[Supratentorial neuroepithelial tumor with PLAGL1 gene fusion - a new type of morphologically variable pediatric brain neoplasm defined by a distinct DNA methylation class. A case report and literature review]. / Supratentorial'naya neiroepitelial'naya opukhol' so sliyaniem gena PLAGL1 u detei ­ novyi tip opukholi mozga s raznoobraznoi morfologicheskoi kartinoi i otlichitel'nym metilyatsionnym profilem(sluchai iz praktiki i obzor literatury).

BACKGROUND: Methylation analysis has become a powerful diagnostic tool in modern neurooncology. This technique is valuable to diagnose new brain tumor types. OBJECTIVE: To describe the MRI and histological pattern of neuroepithelial tumor with PLAGL1 gene fusion. MATERIAL AND METHODS: We present a 6-year-old patient with small right frontal intraaxial tumor causing drug resistant epilepsy. Despite indolent preoperative clinical course and MRI features suggesting glioneuronal tumor, histological evaluation revealed characteristics of high-grade glioma, ependymoma and neuroblastoma. RESULTS: Methylation analysis of tumor DNA confirmed a new type of a recently discovered neoplasm - neuroepithelial tumor with PLAGL1 fusion (NET PLAGL1). PCR confirmed fusion of PLAGL1 and EWSR1 genes. No seizures were observed throughout the follow-up period. There was no tumor relapse a year after surgery. CONCLUSION: Methylation analysis in neurooncology is essential for unclear tumor morphology or divergence between histological and clinical data. In our case, this technique confirmed benign nature of tumor, and we preferred follow-up without unnecessary adjuvant treatment.

[Surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele]. / Khirurgicheskoe lechenie patsientov so strukturnoi fokal'noi farmakorezistentnoi epilepsiei, assotsiirovannoi s visochnymi entsefalotsele.

BACKGROUND: In recent years, temporal lobe encephalocele has become more common in patients with focal drug-resistant epilepsy. Despite available experience, there are still no clear recommendations for choosing the extent of surgery in these patients. OBJECTIVE: To evaluate the effectiveness of diagnosis and surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele. MATERIAL AND METHODS: The study included 21 patients with focal temporal lobe epilepsy and temporal lobe encephalocele. All patients underwent continuous video-EEG monitoring and MRI of the brain. There were 12 (57.4%) selective encephalocele resections and 9 (42.6%) anterior temporal lobectomies. The median follow-up period was 31 months. RESULTS: The overall effectiveness of surgical treatment with postoperative Engel class I was 76% (16 cases). Selective encephalocele resection was followed by postoperative Engel class I in 10 patients (83%). There were 6 (67%) patients with similar outcomes after temporal lobectomy. Mean volume of resected tissue adjacent to encephalocele was 8.3 cm3. CONCLUSION: Surgery is a highly effective treatment for patients with epileptic seizures following temporal lobe encephalocele. In our sample, favorable postoperative outcomes were achieved in 76% of patients (Engel class I). There were no significant differences in effectiveness between selective resection and temporal lobectomy. Further research is necessary for a clear protocol of surgical treatment of focal drug-resistant epilepsy associated with encephalocele.

[Crossed aphasia in neurosurgical practice: case report and literature review]. / Perekrestnaya afaziya (Crossed aphasia) v neirokhirurgicheskoi praktike: opisanie sluchaev i obzor literatury.

BACKGROUND: Crossed aphasia in dexstral (CAD) is an extremely rare disorder. To date, about 200 cases have been described in the literature. MATERIAL AND METHODS: The authors report 4 cases of CAD. Luria's neuropsychological examination was carried out. Functional asymmetry in the arm was analyzed using the Annett questionnaire. MRI and intraoperative findings confirmed localization of lesion in the right hemisphere in all patients. RESULTS: A 59-year-old right-handed man developed frontotemporal aphasia after previous resection of right-sided glioblastoma of fronto-insular-temporal region. In a 31-year-old right-handed man, crossed aphasia occurred after extensive hemorrhage from arteriovenous malformation into the right frontotemporoparietal region. A 39-year-old right-handed man developed severe combined aphasia after resection of diffuse glioma of the right insular-temporal region. A 10-year-old right-handed boy developed aphasia with word forgetting after resection of a large tumor of the right temporal lobe. All patients had impaired dynamic praxis. Other cognitive functions were intact. CONCLUSION: The authors discuss possible combinations of functions in one hemisphere for both right-handers and left-handers and emphasize diverse types of functional asymmetries described in the literature. Different localizations of functions in right-handers and left-handers indicate that local zones with different methods of information processing (successive or simultaneous) can coexist in one hemisphere. Therefore, the functions requiring different processing of information (for example, speech with praxis or spatial functions) can develop in one hemisphere. Cognitive impairment depends not only on the type of functional asymmetry, but also on localization of lesion. Crossed aphasia in right-handers indicates the rarest normal type of functional brain asymmetry with localization of speech functions in the right hemisphere. Possible aphasia in right-handers following damage to homologues of speech zones in the right hemisphere can require additional examination of these patients.

[Application of intraoperative electromagnetic frameless navigation in transcranial and endoscopic neurosurgical interventions]. / Primenenie bezramnoi sistemy intraoperatsionnoi élektromagnitnoi navigatsii pri transkranial'nykh i éndoskopicheskikh neirokhirurgicheskikh vmeshatel'stvakh.

The paper summarizes the experience in using a system of electromagnetic intraoperative frameless navigation in various neurosurgical pathologies of the brain. The electromagnetic navigation technique was used for 102 operations in 98 patients, including 36 transnasal endoscopic interventions. There were no intraoprtative and postoperative complications associated with the use of the system. In the process of using the system, factors influencing the accuracy of navigation and requiring additional control by the surgeon were identified. PURPOSE: The study purpose was to evaluate the use of electromagnetic navigation in surgical treatment of patients with various brain lesions. MATERIAL AND METHODS: The system of electromagnetic navigation was used for 102 operations in 98 patients (42 males and 56 females, including 18 children; median age, 34.8 years (min, 2.2 years; max, 69 years)) in the period from December 2012 to December 2016. In 36 patients, the system was used for endoscopic interventions. In 19 patients, electromagnetic navigation was used in combination with neurophysiological monitoring. RESULTS: In our series of cases, the frameless electromagnetic navigation system was used in 66 transcranial operations. The mean error of navigation was 1.9±0.5 mm. In 5 cases, we used the data of preoperative functional MRI (fMRI) and tractography for navigation. At the same time, in all 7 operations with simultaneous direct stimulation of the cortex, there was interference and significant high-frequency noise, which distorted the electrophysiological data. A navigation error of more than 3 mm was associated with the use of neuroimaging data with an increment of more than 3 mm, image artifacts from the head locks, high rate of patient registration, inconsequence of touching points on the patient's head, and unsatisfactory fixation to the skin or subsequent displacement of a non-invasive localizer of the patient. In none of the cases, there was a significant effect of standard metal surgical tools (clamps, tweezers, aspirators) located near the patient's head on the navigation system. In two cases, the use of massive retractors located near the patient's localizer caused noise in the localizer and navigation errors of more than 10 mm due to significant distortions of the electromagnetic field. Thirty-six transnasal endoscopic interventions were performed using the electromagnetic frameless navigation system. The mean navigation error was 2.5±0.8 mm. CONCLUSION: In general, electromagnetic navigation is an accurate, safe, and effective technique that can be used in surgical treatment of patients with various brain lesions. The mean navigation error in our series of cases was 1.9±0.5 mm for transcranial surgery and 2.5±0.8 mm for endoscopic surgery. Electromagnetic navigation can be used for different, both transcranial and endoscopic, neurosurgical interventions. Electromagnetic navigation is most convenient for interventions that do not require fixation of the patient's head, in particular for CSF shunting procedures, drainage of various space-occupying lesions (cysts, hematomas, and abscesses), and optimization of the size and selection of options for craniotomy. In repeated interventions, disruption of the normal anatomical relationships and landmarks necessitates application of neuronavigation systems in almost mandatory manner. The use of electromagnetic navigation does not limit application of the entire range of necessary intraoperative neurophysiological examinations at appropriate surgical stages. Succession in application of neuronavigation should be used to get adequate test results.

[Amygdalohippocampectomy in treatment of epilepsy in patients with temporal lobe cavernomas].

BACKGROUND: In some cases, single-stage or delayed amygdalohippocampectomy (AHE) can be used for effective treatment of epileptic syndrome upon resection of temporal lobe cavernomas. The efficacy of AHE in treatment of temporal epilepsies is proved in general; however, the indications for surgery in patients with cavernomas are not developed. OBJECTIVE: The study objective was to evaluate the efficacy and safety AHE in the treatment of epilepsy in patients with temporal lobe cavernomas and to define the indications for surgery. MATERIAL AND METHODS: Of 14 patients with temporal lobe cavernomas, which manifested as epileptic seizures, 10 patients underwent selective AHE, and 4 patients underwent anteromedial temporal lobectomy. In 12 cases, AHE was performed simultaneously with cavernoma resection. Delayed AHE was carried out in 2 cases. All patients underwent preoperative MRI and EEG. Preoperative video-EEG monitoring was performed in 3 cases. The duration of postoperative follow-up was at least 1 year (mean follow-up was 3.3 years). RESULTS: Improvement in the epileptic syndrome was observed in all patients. In the postoperative period, 7 patients had no seizures (Engel class IA); of them, 3 patients discontinued anticonvulsants. The surgery outcome depended on the disease duration. Significant postoperative complications in the form of reversible hemiparesis were observed in 1 case. CONCLUSION: Amygdalohippocampectomy is highly efficient in treatment of severe forms of epilepsy in patients with temporal lobe cavernomas. In the case of long history of typical temporal seizures and pharmacoresistant epilepsy, AHE can be performed simultaneously with cavernoma resection.

[Hippocampal sclerosis: pathogenesis, clinical features, diagnosis, and treatment]. / Skleroz gippokampa: patogenez, klinika, diagnostika, lechenie.

Hippocampal sclerosis, also known as Ammon horn sclerosis or mesial temporal sclerosis, is usually associated with intractable epilepsy and characterized by specific patterns of neuronal loss and gliosis in the medial temporal lobe structures. Hippocampal sclerosis manifests clinically as epilepsy, often intractable epilepsy; in most cases, this condition is surgically treatable. As the most common histological diagnosis in adult patients subjected to epilepsy surgery, hippocampal sclerosis is characterized by complex pathogenesis and requires a multidisciplinary approach to its diagnosis and treatment. This article reviews the pathologic features, natural history, pathogenesis, and electroclinical and MRI signs of hippocampal sclerosis.

[Supracerebellar transtentorial approach to tumors of the posterior portions of the medial temporal region].

OBJECTIVE: Despite the advances in microsurgery, the choice of the most adequate approach to the posterior part of the medial temporal region (MTR) remains a very controversial issue. The supracerebellar transtentorial approach (STA) is considered as the most preferable one, since it provides the optimal balance between retraction, incision, and resection of the brain tissue. Here, we present our consecutive series of 20 patients who underwent STA surgery. MATERIAL AND METHODS: Twenty patients with glial tumors affecting the posterior MTR underwent STA surgery between 2006 and 2014. The mean age of the patients was 20 years. Benign tumors were predominant (18 out of 20 cases). RESULTS: Resection of the posterior and middle MTRs was conducted in 16 cases. The anterior MTR was accessed through STA in 1 patient only; in 2 patients, STA was combined with the infraoccipital approach. Cerebellar edema occurred in 4 patients, with hemiparesis persisting in one of the cases for 1 year after surgery. Of 8 patients with drug resistant epilepsy, the Engel class 1 or 2 outcome was achieved in 6 cases within 1 year after surgery. CONCLUSION: STA provides an excellent surgical route to the posterior and middle MTR portions; however, the anterior MTR portions cannot be reached safely. The operative risks of STA increase as the surgeon proceeds with resection of the anterior MTR portions. Anterior MTR structures can be removed using a combination of the supracerebellar and infraoccipital transtentorial approaches or two-stage resection.

[Intraparenchymal brainstem schwannomas: report of three cases and literature review]. / Intraparenkhimatoznye shvannomy stvola mozga: opisanie trekh sluchaev i obzor literatury.

We report three patients with intraparenchymal brainstem schwannomas that were confirmed by surgery and pathological examination: tumors originated from the midbrain parenchyma, the dorsal aspect of the pontomedullary junction and the medulla oblongata respectively. Two of the presented patients differed in age of disease presentation which may reflect different mechanisms of tumor development according to the current views on origin of intraparenchymal schwannomas. One of the cases illustrates treatment of non-communicating hydrocephalus associated with ventricular tumors by preventive stenting of the cerebrospinal fluid pathways.

[Long-term epilepsy-associated tumors]. / Glioneironal'nye opukholi, assotsiirovannye s epilepsiei.

OBJECTIVE: The goal of this review is to summarize data concerning radiological and histological diagnosis of epileptogenic tumors, as well as to discuss their surgical treatment. MATERIAL AND METHODS: PubMed literature database was searched for relevant articles, we also used our own clinical experience. RESULTS: Benign glioneuronal tumors are the second most common cause of drug resistant epilepsy in adults after hippocampal sclerosis. Exceptionally slow growth (more than 2 years), localization in the brain cortex, presence of differentiated neuronal tissue in tumoral body and long history of epilepsy are the key features of these tumors called LEAT (long-term epilepsy-associated tumors). Management of LEAT requires epileptological as well as neurooncological approach. The epileptogenic zone commonly spreads beyond the tumor borders and simple lesionectomy alone may not be sufficient for achieving seizure freedom. CONCLUSION: LEAT typically exhibit low proliferative activity, however they should be thoroughly differentiated from more aggressive glial tumors; while this task is sometimes quite challenging, it's achievable by means of histological and immunohistochemical examination.

[Ventriculoperitoneal and ventriculoatrial shunts in treatment of patients with brain tumors].

Two 234 patients with tumor-associated hydrocephalus underwent their first shunt implantation at Burdenko Neurosurgery Institute between 2004 and 2008. Age of the patients ranged from 18 to 77 years (mean 44). The follow-up was available in 162 patients (72%). The median follow-up was 10 months. Shunt failure occurred in 29,2% of 162 patients. Kaplan-Mayer analysis showed that the probability of shunt failure free interval was 72, 69, 60% at one, two and three years after insertion, respectively. The most frequent cause of shunt failure was malposition of the ventricular catheter (30,6%), occlusion of the ventricular and abdominal catheter occurred in 20,4 and 22,2%, respectively. Shunt infection was encountered in 24,5% of cases. Among the analyzed factors (position of the catheter within the ventricle, additional operations such as tumor resection, implantation of external ventricular drain before shunt surgery, malignancy of the tumor and type of hydrocephalus) only presence of external ventricular drain increased the risk of shunt infection. The duration of external drainage positively correlated with the risk of shunt infection. In most cases shunt failure can be prevented. The results of the study may help the surgeon to choose the optimal treatment strategy for patient with tumor-associated hydrocephalus.