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The OBJECTIVE was to analyze the results of endoscopic transsphenoidal removal of neoplasms of the chiasmalsellar region in children.MATERIALS AND METHODS. We studied 23 patients aged 3 to 17 years who underwent endoscopic transsphenoidal removal of neoplasms of the chiasmal-sellar region. Neurological, ophthalmological, endocrine disorders and otorhinolaryngological pathology were evaluated. Neuroimaging data (MRI and CT), results of laboratory tests were studied. In addition, the analysis of surgical treatment including intraoperative and early postoperative complications was performed.RESULTS. In 39.1 % of cases, neoplasms of supraparainfracellar localization were diagnosed. In 34.8 % of patients, the size of the neoplasms did not exceed 2 cm. In 1 patient, the volume of neoplasm was 53.7 cm3. According to the type of pneumatization, the sellar type prevailed. Сonch type was observed in 1 child. Hemostatic material «Surgicel Fibrillar» was used more often (86.9 %). Plastic surgery of the skull defect was carried out on a floor-by-floor basis with «Tachocomb» plates (62.5 %). Total tumor removal was achieved in 75 % of children. The majority of neoplasms were represented by craniopharyngioma – 30.43 %. In the early postoperative period, there was a significant regression of neurological disorders – by 69.6 %, as well as ophthalmological – by 34.8 % and endocrine – by 37.8 %. There were no cases of nasal liquorrhea and epistaxis in children.CONCLUSION. Our study demonstrated the success of removing neoplasms in children from 3 years old, regardless of the size of the nasal cavity and pneumatization of the sphenoid sinus. It is possible to use endoscopic transsphenoidal access in children to remove the neoplasms of the chiasmal-sellar region of more than 5 cm, since the risk of intra – and postoperative complications is minimized.

The number of diseases requiring continuous neurological monitoring of the main parameters of the craniospinal system increases year on year. Retrospective analysis of results from monitoring intracranial pressure supported the development of an original technology for evaluating the magnitude and dynamics of craniospinal compliance in pathology of cerebrospinal fluid circulation, including a means for quantitative assessment of craniospinal compliance from the amplitude of pulsatile oscillations in intracranial pressure and instrumented implementation of the method in the form of a technically perfected medical device, i.e., the Neurocom craniospinal compliance monitor, as well as a means of applying the technology to different purposes in neurosurgery and neuroresuscitation.

The paper addresses the relatively rare inherited neurodermal disorder – Sturge-Weber syndrome that can manifest in epileptic seizures. We describe updated concepts, epidemiology, etiology, pathogenesis, clinical manifestations, and surgical treatment of the disease. We examined medical records of 21 patients (aged from 1 to 11 years) with Sturge-Weber syndrome treated over the period of 1996-2016. After surgical treatment of 10 patients (five cases with hemispheretomy and five – with multifocal resection), positive outcomes (Engel class I, II) were found in 70% of cases, and negative (Engel class III, IV) – in 20% of cases. Оne child suddenly died during epileptic seizures. In non-operated children (age from 2 to 5 years) under our observation, an improvement was noted in six cases, no changes – in three cases, and a further progression of the disease – in three cases. In this article, we analyze two of these cases in detail.

Pharmacotherapy is the first-line treatment modality for epilepsy. However, in 20-40% of patients, epilepsy is resistant to pharmacotherapy. These numbers have not changed for decades despite the development and use of antiepileptic drugs with novel mechanisms of action. Drug-resistant epilepsy is now considered a separate pathophysiologic and clinical entity. The existing hypotheses on its pathogenesis could be divided in two groups. Firstly, drug-resistance might be caused by an abnormal pharmacokinetics or pharmacodynamics of antiepileptic drugs as a result of congenital or acquired dysfunction of the transporter or receptor proteins. Secondly, it might be a consequence of inherent features of epilepsy per se, such as the so-called “intrinsic severity” or some disorder of the connectome. Taking into account the complexity of this phenomenon, the issue of drug resistance continues to remain in the focus of the current research efforts.

About 30% of patients with temporal epilepsy are resistant to antiepileptic drugs; in such cases, surgical treatment is considered to be the best option. Existence of an extra-temporal epileptogenic focus in the insula is a possible cause of seizures after temporal lobe surgery in about 20% of patients. Because of its localization, the anatomical features of the insular lobe, as well as the entire concept of insular epilepsy have not been well documented. Yet, it has been established that the seizures developing from the insular cortex can mimic the paroxysms arising from the temporal lobe. When the insula is involved in the epileptic process, resection of the insular cortex improves the outcome of surgical treatment. Rational and well-based selection of candidates for surgical treatment is of paramount importance for patients with drug-resistant epilepsy. The article presents a review of the up-to-date literature on this matter.

Complexity of epilepsy and a seizure as clinical and pathophysiological entities is reflected in a large number of experimental models of epilepsy. They could be stratified into acute and chronic, chemical and electric. The present review describes some most typical non-genetic models of different kinds used on laboratory rats. Also, the results of application of these models for research of vagus nerve stimulation as a novel treatment of drug resistant epilepsy are summarized.

Objective. To identify predictive factors of favourable outcomes in children with drug resistant epilepsy (DRE) associated with structural brain changes, who received surgical treatment. Methods. We performed a retrospective analysis of surgical treatment in 239 children with drug resistant epilepsy treated at the Pediatric Neurosurgery Department of the A. L. Polenov Russian Neurosurgical Institute from 2004 to 2014. The age of the patients varied between 2 and 18 years. The follow-up time ranged from 1 year to 10 years (on average 4.3 years). Results. According to magnetic resonance imaging (MRI), 223 (93.3%) patients had different structural brain changes. Among the patients, 47 (21.1%) had cerebral cicatrix, gliosis, cysts and atrophic changes, 33 (14.8%) had malformations of the cortical development, 43 (19.3%) had benign brain tumors, 47 (21.1%) had vascular malformations, 21 (9.4%) had arachnoid cysts, 12 (5.4%) had hippocampal sclerosis, 11 (4.9%) had phacomatosis, 5 (2.2%) had Rasmussen’s encephalitis, and 4 (1.8%) had hypothalamic hamartoma. Positron emission tomography revealed the areas of gray matter hypometabolism in the brain, which correspond to the areas of a metabolic deficiency around the epileptic focus. Favorable long-term outcomes of epilepsy surgery (class I according to Engel scale) were registered in 67.6% of the cases. The most favorable results (class I according to Engel scale) were achieved after lesionectomy (81.9%), temporal resections (78.4%), and hemispherectomy (72.7%). Conclusions. The most important predictive factors of favorable outcomes after epilepsy surgery are as follows: detection of epileptogenic morphological changes of the brain, completeness of resection of an epileptic and epileptogenic areas, and absense of seizures in the early postoperative period.

To determine the clinical features of the course of the disease when the insular lobe is involved in the epileptic process.A comparative analysis of the results of diagnosis and treatment of 55 patients with temporal lobe epilepsy and 46 patients with temporal plus epilepsy was carried out. The results of neuroimaging, clinical and EEG studies were compared.Autonomic paroxysms of nausea and hypersalivation, skin changes, simple motor seizures, emotional seizure and sensory paroxysms are the most reliable signs of the involvement of the insular lobe in the epileptic process in temporal plus epilepsy. For 'pure' temporal lobe epilepsy, cognitive paroxysms of the déjà vu and aura are common. When the insular lobe is involved in the epileptic process, the seizures have a reliably high frequency, polymorphism and are more complex by structure.Paroxysmal syndrome in temporal plus epilepsy with involvement of the insular lobe undergoes significant changes in the form of increased seizures and the appearance of specific seizures and characteristic polymorphism.Определение клинических особенностей течения височной эпилепсии при вовлечении в эпилептический процесс островковой доли.Проведен сравнительный анализ результатов диагностики и лечения 55 больных височной эпилепсией и 46 больных височной-плюс эпилепсией. Сравнивались результаты нейровизуализационных, клинических и электроэнцефалографических исследований.Было установлено, что вегетативные пароксизмы в виде тошноты и гиперсаливации, кожные проявления, простые моторные приступы, эмоциональные кризы и сенсорные пароксизмы являются наиболее достоверными признаками вовлечения островковой доли в эпилептичесий процесс при височной-плюс эпилепсии. Для височной эпилепсии характерны когнитивные пароксизмы по типу déjà vu и наличие ауры в составе приступа. При вовлечении в эпилептический процесс островковой доли приступы имеют достоверно более высокую частоту, полиморфизм и более сложную структуру.Пароксизмальный синдром при височной-плюс эпилепсии с вовлечением островковой доли претерпевает существенные изменения в виде учащения приступов и появления специфических приступов.