Background. Given the difficulties in identifying absences and assessing the level of consciousness in epilepsy patients, it is extremely relevant to develop digital programs for automatic registration and testing of this type of epileptic seizures and related electroencephalographic (EEG) patterns, including those based on artificial intelligence.Objective: development of an algorithm for automatic detection of absence seizures to test real time patient's consciousness level during long-term video-EEG monitoring.Material and methods. The work on creating an algorithm was carried out during joint doctor/engineer cooperation. Doctors prepared a set of labeled EEG recordings of patients with verified absence epilepsy. Two independent experts in the generated examinations database mapped typical episodes of absence seizures that allowed to develop training and testing samples for a neural network algorithm to detect EEG absence epiactivity. Next, trained neural network was incorporated into Neuron- Spectrum.NET software to compare its accuracy with similar approaches published elsewhere.Results. A neural network algorithm was developed and trained using a mapped database to detect EEG absence epiactivity. A comparative analysis of the effectiveness for the proposed method vs. other approaches showed that the former is comparable in quality, whereas in some aspects – even superior to the latter. Accuracy was assessed using a publicly available database with mapped epiactivity episodes.Conclusion. A hardware and software system for automated assessment of patient’s consciousness level during absence seizure in continuous video-EEG monitoring was proposed. Potentially, neural networks may be applied not only to assess patient’s consciousness level, but also to stop stimulation-mediated seizure onset in the future.

Dyke–Davidoff–Masson syndrome is a possible cause of several pathologies and has rare appearance in clinical practice. One of these causes is a perinatal stroke. The man 59‑year‑old applied to the Federal center of brain and neurotechnologies had this one. The patient had bilateral tonic‑clonic seizures with loss of consciousness, and remission was observed during the last 4 years with antiepileptic drugs treatment. Complex radiological and func‑ tional diagnostics were performed with electroencephalography, magnetic resonance imaging, magnetic resonance tractography, functional magnetic resonance imaging. As a result of research many signs of Dyke–Davidoff–Masson syndrome were found. On the example of damage in the perinatal period and subsequent adaptation of the brain, its plasticity with respect to speech function was shown.

OBJECTIVE:To examine the state of the epileptological service in Russia by conducting a survey of epileptologists working in different regions of the Russian Federation.MATERIAL AND METHODS:To assess the structure and mechanisms of the antiepileptic service in the Russian Federation, the Russian League Against Epilepsy (RLAE) offered a questionnaire to 63 epileptologists, which has been answered by 37 epileptologists from 28 regions of the country.RESULTS AND CONCLUSION:Among the respondents, 43% are focused on the care of outpatients, 35% work both in outpatient and inpatient appointments, 22% work only in hospitals. 24% of doctors are employees of neurological departments. 84% of epileptologists noted the neurological profile of their medical institution. The most common patient routing options were: «therapist-neurologist-epileptologist» (59%), «Neurologist-epileptologist» (81%), self-referral to an epileptologist (22%), «Psychiatrist-epileptologist» (16%). 59.5% of epileptologists work with adults and with children. 27% of respondents noted that they work with psychiatric patients. Interaction between the departments of neurology and practical health care is carried out in the form of consultations and training events, as noted by 68% of epileptologists. 73% of the epileptologist reported the willingness to actively interact with the RLAE. 54% of the respondents conduct socially oriented schools for patients and their relatives. In the context of the planned reorganization of the health care system, the epileptologists gave the outlook on the work of the epileptological service.CONCLUSION:The main initiatives mentioned by the respondents were the following increasing the availability of medicines and examination methods, increase of continuity in the work of the epileptological service, creation of unified databases, creation of specialized epileptological centers and/or offices of epileptologists.

Electroencephalography (EEG) is one of the main non-invasive methods for examining brain activity. Cranial defects caused by trauma or surgery can lead to artifacts on the EEG such as breach-rhythm. In this article we provide a literature-based definition, pathophysiological features of the breach-rhythm, differential diagnosis between breachrhythm and epileptiform activity, as well as our own clinical case of a patient with epilepsy and breach-rhythm.

Objective: to assess the status of the epileptological service in Russia by conducting a questionnaire survey of epileptologists working in different regions of the Russian Federation.Material and methods. To assess the structure and mechanisms of the antiepileptic service in the Russian Federation, The Russian League Against Epilepsy (RLAE) offered a questionnaire to 63 epileptologists, among which answers were provided by 37 subjects from 28 regions of the country. The questionnaire data are presented as diagrams and expressed as a percentage.Results. The analysis of data obtained allows to assess differences in the structure of the epileptological service in diverse regions of Russia, the level of care transition and interaction between outpatient and inpatient specialists, specialists in various fields as well as those involved in the scientific and practical fields of healthcare. Besides, it has also started to shape insight into further development of epileptological service in Russia envisioned by practicing epileptologists.Conclusion. A feedback from geographic regions is an ultimate goal posed by the Russian League Against Epilepsy. It is necessary to continue involve medical specialists in diverse regions to cooperate and develop new types of collaboration.

OBJECTIVE:To evaluate the effectiveness of antiepileptic therapy of epileptic syndromes associated with myoclonic seizures.MATERIAL AND METHODS:One hundred and three patients with epilepsy, in whom myoclonic seizures were identified during the observation, were examined. The observed myoclonic seizures entered the structure of 11 different epileptic syndromes.RESULTS AND CONCLUSION:Drug remission is achieved in 67% of patients, a decrease in the frequency of seizures by 50% or more was noted in 23,3%, the lack of effect in 9,7%. The effectiveness of antiepileptic therapy was significantly different in different groups of epileptic syndromes. The high effectiveness of antiepileptic therapy in the general group of patients in this study is mainly due to the prevalence of patients with juvenile myoclonic epilepsy, in which the highest percentage of remission was observed.

Here we provide a literature-based definition as well as etiological factors, clinical picture, prognosis, diagnostic algorithm, and treatment for the New-Onset Refractory Status Epilepticus (NORSE syndrome). In addition, we also present our personal experience by describing a female patient with refractory focal epilepsy associated with the cryptogenic new-onset refractory status epilepticus.

Introduction. One of the most serious periods in the treatment and follow-up of adolescent patients with early debut epilepsy is the transition to a medical center for adults. It is important to carefully watch any possible replacement of antiepileptic drugs (AEDs), which may affect the further course of epilepsy.Aim: to study the algorithms and procedures for bringing patients with epilepsy from a pediatric to adult outpatient practice, and also to evaluate the efficacy of therapy with synonymously replaced AEDs during this period.Materials and methods. A retrospective study involved 218 patients who were transferred from pediatric care to an adult outpatient network. The inclusion criteria were the presence of epilepsy diagnosed in childhood (before age of 18 years), continuous treatment with AEDs, age 18 through 20 years. The exclusion criteria were: irregular observations, failure to comply with medical prescriptions. In case of unplanned pregnancy or failure to take AEDs, the patient was excluded from the ongoing study. History, neurological status, EEG, video EEG monitoring and neuroimaging data were examined. The efficacy of AEDs was graded as “complete remission” (in the absence of epileptic seizures), “incomplete remission” (if the number of seizures dropped by 50% or more), and “without effect” (if the seizures continued). Two groups of patients were analyzed: those receiving original AEDs and those receiving generic AEDs.Results. The study showed that during the transition to an adult outpatient network, the specialized medical care continued: the young patients were monitored by epileptologists; at the same time, we noted a significant increase in the rate of replacement of original AEDs with the respective generics (54 patients). Of these patients, 25 individuals (46.3%) had recurrent seizures.Discussion. Maintaining the continuity of specialized medical care depends on the availability of the city center of epilepsy for children. In addition, the network of outpatient clinics for adult patients with epilepsy and other paroxysmal conditions should be organized in each administrative district. The procedure of synonymous AED replacement is regulated by medical and legal documents; thus, the drugs are prescribed according to their INNs, and pharmacies dispense the medicines under the same INNs but with the trade names that are currently available in the stock. According to reports, the seizures are controlled significantly better under therapy with original AEDs compared to generics.Conclusion. The present study demonstrates the relevance of medical care continuity during transition of young patients with epilepsy from a pediatric to adult outpatient network. However, more extensive comparative studies on the efficacy and safety of synonymous replacement of antiepileptic drugs with the same INNs are needed.All authors contributed equally to this article.

The article presents the main definitions, classification, and electroclinical characteristics of epileptic myoclonus.

This article presents the anamnestic, clinical, electro-encephalographic and neuroimaging findings in 5 patients with epilepsy combined with Wolf-Hirschhorn syndrome (WHS). According to our data and the results of others, this combination has its specific characteristics. These include: a high incidence of epilepsy in patients with WHS (50-100% of cases), an early debut of seizures (mainly in the first year of life), fever-provoked seizures, and a variety of seizure types – focal paroxysms, bilateral tonic-clonic seizures, atypical febrile seizures, atypical absences and epileptic spasms. In addition, there may be frequent epileptic seizures tending toward status epilepticus, a slowing of the major EEG activity, a local EEG slowing (mainly in the posterior and bi-frontal areas), and regional / multiregional epileptiform activity. In more than 50% of cases, the diffuse peakwave activity is observed; the broad spectrum anti-epileptic drugs are highly efficient in 80% of cases. Based on this study, we propose recommendations for the management of patients with epilepsy combined with WHS.

GLUT 1 transporter deficiency syndrome (De Vivo syndrome, GLUT 1 deficiency syndrome, De Vivo Disease) is a rare genetic disease associated with a deficiency of the glucose transporter GLUT 1. Due to this deficiency, diffusion of glucose through the blood-brain barrier is limited or completely blocked. As a result, a clinical symptom complex of neurological disorders – epileptic seizures, cognitive deficit, and motor disorders – develops. The article provides a review of the literature on the variety of epilepsy manifestations in this syndrome, including idiopathic generalized epilepsy with absences, myoclonic-astatic epilepsy, and focal epilepsy. The inability of the basic antiepileptic therapy to stop the seizures is noted. The main treatment method is the ketogenic diet. A clinical observation made by these authors is also presented.

Epileptic seizures affect the heart function and can provoke heart rhythm abnormalities and conduction disorders. Dysfunctions of the supra-segmental vegetative structures are thought to play a significant role in the development of arrhythmic syndrome in epilepsy. The asymmetric influence of the left and right cerebral hemispheres on the heart rate, the impact of antiepileptic drugs and other factors on the heart rhythm are still under discussion. The role of heart rhythm abnormalities in the pathogenesis of sudden unexplained death in epilepsy is also reviewed here. We analyze the pathogenesis of cardiac arrhythmias in epilepsy, the occurrence and risk factors, the types of disorders, the role of antiepileptic therapy and the significance of cardiac arrhythmias for the development of sudden death in patients with epilepsy (SUDEP).

Objective. To conduct a survey of adult patients with focal epilepsy and ictal disturbances of cardiac rhythm and conduction, considering the disease classification, anamnesis, clinical, electroencephalographic and neuroimaging data. Materials and methods . We selected 70 patients aged 18 to 51 years old with confirmed diagnoses of focal epilepsy with epileptic seizures persisting at the time of the study on the background of antiepileptic therapy without concomitant cardiac disorders but with confirmed ictal changes in the cardiac rhythm and conduction. Results . Among the examined patients, those with long lasting “structural focal epilepsy”, frequent epileptic attacks, temporal and frontal-temporal epileptogenic foci, taking several antiepileptic drugs, prevailed. Most of the heart rhythm and conduction abnormalities occurred in male patients with serial paroxysms, bilateral tonic-clonic seizures, or sleep seizures. Сonclusion . The timely detection and examination of patients with high risk of ictal arrhythmias is able to prevent disturbances of cardiac rhythm and heart conduction during epileptic seizures, including those with a life-threatening potential.

The article reviews the use of antiepileptic drugs in various forms of epilepsy. We present a complex case of juvenile myoclonic epilepsy, the diagnostic process and antiepileptic therapy. Our own data and the results of others demonstrated a high efficacy and tolerability of valproate drugs, and a low aggravation potential in all types of epileptic seizures.

AIM:To study knowledge and opinions of neurologists about priorities in using antiepileptic drugs (AEP).MATERIAL AND METHODS:Eighty-one neurologists from Moscow and surrounding regions were surveyed to identify the factors that influenced the choice of AEP.RESULTS AND CONCLUSION:Valproic acid was the most frequently used drug followed by levetiracetam and carbamazepine, which reflected the overall picture of PEP indication in patients with epilepsy in the Russian population. Levetiracetam occupies a leading position as the starting drug for treatment of epilepsy; most often prescribed to women, patients with generalized seizures and idiopathic epilepsy. It is the drug of choice as adjuvant remedies for Duo therapy.

The article presents a clinical case of infantile cerebral palsy combined with BEDC (with no epileptic seizures) in dizygotic twins. The case supports the hypothesis of genetic nature of the BEDC patterns. The variety of EEG characteristics and clinical courses of the disease in children with cerebral palsy and BEDC are reviewed. Correct interpretation of the EEG findings provides an accurate prognosis of seizures and cognitive functions, as well as the patient management tactics. In these patients, continuous video EEG monitoring (both awake and sleeping) is recommended for a long-time follow-up.