OBJECTIVE:To assess the efficacy and safety of sampeginterferon-β1a (samPEG-IFN-β1a) 180 μg and 240 μg administered once every 2 weeks compared to placebo and low dose interferon beta-1a (LIB) 30 μg administered once weekly.MATERIAL AND METHODS:Patients with relapsing-remitting multiple sclerosis aged 18-60 years, with Expanded Disability Status Scale score ≤5.5 were randomized at a ratio of 2:2:2:1 to the following groups: samPEG-IFN-β1a 180 µg, samPEG-IFN-β1a 240 µg, LIB, placebo. After 20 weeks, the placebo group completed the study. After week 52, the final analysis was performed, which included the primary endpoint analysis, the LIB group patients completed their participation in the study. The patients in samPEG-IFN-β1a groups continued to receive therapy with samPEG-IFN-β1a 240 µg until week 100 inclusive. The results of the final analysis after 52 weeks have been previously published. The current article presents a long-term efficacy and safety of samPEG-IFN-β1a after 104 weeks of the trial.RESULTS:The annualized relapse rate over the second year was 0.16 in the samPEG-IFN-β1a 180 μg group and 0.09 in the samPEG-IFN-β1a 240 μg group. By week 104, the proportion of relapse-free patients was 77.0% (87/113) and 83.3% (95/114) in the samPEG-IFN-β1a 180 μg and 240 μg groups, respectively. There were no negative dynamics of MRI markers, neurological deficit parameters and cognitive functions by scales and tests. The safety profile of samPEG-IFN-β1a was consistent with the known safety profile of IFN-β therapy.CONCLUSION:Treatment with samPEG-IFN-β1a is an effective and safe first-line therapy for relapsing-remitting multiple sclerosis patients.

Objective To present clinical and epidemiological aspects of neuromyelitis optica spectrum disorders (NMOSD) in the Russian Federation. Material and methods We studied 142 patients who met diagnostic criteria of 2015 for NMOSD. Sex, age at disease onset, presence or absence of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), mail clinical symptoms, oligoclonal IgG, therapy for the treatment of exacerbations and prevention of exacerbations, compliance with 2006 diagnostic criteria were assessed. Results The prevalence of women is 4.26:1, the most frequent age at disease onset is 18-29 years (36% of cases). The laboratory aspects of the disease are characterized and approaches to the treatment and prevention of exacerbations of NMOSD in patients of the Russian population are evaluated. Approaches to diagnostics are compared depending on the applied diagnostic criteria (34% of patients do not meet neuromyelitis optica 2006 diagnostic criteria). A prognosis for the prevalence of NMOSD in the Russian population has been proposed: 0.45-4.21/100000. Conclusion This is the first published data on clinical and epidemiological characteristics of NMOSD in the Russian Federation.

Background. Inflammatory polyneuropathies (IPNP) are diseases caused by an immune response against antigens in the peripheral nervous system. Epidemiological research is essential for health resource planning.Objective: to assess the clinical and epidemiological characteristics of acute and chronic IPNP in adults in the Leningrad region.Materials and methods. We analyzed the incidence of acute and chronic IPNP the Leningrad region for the last 24 years.Results. A gradual increase in the incidence rate since 2003 with slight fluctuations has been shown. A particularly significant increase was recorded in 2015 from 14 (2014) to 22 patients, while in previous years fluctuations ranged from 4 to 10 people. In 2016 and 2017, the number of cases increased even more to 26–27 per year (there are 1 600 000 residents over 18 years of age in the region). At the same time, an increase in the incidence of both Guillain–Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) was noted. The incidence of GBS in the last 10 years was 0.18–0.88, the incidence of CIDP was the same 0.18–1.0 per 100 000 population. With GBS, women were more likely to get sick, with CIDP – men. With GBS, the debut was more often in winter (35 %), in summer and autumn, 25 % each, less often in spring – 17 %. The average age of GBS development in our study was 50.3 years. CIDP also fell ill at all age periods from 19 to 84 years (average age 55.6 years). The most common GBS triggers were acute respiratory viral infections in 36 % and enterocolitis in 13 %. Respiratory disorders requiring mechanical ventilation were observed in 8 % of patients with GBS.Conclusions. The incidence of IPNP in adults, both acute and chronic in the Leningrad region, is growing with some fluctuations. This should be taken into account for health resource planning.

Анализируются 200 пациентов с положительными тестами на скрытое левшество и исследованием когнитивных функций с применением авторского скринингового теста. Возраст больных — от 30 до 65 лет (в среднем 41,0 ± 5,3 года), из них мужчин — 116 (58,0 %), женщин — 84 (42,0 %). При использовании стандартного исследования неврологического статуса у них были установлены следующие неврологические коморбидные диагнозы: ишемический мозговой инсульт — у 22 больных (11,0 %), дискогенно-венозная люмбосакральная радикуломиелоишемия — у 74 (37,0 %), рассеянный склероз — у 16 (8,0 %), вегетососудистая дистония с гипоталамическими пароксизмами — у 74 (37,0 %), паркинсонизм — у 14 (7,0 %). Исследование тестов на скрытое левшество и состояние когнитивных функций в дополнение к стандартному изучению неврологического статуса значительно расширяет круг диагностических возможностей. Терапевтические и нейрореабилитационные мероприятия должны проводиться с учетом клинических симптомов и признаков всех коморбидных патологий и соблюдением правил хорошей клинической практики.

Анализируются 200 пациентов с положительными тестами на скрытое левшество и исследованием когнитивных функций с применением авторского скринингового теста. Возраст больных — от 30 до 65 лет (в среднем 41,0 ± 5,3 года), из них мужчин — 116 (58,0 %), женщин — 84 (42,0 %). При использовании стандартного исследования неврологического статуса у них были установлены следующие неврологические коморбидные диагнозы: ишемический мозговой инсульт — у 22 больных (11,0 %), дискогенно-венозная люмбосакральная радикуломиелоишемия — у 74 (37,0 %), рассеянный склероз — у 16 (8,0 %), вегетососудистая дистония с гипоталамическими пароксизмами — у 74 (37,0 %), паркинсонизм — у 14 (7,0 %). Исследование тестов на скрытое левшество и состояние когнитивных функций в дополнение к стандартному изучению неврологического статуса значительно расширяет круг диагностических возможностей. Терапевтические и нейрореабилитационные мероприятия должны проводиться с учетом клинических симптомов и признаков всех коморбидных патологий и соблюдением правил хорошей клинической практики.

The purpose — to systematize the modern concepts of diagnosis and treatment for myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disorder. Material and methods. A search for modern publications was carried out using electronic scientific databases PubMed, Science Direct Open Access, Free Medical Journals. Keywords such as MOG-IgG-associated disorder, MOG-encephalomyelitis, MОG-antibody-associated disease were used for search of literature. Modern publications on the nomenclature, pathogenesis, diagnosis and treatment of MOG-IgG-associated disorder as well as scientific works devoted to the history of the study of this problem published in the period from 1987 to 2020 were selected and analyzed. Results. The diagnosis of MOG-IgG-associated disease is based on a combination of clinical, neuroimaging and laboratory assessments with exclusion of alternative conditions. There are many challenges in pathogenesis and nomenclature. Diagnostic criteria were published in 2018. Presence of certain indications is advisable for conducting a blood serum test for MOG-IgG according to the international recommendations. The key diagnostic test proposed is antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) detected in serum with cell-based assays (indirect fluorescence test or fluorescence-activating cell sorting) with strictly employing conformationally intact full-length human MOG as target antigen. There are no generally accepted standards of therapy.The approaches to therapy recommended by various expert working groups are based on the protocols for the management of patients with AQP4-IgG-positive forms of neuromyelitisoptica spectrum disorder. Conclusion. MOG-IgG-associated disorder is a recently proposed group of inflammatory demyelinating syndromes which are different from other demyelinating diseases. Currently it is advisable to use the diagnostic criteria published in 2018. Current knowledge allowed formulating recommendations for laboratory diagnostics which have limited availability in everyday practice. Treatment issues need to be systematized after achieving eхpert consensus.

Objective: description of a clinical case of the development of longitudinally spread transverse myelitis in a young woman suffering from nonHodgkin’s lymphoma (follicular, 1 degree, IIIA stage, complete remission from February 2017). This is a rare, significant case in clinical practice for both residents and neurologists, and the case is under discussion. It requires certain scientific studies in the search for specific antionkoneural antibodies or a more detailed study of the already known. It is necessary to introduce these studies into laboratory practice in Russia, which would help to accelerate the diagnosis of paraneoplastic myelitis.Materials and methods. Patient B., 30 years old, has been suffering non-Hodgkin’s lymphoma since February 2016 (follicular, I degree, IIIA stage, complete remission from February 2017), receives infusions of Rituximab with a dose of 600 mg once in 2 months, the subacute developed pronounced lower spastic paraparesis. In November 2018, suddenly, against the background of complete well-being, weakness and numbness of the legs appeared. The patient asked for help at the Center for Multiple Sclerosis on 11.17.2018, then she was hospitalized and examined. Patient B. received a pulse therapy with methylprednisolone 1000 mg intravenously № 5. Discharged with positive dynamics with a diagnosis of Demyelinating CNS disease, unspecified. Acute protracted transverse myelitis (LETM), EDSS = 3.5 points. On December 13, due to the increase in spacity and leg weakness, she was urgently hospitalized in the neurological department of the Clinical Hospital.Results. After the completion of laboratory and instrumental examination, the condition was regardedby us as paraneoplastic myelitis. Relapse of lymphoma was ruled out. The treatment was carried out: pulse therapy with methylprednisolone, Plasmapheresis and intravenous administration of IgG (Privigen) with a distinct positive dynamics.Conclusion. This clinical observation allowed us to become more familiar with paraneoplastic syndromes and, in particular, with paraneoplastic myelitis. In the clinical situation previously described, we encountered a number of features: longitudinally extensive transverse myelitis developed without relapse of lymphoma, progressed rapidly, there was no response to treatment (glucocorticosteroids, plasmapheresis, Rituximab). All these signs and MRI data of the cervical and thoracic spine (a symmetric hyper-intensive signal from level CII to level ThXI, mainly involving the central spinal cord T2 MRI mode) testified in favor of the paraneoplastic etiology of longitudinally extensive transverse myelitis.

Central pontine myelinolysis (CPM) is a concentrated, symmetric, non-inflammatory demyelination within the central basis pontis. In 10 % of patients with СРМ, demyelination also occurs in extrapontine regions: the mid brain, thalamus, basal nuclei, and cerebellum. Demyelination occurs in regions of compact interdigitation of white and gray matter as a result of cellular edema, which is caused by fluctuating osmotic forces, results in compression of fiber tracts. The most frequent cause of abrupt change in osmotic pressure is the rapid sodium correction of prolonged hyponatremia. In case that we report, there is a combination of central pontine and extrapontine myelinolysis, which occurs in 71-year-old woman owing to rapid sodium correction of prolonged hyponatremia which by turn developed due to salt-free diet, improper antihypertensive therapy and overheating. The diagnosis was confirmed by magnetic resonance imaging (MRI). The treatment led to significant decrease of neurological deficit.

Acute cerebellitis is a common inflammatory syndrome typically occurring in children and only on very rare occasions in adults. Thus, there was no description of acute cerebellitis, associated with Crohn’s disease, found in Russian sources of literature. We reported a case of acute cerebellitis in 45-year-old woman with a long history of Crohn’s disease treated with Mesalazine as a basic therapy and a resection of ileotransversoanastomosis together with small intestine two months before the onset of neurological symptoms. She first presented with severe headache and left-sided cerebellar dysfunction. The MRI showed signs of pronounced edema of the left hemisphere of the cerebellum, pial Gd-enhancement along the cerebellar folia, difficulty of CSF- flow. The application of lumbar puncture was impeded by the possibility of complications, such as brain herniation. Treatment with high-dose intravenous Methylprednisolone resulted in clinical and radiological improvement. Over the following year, her clinical status remained unchanged, except for one case of epileptic seizures as a result of switching of basic therapy of Crohn’s disease on Adalimumab. In this case, hemicerebellitis most likely has an immune-mediated nature. The temporal relationship with increased activity of Crohn’s disease and a significant positive response to corticosteroid therapy favored this hypothesis. It was known that inflammatory bowel diseases (IBD) had many extra-intestinal manifestations including neurological ones. With regard to that, the alterations in gut microbiota could be a possible common base for systemic inflammatory and autoimmune diseases, such as Crohn’s disease or autoimmune cerebellitis, described in this study.

The objectiveof the study is to evaluate the results and compare the methods of different variants of surgical treatment for the occlusive-stenotic lesion in extracranial parts of the carotid arteries.Material and methods.Comparative analysis of immediate and remote results, and evaluation of patency after 450 carotid endarterectomy performed by classic and eversion technique.Results.A significant advantage of eversion carotid endarterectomy was confirmed.Conclusion.The good results show not only the high qualification of each operating surgeon as well the entire staff, providing competent logistics and selection of patients for both planned and emergency surgical interventions.

Neuroblastoma is a malignant tumor derived from the neuroblasts of the sympathetic nervous system, which develop in any region of the nervous system. Usually, neuroblastoma is detected in children aged 1–2 years. About 90% of cases are diagnosed before the age of 5 years. The incidence of adult neuroblastoma is only 0.3 cases per million people per year. The clinical course and biological activity of adult neuroblastoma is different than children neuroblastoma. Early diagnosis of this disease in adults is necessary for timely start of treatment and increasing life expectancy. In this clinical observation, we present a detailed description of the course of this rare disease in the 34-year-old male and literature review on adult neuroblastoma.

Locked-in syndrome (LIS) is a rare neurological disorder, usually appears as a result of the pons cerebellar damage, mostly after the brain stroke. Locked-in syndrome is characterized by the paralysis of skeletal muscles (respiratory, facial, pharyngeal, lingual and muscles of the extremities). Patient is unable to speak and breath, facial expressions and voluntary movements are also impossible. Acromegaly is a disease that can be described by the increase of the growth hormone (GH) and Insulin-like growth factor (IGF-1) and develops in most cases due to the pituitary adenomas. Pituitary adenoma (PA) can be treated by neurosurgical techniques, pharmaceutical and radiation therapy (RT). We present a clinical case of 33-year-old woman with PA-caused acromegaly, that developed muscle weakness, nausea, vomit and respiratory disturbance in a 2 months after the radiation therapy. Subacute comatose state was developed in the patient. MRI of the brain revealed a multi-focal lesion of the media-basal regions on both sides, frontal corpus callosum and brain stem. Differential diagnosis included an acute demyelination (SD, PML), viral encephalitis and vasculitis. Treatment included methylprednisolone pulse therapy and plasmapheresis. The consciousness cleared up, but there was no spontaneous breathing, tetraplegia persisted. Autoimmune and infectious diseases was excluded. The homozygous mutation PAI-1-675 4G/4G was found. In this case, acromegaly induced endothelial dysfunction was the pathogenesis factor of multiple cerebral infarctions and demyelinating lesions, as well as RT and its proven pathological influence on the vascular wall and the fibrinolytic system. The revealed thrombophilia was also a factor of multiple cerebral infarctions. A Potential combination of pathogenic factors in the development of cerebral should be taken into account in predicting complications of RT.

Locked-in syndrome (LIS) is a rare neurological disorder, usually appears as a result of the pons cerebellar damage, mostly after the brain stroke. Locked-in syndrome is characterized by the paralysis of skeletal muscles (respiratory, facial, pharyngeal, lingual and muscles of the extremities). Patient is unable to speak and breath, facial expressions and voluntary movements are also impossible. Acromegaly is a disease that can be described by the increase of the growth hormone (GH) and Insulin-like growth factor (IGF-1) and develops in most cases due to the pituitary adenomas. Pituitary adenoma (PA) can be treated by neurosurgical techniques, pharmaceutical and radiation therapy (RT). We present a clinical case of 33-year-old woman with PA-caused acromegaly, that developed muscle weakness, nausea, vomit and respiratory disturbance in a 2 months after the radiation therapy. Subacute comatose state was developed in the patient. MRI of the brain revealed a multi-focal lesion of the media-basal regions on both sides, frontal corpus callosum and brain stem. Differential diagnosis included an acute demyelination (SD, PML), viral encephalitis and vasculitis. Treatment included methylprednisolone pulse therapy and plasmapheresis. The consciousness cleared up, but there was no spontaneous breathing, tetraplegia persisted. Autoimmune and infectious diseases was excluded. The homozygous mutation PAI-1-675 4G/4G was found. In this case, acromegaly induced endothelial dysfunction was the pathogenesis factor of multiple cerebral infarctions and demyelinating lesions, as well as RT and its proven pathological influence on the vascular wall and the fibrinolytic system. The revealed thrombophilia was also a factor of multiple cerebral infarctions. A Potential combination of pathogenic factors in the development of cerebral should be taken into account in predicting complications of RT.

The authors share their experiences with examination and surgical treatment of 163 patients with various neoplasms of the mediastinum. The first place among the neoplasms is occupied by tumors of the lymphatic apparatus (33%), the second--by tumors of the thymus (21%). The video-thoracoscopic technique was used in 17 of 131 operations performed. Complications after the operations took place in 6 patients (4.5%), two patients died (1.5%). A conclusion is made that the patients of this category must be treated not only by thoracic surgeons but also by hematologists, neurologists, neurosurgeons and specialists in radiation therapy.