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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.