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. 

Hypertensive crisis as cerebrovascular disease risk factor

Objective. To assess main epidemiological indicators for hypertensive crises (HC) in the population of productive age and to study possible correlations of crisis associated arterial hypertension (AH) with clinical and instrumental phenomenon of chronic cerebrovascular diseases. Materials and methods. Data of several studies presented: cross-sectional studies of 726 people aged 35-64 and 415 people aged 40-59. Observational cohort study of 109 patients aged 57.4±5.8 with uncomplicated AH. For the detection of HC in anamnesis, there were used special criteria which widens standard HC definition for additional account of light and mild severity cases. Results. Overall AH prevalence was 45% (95% CI 41-51), in men - 48% (40.2-55.9), in women - 45% (38.4-51.6). History of HC in anamnesis was 11.8% (95% CI 9-15.2), in men 8.8% (5.4-14) and in women - 13.8% (10-18.7). Proportion of HC associated AH defined at the level of 25-30% of all AH cases. The most prevalence of HC associated AH was found in people with “high normal” (130-139/85-89) arterial pressure - 37%. Prevalence of the complaints on headaches, dizziness, poor memory and lower intellectual productivity was higher in people with HC. Chronic cerebrovascular disease was found 2-fold frequently in HC associated AH. But in generally analysis of possible correlations of HC with clinical and instrumental phenomenon of chronic cerebrovascular diseases didn’t revealed any statistically significant differences. Conclusion. HC burden for healthcare system is serious, because it is important risk factor for cerebrovascular diseases and associated with significant lowering of the quality of life. Prevalence of the HC cases with light and mild severity is underestimated. Despite that the study of the most prevalent forms of HC (rare, light and mild severity) didn’t find any associations with morphological or persistent clinical pathology, functional phenomenon were found statistically significant frequently

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

Global mortality from dementia: Application of a newmethod and results from the global burden of disease study 2019

INTRODUCTION: Dementia is currently one of the leading causes of mortality globally, and mortality due to dementia will likely increase in the future along with corresponding increases in population growth and population aging. However, large inconsistencies in coding practices in vital registration systems over time and between countries complicate the estimation of global dementia mortality. METHODS: We meta-analyzed the excess risk of death in those with dementia and multiplied these estimates by the proportion of dementia deaths occurring in those with severe, end-stage disease to calculate the total number of deaths that could be attributed to dementia. RESULTS: We estimated that there were 1.62 million (95% uncertainty interval [UI]: 0.41–4.21) deaths globally due to dementia in 2019. More dementia deaths occurred in women (1.06 million [0.27–2.71]) than men (0.56 million [0.14–1.51]), largely but not entirely due to the higher life expectancy in women (age-standardized female-to-male ratio 1.19 [1.10–1.26]). Due to population aging, there was a large increase in all-age mortality rates from dementia between 1990 and 2019 (100.1% [89.1–117.5]). In 2019, deaths due to dementia ranked seventh globally in all ages and fourth among individuals 70 and older compared to deaths from other diseases estimated in the Global Burden of Disease (GBD) study. DISCUSSION: Mortality due to dementia represents a substantial global burden, and is expected to continue to grow into the future as an older, aging population expands globally

Use of multidimensional item response theory methods for dementia prevalence prediction: an example using the Health and Retirement Survey and the Aging, Demographics, and Memory Study

Background: Data sparsity is a major limitation to estimating national and global dementia burden. Surveys with full diagnostic evaluations of dementia prevalence are prohibitively resource-intensive in many settings. However, validation samples from nationally representative surveys allow for the development of algorithms for the prediction of dementia prevalence nationally. Methods: Using cognitive testing data and data on functional limitations from Wave A (2001–2003) of the ADAMS study (n = 744) and the 2000 wave of the HRS study (n = 6358) we estimated a two-dimensional item response theory model to calculate cognition and function scores for all individuals over 70. Based on diagnostic information from the formal clinical adjudication in ADAMS, we fit a logistic regression model for the classification of dementia status using cognition and function scores and applied this algorithm to the full HRS sample to calculate dementia prevalence by age and sex. Results: Our algorithm had a cross-validated predictive accuracy of 88% (86–90), and an area under the curve of 0.97 (0.97–0.98) in ADAMS. Prevalence was higher in females than males and increased over age, with a prevalence of 4% (3–4) in individuals 70–79, 11% (9–12) in individuals 80–89 years old, and 28% (22–35) in those 90 and older. Conclusions: Our model had similar or better accuracy as compared to previously reviewed algorithms for the prediction of dementia prevalence in HRS, while utilizing more flexible methods. These methods could be more easily generalized and utilized to estimate dementia prevalence in other national surveys

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.

Global, regional, and national burden of stroke, 1990–2016: a systematic analysis for the Global Burden of Disease Study 2016

© 2019 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license Background: Stroke is a leading cause of mortality and disability worldwide and the economic costs of treatment and post-stroke care are substantial. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) provides a systematic, comparable method of quantifying health loss by disease, age, sex, year, and location to provide information to health systems and policy makers on more than 300 causes of disease and injury, including stroke. The results presented here are the estimates of burden due to overall stroke and ischaemic and haemorrhagic stroke from GBD 2016. Methods: We report estimates and corresponding uncertainty intervals (UIs), from 1990 to 2016, for incidence, prevalence, deaths, years of life lost (YLLs), years lived with disability (YLDs), and disability-adjusted life-years (DALYs). DALYs were generated by summing YLLs and YLDs. Cause-specific mortality was estimated using an ensemble modelling process with vital registration and verbal autopsy data as inputs. Non-fatal estimates were generated using Bayesian meta-regression incorporating data from registries, scientific literature, administrative records, and surveys. The Socio-demographic Index (SDI), a summary indicator generated using educational attainment, lagged distributed income, and total fertility rate, was used to group countries into quintiles. Findings: In 2016, there were 5·5 million (95% UI 5·3 to 5·7) deaths and 116·4 million (111·4 to 121·4) DALYs due to stroke. The global age-standardised mortality rate decreased by 36·2% (−39·3 to −33·6) from 1990 to 2016, with decreases in all SDI quintiles. Over the same period, the global age-standardised DALY rate declined by 34·2% (−37·2 to −31·5), also with decreases in all SDI quintiles. There were 13·7 million (12·7 to 14·7) new stroke cases in 2016. Global age-standardised incidence declined by 8·1% (−10·7 to −5·5) from 1990 to 2016 and decreased in all SDI quintiles except the middle SDI group. There were 80·1 million (74·1 to 86·3) prevalent cases of stroke globally in 2016; 41·1 million (38·0 to 44·3) in women and 39·0 million (36·1 to 42·1) in men. Interpretation: Although age-standardised mortality rates have decreased sharply from 1990 to 2016, the decrease in age-standardised incidence has been less steep, indicating that the burden of stroke is likely to remain high. Planned updates to future GBD iterations include generating separate estimates for subarachnoid haemorrhage and intracerebral haemorrhage, generating estimates of transient ischaemic attack, and including atrial fibrillation as a risk factor. Funding: Bill & Melinda Gates Foundatio

Валидация опросника для оценки качества жизни пациентов с хронической приобретенной полинейропатией (Chronic Acquired Polyneuropathy – Patient Reported Index, CAP-PRI) в России

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.Введение. Хроническая воспалительная демиелинизирующая полинейропатия (ХВДП) – самая частая из иммуноопосредованных полинейропатий. Для ХВДП характерны многолетнее прогрессирующее или рецидивирующее течение, развитие неврологического дефицита и инвалидизации, в связи с чем важен мониторинг качества жизни пациентов. Представлены результаты 1‑го и 2‑го этапов валидации русскоязычной версии опросника Chronic Acquired Polyneuropathy – Patient Reported Index (CAP‑PRI) для оценки качества жизни у пациентов с иммуноопосредованными полинейропатиями.Цель исследования – проведение валидации опросника CAP‑PRI у пациентов с ХВДП с разработкой его русскоязычной версии.Материалы и методы. В исследование было включено 85 пациентов с ХВДП, диагностированной на основании критериев EAN / PNS 2021. На 1‑м этапе проведена лингвистическая валидация оригинальной англоязычной версии опросника согласно общепринятым рекомендациям: 2 русскоязычными профессиональными переводчиками в сфере медицины выполнен прямой перевод оригинальной англоязычной шкалы, обратный перевод осуществлен носителями языка с медицинским образованием. На 2‑м этапе валидации проводилось исследование психометрических показателей разработанного русскоязычного варианта.Результаты. Разработана русскоязычная версия опросника CAP‑PRI с высоким уровнем надежности, содержательной валидности и чувствительности.Заключение. Представленная официальная русскоязычная версия опросника для пациентов с хронической приобретенной полинейропатией (CAP‑PRI) рекомендована для отечественной клинической практики и научно‑исследовательской работы с целью оценки качества жизни пациентов. Дополнительно показана связь между улучшением качества жизни и уменьшением выраженности неврологического дефицита, степени инвалидизации и утомляемости (по результатам оценок по шкалам IRODS, NIS и FSS)

Global, regional, and national burden of epilepsy, 1990 - 2016 : a systematic analysis for the Global Burden of Disease Study 2016

Background: Seizures and their consequences contribute to the burden of epilepsy because they can cause health loss (premature mortality and residual disability). Data on the burden of epilepsy are needed for health-care planning and resource allocation. The aim of this study was to quantify health loss due to epilepsy by age, sex, year, and location using data from the Global Burden of Diseases, Injuries, and Risk Factors Study. Methods: We assessed the burden of epilepsy in 195 countries and territories from 1990 to 2016. Burden was measured as deaths, prevalence, and disability-adjusted life-years (DALYs; a summary measure of health loss defined by the sum of years of life lost [YLLs] for premature mortality and years lived with disability), by age, sex, year, location, and Socio-demographic Index (SDI; a compound measure of income per capita, education, and fertility). Vital registrations and verbal autopsies provided information about deaths, and data on the prevalence and severity of epilepsy largely came from population representative surveys. All estimates were calculated with 95% uncertainty intervals (UIs). Interpretation: Despite the decrease in the disease burden from 1990 to 2016, epilepsy is still an important cause of disability and mortality. Standardised collection of data on epilepsy in population representative surveys will strengthen the estimates, particularly in countries for which we currently have no or sparse data and if additional data is collected on severity, causes, and treatment. Sizeable gains in reducing the burden of epilepsy might be expected from improved access to existing treatments in low-income countries and from the development of new effective drugs worldwide

Use of multidimensional item response theory methods for dementia prevalence prediction: an example using the Health and Retirement Survey and the Aging, Demographics, and Memory Study.

BACKGROUND: Data sparsity is a major limitation to estimating national and global dementia burden. Surveys with full diagnostic evaluations of dementia prevalence are prohibitively resource-intensive in many settings. However, validation samples from nationally representative surveys allow for the development of algorithms for the prediction of dementia prevalence nationally. METHODS: Using cognitive testing data and data on functional limitations from Wave A (2001-2003) of the ADAMS study (n = 744) and the 2000 wave of the HRS study (n = 6358) we estimated a two-dimensional item response theory model to calculate cognition and function scores for all individuals over 70. Based on diagnostic information from the formal clinical adjudication in ADAMS, we fit a logistic regression model for the classification of dementia status using cognition and function scores and applied this algorithm to the full HRS sample to calculate dementia prevalence by age and sex. RESULTS: Our algorithm had a cross-validated predictive accuracy of 88% (86-90), and an area under the curve of 0.97 (0.97-0.98) in ADAMS. Prevalence was higher in females than males and increased over age, with a prevalence of 4% (3-4) in individuals 70-79, 11% (9-12) in individuals 80-89 years old, and 28% (22-35) in those 90 and older. CONCLUSIONS: Our model had similar or better accuracy as compared to previously reviewed algorithms for the prediction of dementia prevalence in HRS, while utilizing more flexible methods. These methods could be more easily generalized and utilized to estimate dementia prevalence in other national surveys