Search

In recent decades, the prevalence of demyelinating diseases in paediatric patients has increased rapidly. In this context, there is an increasing need for the study of this pathology in children to enable timely diagnosis and early prescription of highly effective pathogenetic treatment. Neuromyelitis optica spectrum disorders (NMOSD) are a group of severe demyelinating disorders that are united by a single pathogenetic mechanism and primarily affection of the optic nerves and spinal cord. The review presents the main clinical and epidemiological features of NMOSD in the paediatric population. The issues of pathogenesis, which is based on the formation of antibodies against aquaporin-4, and possible therapeutic targets are discussed in detail. The diagnostic criteria and the underlying clinical manifestations as well as their characteristics in childhood are analysed. We present a differential diagnostic framework of the most common diseases, which have to be distinguished from NMOSD in children. Particular attention is paid to the pathogenetic treatment of exacerbations and targeted therapy to prevent exacerbations, which has relatively recently proven its efficacy and been approved for use in paediatric patients.

Neuromyelitis optic spectrum disorders (NMOSD) are rare, severely disabling diseases of the central nervous system. They are characterized by periodic, unpredictable exacerbations that lead to an accumulation of irreversible neurologic damage. Despite existing possibilities for diagnosing NMOSD, it can be difficult to make the correct diagnosis in clinical practice. Current treatment options for NMOSD include drugs that affect the pathogenesis of the disease, including complement C5 inhibitors, with which experience is still limited, but published data from clinical trials demonstrate high efficacy of these drugs with an acceptable safety profile. The article reviews the main current approaches to the diagnosis and treatment of NMOSD.

Neuromyelitis optica spectrum disorders (NMOSD) are a group of inflammatory diseases of the central nervous system characterized by episodes of immune-mediated astrocytopathy, inflammatory demyelination and axonal damage, predominantly affecting the optic nerves and spinal cord. Currently, there are several unresolved issues that make it difficult to provide medical care to patients with NMOSD. The objective of this publication is to present the results of the work of the Russian experts panel, held in September 2022. The consensus council of experts adopted the main definitions and terms for NMOSD, proposed a therapeutic algorithm to prevent exacerbations in patients with NMOSD with serum IgG antibodies to aquaporin-4 (AQP4-IgG).The experts formulated profiles of patients with NMOSD to whom eculizumab may be recommended, and proposed a risk management plan during the treatment with this drug. The experts worked out the next steps, which are necessary for the improvement of medical care for these patients: development of unified electronic register of patients with NMOSD in the Russian Federation, development and approval of clinical guidelines.

Multiple sclerosis (MS) is a chronic autoimmune inflammatory demyelinating and neurodegenerative disease of the central nervous system. Now 2.8 million people in the world suffer from MS, since 2000 every 5 years there has been an increase in the number of MS patients by 10%. At the same time, the number of cases with highly active MS (HAMS) is increasing, i.e. cases of MS with two or more exacerbations without taking specific pathogenetic therapy or one exacerbation during taking MS disease-modifying therapies (DMTsMS), which is accompanied by the corresponding MRI data of the brain and spinal cord (presence of new or active lesions). Previously, there were no studies of quality of life (QoL) in this subgroup of patients in the world.Objective: to compare the QoL indicators of patients with HAMS with those of healthy people and patients with a typical course of relapsingremitting MS (RRMS).Patients and methods. The study included data on 518 patients from 14 regions of the Russian Federation for 2020–2021 years, in whom HAMS was confirmed during a second examination. We used the results of the analysis of clinical data from neurologists and data from questionnaires filled out by patients with HAMS. Two questionnaires were used in the work – the general (non-specific) Questionnaire for assessing the QoL, SF-36 (The MOS 36-item Short-Form Health Survey) and a special questionnaire for MS patients – MusiQoL (Multiple sclerosis Quality of Life).Results and discussion. Almost all indicators of QoL in HAMS were below the control. When compared with the typical course of RRMS, a more pronounced decrease in daily activity indicators, a significant impact of clinical symptoms on QoL, a negative attitude of patients towards the healthcare system, and a low overall health index were noted. Pearson correlation analysis revealed a stronger effect on QoL parameters of the frequency of exacerbations in HAMS than in RRMS.Conclusion. The data obtained in the study indicate the need for more active management of patients with HAMS using second-line DMTsMS.

Neuromyelitis optica spectrum disorders (NMOSDs) are autoimmune inflammatory disorders accompanied by central nervous system damage, widespread immunemediated demyelination, and axonal damage, involving mainly the optic nerves, spinal cord, and area postrema. The diagnostic capabilities, administration and routing of patients, and therapeutic approaches to this disease need to be improved. During several expert councils held in 2019–2021 in different regions of the Russian Federation, we discussed multiple issues related to various aspects of medical care for patients with NMOSDs. As a result, the experts developed further steps necessary to improve the medical care to these patients: to write and publish clinical guidelines for the diagnosis and treatment of NMOSDs; to consider the possibility of optimizing the NMOSDs diagnostic program including the aquaporin-4 antibodies (AQP4-IgG) testing; to evaluate the implementation of a set of measures aimed at including the corresponding laboratory investigations into the system of state guarantees (together with the institutions of the Ministry of Health of Russia), if there is clinical and economic feasibility; to include the issues of timely NMOSDs evaluation in educational programs initiated by the scientific medical community, in order to raise awareness of primary care neurologists in relation to the clinical and neuroimaging signs of probable NMOSDs; to assess the possibility of introducing routing schemes for patients with NMOSDs at the regional level; to work out a decision on the collection of NMOSDs epidemiological and clinical data in the Russian Federation.

An expansion of the age range of multiple sclerosis (MS) manifestation has been reported recently. Considering the multifactorial etiology, high heterogeneity of the clinical symptoms, and the difficulties of early MS diagnosis in young people, the onset and course of this disease in students can be considered as one of the significant medical and social problems.Objective: to analyze the clinical and epidemiological characteristics of MS in university and secondary specialized educational institutions students in the Republic of Bashkortostan (RB).Patients and methods. This case-control study included 32 patients aged 15 to 24 years studying in universities and secondary schools of the RB. The clinical and epidemiological indicators analysis was carried out based on data from the register of MS patients in the RB. 32 sex-, age-, place of birth and residence-matching patients with MS, who were not students, were included in the control group.Results and discussion. The median age of disease manifestation in students was 18 [16.5; 20] years, in the control group – 16 [14; 19] years (p=0.010). We found a lower «disease onset – diagnosis» in the students group of revealed a lower interval «disease onset – diagnosis» (0.5 [0; 1] years, p=0.006), and a shorter duration of MS compared to the control group ((2.8 [2; 4] years and 4 [2; 7] years, respectively, p=0.020). Students with MS predominantly had movement disorders: central paresis and cerebellar ataxia. Students also had a faster MS progression (0.4 [0.2; 0.6] EDSS scores per year, p=0.001), despite of the disease-modifying therapies.Conclusion. MS manifestation and course in students receiving disease-modifying therapies indicate the need for close follow-up, adherence control, and psychological support in the educational process.

The article presents the results of the discussion of the use of anti-B-cell therapy in multiple sclerosis (MS). These cells play a significant role in immunoregulation in MS, not only by producing antibodies to myelin antigens after transformation into plasma cells, but also by presenting the antigen to T cells, producing activation cytokines, and forming laminar follicles. The article provides an expert consensus statement on different drugs of this class in the MS treatment. In addition, the possibilities of determining the disease prognosis for the initially correct treatment choice are highlighted. Undoubtedly, there is a need for confirmation of the MS diagnosis, possible stratification of patients into different risk groups, and evaluation of the response to therapy. Potential additional research methods included evoked potentials and optical coherence tomography, baseline vitamin D3 level as a prognostic marker of the disease course, neurofilament levels in serum and cerebrospinal fluid to confirm neuron damage. However, it takes much time to study, determine the methodology, reference values, and develop a single standard approach to identify and implement a biomarker, which should then be implemented in routine clinical practice.

The process of neurodegeneration in multiple sclerosis (MS) remains an urgent problem of modern neurology; from the point of view of its therapy, it is especially important to identify this condition during the transition to secondary progression. This paper reflects the opinion of the Council of Leading MS Experts on the early detection of secondary progressive MS and its diagnostic tools, including the use of new online questionnaires. Special attention is paid to the concept of clinical progression verification using the indicator of the confirmed progression of disability and to the prospect for introducing siponimod (Kiendra® ) into clinical practice for the treatment of patients with secondary progressive MS.

The need for optimal treatment of the active forms of multiple sclerosis (MS), especially highly active MS (HAMS), poses a number of difficult problems for specialists, including not only the choice of a highly effective disease-modifying drug for MS (DMDMS), but also conditions for the timely and safe switching from other DMDMS. A group of expert neurologists from various clinics in Russia presents a consensus on the practical aspects of switching patients with HAMS from other DMDMS to cladribine tablets, which has been registered in Russia in March 2020. The paper discusses indications for changing therapy and gives indications, contraindications, and conditions for initiating cladribine therapy. It details the procedure and results of the expert consensus, on the basis of which the recommendations have been developed to switch to cladribine tablets from each of the DMDMS registered in Russia for the treatment of MS.

Currently, special attention is paid to timely treatment for active forms of multiple sclerosis (MS), especially for highly active MS (HAMS), in which there are ≥2 exacerbations and corresponding activity signs that are detected by magnetic resonance imaging (MRI) regardless of previous therapy with MS-modifying drugs (MSMDs) or there is ≥1 exacerbation and corresponding activity signs revealed by MRI during therapy with other MSMDs. A group of expert neurologists from various clinics in Russia, who actively participate in the clinical trials of cladribine tablets in MS, presents a consensus on the practical aspects of using the tablets of cladribine registered in Russia in March 2020 for the treatment of patients with HAMS. The paper mentions the groups of patients in whom the drug, an examination before starting a therapy cycle, routes of administration, and dose, and a monitoring scheme during and after treatment cycles are indicated. This drug acts on the principle of selective immunoreconstitution and may occupy an important place in the treatment of HAMS.

The meeting of experts discussed the clinical and pathophysiological features of secondary progressive multiple sclerosis (MS) (SPMS), clinical trials, and promising treatments for the progressive MS stage, as well as proposals contributing to the improvement of the current state of the problem of SPMS. In particular, the definition of and criteria for SPMS are formulated; the earliest period, when its confirmed progression can be recorded, is stated to be 3 months. The exacerbation-unaffected disability progression confirmed 6 months later may be considered to be more convincing. The introduction of tools for the early assessment of disability progression into routine practice will be able to identify the signs of progression at an earlier stage in order to timely change treatment policy. It is also noted that therapeutic possibilities in establishing secondary progression, especially in the absence of exacerbations, but in maintaining progression, are still insufficient. Certain hopes for slowing the progression in patients with SPMS are associated with the advent of siponimod, a new molecular class of S1P receptor modulators. The confirmed efficiency of siponimod in a large population of patients with SPMS allows the latter to be recommended for its treatment with both persistent disease activity (SPMS with exacerbations) and disability progression without exacerbations (SPMS without exacerbations).

Cardiovascular autonomic symptoms significantly impart quality of life in patients with multiple sclerosis (MR) and, in some cases, pose a threat to their life.Objective: to study cardiovascular autonomic dysfunction by cardiointervalography in MS patients.Patients and methods. Cardiovascular tests (CVT) were carried out in 47 patients with MS (a study group) and in 22 healthy individuals (a control group).Results. Comparative analysis revealed a significant reduction in the values of basic CVTs (Cresp, C30:15, and CVals) in MS patients (p