[Controversial and unresolved aspects of the problem of epilepsy]. / Diskussionnye i nereshennye aspekty problemy epilepsii.

OBJECTIVE: Based on the analysis of a comprehensive examination of patients (clinical, neuropsychological, genetic, neuroimaging) to identify the signs that distinguish/preserve the features of cryptogenic epilepsy (CE) relative to symptomatic and generalized idiopathic epilepsy (SE and IE). MATERIAL AND METHODS: 375 patients with various forms of epilepsy were examined: 231 - with SE, 66 - with IE, and 78 - with CE. All patients underwent a generally accepted clinical examination; patients were divided into two clusters: 1 cluster (to identify the distinctive features of the psychoemotional status of patients, to assess the quality of life and compare them with the severity of seizures and other indicators), 2 cluster (to determine the presence of genetic disorders in CI patients and to conduct fMRI in this group). RESULTS: The features of psychoemotional disorders, indicators of quality of life, genetic characteristics that distinguish CE from IE and SE are established. CONCLUSION: CE is a consequence of a combination of two main risk factors for epilepsy - 1) hereditary and 2) exogenous brain damage, but less pronounced compared to IE and CE.

[Healthcare of Russia today. Epileptological service]. / Zdravookhranenie Rossii segodnya. Epileptologicheskaya sluzhba.

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-epileptologist¼ (59%), «Neurologist-epileptologist¼ (81%), self-referral to an epileptologist (22%), «Psychiatrist-epileptologist¼ (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.

[The efficacy and tolerability of extended release carbamazepine in adult patients with new-onset epilepsy using epileptiform activity index]. / Effektivnost' i perenosimost' terapii prolongirovannym karbamazepinom vpervye vyyavlennoi fokal'noi epilepsii u vzroslykh s uchetom indeksa epileptiformnoi aktivnosti.

OBJECTIVE: To evaluate the efficacy and tolerability of extended release carbamazepine (finlepsin-retard and tegretol CR) in adult patients with new-onset focal epilepsy (FE) with the assessment of epileptiform activity index (EAI). MATERIAL AND METHODS: The study included 62 patients (38 (61.3%) men and 24 (38.7%) women) with new-onset FE aged ≥18 years (mean age 42.9±18.4 years). All patients underwent video-ECG-monitoring with EAI assessment at each visit. Treatment efficacy was assessed using the criteria of seizure absence (medically induced remission), seizure rate decrease by >50% (responders), seizure rate decrease by <50% - insufficient efficacy, retention on treatment and seizure rate increase compared to baseline and/or development of new type of seizures (aggravation). Overall study period was 12 months. RESULTS: By the end of the 12-month follow-up period, there was a 4.3-fold decrease of the total EAI compared to baseline (p<0.001). Retention on carbamazepine treatment during 12 months was achieved in 61.3% (n=38) patients; medically induced remission - in 40.3% (n=25); seizure rate decrease by >50% - in 21.0% (n=13). In 29.1% (n=18) of patients, treatment change was performed; double-drug therapy, including carbamazepine, was prescribed in 9.6% (n=6) of patients. Incidence of adverse events was 29.1% (n=18). CONCLUSIONS: Carbamazepine is an effective and promising drug for initial monotherapy of FE. Its use in the treatment of FE results in a 4.3-fold decrease of EAI (p<0.001), which reflects the efficacy of treatment. EAI is an additional objective measure of treatment efficacy.

[Principles of classification of diseases of the nervous system on the example of two diseases with paroxysmal manifestations - typical facial pain and epilepsy]. / Printsipy klassifikatsii zabolevanii nervnoi sistemy na primere dvukh zabolevanii s paroksizmal'nymi proyavleniyami ­ tipichnoi litsevoi boli i epilepsii.

Two diseases of the cranial region with similar key symptoms - typical facial pain and epilepsy are analyzed. The objects of the study are the International Classification of Headache of the 3rd revision, 2018, fragments of which are facial pain, and the International Classification of Epilepsy and Epileptic Seizures, 2017. The key characteristic of both diseases is paroxysmality, that is, manifestations of the main signs of the disease in the form of seizures. The basic indicators of the disease, which are used in classifications, are similar in both cases - the origin (etiology) and the essence of seizures. However, the approaches here are different and even multidirectional. In the International Classification of Headaches, there is a clear desire to preserve the concept of «idiopathic¼ in the sections of paroxysmal facial pain for cases whose origin is unknown. On the contrary, in the International Classification of Epilepsy and Epileptic Seizures, the use of this term is extremely limited: it is replaced by the term «genetic¼ for forms of epilepsy, where it is proven, and «unknown etiology¼, previously «cryptogenic¼, facial pain and the International Classification of epilepsy and epileptic seizures: in the first case, changes in form, in the second in the content. Another object for analysis is ambiguous terminology. The analysis of paroxysms in two different diseases with paroxysmal symptoms - facial pain and epileptic seizures - reveals the divergence of their assessment according to the criteria of origin - idiopathic, genetic and unknown etiology (cryptogenic), as well as terminology of form and content.

[Dynamics of epileptiform activity, efficacy and tolerability of valproic acid in adults and adolescents with newly-diagnosed epilepsy]. / Dinamika epileptiformnoi aktivnosti, effektivnost' i perenosimost' preparatov val'proevoi kisloty pri vpervye vyyavlennoi epilepsii u podrostkov i vzroslykh.

OBJECTIVE: To evaluate the changes of epileptiform activity index (EAI) as a measure of the efficacy and tolerability of treatment with valproic acid (VA) in patients with newly-diagnosed generalized and focal epilepsy. MATERIAL AND METHODS: The study included 93 patients (55 men and 38 women): 27 with focal epilepsy (FE) and 66 with idiopathic generalized epilepsy (IGE). Patients with idiopathic and age-dependent FE were not included in the study. At each visit, video-EEG monitoring with the analysis of focal, diffuse and generalized epileptiform activity during wakefulness before sleep, during sleep, after sleep and during partial awakenings with quantitative EAI assessment at baseline, after 1, 3, 6 and 12 months of therapy was performed. Therapeutic drug monitoring was performed during dose titration 1 month after the start of treatment or in case of treatment change. Treatment efficacy was assessed based on the absence of seizures, decrease of seizure episodes by more than 50% (responders) and <50% (insufficient efficacy). Adverse effects (AE) were assessed using the Assessing SIDe effects in AED treatment scale (SIDAED). RESULTS: Maximal EAI was observed at baseline both in FE and IGE groups. In patients with IGE, the total EAI (52.8±7.8) was significantly higher compared to FE group (27.1±5.5 (p=0,027)). During wakefulness before sleep and during sleep EAI was significantly lower in the IGE group compared to FE patients (3.4±0. vs. 10.5±5.5 (p=0.003) and 4.3±0.8 vs. 8.9±3.7 (p=0.046), respectively). VA demonstrated the high efficacy and good tolerability in IGE and FE patients: after 12 months of treatment remission was achieved in 69 (74.2%) patients, the decrease of seizure frequency by more than 50% was observed in 22 (23,7%) patients, insufficient efficacy only in 2 (2.1%). AEs were registered only sporadically. CONCLUSIONS: VA remains one of the drugs of choice in IGE and FE. EAI may become an additional objective test in cases of difficult differential diagnosis in IGE and FE, using total EAI, EAI before sleep, during sleep, during partial awakenings in the first months of treatment (1-3 months). EAI objectively reflects the dynamics of VA treatment efficacy.

[Psychogenic pseudoseizures as psychoneurological problem]. / Psikhogennye psevdoepilepticheskie pripadki kak obshchaya problema nevrologii i psikhiatrii.

The complexity of the problem is associated with the lack of reliable differential diagnostic indications for distinguishing epileptic and pseudo-epileptic seizures, as well as with the risk of provoking seizures during psychological sessions in patients with PPS. Therefore, apparently, only one of the Russian-language monographs that were published in recent years and one of the 1999 neurology guidelines pay attention to this problem. We have shown the nosological heterogeneity of patients with PPS, clinical particularities related to it and the need for a differentiated approach to its therapy. PPS is a serious clinical problem that can successfully be solved only by interdisciplinary development.

[Relationships between cerebral and facial venous blood flow]. / Vzaimootnosheniia tserebral'nogo i litsevogo venoznogo krovotoka.

The aspects of disruption of venous blood outflow from face region into skull are well-known; they can occur at any age - from neonatal to old age, however, the possible compensation mechanisms for such disorders are usually difficult to identify. A description is given of a unique case of unilateral epistaxis and a homolateral (right-sided) headache that developed much later. Examination revealed hypoplasia of the left sigmoid sinus and blood overflow of the right sigmoid sinus. After prescription of betahistine in a dose of 24 mg before bedtime and after awakening the headache and nosebleeds disappeared on the second day. The effect was persistent. The case demonstrates previously unknown compensation mechanisms for venous outflow - a retrograde outflow from the skull to the face region.

[New possibilities of pharmacotherapy of epilepsy: eslicarbazepine acetate in treatment of focal epilepsy]. / Novye vozmozhnosti farmakoterapii épilepsii: mesto éslikarbazepina atsetata v lechenii fokal'nykh épilepsii.

The article provides an analysis of the latest antiepileptic drug eslicarbazepine acetate (ESL) for the treatment of focal epilepsy in adults as monotherapy and adjunctive therapy. Pharmacokinetics, pharmacodynamics, drug-drug interactions, indications and dosage regimen are described. The authors present the latest data on the efficacy, tolerability and safety of ESL including an exploratory pooled analysis of data from 14 European clinical practice studies (Euro-Esli) as well as their own observations of 34 patients receiving ESL in monotherapy or combination therapy.

[Back to Jackson or ahead to Jackson? Who brought a contemporary perspective to Jackson's conception?] / Nazad k Dzheksonu ili vpered k Dzheksonu? Kto pridal ucheniiu Dzheksona sovremennoe zvuchanie?

One hundred and twenty years ago, J. Jackson summarized the results of his research in his seminal lecture, where he used epilepsy, as a model for the analysis of the structural-functional organization of the central nervous system. He revealed a mechanism of epileptic seizure and showed that any epilepsy, including the 'genuine' one, had focal start. However, a dichotomic conception became firmly established later. In this article, basic stages of further development of the problem are discussed and contribution of the Russian-Soviet-Russian neurological school to the return on the modern level to Jackson's unitary conception of epilepsy and a role of epileptic systems forming different courses of epileptic activity flow from epileptic focus to other cerebral structures are emphasized.

[Problems of juvenile myoclonic epilepsy. A view through the prism of time]. / Problemy iunosheskoi mioklonicheskoi épilepsii. Vzgliad skvoz' prizmu vremeni.

The authors present a comparative analysis of the results of the study of 72 adolescent patients with juvenile myoclonic epilepsy (JME), which were published in 2010, and a new sample of patients over the last 5 years with the publications of domestic and foreign authors. Difficult clinical cases are described. These data indicate positive shifts in the diagnosis and treatment of JME. However, the question of diagnosis is still a hard problem for neurologists. The questions to be resolved are: gender aspect of the disease, to what extent levetiracetam could be a drug of first choice and to what extent valproats could be replaced; monitoring of children born to mothers with JME, a role of paternal inheritance.

[Brivaracetam in the treatment of patients with epilepsy]. / Brivaratsetam v lechenii bol'nykh épilepsiei.

The newest antiepileptic drug (AED) brivaracetam (Briviac, UCB Pharma) (BRV) was approved in the Russian Federation in 2017 as an adjuvant therapy for the treatment of partial seizures with/without secondary generalization in adults and adolescents over 16 years old with epilepsy. This review contains the data of BRV preclinical studies, pharmacokinetic profile and the results of comparative study of BRV and LEV. The results of main studies of efficacy and tolerability with pooled analysis as well as data from meta-analysis are presented. The authors present conclusions of the Russian leading epileptologists on perspectives of using BRV in different populations of epileptic patients. The use of BRV is able to provide long-term efficacy in terms of seizure control, is well tolerated, keeps quality of life and social activity of people with epilepsy.

[Depakine: 25 years in Russia]. / Depakin: 25 let v Rossii.

The article summarizes domestic and international studies on the development and clinical investigation of valproates including multiple studies of a research team directed by the author. Valproate targets are considered in biological and clinical aspects. A spectrum of action and advantages of the brand drug (depakine) compared to generics and other antiepileptic drugs are discussed. A number of recommendations for practitioners about using valproates are proposed.

[The development of ideas about the organization of the speech system]. / Razvitie predstavlenii ob organizatsii rechevoi sistemy.

This work represents an insight into a problem of studying one of the higher mental functions of humans - speech, its primary role in the development of man as a biosocial being. Various concepts of speech and the problem of the dominance of cerebral hemispheres in language function are analyzed. It touched upon the ontogenesis of hemispheric asymmetry. The problem of 'codominance' of right and left hemispheres depending on the nature of the task solving by the subject and the functional level of information processing is discussed. The authors highlight that the problem has an interdisciplinary nature, it is marked at the junction of neurophysiology, neuropsychology, neurology and defectology.

[Quality of life of adult patients with cryptogenic epilepsy]. / Kachestvo zhizni u vzroslykh bol'nykh s kriptogennoi formoi epilepsii.

AIM: To determine a position of cryptogenic epilepsy (CE) in relation to idiopathic epilepsy (IE) and symptomatic epilepsy (SE) in the aspect of patients' quality-of-life. MATERIAL AND METHODS: One hundred and fifty patients, including 90 with CE, 30 with SE and 30 with IE, were examined. The severity of epileptic seizures assessed with the NHS3, anxiety and depression assessed with the HADS, sleep disorders, quality-of-life measured with the Qolie-89 were studied. RESULTS AND CONCLUSION: CE was similar to SE in many parameters but had a favorable course. The following factors were similar for CE and IE: family history of disease, differences between the severity of seizures and Qolie-89 scores. Quality-of-life was higher in CE compared to SE, despite the marked anxiety of patients. It has been concluded that CE, which is caused by both genetic and environmental factors, has an interim position between IE and SE.

[Perampanel in pharmacotherapy of focal epilepsy: the efficacy and tolerability in routine clinical practice]. / Perampanel v terapii fokal'nykh epilepsii: effektivnost' i perenosimost' v povsednevnoi klinicheskoi praktike.

AIM: To analyze the efficacy and tolerability of the innovative drug perampanel (PER) as add-on treatment of focal epilepsy in patients older than 12 years. MATERIAL AND METHODS: Forty-six patients with focal epilepsy, aged from 12 to 63 years, mean age 31.7 years, were studied. RESULTS AND CONCLUSION: A decrease in the frequency of all types of seizures by >50% was noted in 46,5% of patients. Stopping of all types of seizures was found in 25.6% of patients, i.e. in every fourth patient, stopping of secondary-generalized seizures in 39.5%. Adverse effects (AE) were identified in 13 (28.2%) of patients, including aggressiveness - 6 (13.0%); other AE were less frequent (<10%): sleepiness (8.7%), dizziness (4.3%), postural instability (4.3%), irritability (4.3%). PER was withdrawn in 3 (6.5%) patients due to AE. Mean effective dose was 6 mg/day. Quality-of-life improved in the majority of patients (71.7%).

[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.