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Background: Neurosurgical interventions and trauma are common causes of damage to the optic nerve. This determines the relevance of research for solutions aimed at restoration of the nerve's anatomical integrity, electrical conductivity, and subsequently - restoration of its function. Restore a damaged (cut) optic nerve using n. suralis autograft in vivo., Methods: The experiment involved reconstruction of the optic nerve through injury modulation, graft placement and restored nerve harvest and evaluation. Injury modulation included removal of a fragment of the optic nerve. Autograft harvesting and placement involved resection of a fragment of the sural (sensory) nerve and its subsequent anastomosis in place of the removed fragment of the optic nerve. As an experimental model, a rabbit of the "Burgundy" breed was used. The animal was previously examined for the presence of infectious and other diseases to confirm its health., Results: Four months post operatively when stimulating the operated right eye, low-amplitude components altered in shape are registered. Thus, signs of mild restoration of electrical conductivity on the treated optic nerve were seen., Conclusions: Our initial experience shows the technical feasibility of reconstructing the optic nerve using an autograft, the possibility of axonal growth through the graft and, in the future, using this method for direct optic nerve reconstruction, as well as a bypass method for damage to the optic nerve with various tumor diseases of the optic nerve, tumors of the chiasmatic-sellar localization, orbital injuries., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Meningiomas arising from accessory nerve sheath without dural attachment are rare. To date, only 5 cases are described in the literature. A 53-year-old male presented with long history of occipital pain and headaches. Magnetic resonance imaging revealed a small intradural extramedullary contrast enhanced tumor at the level of foramen magnum. The patient underwent microsurgical resection through minimally invasive midline suboccipital approach. According to intraoperative findings, cystic tumor arose from the left accessory nerve without dural attachment. Gross total resection was achieved without damage to the nerve. Histological analysis revealed angiomatous meningioma. Postoperative period was uneventful without new neurological symptoms. Meningiomas can rarely arise from accessory nerve sheath and mimic schwannoma. These tumors may be totally resected without damage to accessory nerve using minimally invasive surgical approaches.

Dysembryoplastic neuroepithelial tumor (DNET) is a benign mixed neuronal-glial neoplasm (WHO grade 1). DNET is most often localized in temporal lobes and found in children and young people with epilepsy. There a few cases of DNET in ventricular system with dissemination along the ependyma in the world literature., Material and Methods: We present a rare case of T1- and T2-negative ventricular system tumor. Only FIESTA imaging revealed dissemination with multiple focal lesions of the third ventricle, its bottom and lateral walls, anterior horns of lateral ventricles, cerebellar vermis, cervical and lumbar spinal cord., Results: The patient underwent transcortical endoscopic biopsy of the third ventricle tumor with simultaneous ventriculoperitoneal shunting. DNET was diagnosed, and radiotherapy was subsequently performed. Literature data on this issue were analyzed., Conclusion: To date, disseminated forms of DNET are extremely rare. X-ray features and morphological results allow us to establish the correct diagnosis and determine further treatment strategy.

Focal cortical dysplasias are known to be the most frequent and furtive lesions leading to intractable epilepsy in children. Epilepsy surgery in central gyri, been effective in 60-70% of cases, is still significantly challenging due to the high risk of postoperative permanent neurological impairment., Study Aims: Assessment of the outcome after epilepsy surgery in children with FCD in central lobules., Material and Methods: Nine patients, median age 3.7 ys, IQR=5.7 ys (min 1.8- max 15.7 ys) with FCD in central gyri and DR-epilepsy underwent surgery. Standard preoperative evaluation included MRI and video-EEG. Invasive recordings were used in 2 cases, coupled by fMRI in 2. An ECOG and neuronavigation, as well as stimulation and mapping of primary motor cortex were used routinely during the procedure. Gross total resection was achieved in 7 patients according to postoperative MRI., Results and Conclusions: Six patients with new or worsening of already existing hemiparesis recovered within a year after surgery. At the last FU (med 5 ys) favorable outcome (Engel class IA) has been achieved in 6 cases (66.7%), while two patients with persisting seizures reported seizing less frequently (Engel II-III). Three patients were able to discontinue AED-treatment and four children resumed development with improvement in cognition and behavior.

Intramedullary abscesses of the spinal cord are the rarest form of infectious lesions of the central nervous system. We report surgical treatment of a patient with intramedullary cervical abscess caused by L. monocytogenes. Features of this case are absence of primary purulent focus and atypical infectious agent. The patient underwent surgical treatment for intramedullary cervical abscess and subsequent antimicrobial therapy. Subtotal regression of neurological symptoms was noted in early postoperative period. The authors emphasize strict collegial decision-making regarding diagnosis of this disease and choice of optimal treatment strategy.

The authors present an extremely rare case of metastatic brain lesion in a patient with gastrointestinal stromal tumor of the stomach. There are literature data on 23 cases of metastatic lesions of the brain, skull and soft tissues of the head in similar patients. Atypical localization of metastases can lead to some diagnostic difficulties, unreasonable cancellation of chemotherapy and delayed surgical treatment. A feature of our observation was postoperative coma determined by the features of the underlying disease.

Most children with tuberous sclerosis (TS) present with intractable seizures. Various factors including demography, clinical data and surgery option are mentioned to affect the outcome after epilepsy surgery in these cases., Objective: To evaluate some demographic and clinical variables probably related to seizure outcome., Material and Methods: Thirty-three children, median age 4.2 ys (7.5 mths-16 ys), with TS and DR-epilepsy underwent surgery. Within overall 38 procedures (redo surgery was needed in 5 cases), tuberectomy (with or without perituberal cortectomy) was performed in 21 cases, lobectomy - 8, callosotomy - 3, various disconnections (anterior frontal, TPO and hemispherotomy) - 6 patients. Standard preoperative evaluation included MRI and video-EEG. Invasive recordings were used in 8 cases, coupled by MEG and SISCOM SPECT in some cases. ECOG and neuronavigation were used routinely during tuberectomies, and stimulation and mapping were employed in cases with lesions overlapping or near to eloquent cortex. Surgical complications: wound CSF leak ( n =1) and hydrocephalus ( n =2) were noted in 7.5% of cases. Postoperative neurological deficit (most frequently hemiparesis) developed in 12 patients, being temporary in majority of them. At the last FU (med 5.4 ys) favorable outcome (Engel I) has been achieved in 18 cases (54%), while 7 patients (15%) with persisting seizures reported less common attacks and their milder form (Engel Ib-III). Six patients were able to discontinue AED-treatment and 15 children resumed development and markedly improved in cognition and behavior., Results and Conclusion: Among different variables potentially influencing the outcome after epilepsy surgery in cases with TS, the most important one is seizure type. If prevalent, focal type may be a biomarker of favorable outcomes and probability to become free of seizures.

Using the example of a recurrent tumor with a 10-year follow-up, the authors show that mutation of the IDH1/2 genes in astrocytomas is not always an early event in the pathogenesis of glioma, that in rare cases a 1p19q codeletion can be found in astrocytomas, and that IDH-mutant tumors can occur in childhood.

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.

Chondrosarcoma is a rare malignancy composed of transformed cells of cartilage. This cancer is characterized by slow growth. Almost 75% of intracranial chondrosarcomas are observed on the skull base and grow from bone synchondrosis. Other rarer localizations of tumor are cerebral falx, tentorium cerebelli, vascular plexuses of the ventricles, fourth ventricle, convexital surface of the brain, etc. In this manuscript, we report treatment of patient with falcine chondrosarcoma.

Surgery is an effective approach for drug-resistant temporal lobe epilepsy following hippocampal sclerosis. There is still no clear and unanimous opinion about advantages and disadvantages of certain surgical technique., Material and Methods: There were 103 surgical interventions in 101 patients. Females prevailed (1.45:1). Age of patients ranged from 16 to 56 years (median 28). Anteromedial temporal lobectomy and selective amygdaloghippocampectomy were performed in 49 (47.6%) and 54 (52.4%) patients, respectively. In the latter group, 30 patients were operated via a 14-mm burr hole-subtemporal approach. Postoperative outcomes were assessed using the Engel grading system. The follow-up period ranged from 2 to 8 years (median 4 years)., Results: By the 2 nd year, Engel class I was observed in 74 (72%) patients, Engel II, III and IV - in 20 (19.4%), 6 (5.8%) and 3 (2.9%) patients, respectively. Engel class I was achieved after anteromedial temporal lobectomy in 68% of cases, selective amygdaloghippocampectomy via standard approaches in 75% of cases, amygdaloghippocampectomy via subtemporal burr hole approach - in 80% of cases. Neurocognitive impairments after anteromedial lobectomy and selective amygdaloghippocampectomy were similar. At the same time, mental disorders de novo prevailed in the group of anteromedial lobectomy ( p <0.05). There were no severe visual field disorders after subtemporal burr-hole access. In other cases, these disorders occurred in 36.2% of patients ( p <0.05). There were 8 (7.8%) postoperative complications: 5 (10.2%) - after anterior temporal lobectomy, 3 (5.5%) - after selective surgeries via standard approaches. There were no complications after burr-hole surgery., Conclusion: Selective amygdaloghippocampectomy is not inferior to anteromedial lobectomy. Moreover, this procedure is associated with a lower risk of complications and adverse events.

Objective: To evaluate variables that may predict the outcome after hemispherotomy basing on a retrospective study of a large consecutive pediatric cohort of patients from a single institution., Material and Methods: One hundred and one patients with refractory seizures and variable decline in development ( n =78) underwent hemispherotomy (med. age - 43 months, med. epilepsy history - 30 months). Developmental pathology was the anatomical substrate of disorder in 42 patients, while the infantile post-stroke scarring and gliosis was its origin in the majority of 43 cases with acquired etiology. The progressive pathology (the Rasmussen encephalitis, Sturge-Weber angiomatosis and tuberous sclerosis) was the etiology in 16 children. Left-sided hemisphere was impaired in 54 cases; some contralateral anatomical and potentially epileptogenic MRI-abnormalities were noted also in «healthy» hemisphere in ¼ of all cases. Eight patients needed second surgery to complete sectioning of undercut commissural fibers. FU is known in 91 patients (med. - 1.5 years) and 73 of them were free of seizures (80.2%), but only 30 of 40 patients with FU > 2 years were still SF (75%). All but one of re-do hemispherotomies were successful. AED-treatment was discontinued in 46 cases and tapered in other 27 patients. Up to 90% of kids demonstrated some improvement in behavior and cognition., Results and Conclusion: Developmental pathology, infantile spasms and younger age onset of seizures are negative predictors for achievement of SF-status ( p <0.05). Neither bilateral epileptic EEG-signs, nor MRI-abnormalities in «healthy» hemisphere had any relation to outcome, but focal seizure onset was associated positively with further SF-status ( p = 0.03). Kids with multiple lobe unilateral CD do somewhat worse than their counterparts with hemimegalencephaly and acquired etiology. Post-hemispherotomy hemiparesis (either new or worsening of already existed one) has no relation either to the age at surgery, or to the age onset ( p = 0.41). Children with left-sided lesions were less successful in every neurodevelopmental domain except maintaining expressive language. Patients with relapse or persisting seizures have good chances to become SF by re-doing hemispherotomy and should be evaluated for the possibly incomplete hemispheric isolation.

Currently, cabergoline therapy is the main method of treatment with prolactin. The use of the drug in most cases leads to tumor regression, normalization of prolactin levels and restoration of gonadotropic function. The mechanism of its impact on tumor cells in vivo, which is dynamically traced in the same human tumor, is the case of considerable interest. We observed a 30-year-old patient who was operated on twice for a giant prolactinoma before and on treatment by cabergoline. The morphological study after the first surgery (before introducing of cabergoline therapy) revealed a prolactin-positive pituitary tumor with a Ki-67 labeling index of 8% and with strong expression of dopamine type 2 receptors (D2R), CD31 and CD34. After 4 months, during which the patient received cabergoline at a dose starting from 0.5 mg to 1.5 mg per week, a second transsphenoidal surgery was performed with subtotal removal of residual tumor tissue. During the morphological study of the second biopsy sample, the tumor retained a pronounced immunopositivity to prolactin and D2R, with a decrease in the labeling index Ki-67 to 2%, as well as a decrease in the expression of CD31 and CD34. Subsequent cabergoline therapyresulted in persistent normoprolactinemia, restoration of androgen (and reproductive) status, and no tumor recurrence over a 10-year period on cabergoline treatment. Thus, one of the mechanisms of effect of cabergoline that leads to tumor regression is a decrease in the proliferative index and angiogenesis of the tumor.

Objective: Surgery is the first-line treatment option in children with FCD and refractory epilepsy, but the rate of success and patient numbers who became free of seizures vary widely from series to series., Study Aims: To elicit variables affecting the outcome and predicting achievement of the long-term seizure-free status., Material and Methods: One hundred sixty-nine children with cortical dysplasia and DR-epilepsy underwent surgery Preoperative evaluation included prolonged video-EEG and MRI (in all patients) and neuropsychological testing when possible. Fourteen patients underwent invasive EEG, fMRI and MEG were used also in some cases. Including 27 repeat procedures the list of overall 196 surgeries performed consists of: cortectomy (lesionectomy with or without adjacent epileptogenic cortices) – in 116 cases; lobectomy – in 46; and various disconnective procedures – in 34 patients. Almost routinely employed intraoperative ECOG (134 surgeries) was combined with stimulation and/or SSEP in 47 cases to map eloquent cortex (with CST-tracking in some). A new permanent and not anticipated neurological deficit developed post-surgery in 5 cases (2,5%). Patients were follow-upped using video-EEG and MRI and FU which lasts more than 2 years (median – 3 years) is known in 56 cases. Thirty-two children were free of seizures at the last check (57,2% rate of Engel IA). A list of variables regarding patients’ demography, seizure type, lesion pathology and localization, and those related to surgery and its extent were evaluated to figure out anyone associated with favorable outcome., Results: Both Type II FCDs and their anatomically complete excision are positive predictors for favorable outcome and achievement of SF-status (p<0,05). Residual epileptic activity on immediate post-resection ECOG do not affect the outcome., Conclusion: Patients with Type II FCD, particularly with Type IIb malformations are the best candidates for curative surgery, including cases with lesions in brain eloquent areas. Kids with Type I FCD have much less chances to become free of seizures when attempting focal cortectomy. However, some of them with early onset catastrophic epilepsies may benefit from larger surgeries using lobectomy or various disconnections.

Neuroblastoma is a malignancy developing from the embryonic neuroblasts of sympathetic nervous system. Primary sellar neuroblastomas are extremely rare (there are currently only 11 case reports in the literature). Possible development of neuroblastoma in sellar region expands differential diagnosis of local processes due to inclusion of neuroblastoma into the spectrum of suspected tumors. We report a literature review and description of a patient with primary sellar neuroblastoma.

Introduction: Glioneuronal tumors (GNT) are usually found in children (less than 1.5% of all neoplasms of the brain). With rare exceptions, they are benign and usually manifest only by epilepsy, which is quite often resistant to treatment with AE drugs. Tumor removal usually helps to cope with epileptic seizures, however, a number of issues regarding diagnosis and surgical treatment (interpretation of morphological data and classification, epileptogenesis and topography of the epileptogenic zone, the value of intraoperative invasive EEG and the optimal volume of resection) remain debatable., Aim: To describe the morphology, electro-clinical picture and MR-semiology in patients with gloneuronal brain tumors, as well as to analyse the results of their surgical treatment and the factors determining its outcome., Material and Methods: 152 children with a median age of 8 years were treated surgically (There were 64 gangliogliomas, 73 DNT, 15 cases where the tumor classification failed - GNT NOS). In children under 2 years of age, temporal localization of the tumor prevailed. In 81 cases, ECoG was used during the operation. Surgical treatment complications: transient neurological deficit (in 15 cases); hematomas removed without consequences (in 2 cases), infectious (osteomyelitis of bone bone flap in 2 cases). We analyzed: the age of the epilepsy onset (median - 4 years 7 months) and its duration (median - 23.5 months), the type of seizures, as well as the features of MR-semiology and morphology of tumors and adjacent areas of the brain. The volume of tumor resection was verified by MRI (in 101 cases) and CT (in each case). The follow-up was collected through face-to-face meetings, with repeated video EEG and MRI, as well as telephone interviews. We studied the effect of a number of parameters characterizing the patient and features of his/her operation on the outcome of treatmen., Results: Among 102 patients in whom the follow-up history is one year or more (median - 2 years), a favorable outcome (Engel IA) was observed in 86 of them (84%); 55 of them (54%) at the time of the last examination stopped drug AE treatment. Radical tumor removal and younger age at the time of surgery were statistically significantly associated with a favorable result., Conclusion: In children with gloneuronal brain tumors, removal of the tumor is effective and relatively safe in the treatment of symptomatic epilepsy. Radical tumor resection and earlier intervention are the most important prerequisites for a favorable outcome and persistent remission of seizures.

Brain metastases of various types of cancer are diagnosed in 8-10% of all cancer patients. In the world literature, only 30 cases of cancer metastasis to the pituitary adenoma are described. This article presents yet another observation of a patient with breast cancer metastasis into the hormone-inactive pituitary adenoma at the Burdenko neurosurgical center, Russia The patient underwent endoscopic endonasal transsphenoid removal of the neoplasm. During microscopy and immunohistochemical studies of the biopsy, two types of tissue (pituitary adenoma and cancer metastasis) with different Ki-67 treated surgically (1% and over 40%) were found.

Background: Immunohistochemistry is a basic diagnostic technique. Immunohistochemical examination results reflect mainly qualitative and less quantitative characteristics of proteomic status of cells. A combined approach with complex quantitative evaluation of marker expression using colorimetric analysis and computer technologies can expand the diagnostic capabilities of immunohistochemistry. We studied such an approach developed by using expression of the proliferative marker Ki-67 in pituitary adenomas., Methods: A retrospective, blind, randomized, comparative study was performed of Ki-67 expression activity in pituitary adenomas using the traditional Ki-67 labeling index and a simple immunohistochemical cytocolorimetric analysis developed by us with immunohistochemical cytocolorimetric index (ICI) estimation as predictors of relapse, assessing the relationships of these indicators with the time before relapse., Results: Mean Ki-67 labeling index was 3.87% ± 0.29% in the relapse-free group and 4.01% ± 0.29% in the relapse group; the difference was not statistically significant. The average Ki-67 ICI was 24.16% ± 0.51% in the relapse-free group and 30.68% ± 0.64% in the relapse group; the difference was statistically significant. The correlation coefficient of ICI values and time before relapse was -0.302, indicating the presence of a weak negative correlation., Conclusions: We successfully tested an ICI estimation method developed by us to assess Ki-67 expression in pituitary adenomas. The ICI technique can be used both as a prognostic factor for relapse and, in combination with other modern proteomic and genetic methods, as the basis for creation of new multimodal analyzing systems for functional state assessment of cells and tissues., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Currently, there is no single standard defining what rhythmic activity should be considered as the area of ictal pattern onset. Differences in electroencephalographic patterns associated with different types of focal cortical dysplasias (FCDs) have not been defined. Aim - we aimed to identify features of the ictal onset pattern on a scalp EEG, depending on the histology, location, and extension of epileptogenic zone, as well as to elucidate the relationship between concordance of the interictal and ictal activity localization and ictal onset pattern types., Material and Methods: We studied scalp video-EEG monitoring data of 38 FCD patients who underwent surgical treatment for intractable epilepsy in the period between 2010 and 2016. We analyzed the data on interictal and ictal activity localization and compared them with the data on FCD types and lesion location., Results: Two types of the ictal onset pattern on EEG, local and generalized, were identified. The local and generalized types included two and four variants of the ictal onset pattern, respectively. Therefore, 6 combinations of rhythmic activity were identified, which manifested within the first 10 s after the onset of electrographic changes on EEG simultaneously with initial clinical manifestations of seizure. Co-localization of the interictal and ictal activity zones occurred in 42% of cases; of these, 88% of patients had the ictal onset pattern. In the remaining cases (58%), no co-localization of the interictal and ictal activity zones was detected; of these, 76% of patients were assigned to the group with the generalized ictal onset pattern. The local ictal onset pattern was more common in patients with type II FCD, while the generalized ictal onset pattern was more common in patients with type I and type III FCD. No correlation between the ictal onset pattern type and the lesion localization was found., Conclusion: We describe two ictal onset pattern types, local and generalized, on EEG in children with FCD. The co-localization of ictal and interictal activity zones prevails in the local ictal onset pattern group. The local ictal onset pattern is often associated with type II FCD. In the case of type I and type III FCD, the generalized ictal onset pattern predominates. There is no clear relationship between the ictal onset pattern type and the lesion location.

Objective: Analysis of factors affecting life expectancy at patients with primary malignant tumors of anterior and middle parts of the skull base with intracranial invasion., Material and Methods: 139 patients (47 women and 92 men) with primary malignant tumors of the anterior and middle parts of the skull base with intracranial invasion (stage T4 according to TNM classification or stage C according to Kadish classification for estesioneuroblast ) were treated at the NMRCN Burdenko for the period from 2004 till 2018. The study was conducted by the method of total sampling. The observations are divided into 2 groups: primarily operated (group I) and repeatedly operated (group II)., Results: The average age in both groups was 50 years. In most (64.7%) cases, the tumor affected the medial sections of the base of anterior and middle cranial fossae, and in 35.3% of cases it was localized laterally. All tumors were classified to T4 stage according to TNM classification or (9 olfactory neuroblastomas) to stage C according to Kadish classification., Discussion: The impact on life expectancy was largely provided by postoperative radiation therapy, the repeated nature of operation, and the presence of brain infiltration. In the total cohort of patients 5-year OS, 5-year RVS, 5-year-old IDF and 5-year LC were 50.7, 35, 54.2 and 36.4%, respectively. In group I, the medians OS and IDF were equal and amounted to 138.3 months. The median RVS was 43.8 months. 5-year OS equal to 63.6%, 5-year RVS - 40.8%, 5-year-IDF - 64.8%, 5-year LC was up to 65.7%. The survival rate in the analyzed cohort for 1, 2, 3 years was 81.4, 71.8 and 67.8%, respectively. In group II, the treatment results for the group of repeatedly treated patients were significantly worse. There were no cases of 5-year survival. The 1-, 2-, and 3-year survival rates were 59.3, 50.8 and 31.8%, respectively. The median OS was 27.1 months, IDF was 27.1 months, RVS was 18.2 months, and LC was 9.1 months., Conclusion: The results and analysis of literature justify the feasibility of surgical treatment of patients with malignant tumors of craniofacial localization at T4 stage. The purpose of surgical intervention should be: elimination of the immediate threat to the patient's life due to edema and dislocation of the brain; the maximum possible removal of tumor tissue (cytoreduction); if possible, the elimination of the most significant symptoms for the patient (pain, nasal breathing disorders, cosmetic defect). If there are special reserves, it is obligatory to include radiation and chemotherapy in the treatment process.

Sarcoidosis is a multisystem granulomatous disorder of unknown nature. Patients often present with pulmonary, skin, eye, and orbital lesions. Involvement of the central nervous system (CNS) is accompanied by granulomatous leptomeningitis and damage to the basal brain structures with formation of granulomas near the cranial nerves, hypothalamus, pituitary gland, cavernous sinuses, optic chiasm, and intracranial optic nerves. The optic nerves can be affected independently of the other CNS regions, which may be the first manifestation of the disease. The article presents two clinical cases of sarcoidosis affecting the anterior visual pathway. Diagnosis of the disease was associated with certain difficulties. A biopsy revealed a sarcoidosis lesion.

Objective: The purpose of the study is to present the results of successful treatment of a patient with traumatic arterial aneurysm of the middle cerebral artery (MCA)., Material and Methods: A clinical case of traumatic arterial aneurysm of the M4 segment of MCA was studied. A patient with a ruptured traumatic aneurysm was transferred to Krasnogorsk City Hospital #1 for further examination and emergency surgery., Results: On the sixth day after surgery, the patient was discharged in a satisfactory condition with almost complete regression of neurological symptoms., Conclusion: Treatment of traumatic arterial aneurysms requires an individual approach taking into account the past medical history, the clinical presentation, aneurysm location and anatomy, as well as the mechanism of injury. It is extremely difficult to suspect an arterial aneurysm after a patient had a traumatic brain injury as the incidence of this condition is very low. An angiographic study of cerebral vessels is needed for reliable diagnosis, not just solely performing standard computed tomography of the brain. Patients with this pathology require surgical intervention.

Oculomotor nerve neurinoma not associated with neurofibromatosis type II is an extremely rare pathology. According to the topography, cisternal, cisternocavernous, cavernous, orbitocavernous, and orbital tumor groups are distinguished. The clinical picture of the disease is characterized mainly by either oculomotor disorders or pyramidal symptoms, depending on the tumor localization. Neurinomas of the oculomotor nerve rarely occur without oculomotor disorders. However, in some patients with these tumors, the third nerve function remains intact. In this paper, we present clinical cases of two patients with oculomotor nerve neurinomas and analyze the relevant literature.

Introduction: McCune-Albright Syndrome is a rare genetic disease characterized by the formation of fibrous osteodysplasia foci of various localization, including the bones of skull base. Having a gross lesion of the main bone body and the simultaneous formation of the pituitary adenoma, its transnasal removal becomes very difficult., Material and Methods: Two clinical observations are presented where at patients with the classic manifestation of McCune-Albright syndrome we were able to successfully remove somatotropinomas with endoextrasellar growth. In both cases the presence of visual disorder was the indication for surgery., Results: Despite pronounced deviations in the anatomy of the skull base bones in both cases we managed to access the Turkish saddle. Removal of the pituitary tumor did not differ from standard surgery. Improved vision after surgery was observed in two patients. Tumors were removed subtotally and clinical and biochemical remission of acromegaly was not achieved. In both cases, the tumors had morphological signs of atypia. Patients continued to receive therapy with somatostatin analogues and radiation., Conclusion: The possibility of performing transnasal surgery in this category of patients using specific instruments and intraoperative navigation is shown.

The paper describes a clinical case of atypical teratoid/rhabdoid tumor with preserved INI1 expression and SMARCA4 gene mutations in an 8-month-old girl. Genome-wide DNA methylation, hierarchical clustering, and next-generation sequencing were used to make a tumor diagnosis. However, BRG1 immunohistochemical examination may be recommended in the routine practice of diagnosis and study of childhood CNS malignant tumors.

Fluorescence diagnostics has been extensively applied in surgery of malignant brain gliomas. However, the use of this technique in surgery of intracranial meningiomas has remained controversial., Objective: The study objective was to assess the sensitivity of 5-aminolevulinic acid-based (5-ALA) fluorescence diagnostics in surgery of brain meningiomas and to clarify the clinical and biological factors that may influence the fluorescent effect., Material and Methods: The study consistently included 101 patients with intracranial meningiomas of various locations who were operated on using 5-ALA. There were 28 (27.72%) males and 73 (72.27%) females (median age, 54 years). In all patients, surgery was performed using an operating microscope equipped with a fluorescent module; in 24 of these, laser spectroscopy was used. For comparison of chances to observe the fluorescent effect of 5-ALA in patients having meningiomas with different WHO histological grades (Grade I vs Grade II-III), we performed a meta-analysis that included 10 studies (the largest series) on outcomes of surgical treatment of meningiomas using intraoperative fluorescence diagnostics., Results: Of 101 patients included in this series, observable fluorescence was detected in 95 (94.1%) patients: weak fluorescence in 12 (11.9%), moderate fluorescence in 23 (22.8%) cases, and strong fluorescence in 60 (59.4%) patients. There was no statistically significant relationship (p>0.05) between the rate and intensity of observable fluorescence and the tumor growth pattern (primary/continued), location, WHO grade of malignancy, and histological subtype. In the absence of intraoperative bleeding, tumor fluorescence was statistically significantly brighter (p=0.02). Of 26 patients with hyperostosis, bone fluorescence was observed in 11 (42.3%) cases. There was no statistically significant relationship between administration of dexamethasone, its dose, administration of anticonvulsants, gastrointestinal tract diseases, as well as diabetes mellitus and the fluorescence intensity. There was also no significant relationship between the extent of tumor resection (Simpson scale) and the presence of fluorescence as well as its intensity. Comparison of the observable fluorescence intensity and the laser spectroscopy indicators revealed a significant correlation (r=0.75; p=0.005)., Conclusion: Meningioma is a well fluorescent tumor, with the technique sensitivity being 94.1%. In some cases, the use of fluorescence diagnostics in surgery of meningiomas improves identification of residual tumor fragments and enables correction of a surgical approach. To assess the effect of fluorescence diagnostics on the recurrence rate and disease-free duration, further research is required.

Schwannomas (neurinomas) are benign, encapsulated, and slow-growing neoplasms developing from any peripheral, cranial, or vegetative nerve that has the Schwann sheath. Schwannomas of the nasal cavity and paranasal sinuses in adults occur rarely, less than 4% of cases. The world literature lacks similar data for the pediatric age group because these tumors in children are unique and extremely rare. The most common clinical symptoms of sinonasal schwannoma are nasal breathing disorders, hyposmia or anosmia, and snoring at night. Other symptoms are associated with intranasal schwannoma invasion of neighboring organs and tissues and include exophthalmos, epiphora (lacrimation), headaches, and anesthesia in the facial area. In this article, we describe a rare case of diagnosis and successful surgical treatment of a child with intranasal schwannoma destructing the skull base.

Material and Methods: In the period from 2010 to 2016. 14 patients with cavernous hemangioma (CH) and 2 patients with capillary hemangioma (CapH) of the orbit were examined. The age of CH patients varied from 17 to 67 years (median, 53 years); 8 females and 6 males. The age of CapH patients was 35 and 54 years. All patients underwent surgery with subsequent histological verification. CT-perfusion was performed in 10 CH patients and 2 CapH patients according to a developed low-dose protocol (80 kV, 200 mAs, t scan =40 s) with allowance for a target localizer (80 kV, 120 mAs) and at a maximum radiation dose of not more than 4.0 mZv. Neoplasm microcirculation was quantitatively assessed by calculating hemodynamic parameters: blood flow velocity (BFV), blood volume (BV), and mean transit time (MTT). MRI without and with contrast enhancement was performed in 11 CH patients and 2 CapH patients according to the ophthalmologic protocol (Signa GE, 3.0 T) accepted at the Institute: without contrast enhancement - T1, T2, and T2-FLAIR modes, T1 and T2 with a Fat Sat technique at a scan thickness of 3 mm, and DWI MRI; contrast enhancement - T1 (three projections) mode, including the Fat Sat technique. SWAN (n=2) and non-contrast MR perfusion ASL (n=3) were also used. Diffusion-weighted images (DWI) were processed with calculation of the apparent diffusion coefficient (ACD)., Results: In all CH patients, CT-perfusion revealed low perfusion parameters of blood flow: BV CH =0.86±0.37 mL/100 g, BFV CH = 4.89±2.01 mL/100 g/min with a high mean transit time MTT CH =10.13±3.05 s compared to the same parameters of blood flow in the normal white matter: CBV NormWM =1.63±2.22 mL/100 g, CBFV NormWM =9.72±3.13 mL/100 g/min, and MTT NormWM =6.76±2.78 s. In CapH cases, significantly increased blood flow velocity and volume values and a low MTT value in the tumor were observed: BV CapH =10.30±4.10 mL/100 g, BFV CapH =119.72±53.13 mL/100 g/min, and MTT CapH =4.35±1.79 s. In the case of orbital hemangiomas, optimal MRI modes were T1 and T2 with the Fat Sat technique, a scan thickness of 3 mm, and intravenous contrast enhancement. The revealed pattern of contrast agent accumulation by CH, initially in the central part and then in the periphery, may be a useful radiographic sign in the differential diagnosis with other orbital tumors., Conclusion: Modern CT- and MRI-based diagnostics of orbital hemangiomas provides not only the exact location, size, and spread of the lesion but also reveals the characteristic structural features of these tumors, and the use of perfusion techniques visualizes hemodynamics of the tumors. CT-perfusion-based hemodynamic parameters of cavernous hemangiomas typical of this type of hemangiomas may be used in the differential diagnosis with other tumors of this location. The use of contrast enhancement and the Fat Sat technique with a scan thickness of not more than 3 mm is optimal for MRI diagnostics of orbital hemangiomas.

Aim: To analyze the histological features of changes in the brain tissue after ischemic stroke., Material and Methods: Brain tissue samples obtained in autopsy from 9 people died as a result of a left middle cerebral artery ischemic stroke from 3 to 7 days after admission were studied. Tissue samples were taken from 3 areas of the brain: 1) contiguous to the tissue necrotic damage site zone, 2) 5-10 cm distant from the previous one, 3) the contralateral hemisphere zone symmetrical to damage site. Samples were Nissl and hematoxylin-eosin stained. Detection of p53 protein, NSE, GFAP was performed by indirect immunoperoxidase immunohistochemistry., Results: A decrease in the total number of neurons and glial elements, their spatial redistribution, change in cell structure and their functional activity was found. The changes of the artery wall and impaired regional blood flow were detected. The more intense NSE reactivity; p53-positive neurons, reduced neuron-astrocytes distance were identified in zones 2 and 3., Conclusion: Ischemic stroke is accompanied by severe histological changes. These changes with varying degrees of severity occur both in the areas adjacent to necrotic core, and in remote areas, and it is a substrate for neuroplasticity.

We present a series of cases of a rare pathology, intracranial neuroenteric cysts, a review of the international literature, and the experience in treating this pathology., Material and Methods: Seven patients with intracranial neuroenteric cysts underwent surgery at the Neurosurgical Institute in the period between 2000 and 2015., Conclusion: The main and only technique for treatment of intracranial neurenteric cysts is their resection.

Background: An extended endoscopic endonasal approach is increasingly used in surgical treatment of space-occupying skull base lesions. The international literature reports only 20 cases of surgical treatment for fibrous dysplasia (PD) of the skull base using the endoscopic endonasal approach. We present our experience with the endoscopic endonasal approach in surgical treatment for giant fibrous dysplasia of the skull base, spreading to the right orbital cavity and nasopharynx., Clinical Case: A 26-year-old male patient presented with cranial pain, Vth nerve dysfunction on the right, right keratopathy. OD=0.2 (near acuity - 0.3), OS=1.0, OD - incomplete eyelid closure of 2 mm, conjunctival injection, mucous discharge, corneal opacity in the lower pole and paracentrally, OS - normal appearance. Severe right-sided exophthalmos (more than 15 mm), impaired nasal breathing on the right, nasal (hemorrhagic) discharge. Magnetic resonance imaging and spiral computed tomography scans revealed a bone density lesion located in the area of the orbit, nasal cavity, maxillary sinus on the right, and labyrinth of the ethmoid bone. The patient underwent endonasal endoscopic resection of the lesion., Results: The lesion was resected totally, which was confirmed by control SCT. Right-sided exophthalmos partially regressed (on the right: exophthalmos of 8 mm; protrusion: OD=23 mm, OS=15 mm; the eyeball was displaced downward and outward). The visual and oculomotor functions did not change. The neurological status remained at the preoperative level., Conclusion: Fibrous dysplasia of the skull base is an extremely rare disease. Modern techniques expand the indications for surgery of giant tumors of the skull base using minimally invasive approaches, in particular the endoscopic endonasal approach.

Diencephalic cachexia (DС) is progressive weight loss despite a normal caloric intake and a satisfactory state of health, which is caused by hypothalamic lesions. This is a rare (about 100 cases were reported) and potentially fatal disorder of unknown pathogenesis. At present, there is no effective pharmacological therapy for the disorder. Cachexia may regress only if the tumor reduces in size, therefore the timely diagnosis and treatment are of vital importance for the patient. DС is typical of early childhood, and only a few cases have been reported in adults. We present a rare case of DС in a 24-year-old female with papillary craniopharyngioma.

The median overall survival of glioblastoma patients is about 15 months. Only a small number of patients survive 3 years. The factors of a favorable prognosis for the 'longevity phenomenon' in glioblastoma patients are not fully understood., Objective: to determine the occurrence rate of long-living patients with glioblastomas, identify clinical predictors of a favorable prognosis, and identify the presence and prognostic significance of an IDH1 mutation., Material and Methods: Among 1494 patients operated on for glioblastoma at the Burdenko Neurosurgical Institute from 2007 to 2012, there were 84 (5.6%) patients who lived more than 3 years after primary surgery. In all the cases, histological specimens were reviewed, and immunohistochemical detection of a mutant IDH1 protein was performed. Overall survival was calculated from the time of first surgery to the date of the last consultation or death, and the recurrence-free period was calculated from the time of first surgery to MRI-verified tumor progression., Results: The median age of long-living patients with glioblastoma was 45 years (19-65 years). All tumors were located supratentorially. The median Karnofsky performance status score at the time of surgery was 80 (range, 70-100). All patients underwent microsurgical resection of the tumor, followed by chemoradiotherapy. The median recurrence-free period was 36 months (5-98 months). Overall survival of 48, 60, and 84 months was achieved in 23, 15 and 6% of patients, respectively. Among 49 specimens available for the IDH1 analysis, 14 (28.6%) specimens had a mutant protein. There was no significant difference in survival rates in patients with positive and negative results for IDH1 (44.1 vs. 40.8 months; p>0.05)., Conclusion: The significance of various factors that may be predictors of a favorable course of the disease is discussed in the literature. This work is the first part of analysis of prognostically significant factors positively affecting overall survival of glioblastoma patients. In our series, the predictors of a favorable prognosis for long-living patients with the verified diagnosis of glioblastoma were as follows: young age, the supratentorial location of the tumor, a high Karnofsky score before surgery, and tumor resection. In our series, we used immunohistochemical tests and found no prognostic significance of the IDH1 gene mutation; further analysis will require application of direct sequencing. We plan to study other morphological and molecular genetic features of tumors, which explain prolonged survival of glioblastoma patients, as well as the role of various types of combined chemoradiation treatment.

The work explores the molecular genetic features of anaplastic astrocytomas and oligodendrogliomas in a series of 43 cases. The mutational status was studied using domestic chemicals and reagent kits. We revealed clear genetic differences between astrocytic and oligodendroglial tumors and proposed an algorithm to study diagnostic and prognostic markers.

Because of the spread to different anatomical regions, craniofacial tumors (CFTs) usually receive blood supply from several arterial systems, and CFT removal is often accompanied by abundant blood loss., Purpose: The study purpose was to develop an algorithm of diagnostic angiography for planning surgical treatment of CFT patients., Material and Methods: Complex preoperative angiography was performed in 72 patients with craniofacial tumors, aged 10 to 78 years (mean age, 45.5 years), who underwent surgical treatment at the Burdenko Neurosurgical Institute in the period from 2012 to 2015. At the first stage, blood supply to tumors was quantified using SCT perfusion. Then, depending on an assessed degree of tumor vascularization, direct angiography or modern minimally invasive angiographic techniques (3D TOF HR MR angiography, SCT angiography) were applied., Results: In 12 cases of hypervascular tumors, accessible afferents were preoperatively embolized through the external carotid artery, which was accompanied by an increase in the blood supply to tumors via alternative routes of the external and internal carotid arteries. The obtained data were used to plan the surgical approach. A comparative analysis of the SCT perfusion data and the expression level of endothelial markers in histological specimens revealed no significant correlation., Conclusion: The study demonstrated the importance of a comprehensive assessment of the blood supply to CFTs in planning of the surgical treatment and enabled the development of algorithms for preoperative angiographic diagnosis, depending on the baseline clinical and radiological data.

Aim: To identify mutations in cerebral cavernous malformation (CCM) genes in patients with hereditary and sporadic CCMs in the Russian population., Material and Methods: Blood samples from 73 randomly selected patients, including 29 MRI-confirmed familial cases, 8 clinically confirmed familial cases and 38 so-called sporadic cases, were examined. A search for large deletions/duplications was performed using multiplex ligation-dependent probe amplification (MPLA). For MLPA-negative samples, the whole genome sequencing was performed to search for single nucleotide polymorphisms (SNP)., Results: Deletions in three genes (ССМ1, ССМ2, ССМ3) were identified in 14 patients, including 5 without definitely established familial type, in whom the familial character of disease was not confirmed by clinical and neuroimaging results. SNP mutations were found in 13 patients, CCM gene mutations in 27. Mutations were detected in 91.7% of familial cases. In two patients, new CCM3 deletions were identified. Gene distribution was as follows: 60.7 for CCM1, 32.2 for CCM2 and 7.1% for CCM3. In two members of a family with hereditary CCMs, no high effect mutations in the known CCM genes were found. Patients with mutations had greater severity of disease. Two patients with CCM3 mutations demonstrated the most aggressive clinical course. De novo formation and growth of CCM were observed only in patients with mutations., Conclusion: The distribution of pathogenic mutations in known CCM genes is consistent with other large-scale studies. Familial CCMs are associated with more severe disease course and may be caused by mutations beyond the known CCM genes.

The purpose to identify key morphological features of the Astrocytes and Neurons in the acute local cerebral ischemia human cortex., Subjects and Methods: Left middle cerebral artery ischemic stroke died persons (n = 9) brain tissue samples from 3 zones: 1st - contiguous to the tissue necrotic damage site zone, 2nd - 5-10 cm distant from the previous one, 3rd - the damage site symmetrical zone of the contralateral hemisphere. For GFAP, MAP-2, NSE, p53 detection indirect immunoperoxidase immunohistochemical staining method has been used. Also, the samples were Nissl and Hematoxylin-Eosin stained., Results: The most pronounced changes in the quantity and morphological structure of astrocytes and neurons are found in directly adjacent to the necrotic core region of theleft middle cerebral artery ischemic stroke brain. This indicates the prevalence of the inflammation processes around the area of nerve tissueischemic destruction. Morphological changes of neurons and astrocytes, apoptosis, enhanced neuron-astrocyte interaction found in the area bordering on necrotic core (5-10 cm from it), as well as ischemic hearth symmetrical sites of the contralateral hemisphere. This interaction is essential for the neuroplasticityrealization in the local ischemic brain injury., Conclusion: The results obtained were shown the nerve tissue morphological characteristics changes occur in local cerebral cortex ischemic injury not only in the lesion, but also in the contralateral hemisphere. These changes are probably related to the implementation of neuroplasticity.

Unlabelled: Glioblastoma is the most common primary malignant glial tumor of the brain in adult patients., Aim: to define the prognostic value of isocitrate dehydrogenase-1 (IDH-1) mutation and methylguanine-DNA methyltransferase (MGMT) methylation status in patients with glioblastoma (GB) and to analyze the impact of clinical data (gender, age, and tumor site), histological variants of the tumor structure, and time to development of recurrences on the course of the disease., Subjects and Methods: The investigation enrolled 63 GB patients aged 18 to 71 years who had received combined treatment (surgery, chemo- and radiotherapy) at the N.I. Burdenko Research Institute of Neurosurgery, Ministry of Health of the Russian Federation, in the period 2008 to 2011. The investigators performed a morphological examination of all tumor tissue samples and an immunohistochemical examination using anti-IDH-1 R-132 antibody clone («Dianova», Germany) and defined MGMT methylation status by a polymerase chain reaction using the CpGenome DNA Modification Kit («Chemicon International», USA). The data were statistically processed using a package of Statistica 6.0 programs., Results: Patient age, time to development of recurrent glioblastoma, mutations in the IDH-1 gene and MGMT were found to be prognostic factors for overall survival among adult patients in this category., Conclusion: Analysis of clinical findings and identification of molecular genetic aberrations in the tumor cells will be able to elaborate an individual approach to treating patients with glioblastoma in order to increase their survival rates and to improve quality of life.

5-aminolevulinic acid (5-ALA) is a natural precursor of protoporphyrin IX (PP IX), which possesses fluorescent properties and is more intensively accumulated in tumor cells than in normal tissue. Therefore, the use of 5-ALA in the surgical treatment of intracranial tumors, particularly gliomas, has gained popularity in the last years, whereas its use in other intracranial pathological entities including meningiomas has been reported occasionally. This study describes a series of 28 patients with intracranial meningiomas, who were administered 5-ALA for a better visualization of tumor boundaries. Twelve patients underwent also laser spectroscopic analysis in order to confirm the visual impression of tumor tissue visualization. Bone infiltration was readily demonstrated. In one case, the tumor recurrence could have been prevented by removal of a tumor remnant, which would possibly have been better recognized if spectroscopic analysis had been used. Fluorescent navigation (FN) is a useful method for maximizing the radicality of meningioma surgery, particularly if the tumor infiltrates the bone, the skull base, and/or the surrounding structures.

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.

Large parasagittal meningiomas, in particular hyperostotic ones, in children are rare and problematic in the differential diagnosis. The literature reports only single clinical cases related to this issue; opinions about the indications, surgical treatment options, and prognosis are contradictory. This paper presents a clinical case of a hyperostotic parasagittal meningioma with intra-extracranial growth in a 10-year-old boy with neurofibromatosis type II significantly worsening the prognosis. We discuss the epidemiological and clinical features of childhood meningiomas and issues of their diagnosis, treatment, and prognosis.

Objective: The study objective is to present the tactics of surgical treatment of simple (solitary) bone cysts of the clival region and CII body., Material and Methods: Two patients were operated on for simple clival and axis cysts., Results: The first patient with a simple clival cyst underwent transoral clivectomy and bone cyst evacuation. Postoperative control SCT scans revealed that the cyst cavity was lined with a hemostatic material and biological glue. Restoration of bone structures of the clivus occurred after 8 months. The second patient with a simple cyst of the second cervical vertebra (axis) underwent a two-stage surgical treatment: occipitospondylodesis was carried out first, and transoral removal of the pathological process of the CII body was performed at the second stage. Control scans a year after the surgery revealed the formation of bone tissue in the axis body region, an increased cystic cavity in the odontoid process of the axis, and posterior migration of the odontoid process, which caused compression of the brainstem structures. In connection with this, we performed transoral re-intervention with removal of the cystic separated odontoid process. Postoperative control scans revealed complete removal of the cystic odontoid process and decompression of the dural sac. The article provides a detailed analysis of the modern literature that has not reported any cases of a simple bone cyst located in the clivus or upper cervical vertebrae region and, thus, has not described the tactics for surgical treatment of these complex diseases. The article presents illustrative pre-, intra-, and postoperative images and histological specimens., Conclusion: The described cases present successful recurrence-free surgical treatment of simple (solitary) bone cysts located both in the clivus region (1 case) and in the body and odontoid process of the axis (1 case). A feature of the second case was that the patient had an extremely rare combination of a bone cyst of the C2 body and the cystic separated odontoid process of the axis (cystic os odontoideum).

An increased blood level of the thyroid stimulating hormone (TSH) is usually associated with primary hypothyroidism (PHT) but can also be observed in such rare cases as TSH-secreting pituitary tumor. The article describes four clinical cases of elevated TSH blood levels: 1) TSH-secreting pituitary adenoma with hyperthyroidism; 2) TSH-secreting adenoma with hypothyroidism; 3) hormonally inactive pituitary adenoma combined with primary hypothyroidism; 4) reversible thyrotropic hyperplasia. These clinical situations substantiate the importance of considering different diagnoses in a patient with a pituitary gland tumor associated with an increased TSH blood level.

Hemangioblastoma is a rare CNS vascular tumor that develops sporadically and can also be associated with von Hippel-Lindau disease. Hemangioblastomas account for 2-6% of all spinal cord tumors and are ranked third in the structure of intramedullary space-occupying lesions of the spinal cord. For the first time in our practice, we observed a dumbbell paravertebral hemangioblastoma. The international literature reports only 3 cases of the tumor with this growth type.

Unlabelled: Basal ganglia germinomas are a specific group of intracranial germinomas. Their early diagnosis is complicated due to their atypical localization and diversity of neuroimaging and clinical signs., Material and Methods: We describe 4 cases of basal ganglia germinoma in boys of 13, 14, 15, and 16 years of age. The medical history data, clinical features, neuroimaging and histological characteristics of basal ganglia germonomas, and preliminary results of the treatment are presented., Conclusion: Basal ganglia germinomas are usually verified at the late stage of the disease when patients are detected with extended lesions of the basal ganglia and severe neurological and neuroendocrine deficits. This situation is due to clinical and imaging signs that are untypical of common germinomas.

Glioblastomas are characterized by a variety of genetic and epigenetic disorders, identification of which allows constantly expanding a list of genes directly involved in carcinogenesis, thus increasing molecular diagnostics, monitoring and predicting disease. Molecular-genetic studies of patients with glioblastomas allowed revealing changes relevant to this disease and determining their prognostic significance. In the future molecular-biological markers along with clinical and therapeutic factors may play a role of separate and independent factors of prognosis in patients with malignant brain lesions.

Thyrotropinomas (TSH-secreting tumors) are a rare type of pituitary adenomas, which account for about 0.5-2.0% of all pituitary tumors. The criterion of thyrotropinoma is visualization of the tumor in the presence of a normal or elevated level of the thyroid-stimulating hormone (TSH) in the blood and elevated concentrations of free T4 (fT4) and free T3 (fT3)., Objective: To study the clinical, diagnostic, and morphological characteristics and treatment outcomes of TSH-secreting pituitary tumors., Material and Methods: The study included 21 patients aged from 15 to 67 years with pituitary adenoma and a normal or elevated blood TSH level combined with elevated fT4 and fT3 levels who were operated on at the Neurosurgical Institute in the period between 2002 and 2015. Before surgery, in the early postoperative period, and 6 months after surgery, the patients were tested for levels of TSH, fT4, fT3, prolactin, cortisol, the luteinizing hormone (LH), the follicle-stimulating hormone (FSH), estradiol/testosterone, and the insulin-like growth factor (IGF-1). The thyroid status was evaluated using the following reference values: TSH, 0.4-4.0 mIU/L; fT4, 11.5-22.7 pmol/L; fT3, 3.5-6.5 pmol/L. An immunohistochemical study of material was performed with antibodies to TSH, PRL, GH, ACTH, LH, FSH, and Ki-67 (MiB-1 clone); in 13 cases, we used tests with antibodies to somatostatin receptors type 2 and 5 and to D2 subtype dopamine receptors., Results: Thyrotropinomas were detected in patients aged from 15 to 67 years (median, 39 years), with an equal rate in males (48%) and females (52%). Before admission to the Neurosurgical Institute, 11 (52%) patients were erroneously diagnosed with primary hyperthyroidism; based on the diagnosis, 7 of these patients underwent surgery on the thyroid gland and/or received thyrostatics (4 cases). Hyperthyroidism symptoms were observed in 16 (76%) patients. The blood level of TSH was 2.47-38.4 mIU/L (median, 6.56); fT4, 22.8-54.8 nmol/L (median, 36); fT3, 4.24-12.9 pmol/L (median, 9.66). Tumors had the endosellar localization in 4 (19%) cases and the endo-extrasellar localization in 17 (91%) cases. Total tumor resection was performed in 7 (33%) patients. All these tumors had the endosellar and endo-suprasellar localization. No total resection was performed in patients with infiltrative growth of adenoma (invading the skull base structures). An immunohistochemical study of tumor resection specimens detected only TSH expression in 3 (14%) cases; 18 (86%) tumors were plurihormonal and secreted TSH and GH and/or PRL. Of 13 tumors, expression of the type 2 dopamine receptor was detected in 9 (69%) cases; expression of somatostatin receptors type 5 and type 2 was found in 6 (46%) and 2 (15%) cases, respectively., Conclusion: The criterion for total tumor resection was a postoperative decrease in the TSH level to 0.1 mIU/L or less. Total resection was performed in 33% of patients with tumors of only the endosellar and endo-suprasellar localization. In most cases, tumors were plurihormonal and secreted TSH and GH and/or PRL.

Objective: the objective of the study was to determine significant differences in electrocorticographic patterns for various types of focal cortical dysplasias., Material and Methods: 42 patients diagnosed with drug-resistant focal epilepsy were operated on at the Burdenko Neurosurgical Institute in the period from 2006 to 2013. Patients who were histologically diagnosed with focal cortical dysplasia (FCD) and underwent video-electroencephalography and electrocorticography were analyzed., Results: The classification of epileptiform patterns proposed by Palmini in 1995 was used. The sporadic epileptiform activity pattern was predominant in electrocorticographic studies. The continued pattern was more frequent in the case of type II focal cortical dysplasias (FCDs), both combined and isolated; burst and sporadic activity patterns prevailed in combinations in the case of type III FCDs. A uniform distribution of all pattern types of the epileptiform activity was observed in type I FCDs. The data are statistically significant for groups with sporadic and continued patterns., Conclusion: The continued epileptiform activity pattern is predominant in type II focal cortical dysplasia that corresponds to the most pronounced epileptogenesis processes of brain tissue with the presence of pathological forms of neurons. A uniform pattern distribution is observed for type I FCD. Patterns of the epileptiform activity and sporadic spike bursts are predominant in the case of type III FCDs. The sporadic activity is likely to be non-specific and almost uniformly distributed in all types of cortical dysplasias.

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.