Objective: to confirm a therapeutic equivalence and similar safety profile of “Midazolam, oromucosal (buccal) solution” and “Sibazon, solution for intravenous and intramuscular administration” used in children aged from 1 year to 18 years suffering from primary generalized and bilateral tonic, clonic and tonic-clonic seizures.

Material and methods. An open-label, randomized clinical trial on efficacy and safety was conducted with 25 patients having primary generalized and bilateral tonic, clonic and tonic-clonic seizures due to epilepsy or epileptic syndrome. The study used age-appropriate doses of Midazolam with a single buccal administration as well as diazepam (Sibazon) for single intramuscular administration. Midazolam dosing was as follows: 5 mg for children of the younger age group (1 tube-dropper 5 mg/ml), 7.5 mg for children of the middle age group (1 tube-dropper 5 mg/ml and 1 tube-dropper 2.5 mg/ml), 10 mg for older children (2 tube-droppers 5 mg/ml). The drug effectiveness was assessed by primary and secondary criteria. The number of cases of drug administration in each group was used as the primary criteria, in which the convulsions ended up within 10 minutes after using the drug and did not resume within 60 minutes after drug administration. The following criteria were used as secondary: no repeated convulsive seizures within 24 hours after drug administration, no repeated convulsive seizure within 48 hours after drug administration, time before repeated convulsive seizure within 48 hours after drug administration. Clinical assessment was carried out according to clinical data, electroneurophysiologic (electroencephalographic) studies, electrocardiography, clinical blood and urine tests, aswell as biochemical blood tests by measuring glucose, total protein, albumin, total bilirubin, cholesterol, aspartate aminotransferase, alanine aminotransferase, creatine phosphokinase, alkaline phosphatase, creatinine, urea, and creatinine clearance level.

Results. Compliance with the first efficacy criterion after using Midazolam and Sibazon was observed in 11 (84.6%) and 9 (75%) patients in Group 1 and Group 2, respectively, showing insignificant differences (Fisher's exact test (FET): p=0.645). The number of no cases of repeated convulsive seizure within 24 hours after drug administration differed significantly and was 12 (92.3%) and 6 (50%), respectively (FET: p=0.030). The number of cases with no second seizures within 48 hours after drug administration in Group 1 and Group 2 was 12 (92.3%) and 5 (41.7%), respectively, showing insignificant differences (FET: p=0.0112). No serious adverse events were reported during the study. No patients cancelled participation in the study due to developed adverse event.

Conclusion. The data obtained evidence about compatibility of therapeutic efficacy profile and similar safety profile for “Midazolam, oromucosal (buccal) solution” and “Sibazon, solution for intravenous and intramuscular administration” that agrees with multiple data of earlier studies.

Objective: to assess efficacy and safety of vagus nerve stimulation (VNS) in patients with pharmacoresistant epilepsy.

Material and methods. A multi-center retrospective observational program was applied in patients with pharmacoresistant epilepsy by using vagus nerve stimulation for at least 2 years. There were enrolled 151 subjects, patient age on stimulator implantation varied from 5 to 65 years (24.4±13.1 years). Among them, subjects under 18 or at least 18 years of age comprised 58 (38.4%) and 93 (61.6%), respectively. Changes in rate and severity of major group epileptic seizures (highly disabling type) 24 months after VNS-therapy vs. baseline state as well as during 3-, 6-, 9-, 12-month follow-up were compared. There were assessed stimulator-related effects on VNS-therapy as well as patient quality of life 2 years after therapy. The dynamics of the frequency of all types of epileptic seizures was evaluated according to McHugh Outcome scale.

Results. Mean epilepsy duration on stimulator implantation was 170.9±126.8 months, with maximum up to 666 months (55 years). Number of patients with dominant (disabling) seizures on implantation procedure comprised 136 (90.1%). Decline in dominant epileptic seizure rate by 50–99% was recorded in 91 patients (66.9%) 24 months after VNStherapy. Among such subjects were 41 patients (30.15%) featured with disabling seizures including 24 fully seizure free subjects (17.65%). Decreased rate of all group epileptic seizures by more than 50% (responders) was found in 52.9% cases, including subjects under 18 and adults in 63.9% and as few as 46.3% (p<0.05), respectively. While assessing dynamic rate for all groups of epileptic seizures applied with VNS-therapy by using McHugh Outcome scale it was found that class I (lowered seizure rate by 80–100%) was observed in 44 cases (29.1%), including 18 patients under 18 (31%) and 26 subjects above 18 (28%) (insignificant difference). Mean dominant group epileptic seizure rate was also significantly decreased in both age groups from 20 down to 5.7 per month. Severity of epileptic seizures and postseizure condition upon VNS-therapy was decreased in 38.6% and 43.9% patients 24 months after therapy and on final follow-up visit, respectively (more than 24 months after implantation). No serious adverse events as well as adverse effects resulting in therapy cancel were noted. Conclusion. Vagus nerve stimulation is an effective and safe auxiliary treatment method for therapy of pharmacoresistant epilepsy both in children and adults.><0.05) , respectively. While assessing dynamic rate for all groups of epileptic seizures applied with VNS-therapy by using McHugh Outcome scale it was found that class I (lowered seizure rate by 80–100%) was observed in 44 cases (29.1%), including 18 patients under 18 (31%) and 26 subjects above 18 (28%) (insignificant difference). Mean dominant group epileptic seizure rate was also significantly decreased in both age groups from 20 down to 5.7 per month. Severity of epileptic seizures and postseizure condition upon VNS-therapy was decreased in 38.6% and 43.9% patients 24 months after therapy and on final follow-up visit, respectively (more than 24 months after implantation). No serious adverse events as well as adverse effects resulting in therapy cancel were noted.

Conclusion. Vagus nerve stimulation is an effective and safe auxiliary treatment method for therapy of pharmacoresistant epilepsy both in children and adults.

The article presents two clinical cases of intensive care unit (ICU) patients with periodic discharges. Examination according to the American Clinical Neurophysiology Society (ACNS) protocol with the use of electroencephalography and functional probes allows differential diagnosis of periodic discharges, which makes it possible to conduct early pathophysiological treatment, to assess its effectiveness in dynamics and to avoid unjustified prescription of antiepileptic drugs. At the same time, the prognosis of the disease and the effectiveness of treatment are largely determined by the etiology of the disease. Evaluation of periodic discharges of three-phase morphology in ICU, considering its nosological nonspecificity, is rational to carry out according to the ACNS recommendations, indicating the ictal genesis, background activity, response to antiepileptic drugs.

A clinical case of a boy aged 20 months old with early infantile epileptic encephalopathy (EIEE) type 54 due to mutated HNRNPU gene presumably suffering from genetic generalized epilepsy and impaired psychomotor development is described. Exome-wide sequencing was carried out by using NextSeq 500 (Illumina, USA). Video electroencephalographic (VEEG) monitoring was conducted by using NeuroScope NS425 (Biola, Russia). The patient was noted to suffer from neonatal delayed motor development and muscular hypotonia with atypical petit mal epilepsy with regional onset at the occipital-parietal-posterior temporal areas based on VEEG data developed at age of eight months as well as progressive psychoemotional disorders. Ethosuximide and valproic acid administered together were efficient in alleviating EIEE seizures that requires to be further followed up. The data obtained allow to identify a precise etiology of epilepsy and apply a differential approach to administer anti-epileptic agents.

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.

Establishing misdiagnosis “epilepsy” is a common event comprising 25% total cases of “pharmacoresistant” forms. Eventually, the majority of cases resulted in diagnosing psychogenic nonepileptic seizures, or functional seizures, conversion seizures, and dissociative seizures. Here we review publications assessing psychogenic non-epileptic seizures. The scientific resources for analysis were selected in Russian (eLibrary) and international (Pubmed/MEDLINE, Google Scholar) databases, as well as in open access resources. We also present a clinical case of a patient diagnosed with epilepsy. In particular, the patient was admitted to the hospital with complains of paroxysmal conditions starting as headache, nausea proceeding with speech arrest and decreased mindfulness of what happens in personal life. He also informed about at least two episodes of disorientation described as “missed public transport stop” and “finding himself in unfamiliar place”. The patient underwent examination (electroencephalography, brain magnetic resonance imaging) and dynamic follow-up to verify origin of such conditions. As a result, he was diagnosed with psychogenic non-epileptic seizures. Administering proper therapy allowed to achieve stabilized condition and arrest seizures. The criteria provided in the review as well as clinical case report may help clinical practitioners to timely conduct differential diagnostics and deliver specialized medical aid.

The problem of low adherence to therapy in patients with epilepsy is one of the most pressing public health problems. Failure to follow the doctor's instructions leads to an increased risk of hospitalization and sudden death. Knowing the main predictors of non-compliance, the doctor is able to develop appropriate strategies to increase adherence. There are methods of improving compliance that should be actively implemented in the clinical practice of epileptologists. The article presents a review of the literature on this problem, clinical examples from the authors' practice are given.

Juvenile myoclonic epilepsy (JME) is the most common form of genetic generalized epilepsy. Patients with JME are at risk of life-threatening heart rhythm and conduction disorders as well as sudden death syndrome due to several potential mechanisms: genetic, clinical, neuroanatomical, pharmacological, psychological, comorbid. This lecture reviews important elements of knowledge about the pharmacological predictors of cerebral-cardiac syndrome and sudden unexpected death in epilepsy. The arrhythmogenic potential of antiepileptic drugs most often used in JME (valproic acid, levetiracetam, lamotrigine, topiramate and zonisamide) is considered, none of which can be classified as class A (drug without risk of QT interval prolongation or TdP) regarding a risk of QT interval prolongation and cardiac arrhythmias. Patients with JME require dynamic video-electroencephalographic monitoring and 24-hour electrocardiographic monitoring to reduce a risk of life-threatening cardiac arrhythmias.

On March 18, 2021, an online council of experts in the field of epilepsy treatment was held, dedicated to the use of perampanel in the additional therapy of focal seizures (FS) and primary generalized tonic-clonic seizures (PGTCS) in children 4–12 years old. During the event, the features of the use of perampanel in this group of patients were discussed, considering the specifics of everyday clinical practice, current possibilities of PGTCS and FS therapy in children, its goals, as well as unsolved problems in the treatment of pediatric patients with PGTCS and FS. Particular attention was paid to the role of perampanel in the treatment of PGTCS and FS in children and its effectiveness in specific types of focal seizures.