Background. The status epilepticus of focal impaired-awareness seizures (SE FIAS) is a series of focal seizures with loss or change of consciousness, between which there is no complete recovery of consciousness. This status epilepticus occurs in patients with temporal (especially with hippocampal sclerosis) and frontal epilepsy. It is important to differentiate SE FIAS with the absence status epilepticus, with psychiatric disorder, with postictal confusion. As a rule, this status epilepticus is self-terminate, without special treatment.

Objective: to study the features of epidemiology, etiology, diagnosis, therapy and prognosis in patients with SE FIAS.

Materials and methods. The study included 1350 consecutive patients diagnosed with epilepsy.

Results and discussion. A history of SE FIAS was found in 20 patients (14 women and 6 men), it occurred in the age range from 5 to 66 years. 13 patients (65 %) had mesial temporal lobe epilepsy, 5 patients (25 %) had frontal lobe epilepsy, and 2 patients (10 %) had lateral temporal lobe epilepsy. Only in 80 % of patients treatment was adequate before the development of SE FIAS, in 20 % of patients it was inadequate and subsequently caused the development of status epilepticus. In 40 % of patients the occurrence of SE FIAS is associated with their own non-compliance; in 30 % of patients the development of status epilepticus had iatrogenic causes. Measures to prevent the development of status epilepticus were ineffective only in patients with pharmacoresistant symptomatic epilepsy and in non-compliant patients.

Conclusions. SE FIAS occurs in 1 % of patients with epilepsy. Among patients, women with temporal or frontal epilepsy dominate; status epilepticus occurs at any age and is often triggered by changes in therapy due to doctors’ recommendations or patient non-compliance. Usually the status is self-terminating. To prevent its recurrence, adequate antiepileptic therapy is necessary. The prognosis in patients with SE FIAS is favorable; however, the general prognosis remains serious due to the severity of the course of epilepsy.

Background. Due to the high prevalence of multiple sclerosis (MS) among women of reproductive age, special attention is paid to the management of pregnancy in them. The problem concerning the health of infants born to mothers with MS receiving disease-modifying therapy has not yet been addressed. It is necessary to identify the timing of cessation of drugs changing the course of MS, as well as to find the possibility of mitigating exacerbations during pregnancy using corticosteroid therapy.

Objective: to analyze the effects of causal therapy for MS on the health of newborns.

Materials and methods. This study included 154 women with MS residing in Moscow region and receiving disease-modifying therapy. We evaluated the course of pregnancy, delivery, and the condition of infants born to these mothers.

Results and conclusions. Our findings are consistent with the earlier published data in the general cohort of pregnant women and allowed us to conclude that the presence of MS and history of immunomodulatory therapy do not significantly affect the outcome of pregnancy. The incidence and severity of disorders in infants born to MS mothers did not significantly differ from those in the general population. Prolongation of therapy with drugs that change the course of MS up to pregnancy is most appropriate, since it stabilizes the condition of women in the perinatal period, without causing significant harm to the health of newborns. Immunosuppressive therapy increases the risk of various disorders in infants (such as multiple malformations, low birth weight, prematurity). If a woman develops an exacerbation of MS during pregnancy, it is possible to prescribe a short course of pulse therapy with methylprednisolone.

Background. An important condition for adequate drug therapy is the early differential diagnosis of paroxysmal conditions in children, the estab lishment or clarification of their epileptic or non-epileptic nature.

Objective: to demonstrate the necessity, effectiveness and safety of the correction therapy in children with paroxysmal disorders of consciousness according to results of complex investigation, including video-electroencephalographic monitoring.

Materials and methods. A comprehensive examination with the inclusion of video-electroencephalographic monitoring was carried out in 527 pa tients referred by neurologists to clarify the nature of paroxysmal consciousness disorder, clarify the form of epilepsy, and select an adequate treatment.

Results. Based on the results obtained during the comprehensive examination of children with video-electroencephalographic monitoring, in all the examined children the diagnosed was corrected. According to the results of the survey, it was found that 210 children had non-epileptic paroxysms. In the overwhelming majority of cases, the treatment was changed.

Conclusions. The presented results of treatment of children with epileptic and non-epileptic paroxysms after correction of treatment indicate the need for careful analysis of all data (clinical and anamnestic, electroencephalographic, laboratory) for correct diagnosis, identification of causes of resistance and reasonable selection of therapy in each patient.

This article discusses the problem of school education as one of the aspects of social adaptation of children diagnosed with epilepsy and / or children with a history of status epilepticus. The authors provide statistical data on the prevalence of epilepsy among children of different ages and information on disease control. We collected the data of patients followed-up in Mytishchi Children’s Polyclinic No. 4. Using our own experience and publications of other authors, we have developed a number of recommendations that can potentially make the presence of a child with a history of epilepsy and / or status epilepticus in an educational institution more comfortable and safe. In particular, we have suggested creating an emergency plan for each child in case of seizures. This plan should be given to a healthcare professional working in the educational institution, as well as to teachers who are responsible for the life and health of the child during classes.

Hopantomide® is a drug containing calcium salt of hopantenic acid. The drug is registered and produced in Russia (Usolye-Siberian Chemical Pharmaceutical Factory) with the use of own raw materials and goes through a full production cycle, which guarantees quality control at every stage. Hopantomide belongs to nootropics and has neurometabolic, neuroprotective, and neurotrophic properties, as well as anticonvulsant action. According to the package insert and results of the studies evaluating the efficacy of hopantenic acid, Hopantomide has a number of positive therapeutic effects. It increases brain resistance to hypoxia and toxic substances, stimulates anabolic processes in neurons, improves mental and physical performance, combines moderate sedative action with mild stimulating effect, reduces motor excitability with simultaneous regulation of behavior, improves the metabolism of gamma-aminobutyric acid and normalizes its level in individuals with chronic alcohol intoxication and after alcohol withdrawal, has anxiolytic and thymoleptic properties, inhibits the abnormal bladder reflex and detrusor tone. This ensures high efficacy of Hopantomide in the treatment of various nervous diseases in different age groups. In our opinion, the combination of various positive effects of just one drug can significantly reduce the pharmacological burden. In this review, we discuss well-known and potential mechanisms of action, indications for its use with a focus on Hopantomide benefits in pediatric neurological practice, and studies evaluating the efficacy of hopantenic acid in clinical practice. Particular attention is paid to the possibilities of its use in children, including those with attention deficit hyperactivity disorder and developmental delay, as well as the part of combination therapy for epilepsy together with antiepileptic drugs.

Drugs containing hopantenic acid are well tolerated by patients, including children who receive it for a long time.

Despite the success of vaccine prophylaxis and therapy, neuroinfections remain a serious problem due to the epidemic threat, high mortality and residual disabling and maladaptive neurological deficits in half of the cases.

The aim of the literature review was to study modern publications concerning the dynamics of the infectious process in the central nervous system, the nature and predictors of its outcome in children.

The results confirmed the effectiveness of social modification with the help of vaccination, guidelines and increased availability of medical care, leading to a decrease in mortality and morbidity of vaccine-controlled neuroinfections. At the same time, the general morbidity and due to the severity of complications and structural acute phase defect post-infectious neurological deficit in the form of epilepsy, motor, cognitive and behavioral disorders remain the same.

The study of the outcomes of neuroinfections, their clinic and treatment at all stages, including the remote one, is necessary to develop an optimal protocol for the treatment and rehabilitation of children who have undergone meningitis and encephalitis in order to improve their quality of life.

The review provides an analysis of 73 full-text articles, the source of which was the Medline, OMIM, NCBI, Pubmed, Scopus, eLibrary.ru databases. The data of studies of the main pathogenetic mechanisms of the formation of the cerebral palsy (CP) phenotype, such as chromosomal aberrations, copy number variations, single nucleotide polymorphisms, associated with the development of the CP phenotype, are reviewed and analyzed. Epigenetic effects on the genome, as well as the effects of the genome on the mechanisms of epigenomic regulation, are examined in detail. The data on the genetic determinism of concomitant pathology and reactivity to therapeutic tactics are presented. Based on the study of data from numerous studies, the authors draw the following conclusions:

1) the pathogenesis of the phenotype of CP includes a large number of genes that determine violations of cellular metabolism, neuroontogenesis, brain resistance to hypoxia, etc;

2) genes whose abnormalities form a syndromic pathology are involved in the pathogenesis of CP;

3) the multidirectionality and breadth of the effects of the gene pool with the outcome in a syndrome-specific distinctive picture of the CP allows us to propose the concept of a neurotropic genome;

4) the mechanisms of gene involvement can vary from aberrations to epigenetic imbalances;

5) different groups of genes can differentially influence the formation of individual syndromes in the phenotype of CP;

6) there are data indicating a genetic determinism of the tendency to contracture, pharmacoreactivity to drugs that reduce muscle tone, reactivity to habilitation effects;

7) genomic-epigenomic interactions normally ensure the body’s adaptation to environmental conditions, and with pathology, they increase the likelihood of regulatory breakdowns that lead to the formation of a CP phenotype;

8) the exclusion from the diagnosis of CP of genetically determined cases of phenotype development is incorrect.

The authors present two anthropogenic reasons for the increase in the frequency of occurrence of de novo identified gene abnormalities:

1) anthropogenic impact on the environment, increasing the number of anomalies of the genome de novo; 2) iatrogenic effects of technologies for preserving life, vitality and reproductive ability of carriers of genomic anomalies. This effect leads to the fixation of anomalies in the genome of the population.

A paradox is formulated, according to which, in the presence of technologies capable of preserving the life of carriers of genomic anomalies, in vivo technologies for genome correction are only just beginning to be put into practice. Based on this, it is concluded that it is necessary to intensify the development of methods for prenatal diagnosis and gene therapy of CP.

We report a case of a girl with a chromosomal disorder that has never been described in the literature: inversion of chromosome 4 with an unbalanced translocation between the short arm of chromosome 4 and long arm of chromosome 18. Clinical manifestations of this syndrome included severe growth retardation, very slow weight gain, optic nerve hypoplasia, pronounced delay in mental and motor development, and epilepsy with focal hemiclonic fever-related seizures of varying location. The patient has multiple stigmas of dysembryogenesis, but no abnormalities in the development of internal organs. The somatic status is complicated by chronic liquid aspiration and sleep apnea. Magnetic resonance imaging has demonstrated agenesis of the corpus callosum. In this article, we have summarized the results of clinical observation and electroencephalography findings obtained during several years. The type of epilepsy in this girl does not match Wolf–Hirschhorn syndrome (which is determined by her karyotype), but is similar to epilepsy in patients with aberrations of the long arm of chromosome 18.