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Epidemiological characteristics of multiple sclerosis in Stavropol region 斯塔夫罗波尔州多发性硬化症的流行病学特征
Q4 Medicine Pub Date : 2024-03-12 DOI: 10.30629/2658-7947-2024-29-1-46-51
G. V. Larin, E. M. Larina, A. Raevskaya, I. Vyshlova, S. M. Karpov
Multiple sclerosis (MS) is a chronic autoimmune, infl ammatory and degenerative disease of the central nervous system and is the most common neurological disorder in young people.Purpose: to determine the clinical and epidemiological features of multiple sclerosis usig the example of the Stavropol region (SR).Materials and methods. A retrospective analysis of 655 medical records of patients for 2021–2022, hospitalized in the neurological department of a multidisciplinary hospital with a diagnosis of MS, was carried out. The age, gender, place of residence of the patient, age of onset of the disease, type of MS, degree of disability, and rate of disease progression were assessed. Statistical processing was carried out using Microsoft Excel and Statistica 10 software.Results. The incidence of MS during this period was 22.7 per 100,000 population. The share of women was 70.2%, men — 29.8%. Average age — 40.04 ± 10.8 years. The vast majority of cases of onset of MS (83.2%) occur between the ages of 21 and 50 years — 82.8% of women and 84% of men. When analyzing the severity of neurological defi cit, the average score on the extended Kurtzke scale of disability was 3.53 ± 1.7 points. The rate of progression was 1.21 ± 1.1 points per year, which corresponds to a fast pace. Among those hospitalized, 65.4% have a disability group: third — 41.9% (275 patients), second — 21.4% (140 patients), fi rst — 2.1% (14 patients).Conclusion. The Stavropol Territory is one of the regions with a moderate risk of developing multiple sclerosis. The results of the work may be useful in planning medical and social care in certain territories of this region.
多发性硬化症(MS)是一种慢性自身免疫性、炎症性和中枢神经系统退行性疾病,也是年轻人最常见的神经系统疾病。对 2021-2022 年期间在一家多学科医院神经科住院并被诊断为多发性硬化症的 655 名患者的病历进行了回顾性分析。对患者的年龄、性别、居住地、发病年龄、多发性硬化症类型、残疾程度和疾病进展速度进行了评估。统计处理使用 Microsoft Excel 和 Statistica 10 软件进行。在此期间,多发性硬化症的发病率为每 10 万人 22.7 例。女性占 70.2%,男性占 29.8%。平均年龄为 40.04 ± 10.8 岁。绝大多数多发性硬化症的发病年龄(83.2%)在 21 至 50 岁之间,其中女性占 82.8%,男性占 84%。在分析神经功能缺损的严重程度时,扩展库尔茨克残疾量表的平均得分是 3.53 ± 1.7 分。病情发展速度为每年 1.21 ± 1.1 分,速度较快。在住院患者中,65.4%的人属于残疾组:第三组--41.9%(275名患者),第二组--21.4%(140名患者),第一组--2.1%(14名患者)。斯塔夫罗波尔边疆区是多发性硬化症发病风险中等的地区之一。这项工作的结果可能有助于规划该地区某些地区的医疗和社会保健工作。
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引用次数: 0
Quality of life of patients with multiple sclerosis after high-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation 自体造血干细胞移植大剂量免疫抑制治疗后多发性硬化症患者的生活质量
Q4 Medicine Pub Date : 2024-03-12 DOI: 10.30629/2658-7947-2024-29-1-25-35
A. Polushin, E. Lopatina, A. Tsynchenko, Yury R. Zalyalov, I. Skiba, A.  V. Shegurova, N. Totolyan, A. D. Kulagin, T. Ionova, T. P. Nikitina, N. Porfirieva, A. A. Skoromets
Introduction. Multiple sclerosis (MS) is a chronic autoimmune and neurodegenerative disease of the central nervous system (CNS). High-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation (HDIT–AHSCT) is the most eff ective method of treating MS, which has a positive eff ect on the quality of life of patients and inhibition of the progression of disability.The aim of the study was to analyze the dynamics of quality of life in patients with MS who underwent the HDIT– AHSCT method in groups with diff erent clinical effi  cacy of this method.Material and methods. The single-center observational study included patients with MS (n = 21, mean age 35.5 ± 6.2 (28–50) years; men/women — 11/10; remitting MS (RMS) n = 16, secondary-progressive (SPMS) n = 4, primaryprogressive (PPMS) n = 1), after HDIT–AHSCT (cyclophosphamide-rituximab conditioning regimen). Assessment of the quality of life (SF-36, FAMS, CSP-MS42, HADS) and the degree of disability in all patients was carried out before and 12 months after HDIT–AHSCT.Results. 12 months after HDIT–AHSCT, more than 50% of patients showed signifi cant improvement in quality of life according to the scales of viability and social functioning of the SF-36 questionnaire, as well as according to the scales of general well-being and additional problems of the FAMS questionnaire. 57% of patients had a signifi cant improvement in the overall FAMS score. According to the CSP-MS42 questionnaire, a decrease in weakness (p < 0.05), feelings of constant fatigue and rapid fatigue during exercise was revealed. After transplantation, there was a slight decrease in anxiety (p = 0.91) and a signifi cant decrease in depression (p = 0.011).Conclusion. The expected dynamics was established: in patients with clinical improvement, there was an improvement in the quality of life for most indicators; in patients with stabilization, most quality of life indicators were similar before and after HDIT–AHSCT; with clinical deterioration after HDIT–AHSCT, quality of life parameters worsened. These data indicate the expediency of further optimization of patient selection criteria and various stages of the HDIT–AHSCT in order to obtain optimal clinical results and, accordingly, improve the quality of life of patients.
导言。多发性硬化症(MS)是一种慢性自身免疫性中枢神经系统(CNS)神经退行性疾病。高剂量免疫抑制治疗联合自体造血干细胞移植(HDIT-AHSCT)是治疗多发性硬化症最有效的方法,对提高患者生活质量和抑制残疾进展具有积极作用。本研究旨在分析接受 HDIT- AHSCT 方法的多发性硬化症患者在不同临床疗效组的生活质量动态。这项单中心观察性研究纳入了接受HDIT-AHSCT(环磷酰胺-利妥昔单抗调理方案)治疗的多发性硬化症患者(n=21,平均年龄35.5 ± 6.2 (28-50)岁;男性/女性-11/10;缓解型多发性硬化症(RMS)n=16,继发性进展型(SPMS)n=4,原发性进展型(PPMS)n=1)。所有患者的生活质量(SF-36、FAMS、CSP-MS42、HADS)和残疾程度在HDIT-AHSCT前和HDIT-AHSCT后12个月进行评估。HDIT-AHSCT治疗12个月后,根据SF-36问卷的生存能力和社会功能量表,以及FAMS问卷的一般幸福感和其他问题量表,50%以上的患者生活质量有明显改善。57% 的患者的 FAMS 总分有明显改善。根据 CSP-MS42 问卷调查,患者的乏力感(p < 0.05)、持续疲劳感和运动时的快速疲劳感均有所减轻。移植后,焦虑略有减少(p = 0.91),抑郁明显减少(p = 0.011)。预期的动态变化得到了证实:在临床症状改善的患者中,大部分生活质量指标得到了改善;在病情稳定的患者中,大部分生活质量指标在HDIT-AHSCT前后相似;在HDIT-AHSCT后临床症状恶化的患者中,生活质量指标恶化。这些数据表明,为了获得最佳临床效果,提高患者的生活质量,有必要进一步优化患者选择标准和 HDIT-AHSCT 的各个阶段。
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引用次数: 0
In-hospital ischemic stroke in ophthalmic patients 眼科患者院内缺血性中风
Q4 Medicine Pub Date : 2024-03-12 DOI: 10.30629/2658-7947-2024-29-1-59-66
S. V. Kolomentsev, I. A. Voznjouk, E. I. Shermatyuk, A. V. Kolomentseva, P. A. Polezhaev, M. S. Yaroslavtseva, A. A. Kirpichenko, Nikolay V. Tsygan, I. Litvinenko
Justifi cation. Due to the high prevalence of diseases of the visual organs (cataracts, glaucoma, etc.) and the large number of surgical interventions performed annually in elderly and senile people, the prevention of in-hospital ischemic stroke (IHS) is an urgent task.The purpose of the work. To study the risk factors of development, characteristics of care and outcomes of IHS in ophthalmic patients.Material and methods. The study was performed in the period from 01.01.2022 to 31.12.2022 on the basis of ophthalmological departments specializing in the performance of planned vitreoretinal surgical interventions, two large multidisciplinary hospitals in St. Petersburg.Results. It has been established that perioperative ischemic stroke (included in the structure of the IHS) is a rare complication of minimally invasive ophthalmic operations. Its share was 0.07% (n = 5) of the total number of patients who underwent elective ophthalmological intervention during the year; the total share of patients with IHS (taking into account 4 cases of ischemic stroke in the preoperative period) was 0.13%. Most strokes (n = 5; 55.6%) belonged to the cardioembolic subtype, the proportion of using reperfusion techniques was high and amounted to 33.3% (2 endovascular interventions, 1 systemic thrombolysis); the proportion of adverse outcomes was 22.2% (n = 2). A distinctive characteristic of patients with IHS was a combination of high comorbidity with insuffi  cient antithrombotic prophylaxis, which consisted in the cancellation of antiplatelet agents and anticoagulants in the preoperative period.Conclusion. Current recommendations on perioperative management of patients indicate the need to continue taking antiplatelet and anticoagulant drugs for most ophthalmic operations, due to the predominance of threats of thromboembolic complications over the risk of retrobulbar bleeding.
理由。由于视觉器官疾病(白内障、青光眼等)的高发病率以及每年对老年人和高龄者进行的大量外科手术,预防院内缺血性中风(IHS)是一项紧迫的任务。研究眼科患者发生 IHS 的危险因素、护理特点和结果。研究时间为 2022 年 1 月 1 日至 2022 年 12 月 31 日,研究对象为圣彼得堡两家大型多学科医院专门从事计划性玻璃体视网膜手术干预的眼科部门。经证实,围手术期缺血性中风(包括在 IHS 结构中)是微创眼科手术的罕见并发症。该年接受眼科择期手术的患者总数中,缺血性中风患者占 0.07%(n = 5);IHS 患者总数占 0.13%(考虑到术前缺血性中风的 4 例)。大多数中风(5 例;55.6%)属于心肌栓塞亚型,使用再灌注技术的比例很高,达到 33.3%(2 例血管内介入治疗,1 例全身溶栓治疗);出现不良后果的比例为 22.2%(2 例)。IHS患者的一个显著特点是合并症多,抗血栓预防不足,包括术前取消使用抗血小板药物和抗凝剂。目前关于患者围手术期管理的建议表明,由于血栓栓塞并发症的威胁大于球后出血的风险,因此大多数眼科手术都需要继续服用抗血小板和抗凝药物。
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引用次数: 0
Assessment indexes of cognitive status, anxiety and depression in senior women with diff erent severity of frailty 不同虚弱程度老年妇女的认知状况、焦虑和抑郁评估指数
Q4 Medicine Pub Date : 2024-03-12 DOI: 10.30629/26587947-2024-29-1-36-45
A. N. Kodintcev, N. V. Izmozherova, A. A. Popov, L. Volkova
Frailty is a key geriatric syndrome characterized by an age-associated diminution of the physiological reserve and functions of many body systems leading to increased vulnerability of the elderly people to the eff ects of endo- and exogenous factors. The combination of frailty, cognitive impairment, anxiety and depression is a signifi cant clinical predictor for an unfavorable prognosis.Purpose. Identifi cation and study of the relationship of cognitive impairment, anxiety, depression and frailty assessment indexes in female patients over 60 years old, observed on an outpatient basis.Material and methods. The study included 80 women over 60 years old. All participants were divided into 4 groups: non-frail (10 women), pre-frail (44 women), mildly frail (20 women), more-frail (6 women). A comprehensive clinical examination was carried out: Montreal cognitive assessment (MoCA), Mini-Mental State Examination (MMSE), frontal assessment battery (FAB), phonetic association test, semantic association test, Beck anxiety inventory, geriatric depression scale–15 (GDS-15), questionnaire “Age is no barrier”, Frailty index (FI, Hoover et al., 2013).Results. All women in 4 groups did not signifi cantly diff er in age. There was no statistically signifi cant diff erence in the MMSE, FAB data and mild cognitive impairment (MCI) prevalence among the groups. Lower rates of MoCA scale were associated with higher probability of frailty (odds ratio (OR) = 0.78; 95 confi dence interval (CI) CI: 0.63–0.96, p = 0.016). The cut-off  level of 26 and lower according MoCA has sensitivity 69% and specifi city 50% to detect of high frailty risk (according FI). Questionnaire “Age is no barrier” has a sensitivity of 86% and a specifi city of 53% to detect prefrailty (score 1–2) and a sensitivity of 69% and a specifi city of 87% to detect the presence of frailty (score ≥ 3) regarding FI. Combined sensitivity of MoCA (≤ 26 score) and questionnaire “Age is no barrier” (≥ 3 score) of 78%, combined specifi city of 69%. Higher rates of Beck anxiety inventory and GDS-15 were associated with frailty (OR = 1.12; 95 CI: 1.02–1.22, p = 0.0134 and OR = 1.39; 95 CI: 1.05–1.84, p = 0.0201, respectively). A higher index of the Beck anxiety inventory was also associated with mild cognitive impairment according MoCA (OR = 1.12; 95% CI: 1.04–1.2, p = 0.003). Arterial hypertension was signifi cantly more common for women with FS (χ2 = 4.23, p = 0.04).Conclusion. There was no signifi cant association between MMSE, FAB, MCI prevalence and the severity of frailty in senior women due to the insuffi  cient study power (< 80%). MoCA cut-off  ≤ 26 score is associated with higher burden of frailty. The questionnaire «Age is no barrier» is an acceptable screening method for determining the necessity of additional geriatric examination of independently living women over 60 years old. Higher rates of the Beck anxiety inventory, GDS-15, BMI, lower rates of MoCA scale and arterial hypertension are associated w
虚弱是一种重要的老年综合症,其特征是与年龄相关的生理储备和身体多个系统功能的减退,导致老年人更容易受到内源性和外源性因素的影响。虚弱、认知障碍、焦虑和抑郁的综合征是预后不良的重要临床预测因素。确定并研究门诊 60 岁以上女性患者的认知障碍、焦虑、抑郁和虚弱评估指标之间的关系。研究对象包括 80 名 60 岁以上的女性。所有参与者被分为 4 组:非虚弱组(10 名女性)、前期虚弱组(44 名女性)、轻度虚弱组(20 名女性)和较虚弱组(6 名女性)。研究人员进行了全面的临床检查:进行了全面的临床检查:蒙特利尔认知评估(MoCA)、小型精神状态检查(MMSE)、额叶评估电池(FAB)、语音联想测试、语义联想测试、贝克焦虑量表、老年抑郁量表-15(GDS-15)、"年龄不是障碍 "问卷、虚弱指数(FI,Hoover 等人,2013 年)。4 组所有妇女的年龄均无显著差异。各组之间的 MMSE、FAB 数据和轻度认知障碍(MCI)患病率在统计学上没有显著差异。较低的 MoCA 评分与较高的虚弱概率相关(几率比(OR)= 0.78;95 置信区间(CI)CI:0.63-0.96,P = 0.016)。在检测高虚弱风险(根据 FI)方面,MoCA 临界值为 26 及以下的灵敏度为 69%,特异度为 50%。年龄不是障碍 "问卷对检测虚弱前期(1-2 分)的灵敏度为 86%,具体灵敏度为 53%;对检测是否存在虚弱(≥ 3 分)的灵敏度为 69%,具体灵敏度为 87%。MoCA(≤ 26 分)和 "年龄不是障碍 "问卷(≥ 3 分)的综合灵敏度为 78%,综合特异度为 69%。贝克焦虑量表和 GDS-15 的较高比率与体弱有关(OR = 1.12;95 CI:1.02-1.22,p = 0.0134 和 OR = 1.39;95 CI:1.05-1.84,p = 0.0201)。贝克焦虑量表指数越高,MoCA结果显示的轻度认知障碍越严重(OR = 1.12;95% CI:1.04-1.2,p = 0.003)。结论:患有 FS 的女性动脉高血压患者更常见(χ2 = 4.23,P = 0.04)。由于研究力量不足(< 80%),高龄女性的 MMSE、FAB、MCI 患病率与虚弱严重程度之间没有明显关联。MoCA 临界值≤26 分与较高的虚弱负担有关。年龄不是障碍 "问卷是一种可接受的筛查方法,用于确定是否有必要对 60 岁以上独立生活的女性进行额外的老年病检查。贝克焦虑量表、GDS-15、体重指数(BMI)较高,MoCA量表和动脉高血压较低,这些指标与高虚弱率相关,可能是虚弱进展的预测因素。
{"title":"Assessment indexes of cognitive status, anxiety and depression in senior women with diff erent severity of frailty","authors":"A. N. Kodintcev, N. V. Izmozherova, A. A. Popov, L. Volkova","doi":"10.30629/26587947-2024-29-1-36-45","DOIUrl":"https://doi.org/10.30629/26587947-2024-29-1-36-45","url":null,"abstract":"Frailty is a key geriatric syndrome characterized by an age-associated diminution of the physiological reserve and functions of many body systems leading to increased vulnerability of the elderly people to the eff ects of endo- and exogenous factors. The combination of frailty, cognitive impairment, anxiety and depression is a signifi cant clinical predictor for an unfavorable prognosis.Purpose. Identifi cation and study of the relationship of cognitive impairment, anxiety, depression and frailty assessment indexes in female patients over 60 years old, observed on an outpatient basis.Material and methods. The study included 80 women over 60 years old. All participants were divided into 4 groups: non-frail (10 women), pre-frail (44 women), mildly frail (20 women), more-frail (6 women). A comprehensive clinical examination was carried out: Montreal cognitive assessment (MoCA), Mini-Mental State Examination (MMSE), frontal assessment battery (FAB), phonetic association test, semantic association test, Beck anxiety inventory, geriatric depression scale–15 (GDS-15), questionnaire “Age is no barrier”, Frailty index (FI, Hoover et al., 2013).Results. All women in 4 groups did not signifi cantly diff er in age. There was no statistically signifi cant diff erence in the MMSE, FAB data and mild cognitive impairment (MCI) prevalence among the groups. Lower rates of MoCA scale were associated with higher probability of frailty (odds ratio (OR) = 0.78; 95 confi dence interval (CI) CI: 0.63–0.96, p = 0.016). The cut-off  level of 26 and lower according MoCA has sensitivity 69% and specifi city 50% to detect of high frailty risk (according FI). Questionnaire “Age is no barrier” has a sensitivity of 86% and a specifi city of 53% to detect prefrailty (score 1–2) and a sensitivity of 69% and a specifi city of 87% to detect the presence of frailty (score ≥ 3) regarding FI. Combined sensitivity of MoCA (≤ 26 score) and questionnaire “Age is no barrier” (≥ 3 score) of 78%, combined specifi city of 69%. Higher rates of Beck anxiety inventory and GDS-15 were associated with frailty (OR = 1.12; 95 CI: 1.02–1.22, p = 0.0134 and OR = 1.39; 95 CI: 1.05–1.84, p = 0.0201, respectively). A higher index of the Beck anxiety inventory was also associated with mild cognitive impairment according MoCA (OR = 1.12; 95% CI: 1.04–1.2, p = 0.003). Arterial hypertension was signifi cantly more common for women with FS (χ2 = 4.23, p = 0.04).Conclusion. There was no signifi cant association between MMSE, FAB, MCI prevalence and the severity of frailty in senior women due to the insuffi  cient study power (< 80%). MoCA cut-off  ≤ 26 score is associated with higher burden of frailty. The questionnaire «Age is no barrier» is an acceptable screening method for determining the necessity of additional geriatric examination of independently living women over 60 years old. Higher rates of the Beck anxiety inventory, GDS-15, BMI, lower rates of MoCA scale and arterial hypertension are associated w","PeriodicalId":36724,"journal":{"name":"Russian Neurological Journal","volume":"120 19","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140250479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acute disseminated encephalomyelitis as an emergency 急诊急性播散性脑脊髓炎
Q4 Medicine Pub Date : 2024-03-11 DOI: 10.30629/2658-7947-2024-29-1-14-18
S. Yevtushenko, Yu. N. Sorokin
Acute disseminated encephalomyelitis (ADEM) is a common acute multifocal autoimmune infl ammatory demyelinating disease of the central nervous system (CNS) with a predominant lesion of the white matter of the brain, brainstem and optic nerves, the conductors of the spinal cord, less often — gray matter of the CNS. ADEM is usually a self-limiting condition with spontaneous improvement and a favorable prognosis. Along with the possibility of a severe course in the classical form of ADEM, the development of hyperacute forms with a much more unfavorable prognosis is possible. Such variants of the course of ADEM are acute hemorrhagic leukoencephalitis (AHLE), acute hemorrhagic leukoencephalomyelitis and acute necrotizing hemorrhagic leukoencephalitis of Hurst, associated with the development of a cascade of hyperacute uncontrolled autoimmune reactions. The severity of these conditions (grouped under the term OHLE) is associated with rapid progression, brainstem involvement, large lesions is due to the development of acute edematous necrosis and hemorrhage with a high possibility of disability or death. The aggressive and severe course of АHLE requires the immediate initiation of decongestant and immunosuppressive therapy.
急性播散性脑脊髓炎(ADEM)是一种常见的急性多灶性中枢神经系统(CNS)自身免疫性炎症性脱髓鞘疾病,主要病变部位为脑白质、脑干和视神经、脊髓导体,少数病变部位为中枢神经系统灰质。ADEM 通常是一种自限性疾病,可自发好转,预后良好。除了传统形式的 ADEM 病程严重外,还可能出现预后更差的超急性 ADEM。急性出血性白质脑炎(AHLE)、急性出血性白质脑脊髓炎和赫斯特急性坏死性出血性白质脑炎是 ADEM 病程的变异型,与一连串超急性、失控的自身免疫反应有关。这些病症(归类于 OHLE 术语)的严重程度与病情进展快、脑干受累、病变大有关,是由于急性水肿性坏死和出血的发展所致,极有可能致残或致死。АHLE 病程凶险而严重,需要立即开始减充血剂和免疫抑制剂治疗。
{"title":"Acute disseminated encephalomyelitis as an emergency","authors":"S. Yevtushenko, Yu. N. Sorokin","doi":"10.30629/2658-7947-2024-29-1-14-18","DOIUrl":"https://doi.org/10.30629/2658-7947-2024-29-1-14-18","url":null,"abstract":"Acute disseminated encephalomyelitis (ADEM) is a common acute multifocal autoimmune infl ammatory demyelinating disease of the central nervous system (CNS) with a predominant lesion of the white matter of the brain, brainstem and optic nerves, the conductors of the spinal cord, less often — gray matter of the CNS. ADEM is usually a self-limiting condition with spontaneous improvement and a favorable prognosis. Along with the possibility of a severe course in the classical form of ADEM, the development of hyperacute forms with a much more unfavorable prognosis is possible. Such variants of the course of ADEM are acute hemorrhagic leukoencephalitis (AHLE), acute hemorrhagic leukoencephalomyelitis and acute necrotizing hemorrhagic leukoencephalitis of Hurst, associated with the development of a cascade of hyperacute uncontrolled autoimmune reactions. The severity of these conditions (grouped under the term OHLE) is associated with rapid progression, brainstem involvement, large lesions is due to the development of acute edematous necrosis and hemorrhage with a high possibility of disability or death. The aggressive and severe course of АHLE requires the immediate initiation of decongestant and immunosuppressive therapy.","PeriodicalId":36724,"journal":{"name":"Russian Neurological Journal","volume":"25 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140253851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Paraneoplastic neurological syndromes: upgraded approaches to diagnosis 副肿瘤性神经综合征:升级诊断方法
Q4 Medicine Pub Date : 2024-03-11 DOI: 10.30629/2658-7947-2024-29-1-4-13
V. Grigoryeva, E. A. Ruina
Paraneoplastic neurological syndromes (PNNS) are neurological disorders due to autoimmune attack on the nervous system, induced by a tumor remote from these structures. The existence of relatively stable associations between the neurological syndrome, the type of antineuronal antibodies (Ab) and the nature of tumor has been proven. At the same time, there are no pathognomonic combinations of this kind: the same antibodies can be detected in diff erent types of tumors and in diff erent syndromes. In addition, level of reliability of the diagnosis of PNNS is considered, taking into account the phenotype of the neurological disorder, the presence of certain antibodies in the blood serum, the verifi cation of oncopathology and the duration of the observation for the patient.
副肿瘤性神经综合征(PNNS)是由远离神经系统结构的肿瘤诱发的神经系统自身免疫性疾病。神经系统综合征、抗神经元抗体(Ab)类型和肿瘤性质之间存在相对稳定的联系已得到证实。与此同时,也不存在这种病理组合:在不同类型的肿瘤和不同的综合征中可以检测到相同的抗体。此外,考虑到神经系统疾病的表型、血清中某些抗体的存在、肿瘤病理学的验证以及对患者的观察时间,PNNS 诊断的可靠程度也要考虑在内。
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引用次数: 0
ATP1A3-alternating hemiplegia ATP1A3 交替性偏瘫
Q4 Medicine Pub Date : 2024-03-11 DOI: 10.30629/2658-7947-2024-29-1-19-24
M. P. Afanasyeva, E. D. Belousova, A. A. Sharkov
Alternating hemiplegia (AH) is a rare and little researched disease. AH is characterized with early episodes of hemiplegia (from a few minutes to a few days), abnormal movements and cognition, and probable development of neurological defi ciency. Studies of genetic basis have led to discovery of mutations in gen ATP1A3, which codes alfa-3 subunit of Na+/K+-ATPase. For research, genetic data are considered most important prognostic information. Its diagnostics is diffi cult due to similarity of some symptoms to epilepsy and absence of deviations at neuro-visualisation. This article is an attempt to generalize world scientifi c experience and to determine most important research derections.
交替性偏瘫(AH)是一种罕见且研究较少的疾病。交替性偏瘫的特征是早期发作性偏瘫(从几分钟到几天不等)、异常运动和认知,并可能发展为神经系统缺陷。对遗传基础的研究发现,编码 Na+/K+-ATPase alfa-3 亚基的 ATP1A3 基因发生了突变。在研究中,基因数据被认为是最重要的预后信息。由于该病的某些症状与癫痫相似,且在神经视觉上没有偏差,因此诊断难度很大。本文试图总结世界科学经验,确定最重要的研究方向。
{"title":"ATP1A3-alternating hemiplegia","authors":"M. P. Afanasyeva, E. D. Belousova, A. A. Sharkov","doi":"10.30629/2658-7947-2024-29-1-19-24","DOIUrl":"https://doi.org/10.30629/2658-7947-2024-29-1-19-24","url":null,"abstract":"Alternating hemiplegia (AH) is a rare and little researched disease. AH is characterized with early episodes of hemiplegia (from a few minutes to a few days), abnormal movements and cognition, and probable development of neurological defi ciency. Studies of genetic basis have led to discovery of mutations in gen ATP1A3, which codes alfa-3 subunit of Na+/K+-ATPase. For research, genetic data are considered most important prognostic information. Its diagnostics is diffi cult due to similarity of some symptoms to epilepsy and absence of deviations at neuro-visualisation. This article is an attempt to generalize world scientifi c experience and to determine most important research derections.","PeriodicalId":36724,"journal":{"name":"Russian Neurological Journal","volume":"48 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140252337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Anatomical focus on the sacrococcygeal region importance in neurology 骶尾部解剖重点在神经学中的重要性
Q4 Medicine Pub Date : 2024-03-11 DOI: 10.30629/2658-7947-2024-29-1-67-76
M. Sankova, V. N. Nikolenko, M. V. Oganesyan, A. V. Sankov
{"title":"Anatomical focus on the sacrococcygeal region importance in neurology","authors":"M. Sankova, V. N. Nikolenko, M. V. Oganesyan, A. V. Sankov","doi":"10.30629/2658-7947-2024-29-1-67-76","DOIUrl":"https://doi.org/10.30629/2658-7947-2024-29-1-67-76","url":null,"abstract":"","PeriodicalId":36724,"journal":{"name":"Russian Neurological Journal","volume":"46 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140253119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Differential diagnosis of chronic acquired demyelinating polyneuropathies 慢性获得性脱髓鞘多发性神经病的鉴别诊断
Q4 Medicine Pub Date : 2024-01-08 DOI: 10.30629/2658-7947-2023-28-6-41-50
D. Grishina, N. Suponeva, M. Piradov
Introduction. The rarity of chronic acquired polyneuropathies (PNP) with the demyelinating nature of peripheral nerve damage causes the difficulties of their differential diagnosis that persist in our country and abroad. Objective: to identify significant clinical, neurophysiological and sonographic differential diagnostic markers in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and non-IgM paraproteinemic demyelinating polyneuropathies (PDP).Material and methods: 80 patients were included in the study: 30 with CIDP, 30 with non-IgM-PDP associated with monoclonal gammapathy of unclear significance (PDP-MGUS), and 20 with non-IgM-PDP associated with lymphoproliferative disease (PDP-LPD). The patients included in the study underwent clinical evaluation of neurological disorders according to the MRC, NIS, VAS, INCAT, IRODS, SARA scales; ENMG and ultrasound studies of peripheral nerves.Results. The predominance of men in all groups was noted (p > 0.05). Compared with patients with CIDP, patients with PDP were significantly older, they were more likely to have neuropathic pain syndrome and trophic disorders (p < 0.05). In patients with PDP-LPD, in contrast to CIDP and PDP-MGUS, there was a predominance of the distal pattern of muscle weakness distribution and a greater severity of sensitive ataxia (p < 0.05). During NCV studies in patients with CIDP, compared with patients with PDP, blocks of conduction and dispersion of M-waves were signifi -cantly more often recorded in the study of motor fibers of the nerves of the hands (p < 0.05); and in the study of motor nerves of the legs, non-excitability of motor fibers was significantly less often noted (p < 0.05). Ultrasound examination of peripheral nerves showed no significant differences between patients (p > 0.05).Conclusion. Clinical phenotype, neurophysiological and sonographic changes in patients with CIDP and PDP do not have highly specific differences. Electrophoresis of serum proteins with immunofixation makes it possible to differentiate CIDP and PDP, and further examination by an oncohematologist with paraproteinemia makes it possible to distinguish MGUS from LPD.
导言。慢性获得性多发性神经病(PNP)具有脱髓鞘性质的周围神经损伤,其罕见性使其鉴别诊断在国内外一直存在困难。目的:确定慢性炎症性脱髓鞘性多发性神经病(CIDP)和非IgM副蛋白血症性脱髓鞘性多发性神经病(PDP)的临床、神经电生理和超声诊断指标:材料和方法:研究共纳入了 80 名患者:30 名 CIDP 患者,30 名非 IgM-PDP 患者伴有意义不明确的单克隆丙种球蛋白病(PDP-MGUS),20 名非 IgM-PDP 患者伴有淋巴增生性疾病(PDP-LPD)。参与研究的患者根据 MRC、NIS、VAS、INCAT、IRODS、SARA 等量表对神经系统疾病进行了临床评估;对周围神经进行了 ENMG 和超声波检查。所有组别均以男性为主(P>0.05)。与 CIDP 患者相比,PDP 患者的年龄明显偏大,他们更有可能患有神经病理性疼痛综合征和营养障碍(P < 0.05)。与 CIDP 和 PDP-MGUS 相反,PDP-LPD 患者的肌无力分布以远端模式为主,敏感性共济失调的严重程度更高(P < 0.05)。在对 CIDP 患者进行 NCV 检查时,与 PDP 患者相比,手部运动神经的传导阻滞和 M 波弥散显著增加(p < 0.05);而在腿部运动神经的检查中,运动神经的不兴奋显著减少(p < 0.05)。对周围神经的超声波检查显示,不同患者之间没有显著差异(P > 0.05)。结论:CIDP和PDP患者的临床表型、神经电生理和超声波检查变化没有高度特异性。通过血清蛋白电泳和免疫排斥可以区分CIDP和PDP,通过副蛋白血症的血液学专家的进一步检查可以区分MGUS和LPD。
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引用次数: 0
Tolosa–Hunt syndrome 托洛萨-亨特综合征
Q4 Medicine Pub Date : 2024-01-08 DOI: 10.30629/2658-7947-2023-28-6-56-61
N. A. Kayleva, A. A. Kulesh, N. L. Starikova, Yuliya V. Karakulova, A. R. Galimshin
A description of a clinical case of Tolosa–Hunt syndrome (THS) and a brief review of the literature are presented. The described clinical case is characterized by three features that have not received sufficient attention in the literature: 1) the presence of recurrent pain outside the orbit in combination with ptosis without diplopia several years before the development of a typical episode of THS; 2) insufficient and unstable clinical response to standard doses of glucocorticoids with high efficiency of pulse therapy with methylprednisolone followed by oral administration of prednisolone; 3) a longer than required according to the international classification of headaches (ICHD-3) time interval between the development of cephalalgia and the appearance of oculomotor disorders. This clinical observation expands the understanding of the clinical picture and treatment of THS.
本文描述了一个托洛萨-亨特综合征(Tolosa-Hunt Syndrome,THS)的临床病例,并简要回顾了相关文献。该临床病例有三个特点,但在文献中并未引起足够重视:1)在THS典型发作前数年出现眼眶外反复疼痛,同时伴有上睑下垂,但无复视;2)对标准剂量糖皮质激素的临床反应不充分且不稳定,使用甲基强的松龙脉冲治疗后再口服强的松龙的疗效很好;3) 从出现头痛到出现眼球运动障碍的时间间隔比国际头痛分类(ICHD-3)所要求的时间间隔要长。这一临床观察结果拓展了人们对 THS 临床表现和治疗的理解。
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引用次数: 0
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Russian Neurological Journal
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