This article provides a detailed description of benign epileptiform discharges of childhood (BEDC) as one of electroencephalography patterns in children. We emphasize that BEDC are not specific for both idiopathic (genetic) focal epilepsy and epilepsy in general. BEDC can be observed in a number of various disorders and also in neurologically healthy children. We developed a detailed classification of BEDC-associated states that are divided into 3 groups: neurologically healthy children, children with various forms of epilepsy, and patients with various neurological disorders without epilepsy. We found that there are 2 main factors responsible for BEDC in electroencephalogram: genetic predisposition and white matter lesions, usually occurring during the antenatal or perinatal period. BEDC should be considered rather a manifestation of congenital abnormalities in brain maturation than a marker of epilepsy. We also discuss the need for therapy in patients with BEDC-associated states.

Background. The similarity of the clinical manifestations of encephalitis (E), disseminated encephalomyelitis (DEM) and multiple sclerosis (MS) in children, the complexity of predicting the nature of the course and outcome of diseases determines the search for additional diagnostic and prognosis criteria.

Objective: a comparative characteristic of laboratory indicators for E, DEM and RS in children to assess their diagnostic and prognostic value.

Materials and methods. Fifty six children (14 children with E, 14 – with DEM, 28 – with MS) and 16 children of the control group (with acute respiratory virus infection) at the age from 10 to 17 years were examined. Laboratory methods included standard studies of cerebrospinal fluid (protein, cytosis), determination of concentrations of albumin and immunoglobulin G (IgG) in cerebrospinal fluid and serum with subsequent calculation of protein indices (albumin, immunoglobulin, intrathecal immunoglobulin G indexes).

Results. With all nosological forms in the serum there were no significant differences between albumin and IgG from the control group, whereas in the cerebrospinal fluid an excess of the concentration of IgG was detected at a normal level of albumin. An increase in the albumin index was found only in E, whereas the immunoglobulin index was significantly higher than the norm in all groups of patients. A significant spread of the intrathecal IgG index (from 0.1 to 11.9) was found, which determined the analysis of clinical and laboratory parameters by subgroups, depending on its magnitude. The maximum incidence of increased intrathecal IgG synthesis was detected in children with MS (with exacerbation of MS – 74 %, in remission – 67 %), whereas in E and DEM, an increase was observed in about half of the patients surveyed and associated with a more favorable course of the disease.

Conclusion. The data obtained make it possible to assert that, despite the commonness of some pathogenetic mechanisms, there are differences in the degree of impaired permeability of the blood brain barrier, the intensity of the systemic and intrathecal humoral immune response in E, DEM and RS, which may determine the features of their course and the outcome of the disease, including the transformation of the DEM in the RS.

Background. Adequate selection of the 1st antiepileptic drug (AED) is an obligatory condition for the successful treatment of epilepsy. It is well known that the first drug in the treatment of infantile spasms (IS) in tuberous sclerosis complex (TSC) is vigabatrin (VGB). With regard to focal seizures (FS) in TSC, there are certain differences: some authors insist on VGB (P. Curatolo, 2012), others as the first choice drugs are mentioning carbamazepine and valproate (A. Saxena, 2015). Data in general on the possible effectiveness of medical treatment of epilepsy in TSC, and the effectiveness of different AEDs are also contradictory.

Objective: to make a comparative evaluation of the efficacy of various AEDs in the treatment of epilepsy in patients with TC.

Materials and methods. Retrospective analysis of medical records of patients hospitalized with epilepsy and TSC in the Department of Epileptology and Psychoneurology, Research and Clinical Institute for Pediatrics named after Yu.E Vel’tishev of N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, for the last 2 years was completed. Efficacy analysis was conducted in 134 patients (91 (67.9 %) with FS and 43 (32.1 %) with IS). Efficacy was estimated as the remission of epileptic seizures during 6 months. The percentage of patients with remission was also evaluated (seizures were stopped for 1 year or more) and percentage of patients with a decrease in the number of seizures. The results of the first monotherapy and the subsequent administration of two and three AEDs were analyzed.

Results. Low efficiency of AEDs in starting monotherapy was noted – the remission of seizures within 6 months on any AED was only 27.5 % (25 from 91) with FS, with IS – 13.9 % (6 from 43). Remission on any 1st AED was observed only in 13.2 % (12 from 91) and 6.9 % (3 from 43), respectively. The effectiveness of individual AED is low, especially in achieving stable remission. So, valproate caused remission of FS in 14 (22.2 %) from 63, but in the future remission was stable only in 11 (17.5 %) from 63. VGB as the first monotherapy proved to be effective in 5 from 6 patients with FS and in 4 from 6 patients with IS. The introduction of the 2nd drug added another 13.3 % and 38.6 % of patients with remission of seizures, the 3rd AED – 7.3 % and 7.7 % in FS and IS, respectively. Most often, an effective drug in additional therapy was VGB. The efficiency of VGB was reduced if it was used not as the first, but as the second and third AED. The percentage of unsuccessful treatment (including combined therapy) is estimated as 51.5 % and 47.8 % of patients with FS and IS, respectively.

Conclusion. Epilepsy associated with TSC is less sensitive to AEDs and gives a smaller percentage of remissions. Perhaps in our country this is due to the difficulties of prescription of VGB as a starting therapy for epilepsy in the patients with tuberous sclerosis complex.

Background. Despite significant advances in epileptology, approximately 30 % of patients suffer from drug-resistant epilepsy. Numerous approaches are currently available to treat epilepsy; however, there are still many patients with treatment-resistant disease, in whom surgery is impossible and alternative methods (vagus nerve stimulation and ketogenic diet) are ineffective. Thus, searching for new effective antiepileptic drugs (AED) for these patients remains highly relevant. In this article, we reviewed available publications and provided own results on the efficacy and tolerability of brivaracetam (Briviact®) in patients with intractable focal epilepsy.

Materials and methods. The study included 8 patients aged between 16 and 35 years (mean age 18.3 years; 2 males and 6 females) with intractable focal epilepsy treated at the Svt. Luka’s Institute of Child Neurology and Epilepsy between February 1st, 2017 and September 1st, 2018.

All patients received brivaracetam as an additional AED for the treatment of focal and bilateral convulsive seizures. Patients were followed up for 1 to 7.5 months. Brivaracetam was added to 1 or 2 AED (valproate, topiramate, or carbamazepine/oxcarbazepine) at a dose of 100–200 mg/day divided into 2 doses.

Results and discussion. Good therapeutic effect (more than 50 % reduction in seizure frequency) was registered in 4 patients (50 %). Two patients (25 %) achieved a 25–50 % reduction in seizure frequency. Minimal clinical efficacy with no effect was observed in one patient (12.5 %). One patient (12.5 %) had aggravation of focal and motor seizures. Brivaracetam significantly reduced the severity (intensity and duration) of epileptic seizures in 70 % of patients. Four patients demonstrated substantial improvements on electroencephalogram (decreased epileptiform activity). One patient had complete suppression of epileptiform activity. Brivaracetam was most effective for bilateral convulsive seizures: 4 out of 5 patients experienced complete relief of these seizures.

Brivaracetam demonstrated good tolerability: no side effects were registered in this study. Six out of 8 participants (75 %) currently continue treatment with brivaracetam. It is important that none of the patients had to stop brivaracetam due to poor tolerability. Of note, all of study participants started to receive brivaracetam because they had seizures resistant to multiple (more than 2–3) AED.

Conclusion. Our findings suggest high efficacy and good tolerability of brivaracetam in patients with focal epilepsy. Our results are also consistent with the data reported by foreign authors.

Attention deficit hyperactivity disorder (ADHD) is the most common cause of behavioral disorders and learning difficulties in preschool and school-age children. Patients with ADHD are often diagnosed with concomitant diseases, which creates additional diagnostic and therapeutic challenges and leads to a more significant reduction in the quality of life. ADHD is often associated with epilepsy: ADHD manifestations are more common in individuals with epilepsy, and vice versa, patients with ADHD are more likely to have epilepsy. The estimated prevalence of ADHD in children is 7–9 %, whereas in children with epilepsy, it reaches 20–50 %. Epilepsy is also one of the most common diseases in children (affecting approximately 1 % of the pediatric population), which is often aggravated by concomitant diseases, including cognitive, behavioral and emotional disorders. Various factors, such as characteristics of epileptic process and lesions in particular portions of the brain, can underlie the development of ADHD in epilepsy. Epileptiform activity and adverse effects of antiepileptic drugs can also play an important etiological role. Some antiepileptic drugs (such as barbiturates) may cause symptoms similar to those in ADHD (in this case, inattentiveness and hyperactivity shall be considered as adverse events that can be reduced or eliminated after cessation of the drug) or exacerbate ADHD symptoms in patients with these disorders. Therefore, the drugs with no negative impact on concomitant diseases or with a positive therapeutic effect for both diseases are preferable in these cases.

High prevalence of the ADHD/epilepsy combination leads to a greater reduction in the quality of life, suggesting high relevance of this problem and requiring a revision of therapeutic approaches.