Creutzfeldt-Jakob disease is a rare degenerative, prion disease of the brain. The causative agent of prion diseases is an infectious prion protein Prion Protein-scarpie (PrPSc), formed as a result of changes in the normal (non - infectious) cell protein Prion Protein-cellular (Rgrs). Accumulation of the pathological form of PrPSc leads to mass death of nerve cells. The main clinical manifestations of the disease include rapidly progressive cognitive impairment, myoclonus, dystonia, kinetically syndrome, spasticity, hyperreflexia, ataxia, visual disturbances, in the later stages of the disease akinetic mutism. About a third of the cases are epileptic seizures. The average life expectancy is about 5 months. More than 90% of patients die within 1 year due to aspiration pneumonia in the state of akinetic mutism. The complexity of diagnosis of CDR is associated with the rarity of the disease, clinical polymorphism and lack of awareness of doctors.

The surgical approach of spinal metastases treatment is becoming more common and it requieres a certain optimal surgical critical care that at minimum surgical trauma it will increase the life quality and prolong the patients lifetime. The prospective analysis of clinical and lab data of the patient cases was carried out. The cases were reviewed from October of 2016 to August of 2018 to evaluate the life quality of the patients after their surgery for metastatic spine lesions at the Department of Neurosurgery GBUZ SO SOOD Hospital. Evaluated parameters: performance status, Karnofsky performance score, SF-36 and EQ-5D health questionnaires, Tomita, Tokuhashi, Frankel scores, VAS, SINS score. All the cases after the surgery that had a histological confirmation of metastatic spine lesions except myeloma cases were reviewed. The life quality was evaluated and the Visual Analog scale of the patients was calculated at the pre-operative stage and the post-operative stage after 3, 6 and 12 months. We found positive changes in the pain syndrome, life quality and level of functional activity in post-operative stage. This evidences the viability of the combined modality treatment of the patients with metastatic spine lesions.

Spinal muscular atrophy is a genetic disorder characterised by loss of motor neurons, progressive symmetrical muscle wasting, hypotonia, different muscle group fasciculation, without sensitive and cerebellar disorders. Kugelberg-Welander disease is one of the spinal muscular atrophy. This article shows the case of Kugelberg-Welander spinal amyotrophy of adult patient.

Limbic encephalitis (LE) is an autoimmune disease of the сentral nervous system with a predominant destruction of the limbic system, including the hippocampus, the amygdala, the anterior nucleus of the thalamus and the limbic cortex. The clinical picture is characterized by subacute disorders of higher cortical functions (memory, learning, emotions), epileptic seizures and (or) mental disorders. Autoimmune reaction in LE can be caused by tumor processes, in-fectious or systemic disease, when it is not possible to identify the cause, LE is called "idio-pathic". There are no specific clinical and instrumental criteria for the diagnosis of the disease, often there is an erroneous or late diagnosis of LE. The given clinical example is an illustration of idiopathic limbic encephalitis with characteristic clinical and neuroimaging signs, effective treatment with glucocorticosteroids.

The aim of the study was to study the etiological aspects of development, the search for ways to prevent and improve the results of treatment of nonspecific hematogenous spondylitis in patients who were on chronic program hemodialysis. The study involved 7 patients (5 women, 2 men) who were on chronic program hemodialysis due to terminal chronic renal failure. All patients were hospitalized at the Regional Hemodialysis Center on the basis of GBUZ SOKB No. 1 in connection with a catheter-associated infection of the blood flow, a septic condition of unclear genesis, progressive neurological disorders, which became the basis for consulting a neurosurgeon, conducting neurovisualization methods, which led to the diagnosis of a non-specific hematologist. spondylodiscitis. All 7 patients were operated on, 1 of them died from pulmonary embolism, the rest had good and satisfactory outcomes due to the absence of complications and a significant regress of complaints.

Impairment of locomotion and balance are common symptoms of Parkinson's disease (PD). Postural instability leads to an increased frequency of falls and traumatization in these patients, which contribute to reduced quality of life and mortality. The objective of the research was to study impairment of voluntary postural control and possibilities of its rehabilitation in the patients with PD, utilizing methods of videomotion analysis and biological feedback. We studied 87 patients with PD, using system for videomotion analysis. During the course of rehabilitation, which includes biofeedback training, we observed statistically significant increase of quotients, which reflect quality of voluntary postural control (paired Wilcox test, asymmetric alternative hypothesis, p<0.05).

The study was devoted to investigation of relationship between level of the brain-derived neurotrophic factor (BDNF) in the peripheral blood and pain, psychoemotioal state in patients with chronic tension-type headache (CTTH). The chronic form of TTH had a well-marked anxiety and depressive and asthenic symptoms, which leads to a significant deficiency of BDNF in the peripheral blood.

Dissection of the cervicocephalic arteries is one of the main causes of acute ischemic cerebrovascular disorders in the young. Intramural hematoma is formed by blood penetration between the layers of arterial wall through the arterial intima defect and narrows the lumen of the artery, which is clinically manifested by symptoms of cerebral ischemia. A lot of cases of cervicocephalic dissection occurs in internal carotid and less common in vertebral arteries. Often fast movements in the neck, head and neck trauma or physical exercises provoke the development of dissection. Dissection of the middle cerebral artery is a rare localization and in the period from 1966 to 2013 only 59 cases of stroke and 2 cases of transient ischemic attack were described.

Multiple sclerosis is a dysimmune-neurodegenerative disease of the central nervous system that affects mostly young people of working age, manifested in a variety of clinical symptoms and inevitably leading to disability at a certain stage of its development. Timely diagnosis of the disease and proper treatment of its symptoms is extremely important and often determines not only the characteristics of its course and the quality of life of the patient, but also a long-term prognosis for the patient. With multiple sclerosis, certain complications can occur not only at the stage of diagnosis, but also with the appearance of new symptoms of the disease, that may require correction of treatment tactics. So, many of the newly emerging symptoms within the framework of an exacerbation may not respond to traditional symptomatic therapy and require the appointment of a pathogenetic treatment aimed at immunopathological mechanisms of the disease. In the article the authors give their own clinical observation of the patient with 12 years’ anamnesis of the disease, who suffered from neuralgia of the trigeminal nerve for many years. Intensive treatment, including neurosurgical treatment, is ineffective, and only the appointment of pathogenetic therapy (a glucocorticoid) provides a great clinical effect. The presented clinical case allows to demonstrate the importance of correct treatment of the symptoms of the disease with multiple sclerosis, which is the key to successful therapy.

The article describes a clinical case of an autosomal dominant dopa-responsive torsion dystonia, analyzes the pedigree, differentiate the features of the debut of the disease in the family. Also the neurological status described of a particular patient and the results of therapy with preparations of levodopa and the method of biological feedback (BFB).

Rendu-Osler-Weber disease is a rare inherited disease of the vasculature, characterized by the development of multiple telangiectasias of the skin and mucous membranes, as well as hemorrhagic syndrome of various localization. We present a case report of recurrent acute ischemic strokes in a young patient with Rendu-Osler-Weber disease. In the first part of the article, literature data on the incidence, etiology, pathogenesis, features of clinical manifestations of hereditary hemorrhagic telangiectasia, modern methods of diagnosis and treatment are presented. In the second part of the article, a rare case of recurrent acute ischemic strokes in a young patient with Rendu-Osler-Weber disease, caused by a paradoxical embolism due to pulmonary arteriovenous malformation was demonstrated. The chest CT detected a pulmonary arteriovenous malformation. The data of medical, including anamnesis, a description of the objective and neurological status, the results of laboratory and instrumental methods of examination are presented. The patient was recommended angiopulmonography, during which the pulmonary arteriovenous malformation was successfully embolized. This case indicates the need for targeted examination and monitoring of patients with hereditary hemorrhagic telangiectasia. For the purpose of early detection of vascular malformations in patients with Rendu-Osler-Weber disease and the timely administration of therapy to prevent possible serious cerebrovascular complications, we recommend performing the chest CT.

The article describes a clinical case of treating a giant schwannoma (neuroma) at the level vertebrae of C6-Th4 with paravertebral growth originating from the C6-C7 roots on the left

Firstly described by Van Berge et al. in 2003, leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) is an autosomal recessive inherited leukoencephalopathy caused by compound heterozygous mutations in the DARS2gene, which codes the mitochondrial aspartyl-tRNA synthetase. The disease is included in a list of rare diseases that have been established by TheMinistry of Health. LBSL mainly affects children and juvenile patients with a slow progressive cerebellar ataxia with spastic paraparesis and proprioceptive loss. The diagnosis of LBSL can be made with confidence in persons with characteristic abnormalities observed on brain and spinal cord MRI and genetic test. The treatment of LBSL doesn’t exist.

Acute disseminated encephalomyelitis (ADEM) is a monophasic demyelinating disorder. But 1,8-3,4% of children may develop relapsing disease. We introduced a boy diagnosed with multiphasic ADEM and experienced residual focal neurological deficit. We emphasize the importance of long-term follow-up and recognizing risk factors of relapse in demyelinating disorders.

The article presents data on the analysis of clinical and functional manifestations of septo-optical dysplasia in both the perinatal and postnatal periods, as well as the determination of factors affecting the occurrence of a congenital developmental disorder of the CNS both in isolation and in combination with other congenital malformations diagnosed in the Sverdlovsk region. The study includes 29 cases of septo-optical dysplasia, both in isolated form and in combination with other defects of the central nervous system, as well as in combination with defects of other internal organs. The diagnosis is based on the presence of a burdened obstetric and gynecological history, prenatal diagnosis, neuroimaging data - brain MRI, neurosonography. The article shows the clinical course and diagnostic symptoms, prenatal diagnosis of pregnant women, MRI and NSG-picture of the brain.

Schizencephaly is a rare abnormality associated with the formation of a cleft of the brain, as a result of which the lateral ventricles communicate with the subarchnal space. A clinical case of a child diagnosed with congenital malformations is presented: anterior abdominal wall defect (gastroschisis), anomaly of brain development - open schizencephaly of the left frontal-parietal region. Outbreaks of bilateral epileptiform activity in the form of acute-slow waves were revealed during an electroencephalographic study, structural epileptic encephalopathy were diagnosed.

In the literature, there are some reports of the carotid artery thrombosis cases after carotid endarterectomy performing. More often, thrombosis is detected in the early postoperative period and less commonly verified intraoperatively. Intraoperative carotid artery dissection is also a rare complication of carotid endarterectomy. Due to the small number of such complications cases occurrence, there is no uniform tactic for managing these patients. Some authors report the facts about appropriate using of operative intervention with dual disaggregant therapy or intraoperative heparin injection or other methods for reducing the risk of the operated artery segment thrombosis. The issues of using the intraluminal shunt in case of intraoperative detection of the collateral blood flow insolvency and are not solved. The question of intraoperative control of the patient's neurological status and prevention of embolic complications is not solved too. A clinical case study demonstrating a series of intraoperative complications is presented, including intraoperative thrombosis and subsequent recanalization of the distal section of internal carotid artery during eversion carotid endarterectomy operation and the occurrence of dissection of the common carotid artery after using the intraluminal shunt. The surgical tactics is shown in identifying the specified complications, the discussion of encountered problems is offered to find the way to solve and prevent them. In a proven clinical case, carotid endarterectomy was performed under local anesthesia, and the patient received double disaggregant therapy.

This article presents data on the effect of occupational risk factors on the prevalence of low back pain, and the effect of physical activity at the workplace and outside of work was also studied. For a more complete picture of the influence of these factors, a multifactorial analysis was conducted.

Epilepsy - polyethylene, highly comorbid pathology, which it can be combined with intractable pathologies. In this case, cause-effect relationships between different diseases can be caused by a random combination, genetic comorbidity or a consequence of each other. In the presented clinical case, genetic epilepsy in a child was combined with youthful myasthenia gravis. This observation of a combination of epilepsy and neuromuscular disease is quite rare, and therefore of interest to clinicians. Dynamic observation, correct tactics of management of the patient allowed to achieve stable medical remission of these diseases.

The article reviews clinical picture and treatment of patients with gliomas. The epidemic characteristic is presented. The peculiarities of rendering medical aid in the conditions of the neurosurgical department and multidisciplinary oncology dispensary are revealed. The analysis of primary brain tumors is made. The quality of life of neuro-oncological patients for the period 2016-2017 was assessed.

There is a description of clinical case of the development of bilateral lesion of the ulnar nerve in the area of the cubital canal in a 45-year-old man. The patient was referred for surgical treatment for the cubital canal syndrome on the left and a tumor formation was detected during the operation. Two years later, the patient noted paresthesia on the ulnar surface of the hand on the other hand. An operative treatment was performed for an intracranial ganglion cyst. Histologically, in both cases, neurofibroma with focal angiomatosis of soft tissues was diagnosed. Due to the negative clinical dynamics, repeated operations were performed on the right ulnar nerve, during which it was not possible to separate the neoplasm tissue from the nerve because of its germination into the nerve tissue. After the second operation on the right hand, the increase of ENMG parameters of the block of conduction of the ulnar nerve in the cubital canal on the right is noted.

The article provides an overview of recent scientific publications on the problem of damage to the nervous system in multiple myeloma. In half of the patients, myeloma is debuted with persistent pain syndrome associated with destructive lesion of the flat bones and compression of the roots and spinal cord. In 20% of cases, polyneuropathy develops as a manifestation of the underlying disease, as well as a complication of chemotherapy used to treat myeloma. Involvement of the nervous system is always secondary, but usually develops before the clinic of the main hematological disease or symptoms of damage to the nervous system muffle the clinic of multiple myeloma. There is a need for diagnostic alertness of specialists regarding oncohematological pathology. After chemotherapy, most patients need advice from neurologists in connection with polyneuropathy developing on this background. Over the past decades, the mechanisms of damage and the pathogenesis of polyneuropathy on the background of tumor blood diseases, which are presented in the article, have been described. The same is a clinical case of myeloma, manifested by neurological symptoms.

The study of human fetal heart veins has recently acquired significant relevance due to the development of fetal cardiac surgery and the transition of Russia to the new criteria of live birth recommended by the World Health Organization (WHO). Modern clinical practice requires a detailed topographic and anatomical characteristics of the fetal heart veins; at the same time, the need to use these veins in diagnostic and therapeutic manipulations in fetuses and preterm infants with extremely low body weight gave a powerful impetus to new anatomic studies

The aim of the study was to improve the results of the treatment of patients with the osteoarthritis of large joints through the development of minimally invasive combined methods of treatment and their introduction into clinical practice. Combined methods of treatment of patients with the osteoarthritis of large joints were applied in 1033 patients. As a result of the development of combined methods, it was possible to increase the percentage of good results by 3 times.

Deforming arthrosis of the ankle joint (Crusarthrosis) (Fig. 1) is characterized by severe pain, impaired movement in the joint, the need to use additional support (walking stick, crutches), which entails a deterioration in the quality of life, disability and impaired biomechanics in adjacent joints. At the moment, there are a large number of methods for the surgical treatment of deforming arthrosis of the ankle joint, such as: arthrodesis, endoprosthetics, arthroplasty. Each of the techniques has advantages and disadvantages of both medical and financial nature. This article presents a comparative experience in the surgical treatment of patients with stage III-IV ankle joint arthritis (according to the classification of J.H. Kellgren and J.S. Lawrence) using arthrodesis. In our clinic, we analyzed the experience of arthrodesis of the ankle joint from 2016 to 2018. A total of 25 operations were performed: 16 people (64%) were operated on by the arthroscopic method with subsequent fixation with screws, 7 people (28%) - by the open method with subsequent installation of the external fixation device, 2 people (8%) - by the open method with pin installation.

The study examined the developmental status of children with dysplasia of the urinary system aged 6 to 10 years with a duration of the disease from 1 month to 5 years. On the basis of the obtained data of the conducted research positive dynamics of the indices of psychological development was revealed for all the considered test evaluations in children receiving the drugs of the exercise therapy in the optimal volume.

113 patients (gestational age: 12-40 weeks) with acute gestational pyelonephritis were treated within 2 years. To evaluate ozone therapy efficiency, these patients were divided into 2 groups. Group I (n = 67) underwent the therapy as a part of combined therapy. Group II (n = 46) that did not experience ozone therapy was used as a control group. Clinical data and systemic inflammatory response parameters (i.e., IL1ß, IL4, plasma cortisol) were assessed in both groups on Day 1 and Day 7. As compared with Group II, on Day 7 Group I demonstrated significantly decreased IL1 ß and cortisol values, as well as markedly increased IL4 level. The results indicate inflammatory regression and ozone therapy efficiency. Clinical data and systemic inflammatory response parameters (i.e., IL1ß, IL4, plasma cortisol) were assessed in both groups on Day 1 and Day 7. As compared with controls, on Day 7 Group I demonstrated significantly decreased IL1 ß and cortisol values, as well as markedly increased IL4 level. The results indicate inflammatory regression and ozone therapy efficiency. The research confirms ozone therapy efficiency to treat acute gestational pyelonephritis.

The problem of supportability of the population with renal replacement therapy is one of the key problems in the modern healthcare system. The quality of medical care in general depends on its solution. In recent years, positive trends have been observed in the activities of the domestic renal replacement therapy service, but these changes are not enough to meet the needs of the population adequately.

The article presents the results of two sociological studies carried out in 2014 and 2018 on assessing the views of nurses of the Sverdlovsk region of their social position and prestige of the profession. It is established that most of them evaluate their social status as low, and only about 60% consider their profession prestigious. The main way to increase the income of nurses is additional part-time job. According to the data obtained in the survey, its value in 2018 comparing to 2014 has increased a bit, but the ratio of average wages in the region has decreased - from 66% down to 62,8%. Nurses attribute the increase in their social status to the increase in wages and pensions, as well as the strengthening of legal protection of their professional activities.