The article deals with epileptic manifestations with malformations of the corpus callosum of the example of two disembriogenetic syndromes (Aicardi syndrome and Pierre-Robin syndrome). These hereditary syndromes have in common is expressed epileptic manifestations, malformations of the corpus callosum and other somatic congenital abnormality. The treatment is mainly symptomatic. Unlike most other neurogenetic syndromes with known molecular defect, DNA testing at Aicardi syndrome and Pierre-Robin syndrome is very difficult task, and the focus of the diagnosis of the disease belongs to the complex clinical and neuroimaging studies. Timely detection of these syndromes is a difficult task that requires a great deal of clinical experience, practical knowledge, not only in neuroscience but also in the related fields of medicine.

Pathological electrical activity in proximity as well as distant cortical and subcortical zones induced by brain tumors alter the functioning of higher regulatory centers. These bioelectric changes in intricately connected brain functional zones lead to misbalances in compensatory mechanisms in turn influencing unfavorable disease outcome.

Objective. This study is aimed at analyzing electroencephalographic (EEG) spectral peculiarities of as well as the peculiarities of cardiac rhythm variability in patients with epilepsy associated brain tumors.

Materials and Methods. 72 patients participated in the survey aged from 22 to 83 years, divided into 2 groups: Brain tumor patients with epileptic seizures and brain tumor patients without seizures. The patients included in the study were those admitted to the neurosurgical department at Ryazan state regional hospital. Statistical, geometric and spectral parameters of heart rate variability as well as coherence and cross-correlational EEG analyses used to access the functional states of the patients.

Results. The study revealed sympathetic predominance of the autonomic nervous system in patients with epilepsy associated brain tumors as opposed to parasympathetic predominance in non epilepsy associated brain tumors indicating significant stress to regulatory systems and depletion of subcortical reserves of the cardiovascular system, further more stretching the adaptation limits, ultimately leading to depression of autonomic regulatory mechanisms and predominance of central mechanisms of regulation and adaptation.

Conclusion. Frequency-amplitude analysis of electroencephalogram and cardiointervalogram allow us to assess the degree of distress of different cortical and subcortical regulatory systems in patients with brain tumors, and is recommended as an additional method of investigation in these patients.

Mental disorders persistent identified in patients with epilepsy as significant personal and affective disorders, especially depressive and anxiety spectrum observed in the interictal period of the disease.

Purpose. Study the frequency of nonpsychotic mental disorders in patients with epilepsy; highlight the clinical features of non-psychotic affective disorders and to analyze the relationship of different variants affective disorders with symptoms of pharmacological resistance.

Material and methods. The study involved 160 patients with epilepsy: 80 patients with drug-resistant epilepsy (DRE), 80 patients with controlled epilepsy using a battery of psychometric scales.

Results. The variety of depressive and anxious psychiatric condition detected in 59.4% of patients with epilepsy, in 86.3% of patients with DRE, in 32.5% – with controlled epilepsy. Clinical and psychopathological analysis of non-psychotic mental disorders showed that in patients with DRE have statistically more frequent affective disorders, also a great “brutality”, “structural complexity” and the depth of depressive and anxiety disorders than in patients controlled epilepsy. We established that patients with affective symptoms have earlier onset and a longer course of disease.

Conclusion. The therapeutic resistance of patients with epilepsy accompanied by the presence of various non-psychotic affective mental disorders represented, mainly, depressive and anxiety psychopathological conditions. In terms of clinical and psychopathological a characteristics they are close to an endogenous affective mental disorders and, essentially, represent their “organic model.”

Limbic encephalitis (LE) may be manifested by non-convulsive status epilepticus (NCSE). Two clinical cases of LE presenting with NCSE, one of them with a background ovarian theratoma, and the value of electroencephalographic triphasic and delta-beta waves in diagnosis of LE, are discussed in this paper. Course of glucocorticoid pulse-therapy, complimented by long-term regiment of valproic acid, is shown as a successful treatment modality for LE with NCSV.

Differential diagnosis of epileptic seizures with non epileptic paroxysmal events often have serious difficulties. The most various states which are observed at somatic, neurologic, psychiatric disorders and even at healthy children imitate an epilepsy.

Objective. Evaluation of reliability of the diagnosis “epilepsy” in patients referred to the epilepsy center.

Material and methods. 2015 patients referred to our epilepsy center for evaluation and treatment for 3-year period. Age from 0 to 21 years. Methods of diagnosis: clinical examination, video-EEG monitoring (VEEG) of 6 to 120 hours, brain MRI.

Results. The diagnosis of “epilepsy” was reliably confirmed in 1552 patients (77%). Among these, 101 (6,5%) patients were diagnosed late (later than 6 months from the onset with seizures frequency more often than 1 time per month). At 463 (23%) patients overdiagnosis has been established – the diagnosis epilepsy with the current seizures was established unreasonably. Among them we have identified the following 3 groups: healthy children – 57 patients (12.3%); somatic and neurological disorders – 323 patients (70%); psychogenic non-epileptic seizures (PNES) – 83 patients (18%). The most frequently seizures was imitated by hyperkinetic movement disorders – 40.4% (187 patients), including paroxysmal dyskinesia, hyperkinetic eye movements, pathological nonepileptic myoclonus, jitteriness, shuddering attacks, benign neonatal sleep myoclonus, tics, hyperekplexia, benign myoclonus of early infancy, benign paroxysmal torticollis, spasmus nutans. Among the frequent reasons of over-diagnosis of epilepsy were also parasomnias and PNES.

Conclusions. The most frequent diagnostic errors were linked to the lack of objective information about the nature of paroxysms and misinterpretation of the EEG. VEEG is the most valuable tool in the differential diagnosis of epilepsy

The article presents modern data on the use of perampanel in antiepileptic drug therapy resistant epilepsy in routine clinical practice in adolescents. Despite the well-developed diagnostics and treatment of epilepsy system, about onethird of patients with newly diagnosed disease is resistent to medical therapy. When you select a combination of AEDs for children and adolescents should be borne in mind that school performance can largely predetermine their future. In this regard, the most feared cause such adverse events (AEs) as cognitive, neuropsychological, psychiatric and behavioral disorders. In the review perampanel demonstrates efficacy and safety, including among adolescents. There were shown the prospects of the usage of perampanel as add-on therapy of focal seizures with or without secondary generalization and a clinical case of application perampanel a patient with refractory epilepsy in adolescence.

Often, stroke, regardless of their type, are complicated by epileptic seizures, in different periods of the disease – both in the acute period, and in the late period of the disease.

Objective – to identify the characteristics of symptomatic epilepsy, occurred against the backdrop of stroke in children.

Materials and Methods. The study involved 94 children aged from 3 months to 18 years with the effects of stroke and 21 children with acute stroke. A brain MRI, routine EEG, clinical tests.

Results. Among infants prevailed hemorrhagic stroke, in most cases due to the DIC syndrome. Among older children, prevailed children with ischemic stroke, the most common cause of which were congenital and acquired heart defects. In 80.9% of cases were noted early seizures in the acute phase of stroke, more common in children with hemorrhagic stroke. In the acute phase of the disease seizures were mostly secondary generalized to partial component (68; 73.1%). On brain MRI in patients with ischemic stroke lesions localized cortical and subcortical. Late seizures were observed in 48 (41.7%) patients in the MRI of the brain of these patients had cystic atrophic changes in the brain in patients with primary generalized seizures subcortical structures of the brain were involved in the process.

Conclusions. Hemorrhagic strokes are more common in children under 1 year of age in subsequent periods prevalent ischemic strokes and nature of seizures depends on the location and massive fireplace.

Perinatal hypoxic-ischemic encephalopathy (HIE) is a one of common cause of damage to neonatal CNS. Electroencephalography (EEG) has diagnostic value for choice of therapeutic interventions and predicting of outcomes. Sarnat classification is used EEG findings. EEG-monitoring and amplitude-integrated EEG could be used in Intensive Care Unit to provide information about the prognosis for the infant. Comprehensive assessment based on EEG data and serum determination of biochemical markers of brain damage could be feasible for identifying risk of subsequent brain damage that might benefit for treatment and predict of outcomes after HIE. Further studies in this area are clearly needed.

The types of anaesthesia care for patients with drug-resistant epilepsy during stereotaxic operations for epilepsy pathways have been analyzed.

Objective: to assess the adequacy of anesthesia and optimal values of EEG for total intravenous anesthesia based of Propofol, Midazolam and Fentanyl in stereotaxic operations for epilepsy.

Materials and Methods. We enrolled 60 patients aged 17 to 35 years old. The study group included 40 patients with drug-resistant epilepsy while the control group consisted of 20 patients with brain tumors and epileptic seizures.

Results. Comparative analysis of systemic hemodynamic parameters and adequacy of anaesthesia regimen revealed that total intravenous anaesthesia based on Propofol, Midazolam and Fentanyl at stage 3 on EEG by Courtin as an adequate protection from surgical stress decreasing the risk of intraoperative kindling without new foci of epileptic activity.

Conclusion. The selected method of anesthesia provides electrophysiological identification of the focus of paroxysmal activity. It has been noted that a cooperation between a surgeon, an anesthesiologist and a neurophysiologist regarding stereotaxic operations for epilepsy is of great importance for successful operation.

Objective: to generalize the Russian experience with perampanel (PER) in routine clinical practice; to do this, the results of its use as an adjuvant partial epilepsy medication were retrospectively assessed. The study is still in progress now; therefore, the paper gives its preliminary results.

Materials and methods. The investigation included 52 patients with drug-resistant partial epilepsy. Their mean age was 28.92±14.02 years (a small number of the patients had not attained the age of 12 years); the proportion of men was 56%; the disease duration was over 10 years (69.2%); symptomatic epilepsy was in 76.9% with an epileptic focus being in the frontal (46.2%) and temporal (44.2%) regions. PER was prescribed to the majority (71.2%) of patients after three previous therapy lines. The baseline monthly rates of all types of seizures were 127.29±82.29; those of generalized seizures were 6.72±1.90.

Results. After addition of PER to therapy just within the first month, there was a significant reduction in the frequency of all types of seizures to 52.06±29.26 per month (Sign test; p=0.00001) and in that of secondary generalized seizures to 3.71±1.71 (Sign test; p=0.00001). The duration of PER administration was more than 6 months in the overwhelming majority of cases. In 58% of the patients, the frequency of seizures decreased by more than 50% (respondents). The lack of all types of seizures was noted in 8%; that of only secondary generalized seizures was in 31%. Adverse events were observed in 30.1% of the patients (aggression in 11.5% and somnolence in 9.6%; others were seen more rarely). The dose of PER was decreased because of side effects in 7 (13.5%) patients; the drug was discontinued in 4 (7.7%). The mean dose of PER for adults was as high as 6 mg.

Syndromes with continuous spikes and waves during sleep (CSWS) are being actively studied now. Etiological factors include both genetic causes and structural brain disorders; frequently – the damage of the thalamus, and congenital malformations. In most cases, the persistence of CSWS pattern leads to a neurocognitive dysfunction. Two mechanisms of the intellectual regression are supposed: a violation of brain maturation processes and the effect of the remote inhibition. The most effective treatment is considered to be a combination of hormones and antiepileptic drugs. It is often possible to restore a normal level of child development in case of the early diagnosis and rational therapeutic tactics. However, if more than 18 months has passed since the onset of seizures, as a rule, intellectual functions can’t be improved.