Search

Objective: analysis of treatment tactics for patients with magnetic-resonance-imaging (MRI) negative epilepsy, characteristics of morphological data and assessment of the treatment results.Material and methods. Within the period of 2013–2021, 31 patients under the age of 18 with drug-resistant nonlesional epilepsy were examined according to the “epilepsy” MRI-protocol. Patient distribution by gender, age, duration of illness, age of onset, number of anticonvulsant protocols, frequency of seizures at the time of initiated treatment, side and lobe of the seizure generation zone was analyzed. The instrumental diagnostic methods were noted in a specially designed study form and reported contribution of relevant result to epileptogenic zone. The distribution of patients according to surgical intervention and histological data was also analyzed.Results. Resection surgery was carried out in 26 (83.9%) patients. Palliative operations were performed in 5 (16.1%) cases with bilateral localization of the epileptic focus: callosotomy (3) and vagal nerve stimulation (2). Twelve months post-therapy outcomes by Engel class I were found in 19 (61.3%) patients, class II – in 6 (19.4%), class III – in 5 (16.1%), and class IV – in 1 (3.2%). Long-term results of surgical treatment were assessed 3 and 5 years later.Conclusion. Surgery of MRI-negative epilepsy can be effective with seizure-free outcome in 61.3% of cases, but requires use of additional diagnostic methods in relation to standard pre-surgery examination to localize epileptogenic zone.

Background. Neuronal and mixed neuronal-glial tumors are rare brain tumors that are most commonly observed in pediatric patients. The main clinical manifestations are presented by epilepsy substantially affecting child’s physical and neurocognitive development.Objective: to assess short-term and long-term changes in seizure frequency and neurocognitive status in pediatric patients who underwent surgery for mixed neuronal-glial tumors.Material and methods. Thirty-eight pediatric patients underwent surgery for mixed neuronal-glial tumors at the Almazov Research Medical Centre within the period from 2008 to 2018. All patients had comprehensive diagnostic examination including clinical neurological examination, neuroimaging tests, electroencephalography and electrocorticography before and after surgery. The postoperative changes in the seizures rate were assessed by using the 1993, 1996 Engel classification. Neurocognitive status was evaluated using The Child Behavior Checklist (a component of the Achenbach System of Empirically Based Assessment).Results. During the postoperative period, 84% of patients were found to be seizure-free; 42.6% demonstrated no clinically relevant behavioral disturbances or social adaption issues after the surgery. However, 76% of children experienced poor concentration. In a long-term perspective, seizures reoccured in 25% of patients and were not associated with any magnetic resonance signs of tumor progression.Conclusion. A gross total resection of the brain tumor was the main factor that affected changes over time in seizure rate. Assessment of long-term changes over time has shown that the patients tended to retain concentration difficulties, which should be taken into account while planning school education programs.

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.

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.

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.

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.

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.