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Steroid myopathy is a common drug-induced non-inflammatory myopathy that affects patients requiring long-term glucocorticoid treatment for various autoimmune, inflammatory and oncological diseases. According to the neurology clinical practice guidelines, non-fluorinated glucocorticoids are the first-line pathogen-directed therapy for a number of dysimmune neuromuscular disorders, including myasthenia gravis. Long-term high-dose steroid treatment regime for myasthenia gravis leads to both acute and chronic development of glucocorticoids-induced proximal muscle weakness and atrophy. Steroid myopathy, along with other undesirable side effects of glucocorticoids therapy, impact health-related quality of life, patient satisfaction and adherence to treatment. Hence, further studies are required to expand our knowledge of clinical evaluation, diagnostic testing and prevention approaches for glucocorticoids-induced myopathy. The aim of this literature review is to analyze existing data on pathogenesis, diagnostic tools and treatment strategies for steroid myopathy.

An erratum on « Insufficiency/deficiency of vitamin B12 in patients in the endocrinological practice» by Natalya G. Mokrysheva, Marina V. Shestakova, Alexander S. Ametov, Mikhail B. Antsiferov, Igor G. Bakulin, Tatiana V. Vavilova, Gagik R. Galstyan, Tatiana Y. Demidova, Fatima K. Dzgoeva, Tatiana L. Karonova, Elena A. Lukina, Ashot M. Mkrtumyan, Rodion V. Ponomaryov, Natalia A. Suponeva, Olga Y. Sukhareva, Minara S. Shamkhalova (2024). Diabetes mellitus. 27(3). doi: 10.14341/DM13181An error was made in the list of authors: Nina A. Petunina was not indicated as author of this article. The correct list of authors: Natalya G. Mokrysheva, Marina V. Shestakova, Alexander S. Ametov, Mikhail B. Antsiferov, Igor G. Bakulin, Tatiana V. Vavilova, Gagik R. Galstyan, Tatiana Y. Demidova, Fatima K. Dzgoeva, Tatiana L. Karonova, Elena A. Lukina, Ashot M. Mkrtumyan, Nina A. Petunina, Rodion V. Ponomaryov, Natalia A. Suponeva, Olga Y. Sukhareva, Minara S. Shamkhalova.The identifier in the ORCID system for F.K. Dzgoeva was also indicated incorrectly. The correct link is: https://orcid.org/0000-0002-0533-7652.The editorial board apologize for this error and state that this does not change the scientific conclusions of the article in any way.The original article has been updated.

In recent years, the development of instrumental diagnostics has made it possible to identify a subgroup among patients with prolonged disorders of consciousness (PDoC) in which the phenomenon of “covert cognition” occurs, characterized by a dissociation between the clinical assessment and the data of instrumental diagnostic methods. To identify this phenomenon, we used a set of diagnostic paradigms developed at the Scientific Centre of Neurology in collaboration with a group of neuropsychologists from M.V. Lomonosov Moscow State University and tested on a cohort of healthy volunteers (n = 10) under the control of functional magnetic resonance imaging (fMRI). Objective: to evaluate the results of applying a set of paradigms to diagnose the phenomenon of “covert cognition” in a Russian-speaking cohort of patients with PDoC. Material and methods. In this fragment of a prospective study, after analyzing the medical records of 138 patients, 10 individuals with PDoC of various etiologies were included. Patients underwent a thorough neurological examination and a comprehensive neurophysiological and imaging study with emphasis on fMRI with paradigms. Results. When analyzing the fMRI data, significant activation clusters were detected in five patients in response to some passive paradigms, some of which were comparable to those of healthy subjects. Conclusion. Using the proposed set of fMRI paradigms, we demonstrated the possibility of identifying the phenomenon of “covert cognition” in a Russian-speaking cohort of patients in vegetative state / with unresponsive wakefulness syndrome (1/6), and confirmed by instrumental methods preservation of individual aspects of consciousness in patients in minimally conscious state “minus” (4/4).

On March 20, 2024, an interdisciplinary meeting of the Expert Council on the current problem of B12 insufficiency/deficiency and the prevalence of this condition among endocrine patients was held at the Endocrinology Research Centre (Moscow). The purpose of the meeting was to assess the role of B12 deficiency in reducing the quality of life of patients of different groups and to outline a strategy for the management of patients with vitamin B12 insufficiency/deficiency by endocrinologists.The resolution of the expert council was developed by leading specialists in various specialties.

Assessment of the individual level of consciousness on admission of a patient with brain injury to the intensive care unit (ICU) is a priority task and a mandatory step in the overall assessment of neurological status. The Full Outline of UnResponsiveness (FOUR) scale, developed at the Mayo Clinic (USA) in 2005, is a widely used tool for comprehensive assessment of patients with altered state of consciousness. The lack of a validated Russian-language version of the FOUR scale has hindered its widespread use in clinical practice. Therefore, the official Russian version of the FOUR scale was developed and adapted for use in Russia after the first stage of the validation study (linguistic and cultural adaptation).Aim. To evaluate the psychometric properties of the Russian version of the FOUR scale for comprehensive assessment of patients in altered state of consciousness.Materials and Methods. As part of a prospective multicenter validation study, the psychometric properties of the scale (reliability, validity, and sensitivity) were evaluated in a group of 171 adult patients with altered conscious state of various etiologies, such as ischemic and hemorrhagic stroke, neuroinflammatory conditions, and traumatic brain injury. Patients’ responses were assessed on the first day of ICU stay and 2-3 days later by two ICU neurologists with at least three years of experience.Results. High levels of validity and reliability were obtained for the Russian version of the FOUR scale for comprehensive assessment of unresponsive patients, including Spearman’s rank correlation coefficient r=0.99 (P

Over the past decade magnetic resonance imaging is being increasingly used in revealing pathological changes in peripheral nervous system due to a number of technical innovations and growth of diagnostical strength, and, therefore, due to initiation of research of several magnetic resonance imaging methods which allow to perform quantitative assessment of peripheral nerves. Among them, diffusion tensor magnetic resonance imaging which gives an opportunity to investigate microstructural changes in nerves tissue by water diffusion evaluation should be mentioned first. T2‑relaxometry and magnetization transfer ratio studies allow assessing macromolecular integrity of peripheral nerves elements. Chemical shift‑based fat fraction evaluation in peripheral nerves and corresponding muscles is also of great scientific interest both for diagnostic and therapy effect monitoring purposes. Manuscript presents brief description of above‑ mentioned methods, as well as recent results and perspectives of their application for peripheral nerves evaluation, supplemented with own illustrations of experimental observations.

Background. One of the key distinctions between multifocal motor neuropathy (MMN) and multifocal variant of chronic inflammatory demyelinating polyradiculoneuropathy (mCIDP) lies in the presence or absence of sensory deficits. Nevertheless, existing literature suggests that MMN can also involve sensory fibers of peripheral nerves, complicating the differential diagnosis of MMN and mCIDP, which remains a relevant issue.Aim. To evaluate the profile and intensity of objective sensory deficits and autonomic dysfunction in MMN and mCIDP, as well as to identify differential diagnostic markers.Materials and methods. Out of 65 patients followed up in the study, 30 were diagnosed with MMN and 35 were diagnosed with mCIDP. A retrospective analysis of clinical and epidemiological characteristics was carried out. The evaluation encompassed clinical assessments of sensory symptoms (including on the NTSS-9 scale), assessments of the severity of autonomic dysfunction using the COMPASS-31 questionnaire, and neurophysiological multimodal sensory testing (including electroneuromyography, computer pallesthesiometry, and short-latency auditory evoked potentials).Results. Patients with mCIDP were significantly more likely to have subjective (according to the NTSS-9 and COMPASS-31 scales) and objective (according to clinical examination) sensory deficits and autonomic dysfunction (p 0.05). Electroneuromyography studies revealed significantly lower amplitudes of hand sensory nerve action potentials in the mCIDP group compared to MMN patients (p

Little attention has been paid abroad to the problem of the long-term course of multifocal motor neuropathy (MMN). In our country, catamnestic studies of MMN have not been conducted at all. However, the results of such an analysis are extremely important for understanding the course and prognosis of the disease.Objective: to analyse the clinical and neurophysiological data of patients with MMN with a disease duration of more than 5 years.Material and methods. The study included 28 patients with MMN: 9 women (32%) and 19 men (68%); the median age at admission was 50 [44; 56] years; the median disease duration was 10 [8; 13] years. Medical documentation, medical history, complaints, neurological examination results (scored on the MRC and INCAT scales) and results of electroneuromyography (ENMG) of the long nerves of the hands were analysed.Results. The median time between onset of the disease and diagnosis was 5.5 [2; 10] years. Paresis 0.05). An objective assessment of sensory disorders revealed no changes when testing tactile, pain and temperature sensitivity, while half of the cases (14/28; 50%) showed a disturbance of vibration sensitivity in the lower extremities. The ENMG examination was consistent with the electrophysiological criteria of the disease, one third of the patients showed significant secondary damage to the axons of the motor fibers of the hand nerves, and in half of the cases a slight impairment of the axons of the sensory fibers was registered.Conclusion. MMN is a curable disease. Unfortunately, our retrospective analysis showed that in the Russian Federation there are problems with its diagnosis and quality care of this category of patients. Late diagnosis, delayed start of treatment and non-compliance with the schedule of pathogenetic therapy lead to persistent disability of patients.

Multiple sclerosis (MS) is a chronic autoimmune disease that limits the ability to work and lead an active lifestyle, mostly in young adults. Physical activity, along with drug therapy, is an essential part of the life of a person with MS. In the last decades, rehabilitation of MS patients has made great progress. However, the problem of compliance is still relevant. Virtual reality (VR) based rehabilitation can not only improve the quality of the training process but also increase patient compliance.Objective: to study the literature data on the effectiveness of the techniques of VR in reducing the severity of symptoms of MS and improving the quality of life.Materials and methods. A thorough literature search of the MEDLINE (Pubmed), eLibrary, and Google Schcolar databases was performed. Papers that met two criteria were included in the study: study population – patients with MS; presence of at least one comparison group (standard therapy or no therapy).Results. Based on the results of the analysis, 44 articles were selected. The efficacy of VR in reducing symptoms of MS such as fatigue, balance disturbances, risk factors for falls, mobility, apraxia, impaired hand function was described, as well as the impact on overall quality of life and its components.Conclusions. The VR rehabilitation can have its own place in the general system of physical rehabilitation and also become an effective tool to increase motivation and quality of life at all stages of rehabilitation.

INTRODUCTION An increase in the survival rate of patients with severe brain injuries of various origins determines the relevance of the search for approaches to assessing the prognosis of changes in the state of patients with chronic disorders of consciousness (CDC). Concomitant diseases are predictors of the recovery of consciousness and functional independence of patients with CDC. To assess the impact of the level of comorbidity on the prognosis of the patient state, the Comorbidities Coma Scale (CoCoS) is used abroad. However, the lack of a Russian-language version of this scale limits the practical and scientific areas of work with this category of patients.THE AIM of the study was to evaluate the psychometric properties of the developed Russian version of the Comorbidities Coma Scale (CoCoS).MATERIALS AND METHODS As part of the validation study, an assessment of psychometric properties (reliability, validity, sensitivity) was performed on a group of 52 adult patients with traumatic (18/52) and non-traumatic (34/52) brain damage.RESULTS High levels of validity and reliability were obtained (the Spearman’s Rank Correlation Coefficient r=0.98 (p

Transcranial magnetic stimulation (TMS) is increasingly used in research and clinical practice, therefore, the issues of its safety and tolerability are especially important. Currently, serious adverse reactions (ARs) are extremely rare. The more common mild ARs that can develop during and after stimulation remain less well understood but may affect procedure tolerance and treatment adherence.Objective: to study the tolerability and safety of TMS in patients and healthy volunteers.Material and methods. In a retrospective cohort study using standardized questionnaires, ARs were assessed during and within 24 hours after TMS in patients (n=103) and healthy volunteers (n=74).Results. In total, the data of 1246 sessions were analyzed. ARs were observed during 56.8% of the analyzed sessions and within 24 hours after in 21.5% of the sessions. In most cases, they were mild and did not affect the intent to continue stimulation. TMS withdrawal due to ARs was reported in six cases (3.3% of participants), three of which experienced severe headache (HA) after stimulation. During stimulation, sleepiness (32.4%) and pain in the stimulation area (20.3%) were most often recorded. The most frequent AR within 24 hours after TMS was HA (13.3% of sessions), its characteristics are described and possible diagnostic criteria are proposed.Conclusion. ARs can occur during and after a TMS session and are mild in most cases. TMS-induced HA, which is the most common AR within 24 hours after TMS, can be considered a distinct type of secondary HA, the pathogenesis of which requires further study.

Background. Paired-pulse transcranial magnetic stimulation allows assessing intracortical inhibition. However, a high variability of motor evoked potential (MEP) amplitude is a limitation of its use. Therefore, a new threshold tracking technique became of particular interest, which is based on the measurement not of the amplitude, but of the change of test stimulus intensity required to induce a MEP of a given amplitude.Aim. The assessment of absolute and relative reliability of short-interval intracortical inhibition (SICI) using threshold tracking technique in healthy volunteers.Materials and methods. All healthy volunteers included into the study (n = 12) underwent diagnostic paired-pulse transcranial magnetic stimulation in two consecutive days. The procedure included registration of passive motor threshold; the registration of 30 MEPs with supra-threshold intensity and determination of SICI using threshold tracking technique. At the first day the procedure was performed twice (T1, T2), at the second day – once (T3). Standard error of the measurement (SEM) and SEM% were calculated to assess absolute reliability, and intra-class correlation coefficient – for the assessment of relative reliability.Results. A good or excellent relative reliability were observed for SICI averaged at intervals 1.0–3.0 ms and 1.0–7.0 ms when assessed within a day or at different days. Relative reliability of SICI at separate interstimulus intervals varied in a wide range. SEM% was more than 10 % both for averaged SICI and SICI at all interstimulus intervals. Motor threshold had excellent reliability both assessed within a day and at different days, and low SEM values (5.6 % for Т1–Т2 and 4.39 % for Т1–Т3). High SEM and SEM% were observed for average MEP amplitude, it also had a moderate relative reliability when assessed within a day and a poor one – at different days.Conclusion. Calculation of averaged SICI can be recommended in further studies because of its high reliability values.

Background. The use of rating scales and questionnaires is essential in an evaluation of disease course, treatment response, the disability level and quality of life in patients with chronic inflammatory demyelinating polyneuropathy. The Medical Research Council (MRC) scale and its modification Medical Research Council sum score (MRCss) are widely used for measurement of motor deficit in patients with neuromuscular disorders. However, its usage is limited by the absence of the validated version for Russian-speaking patients.Aim. To validate MRCss scale in patients with chronic inflammatory demyelinating polyneuropathy with development of a Russian version.Materials and methods. We enrolled 50 patients with chronic inflammatory demyelinating polyneuropathy (25 with typical chronic inflammatory demyelinating polyneuropathy and 25 with Lewis–Sumner syndrome). At the first step we conducted linguocultural ratification according to the standard protocol. At the second step the psychometric parameters were evaluated, such as reliability, validity and sensitivity.Results. The developed Russian version of MRCss scale demonstrated the high level of reliability, validity and sensitivity.Conclusion. As a result, we developed a validated Russian version of MRCss scale, recommended for clinical practice and research.

Background. Today, the issues of differential diagnosis of chronic hereditary and acquired demyelinating neuropathies are still relevant. The variety of phenotypic variants of chronic inflammatory demyelinating polyradiculoneuropathy and hereditary neuropathy with liability to pressure palsies, their remitting course and the non-specificity of neurophysiological changes necessitate the identification of clear markers that can help in the differential diagnosis of the neuropathies under discussion already at the stage of the analysis of the electroneuromyographic study data.Aim. To determine neurophysiological differential diagnostic markers in the manifestation of chronic inflammatory demyelinating polyradiculoneuropathy and hereditary neuropathy with liability to pressure palsies.Materials and methods. A retrospective analysis of the data of neurophysiological examination of 25 patients with hereditary neuropathy with liability to pressure palsies and 25 patients with chronic inflammatory demyelinating polyradiculoneuropathy.Results. A combination of such indicators as the age of the onset of the disease 0.7), the value of the conduction velocity along of the motor fibers of the ulnar nerve at the level of the elbow joint 0.8), the conduction velocity along of the sensory fibers of the median nerve at the level of the wrist 0.8), absence of conduction block along the median nerve in any area, and also the presence along the ulnar nerve at the level of the elbow joint is characteristic of hereditary neuropathy with liability to pressure palsies and allows to exclude chronic inflammatory demyelinating polyradiculoneuropathy.Conclusion. Neurophysiological markers have been identified that can help in the differential diagnosis of two chronic remitting demyelinating neuropathies: chronic inflammatory demyelinating polyradiculoneuropathy and hereditary neuropathy with liability to pressure palsies. However, only a combined analysis of clinical, anamnestic and paraclinical data makes it possible to establish a final diagnosis.

Cortical representations of muscles can be probed non‑invasively using transcranial magnetic stimulation (TMS) motor mapping. TMS mapping can be applied both in research and clinical settings to assess motor cortex reorganization under the influence of various factors, to clarify pathophysiological mechanisms of motor system damage in various diseases, and to develop novel biomarkers of this damage. The article discusses the main methodological aspects of TMS motor mapping: choosing stimulation intensity and interstimulus interval, using of grid, neuronavigation and robotic systems, determining of a target muscle, etc. Special attention is paid to the cortical motor representation parameters such as the “hot spot” and center of gravity localization, area, etc. and their reproducibility under various mapping protocols. A review of publications on the use of TMS motor mapping application in brain tumors, stroke and amyotrophic lateral sclerosis is presented. The most significant practical impact of TMS motor mapping of the cortex is seen in brain tumors when used for presurgical localization of eloquent motor areas, as it is associated with the improvement of functional outcomes and progression‑free survival.

While compression neuropathies of the nerves of the lower limbs are not a common pathology, peroneal nerve neuropathy is the most common of them. Peroneal nerve compression most commonly occurs at or around the head of the fibula, but can also occur in the lower leg, ankle, or foot. Neurophysiological and neuroimaging methods are effective in diagnosing peroneal nerve compression and determining the type of damage. The first line of therapy is lifestyle modification, avoidance of compressive postures, ankle joint orthotics, treatment of knee joint instability when detected, kinesiotherapy. An important role in the correction of symptoms is played using anticholinesterase drugs, the effectiveness of which in diseases of peripheral nervous system has been shown in a number of domestic studies. If conservative treatment fails, surgical treatment is recommended. The diagnosis of peroneal neuropathy is associated with a good prognosis and most patients experience complete recovery of nerve function.

Background. Spasticity is a disabling syndrome frequently observed in progressive multiple sclerosis. One of the promising approaches to the treatment of spasticity is the use of therapeutic intermittent theta‑burst transcranial magnetic stimulation. In the last time new metaplasticity‑based protocols are being developed in order to increase the effectiveness of this technique. These protocols consist of several stimulation sessions in a day with an interval between sessions. However, there is no experience of use of such protocols in spasticity so far.Aim. To assess the safety and tolerability as well as provide first evidence of anti‑spastic effects of an original meta-plasticity‑based intermittent theta‑burst stimulation protocol in patients with progressive multiple sclerosis and spasticity.Materials and methods. In total, 5 patients with progressive multiple sclerosis and spasticity (2 females and 3 males, 28–53 y. o., disease duration – 11–18 years, EDSS – 6.5–8.5 points) were included into the study. 3 sessions of stimulation separated by an interval of 1 hour were applied daily, where a single session consisted of 3 protocols of theta‑burst stimulation with standard duration. Stimulation target was the area of cortical representation of the leg muscles, stimulation was applied consequently to both sides during 5 days (15 sessions in total). Before and after the treatment course anti‑spastic effect (modified Ashworth scale) as well as spasticity‑related pain, fatigue and clinical global impression were assessed.Results. No serious adverse events were observed during the study. Mild adverse events (sleepiness, pain at the stimulation site) developed in some cases, which did not affect patients’ willing to continue participation in the study. After the stimulation course decrease in spasticity in the legs was registered in 4 of 5 patients (to 12–39 % from the basic level). Decrease of fatigue (4 / 5) and pain severity (3 / 5) was also observed.Conclusion. According to the first experience, the proposed original metaplasticity‑based transcranial magnetic stimulation protocol is safe, well‑tolerable and potentially effective in patients with progressive multiple sclerosis. Therefore the further investigation of the protocol in a randomized controlled study seems justified.

Background. Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common immune‑mediated polyneuropathy. CIDP is characterized by a long‑term progressive or recurrent course of the disease and the development of neurological deficits and disability, it is important to assess the quality of life of patients. The paper presents the results of the first and second steps of validation of the Chronic acquired polyneuropathy – Patient Reported Index (CAP‑PRI) questionnaire designed to assess quality of life in patients with immune‑mediated polyneuropathy.Aim. To develop the Russian version of the CAP‑PRI questionnaire.Materials and methods. 85 patients with CIDP (according to EAN / PNS 2021 criteria) were enrolled. At the first step we conducted linguocultural validation according to the standard protocol. Two Russian‑speaking professional translators in the field of medicine performed direct translation of the original English‑language questionnaire, and the back translation was performed by native speakers with a medical background. At the second step the psychometric parameters were evaluated.Results. The developed Russian version of the CAP‑PRI questionnaire demonstrated a high level of reliability, validity and sensitivity.Conclusion. The Russian version of the CAP‑PRI questionnaire is recommended for clinical practice and research. In addition, the relationship between improved quality of life and the reduction of neurological deficits, disability and fatigue (as assessed by the IRODS, NIS and FSS scales) has been shown.

Хронические нарушения сознания (ХНС) представляют собой синдромы тяжелого поражения центральной нервной системы, приводящие к длительной грубой инвалидизации и требующие значительных усилий по лечению и реабилитации, которые ложатся на медицинские учреждения и на плечи близких пациентов. ХНС развиваются у пациентов после комы и характеризуются наличием бодрствования при полном или практически полном отсутствии признаков осознанного поведения. К ХНС относятся вегетативное состояние (ВС) и состояние минимального сознания (СМС). Также для описания начальных стадий этих состояний используется термин «продленное нарушение сознания» (ПНС). Отдельно выделяют выход из СМС — состояние, которое формируется по мере восстановления когнитивных функций. Диагностика ХНС основывается на многократном структурированном клиническом осмотре с применением специализированных шкал при условии исключения обратимых причин нарушения сознания. Лечение пациентов с ХНС включает в себя поддержание жизненно важных функций, обеспечение оптимального питания и борьбу с типичными осложнениями и сопутствующими состояниями (пролежни, спастичность, боль, пароксизмальная симпатическая гиперактивность и др.). У пациентов с ХНС должна проводиться реабилитация с участием мультидисциплинарной реабилитационной команды в объеме, который определяется проблемами и возможностями конкретного пациента. Наиболее эффективной реабилитация является при условии ее раннего начала. На данный момент однозначных доказательств эффективности каких-либо специфических методов, направленных на восстановление сознания, не получено; изучается ряд соответствующих фармакологических и нефармакологических вмешательств, обязательным условием применения которых является максимально возможная коррекция соматических проблем пациента. Важную роль в ведении пациентов с ХНС играет вовлечение близких пациента, которые, в свою очередь, нуждаются в получении объективной практической информации о состоянии своего родственника и о направлениях реабилитации, а также в психологической помощи.

The use of high-frequency repetitive transcranial magnetic stimulation (rTMS) in patients with a drug-resistant depressive episode is associated with a high variability of its effect, which determines the relevance of the search for predictive biomarkers of the effect.Objective: to search for neuroimaging biomarkers of the clinical effectiveness of navigational rTMS in patients with a drug-resistant depressive episode.Patients and methods. The study included 30 patients with a drug resistant depressive episode who underwent 20 sessions of navigational highfrequency rTMS of the dorsolateral prefrontal cortex (DLPFC) of the left hemisphere. We performed an analysis of correlations between functional connectivity and gray matter volume (GMV) in given areas (before rTMS) with the degree of reduction in the Beck score for assessment of depression and the presence of a clinically significant response to therapy.Results and discussion. A relationship was shown between the clinical effect and low values of functional connectivity between the subgenual cingulated cortex and DLPFC of the right hemisphere. A relationship was found between a decrease in the severity of symptoms of depression and the volume of GMV in the insula, inferior frontal gyrus, temporal pole, superior temporal gyrus, and median cingulate cortex of the left hemisphere, as well as a relationship between the presence of a significant response to therapy and the volume of GMV in the insula, inferior frontal gyrus, temporal pole and superior temporal gyrus on the left.Conclusion. The obtained results may become a prerequisite for the development of individual predictive biomarkers of the effectiveness of rTMS in patients with a resistant depressive episode.

Background. Carpal tunnel syndrome is the most common compression mononeuropathy. The choice of the preferred conservative treatment method is still relevant, especially for the patients with the moderate severity of the disease. Objective: to perform a comparative study of the effectiveness of local injection therapy and wrist splinting in idiopathic moderate carpal tunnel syndrome; to assess the duration of the clinical effect in the follow‑up period to 6 months. Materials and methods. The study included 105 cases of moderate idiopathic carpal tunnel syndrome. Patients were randomized to 2 groups depending on the treatment method: the main group (n = 54) received 1 or 2 ultrasound‑guided injections with glucocorticosteroid (betamethasone 5 mg + 2 mg/1.0 ml) and lidocaine 2 % 1.0 ml into the carpal canal; the control group (n = 51) was assigned wrist splinting at night for 1 month. The primary criterion for therapy effectiveness was evaluation by clinical scales (SSS, FSS, LANSS, Visual Analogue Scale) after 1 month from the treatment initiation. A secondary criterion for the therapy effectiveness was the assessment of changes in electrophysiological and neurosonographic parameters.Results. The scores for SSS, FSS, LANSS and Visual Analogue Scale were significantly lower at 1 month in the injection therapy group than in the splinting group (p

Identification of complications and control of comorbidities are essential in monitoring the patients with chronic disorders of consciousness and predicting their outcomes. The researchers of the Department of Biotechnological and Applied Clinical Sciences of the University of L'Aquila (Italy) developed the Comorbidities Coma Scale (CoCoS) for a comprehensive assessment of such patients. Lack of an officially validated version of the scale hampers its use in Russia, while using versions which have not been completely validated prevents clinicians from obtaining reliable results when examining patients with chronic disorders of consciousness. Aim. To develop the official Russian language version of the Comorbidities Coma Scale, considering various linguistic and cultural parameters, as a part of the 1st stage of the validation study. Material and methods. The first stage of validation was completed: direct and reverse translation of the scale was performed by two independent medical translators. The translated version was assessed by an expert board including an expert translator, neurologists, and critical care specialists. Pilot test and two meetings of the expert board, before and after testing, were arranged to assess the results and approve the final Russian version of the scale. Results. During the first meeting of the expert board, corrections were made in the Russian language version of the scale in terms of language and cultural adaptation. Pilot testing was carried out based on the inclusion and exclusion criteria. The researchers had no difficulties in understanding and interpreting the instructions for the scale. The second meeting of the expert board was held thereupon, and the final version of the Russian language version of the scale was adopted, which is available on the website of the Center for Validation of Health Status Questionnaires and Scales of the Research Center of Neurology. Conclusion. The first stage of validation, i. e., linguistic and cultural adaptation, was carried out at the Research Center of Neurology (Moscow, Russia). For the first time, the Russian version of the scale for assessing comorbidities in patients with chronic disorders of consciousness was presented and approved for the practical use. The future publications will address the psychometric results of the scale such as sensitivity, validity, reliability.

Amyotrophic lateral sclerosis is a neurodegenerative disease with a steady and rapid progression, manifested, in addition to the loss of strength of the muscles of the limbs and trunk, the development of fatal bulbar and respiratory disorders. Due to the need for early detection of this pathology, a large number of diagnostic tests have been proposed, which make it possible to suspect a motor neuron disease in a patient with a high probability. However, due to the polymorphic clinical picture of the disease, currently there are diagnostic situations in which existing methods do not allow to establish a reliable diagnosis or it becomes possible only at the later stages of the disease. This review examines the modern possibilities of ultrasound examination of peripheral nerves and muscles in the diagnosis and differential diagnosis of amyotrophic lateral sclerosis.

Background. Chronic inflammatory demyelinating polyneuropathy (CIDP) is a treatable dysimmune polyneuropathy. An objective response for pathogenic therapy is essential in diagnosis and management of CIDP. For proper assessment of patient’s complaints and evaluation of disease progression, it is recommended to use validated scales and questionnaires. The paper presents the results of the first step of Inflammatory Neuropathy Cause and Treatment (INCAT) validation in patients with CIDP.Objective: the development of the Russian version of the INCAT scale and its linguocultural ratification.Materials and methods. 15 patients with definite CIDP (according to EFNS/PNS criteria) were enrolled. Linguocultural ratification was conducted according to the standard protocol.Results. The Russian version of the INCAT scale was developed.Conclusion. We conducted the first stage of INCAT scale validation in patients with CIDP.

Transthyretin amyloidosis (ATTR-amyloidosis) is a systemic progressive fatal disease, for which a modifying therapy has recently been proposed that delays the progression of the disease and improves the patient’s quality of life. The delay in the diagnosis of ATTR-amyloidosis is associated with the heterogeneity of the manifestations of the disease, as well as insufficient awareness of doctors of different specialties about the disease. A review of recent studies on the symptomatology, diagnosis, molecular genetic characteristics of ATTR-amyloidosis and the most common forms of the disease with the predominant involvement of peripheral nerves and the heart, as well as the kidneys, gastrointestinal tract, and eyes is presented. The international consensus recommendations for the diagnosis of suspected ATTR-amyloidosis using modern methods that facilitate early and accurate diagnosis are discussed. The reasons and the most frequent misdiagnoses of ATTR-amyloidosis, which also lead to a delay in the timely appointment of therapy, are considered. Molecular genetic testing should be considered early in the evaluation of a patient with unexplained peripheral neuropathy and cardiomyopathy. A diagnostic algorithm based on the initial symptoms and manifestations of the cardiovascular and nervous systems facilitates the identification of a patient with clinical suspicion of ATTR-amyloidosis by the general practitioner. Early diagnosis is critically important for patients with ATTR polyneuropathy, since the early prescription of Vyndaqel (tafamidis), registered in the Russian Federation in 2017, allows a significant clinical effect to be obtained. Timely administration of Vyndaqel significantly slows down the progression of the disease, improves the prognosis and quality of life in patients with ATTR polyneuropathy.

Background. Trigeminal neuralgia is the most common cause of facial pain. Insufficient effectiveness and frequent side effects of pharmacological therapy, as well as the risk of complications of invasive neurosurgical manipulations, determine the relevance of the development of new treatment approaches, one of which is repetitive transcranial magnetic stimulation (rTMS).The aim of the study is to determine the effectiveness of high‑frequency rTMS of the primary motor cortex in terms of pain intensity and quality of life in patients with trigeminal neuralgia, as well as to assess the safety of the stimulation course.Materials and methods. This open‑label non‑randomized single arm study included 20 patients with classic trigeminal neuralgia according to the ICHD‑3 classification. All patients received 10 sessions of navigated high‑frequency rTMS of the primary motor cortex (hand area) of the hemisphere, contralateral to pain syndrome localization. The maximum and average pain intensity was assessed before and after 10 rTMS sessions according to a Numeric Pain Rating Scale (NPRS), as well as quality of life was measured according to the SF‑36 questionnaire, and the severity of affective disorders according was measured to the Beck Depression Inventory (BDI). Safety and tolerability of rTMS were assessed using self‑developed questionnaires for adverse events that occurred during stimulation and within 24 hours after the previous session.Results. A significant decrease in the maximum (p = 0.01) and average (p

Background. The study of the sensitive portion of the medial plantar nerve is relevant not only in the examination of patients with tibial nerve mononeuropathy or its branches, but also in the diagnosis of polyneuropathies.Objective: to analyze the normative parameters of the sensory potential recorded during the study of the medial plantar nerve by the orthodromic method in healthy adults.Materials and methods. 126 sensitive fibers of the medial plantar nerves were studied on the Dantec Keypoint G4 device (Denmark) in 63 healthy individuals (31 men and 32 women; age from 20 to 80 years). 3 groups were identified taking into account age: group 1 included healthy people aged 20 to 39 years (n = 23); group 2 consisted of people aged 40 to 60 years (n = 20); and 3 – older than 60 years (n = 20). The parameters of the sensory potential of the medial plantar nerve are analyzed.Results. The sensory potential in the study of the sensitive portion of the medial plantar nerve was registered in all 126 healthy subjects. Comparative statistical analysis did not demonstrate significant differences between groups 1–3 in the values of such parameters of the sensory potential as the latency of the onset, the duration of the negative phase and the rate of propagation of excitation. At the same time, in groups 2 and 3, the magnitude of the amplitude from peak to peak of the sensory potential was significantly lower compared to group 1, and averaged 8.92 and 7.86 MV, respectively.Conclusion. Knowledge of the regulatory parameters will allow expanding the use of electroneuromyography of the sensitive portion of the medial plantar nerve in clinical and research practice.

Objective: linguistic and cultural adaptation of the original version of Berg Balance Scale (BBS) and assessment its psychometric properties.Patients and methods. The staff of the Validation Center of International Scales and Questionnaires of the Research Center of Neurology received consent from Katherine Berg to validate the BSS in Russia. We carried out the linguocultural ratification during the validation study and prepared a Russian version of the scale. To assess the psychometric properties of the scale (reliability, validity, and sensitivity), we evaluated 55 patients (30 females and 25 males) aged 22–88 years with different neurological disorders (vascular and demyelinating diseases of the central nervous system, peripheral neuropathy, and movement disorders). We analyzed the differences of the total BBS score and the number of patients with high and low risk of falls at the end of rehabilitation compared to baseline to assess the dynamics of changes.Results and discussion. We successfully performed the translation and linguocultural adaptation of the BBS. The scale represents a high level of validity (expert score: 8.6 out of 10 points), reliability (Pearson's correlation coefficient r=0.98, р

INTRODUCTION Determination of the degree of depression of consciousness in patients with brain damage upon admission to the intensive care unit and intensive therapy is a primary task. In order to carry out a quick and at the same time sufficiently complete assessment of this kind, in 1974 neurosurgeons Graham Teasdale and Bryan Jennett from the University of Glasgow developed an algorithm consisting of a sequential series of tests in the form of eye opening, speech and motor responses, called Glasgow Coma Scale. This scale has received worldwide recognition and for many decades has been the main one for determining the state of consciousness in the most severe patients with brain damage. The absence of a validated version of this scale complicates its application in Russia, and the use of currently available versions that have not passed all the necessary stages of validation distorts the originally intended meaning of the scale and does not allow obtaining reliable clinical results when examining patients with acute impairment of consciousness.AIM OF STUDY Development of the official Russian­language version of the Glasgow Coma Scale, taking into account linguistic and cultural characteristics (1st stage of the validation study).MATERIAL AND METHODS The staff of Center for Validation of Health Status Questionnaires and Scales in Russia, Research Center of Neurology obtained consent from G. Teasdale to validate Glasgow Coma Scale in Russia. Two Russian­speaking professional certified translators in the field of medicine performed a direct translation of the original English­language scale, and a reverse translation was carried out by native speakers with a medical education. Pilot study was performed in 15 patients with acute impairment of consciousness, two meetings of the expert committee were held (before and after pilot study).RESULTS Based on the results of the first meeting of the expert commission, a linguistic and cultural adaptation of the text of the scale was carried out. During the pilot testing of the researchers did not have difficulties in understanding and interpreting instructions. As a result, the second meeting of the expert commission was held and the final Russian­language version was approved, which is presented in this article and is available on the website of Center for Validation of Health Status Questionnaires and Scales in Russia, Research Center of Neurology.CONCLUSION For the first time, the Russian language version of the Glasgow Coma Scale was officially presented and recommended for use both in clinical and research practice in Russia and other Russian speaking countries. The next publication will highlight the result of assessing the psychometric properties (reproducibility, inter­expert agreement and sensitivity) of the Russian­language version of the scale.

Patients with epilepsy may require a neurorehabilitation aid particularly due to developing motor alterations related to stroke, sequelae of traumatic brain injury, multiple sclerosis and other brain damage. Modern neurorehabilitation approaches directly or indirectly affect neuroplastic processes altering cerebral cortex excitability, stimulate the afferentation systems, and result in fatigue and may act as factors provoking seizures or aggravated epilepsy. In addition, developing seizures may temporarily coincide but unrelated to the neurorehabilitation activities: e.g., while omitting antiepileptic drug administration, sleep deprivation or long-term stress occurring to patient etc. Here we present a review on recent studies aimed at investigating epilepsy triggers by aligning them with the factors of interventions used in motor rehabilitation. We also emphasize the safety data for routine use of the main neurorehabilitation methods as well as propose actions to reduce the risk of developing epileptic seizure.

Introduction. The heterogeneity of the forms and severity of Guillain-Barre syndrome explains the variability of recovery: from rapid and complete (in most cases) to slow with the development of persistent residual deficiency (rarely). It is unclear how effective the Erasmus Guillain–Barre syndrome Outcome Scores and its modified version are for different forms of the disease.The aim of the study – to demonstrate the features of recovery in acute motor axonal neuropathy with conduction blocks on the example of 2 clinical cases; to show the possibilities of Erasmus Guillain–Barre syndrome Outcome Scores and its modified version in predicting recovery in this form of the disease.Materials and methods. Data from 2 patients with acute motor axonal neuropathy with motor conduction blocks were retrospectively analyzed. Calculation of the score and assessment of the prognosis of walking recovery by 6 months from the onset of the disease were performed using the online calculator International Guillain-Barre syndrome Outcome Study Prognosis tool in the acute period.Results. In both patients, the forecast of recovery of walking by half a year from the onset of the disease on the Erasmus Guillain–Barre syndrome Outcome Scores and modified Erasmus Guillain–Barre syndrome Outcome Scores scales in the acute period was erroneous. In the first case, the total score on the Erasmus Guillain–Barre syndrome Outcome Scores and its modification in the acute period was 5 and 10 points respectively (poor prognosis), which foreshadowed a long rehabilitation process and incomplete recovery. However, the regression of disorders was dramatic and complete, and by the second month of the disease, only minimal motor disorders remained. In the second patient, on the contrary, the total Erasmus Guillain–Barre syndrome Outcome Scores and its modification during the period of increasing symptoms was 3 and 7 points respectively (good prognosis), while recovery was delayed – only by 5 months from the onset of the disease, the ability to move with support was restored.Conclusion. The Guillain–Barre syndrome is a disease with a favorable prognosis for recovery. However, the prediction of regression of motor disorders should be approached carefully, because in some cases, generally accepted criteria and prognostic scales may not work. Acute motor axonal neuropathy with conduction blocks is a unique form of the disease that has pathophysiological and clinical-neurophysiological features, which should be taken into account when managing this category of patients.

Introduction. Atypical variants of chronic inflammatory demyelinating polyneuropathy are marked by its clinical heterogeneity and variable disease course.Aim of the study. To describe clinical, anamnestic and neurophysiological features of patients with atypical variants of chronic inflammatory demyelinating polyneuropathy, characterized by benign disease course, minimal motor involvement and not required pathogenic therapy.Materials and methods. 8 patients (7 men (87 %) and 1 woman (13 %) at the age of 52–77 years) with atypical variants of chronic inflammatory demyelinating polyneuropathy were analyzed: 5 patients (62.5 %) with asymmetric variant – multifocal acquired demyelinating sensorimotor neuropathy and 3 patients (37.5 %) with sensory variant. All patients were observed at the Research Center of Neurology for the period of 2016– 2019. In each patient the proper clinical and laboratory evaluation was performed along with nerve conduction study and nerve ultrasound.Results. The disease duration at the time of first visit was 1–8 years. By INCAT disability score 3 (37.5 %) patients had 0 points (normal), 3 (37.5 %) patients – 1 point and 1 patient had 2 (25 %) points. Nerve conduction study showed multifocal, asymmetric demyelinating changes in motor nerves. For the whole period of observation all patients were stable, so no one required pathogenic therapy.Conclusion. Chronic inflammatory demyelinating polyneuropathy is a clinically heterogeneous disorder, required clinical suspicion in all patients over 50 years with features of multiple nerve involvement; nerve conduction study helps to detect typical changes, including subclinical ones. The primary strategy of management typical and atypical disease variants in stable course and minimal symptoms is a case follow-up with precise assessment of advantages and disadvantages of pathogenic therapy.

Сhronic inflammatory demyelinating polyneuropathy (CIDP) is the most prevalent acquired dysimmune neuropathy with clinical picture of symmetric motor and sensory disturbances. Since the first description of CIDP, many atypical variants have been described, which may reach up to 50% of cases. Diagnosis of atypical CIDP may be challenging due to different clinical presentation and treatment response. Current researches improve our knowledge about dysimmune neuropathies and highlight the importance of its classification. Nowadays CIDP is considered as a spectrum of disorders rather than a separate disease entity. Up to date, more than 15 diagnostic criteria have been proposed reflecting the complexity of СIDP diagnosis. Many polyneuropathies may mimic CIDP, therefore CIDP is frequently a diagnosis of exclusion. The key diagnostic instrument is electroneuromyography; however, the issues related to results misinterpretation and some technical aspects are the most important in CIDP misdiagnosis. Supportive instrumental and laboratory methods have variable sensitivity and specificity, making challenging CIDP diagnosis, especially its atypical forms. The importance of an early and accurate diagnosis of CIDP is supported by an effective pathogenic treatment, which affects the patient's prognosis and level of disability.

Background. Carpal tunnel syndrome is the most common tunnel neuropathy in which the median nerve is compressed at the level of the wrist in the carpal canal. Treatment of carpal tunnel syndrome can be conservative and surgical. Surgical treatment is indicated in case of ineffective conservative treatment. However, the strategy of managing patients with carpal tunnel syndrome in the postoperative period has not yet been determined; there is no clear understanding of the effectiveness and necessity of rehabilitation in the early and long-term postoperative periods.Aim. Follow-up the patients after decompression of the median nerve in the late (up to 3 weeks after surgery) and long-term (3 weeks after surgery) postoperative periods to assess the effectiveness of different methods of rehabilitation.Materials and methods. A randomized controlled study included 108 cases of idiopathic carpal tunnel syndrome (unilateral and bilateral). After surgery, the patients were divided into three groups: the restorative treatment group using magnetic therapy, the kinesiotherapy group, and the control group. Clinical, neurophysiological and ultrasound monitoring was carried out for six months.Results. Patients of all the groups showed similar improvement in the most of the analyzed parameters, without any significant difference.Conclusion. Thus, according to the results of a comprehensive study, it is evident that early diagnosis of carpal tunnel syndrome and a high-quality surgical decompression of the median nerve with a complete dissection of the flexor retinaculum of the hand guarantee improvement within six months or later after surgical treatment without additional rehabilitation measures.

High-frequency repetitive transcranial magnetic stimulation (rTMS) is approved by the Food and Drug Administration (FDA) for the treatment of pharmacotherapy-resistant depressive disorders (DD). Individual target determination for stimulation can be one of the approaches that can increase the efficiency of the technique. Objective: to compare of the effectiveness and tolerability of standard and personalized rTMS protocols.Patients and methods. The investigation enrolled 30 patients with pharmacotherapy-resistant DD who were pseudo-randomized into two groups matched for age, gender, and episode severity. In the study group, the target was located at a point within the left dorsolateral prefrontal cortex (DLPFC) with maximum negative functional connectivity of the subgenual cingulate cortex. In the control group, the stimulation point was 5 cm anterior to the primary motor cortex (hand area). All the patients underwent 20 sessions of high-frequency rTMS DLPFC. For clinical evaluation, the investigators used the Beck Depression Inventory (BDI) and the 36-item Short Form Health Survey (SF-36) questionnaire before and after 10 and 20 rTMS sessions, respectively. Tolerability was assessed using the standardized questionnaires during and within 24 hours after each session. Results and discussion. The study group showed a significant reduction in BDI scores and an increase in the SF-36 (“Mental Health” section) scores after both 10 and 20 rTMS sessions; the control group had those only after 20 sessions. The two groups exhibited no significant differences in the reduction of BDI scores before and after 10 and 20 sessions, respectively. The investigation can be considered to be pilot in searching for algorithms to enhance the efficiency of rTMS DLPFC in pharmacotherapy-resistant depression using the algorithm for personification of target selection. It demonstrated the more rapid onset of a clinical effect in the study group patients. No serious adverse events were reported. The patients had dizziness, headache, and contraction of the facial muscles during the session; headache and mood changes within 24 hours after it. Conclusion. In both groups, rTMS was satisfactorily tolerated and effective; however, personalized target selection accelerated the onset of a clinical effect.

This literature review provides a range of neurological and other organic causes of brachialgia. Features of pain syndrome in different medical conditions are described.

Objective: to investigate changes in grey matter volume in patients with classical amyotrophic lateral sclerosis (ALS) and lower motor neuron syndrome (LMNS) with voxel-based morphometry (VBM).Material and methods. 30 patients with classical ALS, 22 patients with LMNS and 23 age and gender matched healthy controls were enrolled in this study. All participants underwent a T1MPR (multiplanar reconstruction) magnetic resonance imaging with post-processing included spatial normalization, segmentation and smoothing. VBM was used to investigate changes in grey matter volume across the groups.Results. There was a significant decrease in grey matter volume of middle part of left pre- and postcentral gyri, middle part of right precentral gyrus, right and left occipital lobes in patients with classical ALS compared to healthy subjects. There was no difference in grey matter volume between patients with LMNS and healthy controls. Patients with classical ALS showed a significant decrease in grey matter volume of middle part of left preand postcentral gyri, upper part of left precentral gyrus, middle and upper parts of right precentral gyrus, right and left occipital lobes compared to patients with LMNS. There was no significant correlation between grey matter volume and clinical findings in patients with ALS and LMNS.Conclusion. VBM reveals a decrease in grey matter volume of motor and nonmotor brain regions in patients with classical ALS, but not in patients with LMNS.

Objective: to develop a Russian version of the modified Rankin Scale (mRS) and to assess its psychometric properties.Patients and methods. The investigation involved 50 patients (25 women, 25 men) over 18 years of age (mean age, 56 years) with an acute cerebrovascular accident diagnosis. During the validation procedure, the authors carried out the linguocultural ratification of mRS, prepared its Russian-language version, and then assessed its psychometric properties (reliability, sensitivity, validity).Results and discussion. Translation and linguistic adaptation of mRS was successfully completed. The results obtained in assessing the psychometric properties of the developed Russian version of the scale reflect its high reliability and the valid and reliable indicators of its sensitivity. A statistical study of constructive and content validity also determined the high significance of differences.Conclusion. The investigation has resulted in the official Russian version of mRS, which is recommended for use by neurologists and rehabilitation specialists in both daily clinical practice and in clinical trials.

Intravenous high-dose immunotherapy is one of the highly effective proven treatments for a number of autoimmune diseases of the nervous system. Nowadays there is enough knowledge about the spectrum of side effects and approaches to their prevention and monitoring. Most of them are leveled by improving the technology of the production process, but anaphylactic reactions remain one of the uncontrolled, albeit rare, undesirable reactions. The development of allergic reactions is associated with the presence of antibodies to immunoglobulin class A (IgA) in the patient, which is manifested by a decrease in the level of IgA during routine immunological examination. The article provides a review of the literature on the prevalence of IgA deficiency, its causes. A modern view of the need for routine testing of IgA level before the course of intravenous immunotherapy, approaches to reduce the risk of developing serious adverse reactions in such cases is described. A clinical case of treatment of a patient with chronic inflammatory demyelinating polyneuropathy and a reduced level of IgA is presented.

Background. Objectivization of the characteristics of neuropathic pain is an important component of differential diagnosis in various diseases. Scales and questionnaires are topical diagnostic tools for assessing subjective symptoms for patients and health professionals.Objective to development and validation of the Russian version of the the Leeds Assessment of Neuropathic Symptoms and Signs Pain Scale (LANSS).Materials and methods. The study included two stages: linguocultural ratification and assessment of the psychometric properties of the developed Russian version.Results. The study showed a high level of reliability, validity and sensitivity of the developed Russian-language version of the scale. Conclusion. By the results of the study, the official validated Russian-language version of the scale is presented and recommended for use in work with Russian-speaking patients.

International scales and questionnaires have become widespread in Russian neurology. Validation is a procedure of top priority necessary before applying this kind of diagnostic instrument in Russian-speaking population. In this article, validation of the Boston Carpal Tunnel Questionnaire (BCTQ) intended for patients with this disease is described. Use of validated Russian version would allow to objectively evaluate severity of clinical manifestations of carpal tunnel syndrome and follow patient dynamics. We present the official BCTQ version recommended for use in Russia, as well as data that showed high sensitivity and reliability of this instrument for clinical evaluation of carpal tunnel syndrome.

Amyotrophic lateral sclerosis (ALS) is a clinically and pathogenically heterogenic disease. Clinical heterogeneity refers to the initial localization of the pathological process, the variability of the combination of signs of upper and lower motor neuronal damage, the age of the onset, the rate of progression, hereditary predisposition, and the presence of non-motor clinical manifestations. A large number of forms of the disease are described (primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, flail-arm syndrome, and others), which in some cases can be considered as independent diseases due to the localization of the pathological process, disease course and prognosis. In recent years, convincing evidences of the genetic and pathogenetic heterogeneity of ALS have also been obtained. Large heterogeneity makes it difficult to create a unified classification of ALS. Formal classifications used, for example, in the diagnostic criteria of El Escorial or the International Classification of Diseases of the 10th revision, do not take into account many of the important features of the disease. The article describes the clinical heterogeneity of ALS. The proposed classifications are compared. A new classification system that taking into account various features of the disease and can improves understanding of the diversity of ALS is described.

Transcranial magnetic stimulation (TMS) is a rapidly developing method of neuromodulation. The use of TMS has increased significantly in both research and clinical practice. This allows not only to better understand this method, but also assess possible risks and consequences for both healthy individuals and patients. In 1998 and 2009 safety, ethical considerations, and application guidelines for the use of TMS in clinical practice and research were published. These recommendations are now the basis for safe application of the method in clinical practice and research. Safety of brain stimulation includes several aspects: the prevention and treatment of adverse effects, the strategy of patient and stimulation protocols selection, as well as safety and monitoring procedures. The most common adverse effects of TMS include headache and neck pain, syncope, transient hearing impairment. The risk of epileptic seizureis extremely low and can be minimized by careful selection of patients and the use of safe stimulation protocols. Careful selection of patients is important, taking into account a large number of factors that influence the risk of adverse effects. These factors are considered in the questionnaires to identify limitations and absolute or relative contraindications to TMS. Another important part of TMS safety is the choice of the stimulation protocol and parameters such as intensity, frequency, duration of one train of stimuli, and the interstimulus interval. Currently, the recommended limits of stimulation parameters are covered in the safety guidelines. It is also necessary to follow the procedure, including the monitoring the patient's condition during TMS and the providing qualified assistance in case of adverse effects.

Glucocorticoids (GCs) are often prescribed for neurologic patients. Autoimmune diseases of central and peripheral nervous system, neuromuscular disorders are associated with disability and life-threatening complications, and require long-term high-dosage administration of GCs. GC-therapy is a crucial decision to be made by a neurologist in collaboration with a patient, and requires strict observance of clear guidelines on the prevention, diagnosis and treatment of possible complications. One of the most frequent complications of GCs is osteoporosis. According to the Russian and international guidelines, all the patients must undergo preliminary and follow-up laboratory and instrumental examination, as well as administration of calcium and vitamin D during the whole period of GC-therapy. In case of a high fracture risk or if osteoporosis is diagnosed, antiresorptive therapy is indicated.

The consequences of cerebrovascular diseases significantly reducethe quality of life of patients. Recovery of basic motor skills,such as the ability to reach the object, manipulate it, and coordinatethe movements of two hands is one of the top-prioritytasks in restoring patients living and social activity. Training inthe environment as close to the real one as possible, active involvementof the patient, as well as interactive feedback, whichallows patients to control the correct execution of motor tasksand adjust their own efforts are required for successful motorrecovery. The development of computer technology enabled theimprovement of conventional approaches to rehabilitation ofstroke patients. Virtual reality (VR) is extensively used to meetthese conditions and achieve successful targeted training for aspecific motor task. The technical basis of VR includes computermodeling and computer simulation, as well as three-dimensionalvisualization, which provides realistic visualization of movementson the screen. These technologies enable reconstructionof the appropriate working space for motor skill training, andprovide interactive feedback and high intensity of rehabilitation.This article provides information about the development of thesetechnologies in the field of motor rehabilitation of upper extremityfunction, comparative analysis of systems that are currentlyused or being developed, and prospects of VR development inneurorehabilitation.

Navigated rTMS is characterized by a great accuracy of localizingthe stimulated area, which opens up new prospects forneuroplasticity modulation. We conducted an interventionalrandomized double-blind sham-controlled study to evaluate theeffectiveness of navigated rTMS in post-stroke rehabilitation.We found that low-frequency stimulation reliably reduces severityof motor deficit and increases the Barthel Index of Activitiesof Daily Living. High-frequency stimulation reduces spasticity(Ashworth Scale) and severity of central post-stroke pain syndrome.Navigated repetitive transcranial magnetic stimulationeffectively supplements the conventional rehabilitation of poststrokepatients. Compared to routine TMS, the use of navigatedrTMS enables effective control of the neuroplastic processes andprovides new opportunities in development of personalized neurorehabilitationprograms.

Transcranial magnetic stimulation (TMS) new method based on the neurons excitation by an alternating magnetic field. According literature high-frequency TMS could be effectiveness method in the treatment of drug-resistant central post-stroke pain (CPSP). The presented cases report describes the clinical observation of two patients with CPSP, with different localization and volume of thelesion. TMS was applied in the mode of high-frequency stimulation(10 Hz) to the primary motor cortex of the affected hemisphere.After stimulation, we record a significant reduction of painon a visual analog scale (VAS). It is shown that after the end ofTMS pain gradually returned to previous levels after 3-4 months.

We present the results of ultrasound study of 21 patients withchronic inflammatory demyelinating polyneuropathy (CIDP).In 100% cases were observed changes of the ultrasound structureof peripheral nerves. The ultrasound abnormalities were mostprominent in the peripheral nerves of lower extremities. Thechanges of both tibial and ulnar nerves morphology seem to bethe most valuable for the diagnostics. The cross-sectional area ofnerves does not correlate with the duration of disease that hindersthe performance of the ultrasound for the purpose of follow-up.

We carried out an examination of 74 patients who have had,from 3 months to 36 years ago, GuillainBarre syndrome (GBS)with severe impairmenet of motor functions. The average follow-up period was 2.3 years. Fifty-eight subjects had acuteinflammatory demyelinating polyneuropathy (AIDP), and theremaining 16 patients had axonal forms. In the structure ofresidual neurologic deficits of AIDP patients at all stages of earlyrecovery period (follow-up period 1 year) and in late (1 year)period sensory disorders and pain syndrome prevailed. Themajority of AIDP patients (90%) began to walk with supportafter 1 month of onset, and occasionally (3%) six months later. Patients with axonal forms could stand only after 6 months(88%) and later (12% patients up to 12 months). After 1 yearof onset, all AIDP patients walked unassisted, and most of themreturn to their job. Over the equal follow-up period every thirdpatient with an axonal form remained dependent in their dailyliving activities. This confirms that GBS is a disease with a favorableprognosis: even in severe cases most patients resumed walkingafter half a year, regardless of the disease form. Axonal formsshow lower recovery rate and greater severity of residual symptomscompared to AIDP, which affects quality of life in respectivepatients.

Testing for IgG+IgM autoantibodies to gangliosides of peripheralnerves (asialo-GM1, GM1, GM2, GD1a, GD1a, GD1b, GQ1b)was performed in 95 patients with GuillainBarr syndrome (GBS)in Moscow. In 70 patients (74%) acute inflammatory demyelinatingpolyneuropathy (AIDP) was diagnosed, and in 25 patients (26%) either acute motor axonal neuropathy (AMAN) or acute motorsensoryneuropathy (AMSAN). The role of antibodies to gangliosidesfor prognosis of the disease course and response to pathogenictreatment was assessed. Antibodies to gangliosides were found in55 patients (57.9%) more often in patients with AMAN/AMSAN(p0.05). The whole range of antibodies was found both in patientswith AIDP and AMAN/AMSAN. Anti-GM1 were found in bothsubtypes of GBS. Anti-GD1b were associated withAMAN/AMSAN (p0.05). There was a correlation between anti-GM1 and diarrhea (p0.05), anti-asialo-GM1 and campylobacteriosis(p0.05). There wasnt found significant correlation betweenanti-GD1 and axonal subtypes, diarrhea in anamnesis, and campylobacteriosis.Association of severe GBS and elderly age, camplylobacteriosisand axonal subtypes was confirmed. AMAN/AMSANsubtypes, severity of the disease, necessity in mechanical ventilationwere characterized by insufficient efficacy of pathogenic therapy.The same factors and elderly age were associated with unfavorableprognosis in terms of absence of unassisted walking in 6 and12months. Anti-GD1a were associated with sever course of the disease,including necessity in mechanical ventilation. Immunologicfactors are not sufficient in prognosis of the treatment efficacy.Antibodies to GM1 were associated with unfavorable prognosis withabsence of unassisted walking in 6 months. Clinical and neurophysiologicaldata is crucial for making diagnosis and prognosisof the GBS course. Testing for autoantibodies to gangliosidescould be helpful in diagnostics of GBS. It is possible to use thattest for revealing axonal type of the disease (anti-GD1b), prognosticationof severe course of GBS (anti-GD1a) and unfavorablerehabilitation of unassisted walking.