SUMMARY. Hydrocephalus is one of the most common diseases of the nervous system in children. Every year the number of cerebrospinal fluid shunt surgeries is raising but malfunction of shunt systems is also increasing.

PURPOSE OF THE STUDY: define risk factors of malfunctioning of shunt systems in children in the material of the neurosurgical department of the Children’s Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan.

MATERIALS AND METHODS: research performed on 688 children with hydrocephalus that were implanted with ventriculoperitoneal shunt (VPS) in the Republic of Tatarstan from 1995 to 2016.

RESULTS: increase in age and body mass at the time of surgery decreased risk of VPS failure. Mean values of survival in children with post-inflammatory internal hydrocephalus is significantly lower than in patients with hydrocephalus of other etiology. Occlusive hydrocephalus mean values of survival are twice lower when compared to communicating hydrocephalus.

CONCLUSION. main factors influencing malfunctioning of VPS are type of hydrocephalus, age and weight of patients at the time of surgery and implant position of ventricular catheter.

SUMMARY. Glioblastoma is the most malignant primary brain tumor, the gold standard of treatment for which is considered to be combination therapy, including surgical removal of the tumor followed by chemoradiotherapy. Nevertheless, the median survival of patients with this diagnosis is about 14 months. The improvement of radiation therapy has led to the possibility of its implementation during surgery, which is a practical and effective method aimed at getting rid of the remaining tumor cells, reducing the spread of glioma and filling the therapeutic gap between surgery and chemotherapy and radiation therapy.

THE PURPOSE of the study is establishing of the safety of intraoperative radiotherapy of patients with the INTRABEAM system with a dose of up to 40 Gy on the applicator.

MATERIALS AND METHODS. The study involved 15 patients the average age of which was 51 years, the ratio of men and women was 4:6, the average Karnovsky scale before surgery was 97. Next, intraoperative radiation therapy by INTRABEAM (Carl ZEISS) system with a dose of 40 Gy was performed, followed by an assessment of the Karnovsky scale, early postoperative and delayed possible complications.

RESULTS. In all patients operated on with intraoperative radiation therapy and subsequent adjuvant radiochemotherapy, the method was well tolerated, there were no wound healing features or identified complications, there were no cases of wound liquorrhea, wound inflammation.

CONCLUSION. Intraoperative radiotherapy after surgical removal of the tumor can be recommended as a safe method for the treatment of malignant gliomas in addition to existing combination therapy.

SUMMARY.

The aim of the study was to evaluate the effectiveness of various options for decompression and stabilization interventions in patients with metastatic epidural compression of the spinal cord in the early postoperative period.

MATERIALS AND METHODS. The study included 70 patients with metastatic vertebral tumors, operated on from 01.01.2014 to 31.08.2021. Patients underwent surgical treatment in the amount of decompression and stabilization intervention.

RESULTS.. The presence of significant positive dynamics of the functional state in the group of patients was revealed in the absence of statistically significant dynamics of the neurological status during the 30 days after surgical decompression.

CONCLUSION. Decompression and stabilization interventions in the treatment of metastatic tumor compression of neural structures in the early postoperative period show effectiveness in restoring the functional status of patients, but allow regression of neurological disorders only in a statistically insignificant number of patients.

SUMMARY. Improving the efficiency of diagnosis and treatment of drug-resistant epilepsy is an urgent problem of modern neurology and neurosurgery.

PURPOSE OF THE STUDY: to evaluate the efficacy and safety of Dibufelon in drug-resistant epilepsy, at a dose of 800 mg per day (analysis of data from a clinical study and clinical cases).

MATERIALS AND METHODS. The analysis included data from 9 patients. Of these, 4 took part in a clinical study according to Protocol No.: DBF‑01/10: “Multicenter, randomized, double-blind, parallel group, placebo-controlled study to evaluate the efficacy, safety and tolerability of Dibufelon® as adjuvant therapy in patients with partial epileptic seizures. This study was carried out at the RNHI them. A.L. Polenov in 2012–2014. In addition, the data of 5 patients who took Dibufelon after the official registration of the drug and its entry into the pharmaceutical market were taken into account.

RESULTS. 4 patients were participants in a phase III clinical trial according to Protocol No.: DBF‑01/10 (Dibufelon). In another 5 patients, the experience of using Dibufelon was assessed as part of routine practice, after registration of the drug. All patients were regularly taking the study drug at the prescribed dosage. There were no changes in the basic therapy for epilepsy. All patients taking Dibufelon noted its good tolerance, there were no adverse events. The dynamics showed a decrease in the frequency of seizures, an increase in the duration of remission (the absence of epileptic seizures) and an improvement in cognitive functions. In all patients taking Dibufelon, the EEG showed a trend towards positive changes in bioelectrical activity over time. One of the patients noted an improvement in sexual function, which may indicate an improvement in the quality of life in patients with epilepsy while taking Dibufelon

CONCLUSION. The experience of using Dibufelon in the research center has shown its effectiveness and favorable tolerability profile in the adjunctive therapy of focal epileptic seizures in patients with various forms of structural epilepsy.

SUMMARY. The aim of the study — a comparative assessment of the effectiveness of surgical treatment of patients with carpal tunnel syndrome using open decompression and median nerve decompression with endoscopic assistance.

MATERIALS AND METHODS. The article analyzes the results of surgical treatment of patients with carpal tunnel syndrome who underwent surgical treatment from 2011 to 2021. All patients were divided into two groups. Median nerve decompression with endoscopic assistance (Group 1) was performed in 31 women and 3 men (mean age — 57.12±2.86 years) in N.N. Burdenko National Scientific and Practical Center for Neurosurgery. Open decompression (Group 2) was carried out in 5 men and 35 women (mean age — 59.88±2.99 years) in the Clinical Center of the Sechenov First Moscow State Medical University. The results of surgical treatment were evaluated using Visual Analogue Scale (VAS; from 0 to 10 points), and Symptom Severity Scale (SSS) and Functional Status Scale (FSS) of the Boston Carpal Tunnel Questionnaire before surgery, at discharge and 1 year after the surgery. Statistical data were processed using Microsoft Excel 2013 (Microsoft Corp., USA) and Statistica 10 software package (StatSoft Inc., USA).

RESULTS. A statistically significant decrease in the severity of pain syndrome at discharge were registered both in the Group 1 (p <0.001) and in the Group 2 (p<0.05). No statistically significant differences by gender were found. Also, positive dynamics was registered for the Symptom Severity Scale and Functional Status Scale, which was the more pronounced in Group 1.

CONCLUSION. Surgical treatment of carpal tunnel syndrome (open decompression, single-portal decompression with endoscopic assistance) is an effective treatment method contributing to the return of the patients to normal work activity. The results of the study indicate rather more effectiveness of the endoscopic technique in the early postoperative period in terms of more pronounced decrease of pain syndrome and shortening of postoperative rehabilitation period.

RESUME. Brain damage is a significant medical and social problem. At the same time, the main share in its overall structure is occupied by the so-called “light” traumatic brain injury — concussion and bruising of the brain of mild severity.

OBJECTIVE: to study the features of the neuropsychological status and the state of T-cell immunity in the acute period of mild concussion and brain injury.

MATERIALS AND METHODS: 55 patients with mild traumatic brain injury aged 18 to 55 years. A neuropsychological examination was conducted. The content of T-lymphocyte subpopulations was determined in venous blood, the level of cytokines and chemokines was determined in cerebrospinal fluid.

RESULTS: it was found that the basis of cognitive disorders are neurodynamic and regulatory disorders. The most significant indicators of violations in immune status are changes in the absolute values of “naive” Th, Th CM, Th EM cells, absolute and relative TEMRA content in blood serum, as well as pro-inflammatory cytokines IL‑6, IL‑15, IL‑1 RA, G-CSF, sCD40L, and CCL2/MCP‑1, CXCL9/MIG, CXCL10/IP‑10 chemokines in liquor.

CONCLUSION: In the acute period of mild traumatic brain injury, the development of cognitive disorders associated with the defeat of the first and third functional blocks dominates. The “pro-inflammatory” profile of cytokines and chemokines prevails in peripheral blood, which indicates the essential role of immune inflammation in the pathogenesis of injury.

RESUME. Multivariate analysis of information on 457 patients with cervical osteochondrosis made it possible to form a database of various diagnostic factors to identify indications for surgical treatment and quantify them.

PURPOSE OF THE STUDY: To improve the results of complex treatment of neurological complications of cervical osteochondrosis by studying the clinical manifestations of the disease at various stages of treatment and timely identification of indications for surgical treatment.

MATERIALS AND METHODS: The work is based on the analysis of the results of a comprehensive clinical examination, treatment and follow-up of 457 patients who underwent surgical treatment for cervical osteochondrosis. The age of patients is from 22 to 64 years. There were 186 men (40.7 %), women — 271 (59.3 %). The duration of the anamnesis before surgical treatment ranged from 2 to 11 years, but in the vast majority (296 observations 64.8 %) the duration of the disease was from 3 to 5 years. All 457 patients underwent high-tech surgical interventions.

RESULTS. Based on the identified diagnostic factors, a method was developed for “Clinical and digital determination of indications for surgical treatment of cervical osteochondrosis” depending on the severity of these factors. The method is carried out individually by identifying the positive, zero or negative significance of the digital total quantitative value of the expression of the clinical manifestation of cervical osteochondrosis, including the assessment of clinical and neuroimaging manifestations of the disease (MRI — a picture of the severity of the maximum amount of protrusion of the intervertebral disc or osteophyte in the focus of osteochondrosis, the intensity of the pain syndrome, impaired motor functions, the duration of the last exacerbation), functionally significant impairments in vital activity (the patient’s ability to self-service) and violations of the patient’s physiological conditions (sleep disorders, rest).

CONCLUSION. The criterion of a positive total quantitative numerical value of all diagnostic factors is an indication for surgical treatment of cervical osteochondrosis, the criterion of a zero total quantitative numerical value of all diagnostic factors is a relative indication for surgical treatment and requires dynamic monitoring, the criterion of a negative total numerical value of all diagnostic factors is an indication for conservative treatment.

SUMMARY. The use of segmented electrodes in deep brain stimulation (DBS) systems in stereotaxis has proven to be effective against undesirable side effects by changing the stimulation vector. Appearance of modern algorithms for calculating the volume of tissue activated (VTA) around the active contact of the electrode allowed better understanding how stimulation affects the activity of target brain structures. We evaluated the results of VTA modeling using segmented electrodes for deep stimulation implanted in the Globus Pallidum and the Subthalamic nucleus. Activation of the DBS system in the directional stimulation mode at the same amplitude values allowed using more structures in the direction of stimulation than in the non-directional mode, while reducing the involvement of structures around inactive contacts. With a suboptimal position of the electrode, it is possible to achieve the involvement of the required stereotactic target by the activation volume only with a significant increase in amplitude; operation in the directed stimulation mode can improve the results, but the accuracy of electrode implantation still plays a key role in the effectiveness of the operation.

SUMMARY. The article presents the results of diagnostics and surgical treatment of patients with non-traumatic intracranial hematomas using modern technologies. The main causes of intracranial hematomas are: hypertension (55.2 %), cerebral aneurysm (15.7 %), arteriovenous vascular malformations (21.5 %) and other causes (angiopathy, vasculopathy, amyloidosis — 7.8 %).

SUMMARY: Pituitary MRI with contrast enhancement is a standard method of neuroimaging, but, unfortunately, it can be uninformative in 30–50 % of cases when clarifying not only the presence, but also the localization of corticotropinoma. Inferior petrosal sinus sampling is the method of choice in the differential diagnosis of Cushing’s disease, and in addition, it can be used for the purpose of lateralization of pituitary adenoma. However, the lateralization sensitivity according to the latest data ranges from 54 % to 100 %, and these data may well serve as a starting point in choosing the side of intervention by a neurosurgeon, especially in cases where corticotropinoma is not visualized by MRI.

PURPOSE OF THE STUDY: to evaluate the diagnostic accuracy of lateralization gradients.

MATERIALS AND METHODS: cohort single–center retro/prospective study of 53 patients with Cushing’s disease. In order to assess the localization of corticotropinoma, a number of indicators were calculated (lateralization and prolactinadjusted ACTH intersinus gradient). Catheterization results were evaluated in comparison with contrast-enhanced pituitary MRI data and intraoperative data.

RESULTS: the study of the lateralization and prolactin-adjusted ACTH intersinus gradient showed their low diagnostic accuracy. The correct lateralization of corticotropinoma in our study when assessing gradients did not exceed 56.9 %, which in essence does not differ from random guessing. At the same time, an MRI of the pituitary with contrast enhancement allowed to correctly establish localization in 77.4 % of cases.

CONCLUSIONS: lateralization cannot be used as independent predictors of tumor node localization and choice of intervention side.

ABSTRACT. One of the main purpose of various surgical operations is to preserve the nerves, since unintentional damage can lead to neurological deficits in the patient, including numbness, pain, local paralysis, and others. Nerve identification during surgery depends on a variety of parameters, including anatomy, texture, color, and relationship to surrounding structures using white light illumination. The use of fluorescent labeling of nerves may enhance the contrast between nerves and adjacent tissues during surgery, which may lead to improved outcomes.

PURPOSE OF THE STUDY. Analysis of the results of pilot studies in the field of fluorescent biovisualization of nerves in relation to neurosurgical practice.

MATERIALS. The analysis includes publications reporting on the results of the application of methods of fluorescent biovisualization of nerves with different fluorescence inducers.

RESULTS. Currently, promising photosensitizers that have most of the desired characteristics for in vivo image-guided fluorescent nerve surgery are fluorescents GE3126, oxazine 4, Cy5-NP41.

CONCLUSIONS. Under ideal conditions, in vivo fluorescence imaging of nerve fibers is superior to other methods due to its real-time signal acquisition, high spatial resolution, high sensitivity, ease of operation, and low cost. Although fluorescence imaging techniques are not widely used in the clinical identification of nerves, they show exceptional promise for significantly reducing the risk of neurological deficits.

SUMMARY. Respiratory disorders during sleep in children with syndromic craniosynostosis: a comparative analysis of polygraphic data and computer morphometry.

Syndromic craniosynostosis (SCS) is a group of craniofacial malformations that includes the Cruson, Apert, Pfeiffer syndromes, and others, which are accompanied by premature fusion of cranial sutures or other craniofacial anomalies, including hypoplasia of the maxilla, cleft lip or palate, stenosis or choanal atresia, Chiari‑1 malformation, hydrocephalus, tracheobronchial dysplasia.

PURPOSE  of this work was to carry out a comparative analysis of the results of polysomnographic studies and morphometric data of patients with SCS to determine possible morphological predictors of the severity of respiratory disorders.

MATERIALS AND METHODS. A retrospective study was carried out in 43 patients (24 girls (56 %) and 19 boys (44 %) aged 40.55 ± 32.55 (3.6–135.9) months with various forms of SCS, who underwent instrumental assessment of respiratory function in the period from 2013 to 2021. All patients underwent examination and treatment in the conditions of the Federal Institution “National Medical Research Center of Neurosurgery named after Academician N.N. Burdenko. Syndromic diseases were represented by the following forms: 16 cases — Crouzon syndrome (23.2 %), Apert’s syndrome — 11 (25.6 %), Pfeiffer syndrome — 10 (23.3 %), and unspecified syndromes — 6 (13.9 %). Polysomnography (PSG) was performed in 32 patients (74.4 %), in 18 cases. (41.7 %) — computer monitoring pulse oximetry (CMP), among them in 7 cases. (16.3 %) both PSG and CMP were performed.

RESULTS. A moderate significant positive correlation (r = 0.4 p <0.01) was revealed between the values of the minimum saturation and the width of the choanas, i. e. the greater the choanal width, the higher the minimum saturation level. In addition, there was a negative correlation between the degree of obstructive sleep apnea syndrome (OSAS) and choanal height (r = 0.4 p <0.01). A negative correlation was found between the minimum saturation and the degree of dystopia of the cerebellar amygdala (r = 0.6), between the degree of dystopia of the cerebellar tonsils and the OSAS degree (r = 0.6). For patients with moderate and severe OSAS, a CT pattern of “interruption” of the air column at the choanal level is typical, as detected by 3D CT of reconstruction of the upper airways in 73 % cases, versus 16 % cases. (p <0.05) in patients with mild OSAS.

CONCLUSION. 1. Patients with syndromic forms of craniosynostosis are characterized by respiratory disorders, among which obstructive sleep apnea syndrome prevails. 2. Computer monitoring pulse oximetry during night sleep can serve as a method for screening respiratory disorders in patients with syndromic craniosynostosis. If signs of respiratory disorders are detected, patients need to conduct a polysomnographic study, which allows assessing the severity of violations and also clarifying their nature. 3. Despite the obvious relationship of morphological abnormalities detected in patients with syndromic craniosynostosis and respiratory disorders, only a correlation was found between the level of minimal saturation and choanal width. Respiratory disorders are determined by the summation of many factors of morphological abnormalities detected in this group of children. For a full clinical assessment of a patient, it is necessary, in addition to modern imaging diagnostics, to conduct a modern functional examination.

SUMMARY. Trigonocephaly (TC) is a common form of craniosynostosis (10 %). Surgical treatment of patients with TC is traditionally performed by bilateral frontoorbital reconstruction. Minimally invasive endoscopic (ESE) correction in TC has become an effective, safe alternative to open reconstructive surgery. The choice between traditional reconstructive surgery and endoscopic should be carried out with full disclosure of the features of treatment options, therefore, additional study of the advantages and disadvantages of ESE is required.

PURPOSE of this retrospective study is to analyze the results using ESE in combination with cranial orthosis therapy (COT).

MATERIALAND METHODS.  The results of treatment of 22 patients with TC (13 (59 %) boys and 9 (41 %) girls) aged 3.09±0.81 months were analyzed. The study of the clinical status before and after treatment, cephalometric examination to assess the morphological result, as well as the evaluation of the aesthetic result using visual analog scales (VAS) and questionnaires of the parents of patients.

RESULTS. Hemotransfusion was performed in 7 (31.8 %) cases. The duration of surgery was 35.91±8.82 min. The volume of blood loss was 40±32.66 ml. The duration of COT was 8.43±3.74 months. Repeated surgical interventions were performed in 3 cases. A significant increase in the frontal angle was revealed. The ratio of the interfrontal and interparietal diameters was less than normal values, both before and after treatment. The analysis of deformity according to the VAS and 10-point scale revealed significant differences in the shape of the patients’ heads before and after treatment.

CONCLUSION. Endoscopic suturectomy at TC, performed at the optimal time, in an adequate volume and accompanied by optimal COT, allows achieving good and satisfactory functional and aesthetic results in most cases. The immediate morphological results of treatment significantly differ from the preoperative ones, but also differ from the parameters of healthy children. The result of treatment is delayed and can be achieved with proper adherence to the treatment protocol by the patient’s parents and attending physicians. The significant advantages of the method are the absence of an extended postoperative scar, the relatively mild course of the postoperative period, the relatively low probability of blood loss and necessary hemotransfusion, the possibility of shortening the period of inpatient treatment of the patient, the low frequency of undesirable consequences and complications.

SUMMARY. Despite the large number of studies on sagittal balance, we were unable to find data in the literature comparing the use of different techniques for preoperative planning of lumbar lordosis correction in patients with degenerative stenosis.

AIM OF THE STUDY: to compare quality-of-life indicators and the effectiveness of methods for calculating lumbar lordosis correction in patients with degenerative stenosis during decompression and fusion on the lower lumbar spine.

MATERIALS AND METHODS: The results of clinical examination, surgical treatment, and postoperative period were analyzed in 60 patients aged 22 to 72 years (33 women and 27 men) with single- or multilevel lumbar stenosis at the L3 to S1 vertebrae grade C and D according to the Schizaz classification. Patients underwent TLIF surgery with correction of segmental lordosis at the level of the affected segment. All patients were divided into two groups of 30 depending on the method of calculation of lumbar lordosis correction: in the first group, the calculation was based on sagittal radiography of the lumbar spine and pelvis with capturing the femoral heads in standing position using the ideal lordosis formula LL=0.54×PI+27.6. In the second group, the calculation was based on whole-body sagittal radiographs using the FBI index. All patients were assessed preoperatively and postoperatively for pain on the VAS and quality of life on the ODI and RDQ scales. The postoperative follow-up period was 12 months.

RESULTS. No significant statistical differences (at p>0.05) were found between the study groups in terms of age, sex, lesion levels and their number, as well as pain level and quality of life on ODI and RDQ scales both before and after surgical treatment at 6 to 12 months. The results of changes in lumbar lordosis and other spinopelvic and global balance parameters indicate statistically significant changes in lumbar lordosis in both study groups.

CONCLUSION. In short-segment decompression and fusion interventions at the L3 to S1 level, the method of calculation of lumbar lordosis correction does not significantly affect the clinical outcome 6 to 12 months after surgical treatment.

ABSTRACT. In 31–48 % of patients treated for brain metastases in breast cancer, a recurrence of cerebral metastatic lesion develops. A targeted study of recurrent metastatic intracranial lesions in breast cancer aimed at adapting subtypespecific and morphospecific both local and systemic treatment is currently most relevant and has undoubted practical interest.

PURPOSE OF THE STUDY. The study is aimed at determining the nature of recurrent cerebral metastases depending on the method of local first-line exposure, the biological subtype of the tumor, and the morphological properties of the cerebral metastatic process.

MATERIAL AND METHODS. The results of examination and treatment were studied in 162 patients with breast cancer metastasizing to the brain. Recurrent cerebral metastases were diagnosed in 50 (30.8 %) women aged 28 to 80 years. The first-line treatment included tumor resection surgery in 18 (36 %) patients, radiosurgical treatment in 23 (46 %) patients, and whole-brain radiotherapy in 9 (18 %) patients. Patients were stratified by the biological subtype of their primary tumor. All patients were examined according to a standard protocol, applying clinical and laboratory research methods as well as neuroimaging.

RESULTS. Recurrent cerebral metastases were diagnosed in 50 (30.8 %) women. The average age was 52.9±12.3 years. HR+ biological subtype of breast cancer was verified in 16 % (N=8), HR+/HER2+ in 36 % (N=18), and HER2+ and HR-/HER2- in 24 % (N=12) each. In 92 % of cases, metachronous brain metastases were revealed after an average gap of 60.9 months from the verification of the primary tumor. The dependence between the biological tumor subtype and the nature of the primary metastatic brain lesion was not statistically significant (p=0.106). When analyzing how the methods of primary local control influence the variant of a recurrent cerebral lesion, no statistically significant correlation was found (p=0.638). However, a comparative analysis between the studied groups showed significant differences in the nature of cerebral progression. The local recurrence was significantly more common (p=0.001) in the HR+ tumor group, while distant recurrence was inherent to the HR-/HER2- group (p=0.003). The first relapse-free period among all patients was 7.74±8.04 months. None of the biological characteristics specific to the primary tumor influenced the duration of the relapse-free period (p=0.09). The median overall survival in the total number of subjects was 28.5 (Q1 13.25; Q240.75) months. The comparative analysis between groups revealed significant differences in the overall survival rate. The latter was significantly higher in the HER2+ group (p=0.004).

CONCLUSION. Aimed at adapting subtype-specific and morphospecific treatment, both at the local and systemic scale, the study of a recurrent metastatic intracranial lesion in breast cancer is the most relevant option, which demonstrates evident practical efficiency.

ABSTRACT. Microsurgical treatment of patients with distal aneurysms (DA) is an significant problem of vascular neurosurgery.

PURPOSE OF THE STUDY. To investigate hospital and long-term results of treatment of patients with DA.

MATERIALS AND METHODS. The analysis of 40 patients with DA was performed retrospectively. The control group included 40 patients with typical localization of aneurysms. In both groups, a standard set of clinical and instrumental studies was performed in accordance with current clinical guidelines. All patients underwent microsurgical aneurysm clipping. In the long-term period, daily activity, dependence on outside help, the level of disability, cognitive impairment, the level of anxiety and depression were assessed.

RESULTS. Most often, 52 % (21) DA were localized in the middle cerebral artery. In patients in the main group, the average severity prevailed during hospitalization, angiospasm developed much less often, but ICH formed more often. There were no significant differences in the level of disability, the level of self-service, as well as the severity of cognitive and mental disorders in the long-term period in patients from the main and control groups.

CONCLUSIONS. Microsurgical treatment of patients with DA is an effective and relatively safe technique associated with favorable long-term treatment outcomes.

ABSTRACT: Diffuse leptomeningeal glioneuronal tumor is a rare disease of the central nervous system, first classified in 2016. Due to the insufficient number of studies, there are different views on the clinical, pathological and radiological characteristics of this disease. This article presents a clinical case. By keywords, publications were searched in the databases eLibrary, PubMed, Google Scholar, Scopus, CrossRef, ResearchGate. The found publications were analyzed for information about the treatment, age, localization of the tumor, the results of immunohistochemical and molecular genetic studies, and the follow-up period. In this example, the histological picture and immunophenotype of the tumor did not differ from those previously described in the literature. Given that this tumor is predominantly aggressive, a quick and accurate diagnosis is crucial for the timely initiation of treatment.

ABSTRACT

Gangliosidoses are a heterogeneous group of genetically determined metabolic diseases resulting from the insufficiency of the beta-galactosidase enzyme, which leads to the accumulation of GM1-ganglioside and its metabolites in cells. The more the enzyme activity is reduced, the more severe the course and early onset of GM1 gangliosidosis. Depending on the age of the debut, there are three clinical forms of the disease. The most common is GM1-gangliosidosis type 1 — the disease manifests in the first 6 months of life, has a progressive course and an unfavorable prognosis. The main clinical manifestations of the disease are facial and skeletal anomalies, diffuse muscular hypotension, hepatosplenomegaly, cherryred macular spot (a symptom of a “cherry stone” on the fundus), pronounced delay in psychomotor development. The article describes a patient with severe GM1-gangliosidosis type 1, with typical clinical manifestations, including craniosynostosis and hydrocephalus.

SUMMARY. The article introduces the instrumental and personnel basis of high-tech neurosurgery in the Republic of Tatarstan. It provides the quantitative and qualitative parameters of surgical care for the adult population with diseases and trauma of the brain and spinal cord in the Republic. The range of the central nervous system pathology and the spectrum of neurosurgical interventions — open microsurgical, endoscopic, endovascular, and stereotaxic — highlight Kazan as one of the centers of modern neurosurgery.

The article states the goals for further improvement of neurosurgical care for adult residents of the Republic.

SUMMARY. The condition for maximum life expectancy and its high quality in patients with CNS tumors is adherence to clinical guidelines, including those on combined treatment for malignant neoplasms. Considering that the treatment components (surgical tumor removal of subsequent irradiation and chemotherapy) are typically provided in various medical institutions, care management is important for its final result.

This work aims to present medical care management for adult patients with neurosurgical oncology in the Republic of Tatarstan.

MATERIALS AND METHODS: the paper summarizes the experience in the management of diagnostics, surgical treatment, irradiation, chemotherapy, rehabilitation for patients with primary and metastatic brain and spinal cord tumors based on epidemiological studies in a large Russian Federation region.

RESULTS: The main points of the Procedure for healthcare provision to neurooncological patients, stated by the Order of the Ministry of Health of the Republic of Tatarstan, are outlined. The productive experience of the Coordinating Council in the complex treatment of CNS tumors is reported.