Хроническая воспалительная демиелинизирующая полинейропатия у взрослых: принципы диагностики и терапия первой линии

Chronic inflammatory demyelinating polyneuropathy (CIDP) is among the key reasons of chronic polyneuropathies in adults. Diagnostic algorithm of CIDP in adults is presented. Diagnosis of CIDP is based on clinical and electrodiagnostic criteria of European Federation of Neurological Societies/Peripheral Nervous System in 2010. Principles of CIDP treatment are discussed, including modern trends of standard and 10 % IVIG solutions. Хроническая воспалительная демиелинизирующая полинейропатия (ХДВП) входит в структуру причин хронических полинейропатий у взрослых. Представлен алгоритм диагностики ХВДП у взрослых, который основывается на клинических и электрофизиологических критериях, предложенных Европейской федерацией неврологических обществ/периферической нервной системы (European Federation of Neurological Societies/Peripheral Nervous System) в 2010 г. Рассмотрены принципы лечения больных ХВДП и место внутривенной иммунотерапии в современных терапевтических подходах, включая использование 10 % растворов. 

Efficacy of local injection therapy versus wrist splinting in moderate idiopathic carpal tunnel syndrome: the results of a randomized clinical trial

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 <0.0001). A significant change in the electrophysiological parameters of the median nerve (a decrease the distal motor and sensory latency, an increase in the Mand S‑response amplitude, an increase in sensory conduction velocity on the hand) and a significant decrease in the cross‑sectional area of the median nerve at the level of the pisiform bone was noted only in the local injection therapy group. The average duration of the local injection therapy clinical effect surpassed the splinting one and reached 4 months.Conclusion. After 1 month from the start of the treatment, the local injection therapy demonstrated a higher clinical efficacy compared to the wrist splinting, the average duration of the effect reached 4 months. This conservative treatment method is preferred for the patients with moderate idiopathic carpal tunnel syndrome

Validation Chronic Acquired Polyneuropathy – Patient Reported Index (CAP-PRI) in Russia for CIDP patients

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

Global, regional, and national comparative risk assessment of 84 behavioural, environmental and occupational, and metabolic risks or clusters of risks for 195 countries and territories, 1990–2017 : a systematic analysis for the Global Burden of Disease Study 2017

Background: The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2017 comparative risk assessment (CRA) is a comprehensive approach to risk factor quantification that offers a useful tool for synthesising evidence on risks and risk outcome associations. With each annual GBD study, we update the GBD CRA to incorporate improved methods, new risks and risk outcome pairs, and new data on risk exposure levels and risk outcome associations. Methods: We used the CRA framework developed for previous iterations of GBD to estimate levels and trends in exposure, attributable deaths, and attributable disability-adjusted life-years (DALYs), by age group, sex, year, and location for 84 behavioural, environmental and occupational, and metabolic risks or groups of risks from 1990 to 2017. This study included 476 risk outcome pairs that met the GBD study criteria for convincing or probable evidence of causation. We extracted relative risk and exposure estimates from 46 749 randomised controlled trials, cohort studies, household surveys, census data, satellite data, and other sources. We used statistical models to pool data, adjust for bias, and incorporate covariates. Using the counterfactual scenario of theoretical minimum risk exposure level (TMREL), we estimated the portion of deaths and DALYs that could be attributed to a given risk. We explored the relationship between development and risk exposure by modelling the relationship between the Socio-demographic Index (SDI) and risk-weighted exposure prevalence and estimated expected levels of exposure and risk-attributable burden by SDI. Finally, we explored temporal changes in risk-attributable DALYs by decomposing those changes into six main component drivers of change as follows: (1) population growth; (2) changes in population age structures; (3) changes in exposure to environmental and occupational risks; (4) changes in exposure to behavioural risks; (5) changes in exposure to metabolic risks; and (6) changes due to all other factors, approximated as the risk-deleted death and DALY rates, where the risk-deleted rate is the rate that would be observed had we reduced the exposure levels to the TMREL for all risk factors included in GBD 2017. Findings: In 2017,34.1 million (95% uncertainty interval [UI] 33.3-35.0) deaths and 121 billion (144-1.28) DALYs were attributable to GBD risk factors. Globally, 61.0% (59.6-62.4) of deaths and 48.3% (46.3-50.2) of DALYs were attributed to the GBD 2017 risk factors. When ranked by risk-attributable DALYs, high systolic blood pressure (SBP) was the leading risk factor, accounting for 10.4 million (9.39-11.5) deaths and 218 million (198-237) DALYs, followed by smoking (7.10 million [6.83-7.37] deaths and 182 million [173-193] DALYs), high fasting plasma glucose (6.53 million [5.23-8.23] deaths and 171 million [144-201] DALYs), high body-mass index (BMI; 4.72 million [2.99-6.70] deaths and 148 million [98.6-202] DALYs), and short gestation for birthweight (1.43 million [1.36-1.51] deaths and 139 million [131-147] DALYs). In total, risk-attributable DALYs declined by 4.9% (3.3-6.5) between 2007 and 2017. In the absence of demographic changes (ie, population growth and ageing), changes in risk exposure and risk-deleted DALYs would have led to a 23.5% decline in DALYs during that period. Conversely, in the absence of changes in risk exposure and risk-deleted DALYs, demographic changes would have led to an 18.6% increase in DALYs during that period. The ratios of observed risk exposure levels to exposure levels expected based on SDI (O/E ratios) increased globally for unsafe drinking water and household air pollution between 1990 and 2017. This result suggests that development is occurring more rapidly than are changes in the underlying risk structure in a population. Conversely, nearly universal declines in O/E ratios for smoking and alcohol use indicate that, for a given SDI, exposure to these risks is declining. In 2017, the leading Level 4 risk factor for age-standardised DALY rates was high SBP in four super-regions: central Europe, eastern Europe, and central Asia; north Africa and Middle East; south Asia; and southeast Asia, east Asia, and Oceania. The leading risk factor in the high-income super-region was smoking, in Latin America and Caribbean was high BMI, and in sub-Saharan Africa was unsafe sex. O/E ratios for unsafe sex in sub-Saharan Africa were notably high, and those for alcohol use in north Africa and the Middle East were notably low. Interpretation: By quantifying levels and trends in exposures to risk factors and the resulting disease burden, this assessment offers insight into where past policy and programme efforts might have been successful and highlights current priorities for public health action. Decreases in behavioural, environmental, and occupational risks have largely offset the effects of population growth and ageing, in relation to trends in absolute burden. Conversely, the combination of increasing metabolic risks and population ageing will probably continue to drive the increasing trends in non-communicable diseases at the global level, which presents both a public health challenge and opportunity. We see considerable spatiotemporal heterogeneity in levels of risk exposure and risk-attributable burden. Although levels of development underlie some of this heterogeneity, O/E ratios show risks for which countries are overperforming or underperforming relative to their level of development. As such, these ratios provide a benchmarking tool to help to focus local decision making. Our findings reinforce the importance of both risk exposure monitoring and epidemiological research to assess causal connections between risks and health outcomes, and they highlight the usefulness of the GBD study in synthesising data to draw comprehensive and robust conclusions that help to inform good policy and strategic health planning

Validation of the Medical Research Council sum score (MRCss) for use in Russian-speaking patients with chronic inflammatory demyelinating polyneuropathy

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

Validation of the Neuropathy Impairment Score (NIS) for use in Russian-speaking patients with chronic inflammatory demyelinating polyneuropathy

Background. A clinical application of scales and questionnaires is essential for the objective evaluation of treatment response, disease course, quality of life and the disability level in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Neuropathy Impairment Score (NIS) has become widespread in epidemiological and clinical studies of various polyneuropathies, including CIDP. However, its application in Russia is limited by the absence of its validated version for Russian-speaking patients. Aim. To develop the Russian validated version of the NIS for its application in patients with CIDP. Materials and methods. The study involved 50 patients with CIDP (25 with multifocal variant of CIDP (Lewis–Sumner syndrome) and 25 with typical CIDP). The validation process included two stages: translation and linguocultural ratification of the scale according to the standard protocol and an assessment of its psychometric properties, such as reliability, validity and sensitivity. Results. The developed Russian version of the NIS demonstrated the high level of reliability, sensitivity and validity. Conclusion. The validated Russian version of the NIS can be recommended for application in research and clinical practice

Эффективность локальной инъекционной терапии и ортезирования лучезапястного сустава при идиопатическом карпальном туннельном синдроме средней степени тяжести: результаты рандомизированного клинического исследования

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 <0.0001). A significant change in the electrophysiological parameters of the median nerve (a decrease the distal motor and sensory latency, an increase in the Mand S‑response amplitude, an increase in sensory conduction velocity on the hand) and a significant decrease in the cross‑sectional area of the median nerve at the level of the pisiform bone was noted only in the local injection therapy group. The average duration of the local injection therapy clinical effect surpassed the splinting one and reached 4 months.Conclusion. After 1 month from the start of the treatment, the local injection therapy demonstrated a higher clinical efficacy compared to the wrist splinting, the average duration of the effect reached 4 months. This conservative treatment method is preferred for the patients with moderate idiopathic carpal tunnel syndrome.Введение. Карпальный туннельный синдром (КТС) является наиболее частой компрессионной мононевропатией. Вопросы предпочтительного метода консервативного лечения остаются актуальными, особенно для пациентов со средней степенью тяжести заболевания.Цель исследования – выполнить сравнительное исследование эффективности локальной инъекционной терапии и ортезирования лучезапястного сустава при идиопатическом КТС средней степени тяжести, оценить продолжительность клинического эффекта в периоде наблюдения до 6 мес.Материалы и методы. В исследование вошло 105 случаев идиопатического КТС средней степени тяжести. Пациенты были рандомизированы в 2 группы в зависимости от проводимого лечения: основная группа (n = 54) получила 1 или 2 инъекции с глюкокортикостероидом (бетаметазон 5 мг + 2 мг/1,0 мл) и лидокаином 2 % 1,0 мл под ультразвуковым контролем в запястный канал; контрольной группе (n = 51) было назначено ортезирование лучезапястного сустава в ночные часы в течение 1 мес. Первичным критерием эффективности терапии была оценка по клиническим шкалам (SSS, FSS, LANSS, визуально‑аналоговая шкала боли) через 1 мес от начала лечения, вторичным критерием эффективности – оценка изменений электрофизиологических и нейросонографических параметров.Результаты. Через 1 мес после лечения оценки по SSS, FSS, LANSS и визуально‑аналоговой шкале боли в группе инъекционной терапии оказались значимо ниже, чем в группе ортезирования (p <0,0001). Достоверное изменение электрофизиологических показателей срединного нерва (укорочение дистальной латентности Ми S‑ответа, увеличение амплитуды Ми S‑ответа, увеличение сенсорной скорости распространения возбуждения на кисти) и значимое уменьшение площади поперечного сечения срединного нерва на уровне гороховидной кости были отмечены только в группе локальной инъекционной терапии. Средняя продолжительность клинического эффекта локальной инъекционной терапии оказалась больше, чем ортезирования, и составила 4 мес.Заключение. Через 1 мес от начала лечения локальная инъекционная терапия продемонстрировала более высокую клиническую эффективность по сравнению с ортезированием лучезапястного сустава, продолжительность эффекта составила в среднем 4 мес. Данный метод консервативного лечения является предпочтительным для пациентов с идиопатическим КТС средней степени тяжести

Хронические нарушения сознания: клинические рекомендации Общероссийской общественной организации «Федерация анестезиологов и реаниматологов»

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

Валидация шкалы суммарной оценки мышечной силы (MRC sum score) для использования у русскоязычных пациентов с хронической воспалительной демиелинизирующей полинейропатией

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. Введение. Использование международных шкал и опросников является неотъемлемой частью работы с пациентами с хронической воспалительной демиелинизирующей полинейропатией и позволяет объективно оценивать динамику состояния пациента, ответ на терапию, степень инвалидизации и качество жизни. Шкала Medical Research Council для оценки мышечной силы (MRC Muscle Scale) и ее модификация Medical Research Council sum score (MRCss) широко используются у пациентов с поражением периферического нейромоторного аппарата для оценки степени выраженности двигательного дефицита. Однако применение шкалы в Российской Федерации ограничено отсутствием русскоязычной, валидированной версии.Цель исследования – провести валидацию шкалы MRCss у пациентов с хронической воспалительной демиелинизирующей полинейропатией с разработкой русскоязычной версииМатериалы и методы. В исследование включено 50 пациентов с хронической воспалительной демиелинизирующей полинейропатией (25 пациентов с типичным вариантом заболевания, 25 – с синдромом Льюиса–Самнера). Первым этапом проведена лингвистическая ратификация оригинальной англоязычной версии шкалы согласно общепринятым рекомендациям. На 2-м этапе валидации проведена оценка психометрических показателей разработанного русскоязычного варианта шкалы: надежности, содержательной валидности, чувствительности.Результаты. Получена русскоязычная версия шкалы MRCss, которая продемонстрировала высокий уровень надежности, чувствительности и валидности.Заключение. По результатам проведенной работы представлена валидированная, русскоязычная версия шкалы MRCss, рекомендованная для клинической и научно-исследовательской работы у русскоязычных пациентов.