepsiei devochek.

Objective. To investigate the effect of modern antiepileptic drugs on the hormonal profile of girls with epilepsy. Materials and methods. Levels of thyroid-stimulating hormone (TSH), thyrogiobulin antibodies (a/b TG), triiodothyronine (T3), thyroxine (T4), parathyroid hormone (P) and cortisol (C) were determined in the blood of 50 girls with epilepsy, aged from 8 to 17 years. In 19 girls, repeated measures of TSH, a/b TG, T3, T4 and C were taken 2-12 months after the first study. Valproate, carbamazepine, and topamax were used for treatment. Results. The highest content of TSH was found in girls, aged 8-17 years, treated with valproate. The presence of a/b TG was recorded in patients who did not receive antiepileptic drugs. T3, T4 and P levels were found in patients treated with phenobarbital or benzonal, topamax and valproate, respectively. C was detected in girls, aged 8-13 years, treated with phenobarbital or benzonal and in girls, aged 14-17 years, treated with trileptal. Repeated measures revealed the changes in all hormones in 64.86% of patients. Conclusion. Significant differences in the content of all hormones were found in girls treated with various antiepileptic drugs. Choice of drug, dosage and its correction should be made taking into account its impact on children's hormonal profile.

[Unusual behaviors in sleep as "compensatory" reactions, aimed at normalizing sleep-alertness cycles].

The present article is an attempt to perform a conceptual clinical and physiological analysis of a large spec- trum of sleep-related phenomena called parasomnias in children, based on data from three independent in- stitutions. Parasonmias appear in the process of falling asleep, at the time of sleep stage changes, and upon awakening. They are common for both healthy children and those with neurological and psychiatric disorders. Brief descriptions of clinical pictures of several groups of parasomnias and their polysomnographic characteristics are presented. Instances of stereotyped rhythmic movements (e.g. head rocking), paroxysmal somatic and behavioral episodes (night terrors and nightmares), "static" phenomena (sleep with open eyes, strange body positions), as well as somnambulism are specifically described. Common features of parasomnias as a group have been identified (the "Parasomnia syndrome"). It was found that sleep architecture frequently normalizes after a parasomnia episode, whereas parasomnias are self-liquidated after sleep matures (self-cure). The significance of gender differences in parasomnias have been reviewed. Possible compensatory physiological functions of parasomnias acting as "switches" or "stabilizers" of sleep stages to "off-set" deviated or immature sleep-wake mechanisms were discussed.

[Efficacy and safety of the monotherapy with trileptal (oxcarbazepine) in children and adolescents].

A prospective non-randomized non-controlled multicenter trial has been conducted. The trial included 254 children, aged from 11 months to 18 years (mean age 9.3 +/- 4.5 years), with predominantly focal forms of epilepsy treated with trileptal (oxcarbazepine). The observation period was 31 weeks. Efficacy and safety of therapy was assessed in 3 visits: screening and assignment to therapy (visit 1), the end of titration and achievement of maintenance dose (visit 2), assessment of maintenance therapy (visit 3). The percentage of patients with a positive response to the trileptal therapy (the decrease of seizure frequency by 50% and more) was 91.1%. The complete reduction of seizures was achieved in 59.4% of patients. Most of patients (95.3%) continued to receive trileptal until the end of the trial. The significant decrease (p < 0.001) of seizure frequency from visit 1 to visit 3, the reduction of epileptiform activity (p < 0.05) on the awake EEG in visit 3 were found. The mean effective dose of trileptal was 902.4 +/- 442.7 mg/day, i.e. was less than 30 mg/kg/day, daily doses did not exceed 600 mg. Adverse effects were observed in 11.2% of patients but in 40% of cases they seemed not be related to the drug. The adverse effects were from mild to moderate extent. In conclusion, trileptal as the monotherapy is effective and well-tolerated in the treatment of focal epilepsies in the age groups studied.

Characteristics of cognitive functions in children with epilepsy.

The aim of the present work was to study the causes and structure of interictal cognitive and emotionalpersonality impairments in children with different types of epilepsy. A total of 40 children were studied, aged from eight to 18 years. Symptomatic epilepsy was diagnosed in 31 patients (77.5%) and idiopathic epilepsy in nine (22.5%). Cognitive functions were compared in relation to the type of epilepsy, type of seizures, and treatment with antiepileptic and its efficacy. The results showed that intellectual-mnestic disorders were seen in 62% of children with epilepsy, more profound impairments being seen in patients with generalized epileptic seizures. Achievement of stable remission from seizures was associated with good recovery of cognitive functions; in the absence of treatment with antiepileptic agents and when treatment was ineffective, there were marked disorders to the intellectual-mnestic sphere.

[Peculiarities of cognitive functions in children with epilepsy].

A study aims at investigation of causes and structure of cognitive and emotional-personality interictal disturbances in children with different forms of epilepsy. Forty children, aged from 8 to 18 years, have been studied. Symptomatic epilepsy is diagnosed in 31 (77,5%) of patients, idiopathic epilepsy in 9 (22,5%) patients. Cognitive functions have been compared to the form of epilepsy, types of epileptic atacks, antiepileptic therapy and its efficacy. The results of the study reveal intellectual and memory disturbances in 62% of children with epilepsy and more severe disturbances in generalized absence of antiepileptic therapy or its ineffectiveness the marked disorders of intellectual and memory functions are demonstrated.

[Environmental factors and parkinsonian syndrome].

Particular emphasis has been in recent years placed on the study of an integrating role of medicine in the prevention, early diagnosis, and treatment of diseases associated with the influence of environmental factors. Among nervous system diseases associated with the adverse influence of environmental factors, the parkinsonian syndrome has been the particular concern, which is due to its higher incidence rates and early disability in able-bodied persons. In this connection, the purpose of the study was to review the data available in the literature on the neurotoxicity of heavy metals and other environmental factors causing toxic parkinsonism. The mechanisms of development and the clinical manifestations of the parkinsonian syndrome were considered in occupational manganese poisonings, magnesium deficiency, and carbon monoxide intoxication. Profound assessment of the influence of environmental factors on certain population groups is possible only when hygienists, toxicologists, biochemists, immunologists, molecular geneticists, and clinicians combine their efforts.

[Analysis of blood hormone levels in boys of prepubertal and pubertal age with epilepsy treated with different antiepileptic drugs].

Thirty-nine boys with epilepsy, aged 8-17 years, and 61 sex- and age-matched controls have been examined. Changes of blood hormone levels were found in patients received carbamazepines, valproates, polytherapy as well as in non-treated patients. The increase of levels of progesterone, testosterone, FSH and LH, decrease of prolactin and estradiol levels and influence on thyroid gland hormones, as reflected by increasing of T4 and decreasing of T3 compared to carbamazepines, were found during the treatment with trileptal in the older group. The significant changes of decreased blood hormone levels were observed in children received phenobarbital and benzonal. In children treated with topamax, only the changes in FSH and DHEA were shown. No changes in the content of thyroid gland hormones were found in patients received topamax and non-treated patients. The increase of TTH was observed in children of the younger group treated with valproate. In conclusion, the influence of such antiepileptic drugs as carbamazepine, barbiturates may be directly related with the changes of steroid hormone metabolism. Valproic acid, a well-known protein inhibitor, may impact on the content of reproductive hormones in the blood. The changes of blood hormone levels in boys with epilepsy treated with antiepileptic drugs are thought to influence their somatic health and reproductive functions.

[Depakine in the therapy of epilepsy in children and adolescents].

The study included 642 patients, aged from 2 months to 18 years, who received different forms of depakine during the period from 2000 to 2006. Also the treatment with injection form of depakine was analyzed in 14 children. The drug was assessed in mono- and polytherapy. All patients underwent online EEG and MRI monitoring of the brain. Video EEG monitoring was conducted according to plasma concentration level of valproate. The best therapeutic effect was found for idiopathic partial epilepsy. The complete stopping of seizures was observed in 71% of patients from this group. The treatment of idiopathic generalized forms of epilepsy led to stable remission in 64%. In 23% of patients with symptomatic partial epilepsy, 100% efficacy of depakine in the monotherapy was achieved. It has been suggested that depakine is highly effective and tolerable for most of the patients and can be recommended as a drug of choice for all forms of epilepsy in children and adolescents. The use of injection form is effective for stopping of epileptic status.