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Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova最新文献

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[Multiple system atrophy]. [多系统萎缩]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro2023123021144
M P Topuzova, I K Ternovykh, T A Shustova, A Yu Mikheeva, A O Chistyakova, T A Pavlova, N E Dudnikova, M L Pospelova, T M Alekseeva

The article presents a progressive neurodegenerative disease - multisystem atrophy, characterized by a combination of autonomic failure and various motor disorders, including parkinsonism and/or cerebellar ataxia; etiopathogenetic factors and variants of the clinical picture are described. We describe own clinical observation of a 59-old patient with cerebellar and bulbar syndromes, parkinsonism, pyramidal insufficiency, cognitive deficits, and autonomic dysfunction. The differential diagnosis included a whole range of neurodegenerative and hereditary diseases: Parkinson's disease, vascular parkinsonism, progressive supranuclear palsy, spinocerebellar ataxia, FXTAS, mitochondrial encephalopathies. The moderate severity of parkinsonism and the significant predominance of cerebellar symptoms and autonomic dysfunction make this clinical case difficult to diagnose. However, based on the life and disease history, clinical picture and research methods, a diagnosis of multiple system atrophy, cerebellar type (cerebellar, autonomic, bulbar syndrome, parkinsonism, pyramidal insufficiency and moderate cognitive impairment) was established. Differential search in such patients is a difficult task and includes a whole range of neurodegenerative and hereditary diseases due to the similarity of individual clinical and neuroimaging features and, unfortunately, the limited availability of molecular genetic diagnostic methods. However, earlier diagnosis is necessary to focus in time on the development of a personalized approach to the management of each such patient, taking into account the rate of symptoms development and steady progression, in order to ensure the longest possible survival time with an acceptable level of quality of life.

本文介绍了一种进行性神经退行性疾病-多系统萎缩,其特征是自主神经衰竭和各种运动障碍的结合,包括帕金森病和/或小脑性共济失调;致病因素和变异的临床图片描述。我们描述了一名59岁的小脑和球综合征、帕金森病、锥体功能不全、认知缺陷和自主神经功能障碍患者的临床观察。鉴别诊断包括一系列神经退行性和遗传性疾病:帕金森病、血管性帕金森病、进行性核上性麻痹、脊髓小脑共济失调、FXTAS、线粒体脑病。帕金森病的中度严重程度以及小脑症状和自主神经功能障碍的显著优势使该临床病例难以诊断。然而,根据生活和疾病史,临床表现和研究方法,诊断为多系统萎缩,小脑型(小脑,自主神经,球综合征,帕金森病,锥体功能不全和中度认知障碍)。这类患者的鉴别搜索是一项艰巨的任务,由于个体临床和神经影像学特征的相似性,不幸的是,分子遗传诊断方法的可用性有限,因此包括整个范围的神经退行性和遗传性疾病。然而,早期诊断是必要的,以便在考虑到症状发展和稳定进展的速度的情况下,及时集中精力制定个性化的方法来管理每一个这样的病人,以确保尽可能长的生存时间和可接受的生活质量水平。
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引用次数: 0
[The role of the phenosanic acid in the combined treatment of patients with epilepsy]. 【酚酸在癫痫患者联合治疗中的作用】。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro2023123021151
K V Voronkova, A M Alieva, I G Nikitin, G M Musina, E V Surskaya, O S Zaitseva, N G Mashkevich, L V Gomonova, A S Petrukhin

Epilepsy is one of the most studied chronic neurological disorders in the world. Despite significant progress in epilepsy research, some patients continue to experience recurrent seizures. It has been proven that oxidative stress plays an important role in epilepsy. Targeting oxidative stress may provide a novel therapeutic intervention to attenuate epileptogenesis as well as cognitive and psychiatric impairment in patients with epilepsy. One of the promising directions in the search for new strategies for the treatment of epilepsy is the combined use of antiepileptic drugs with antioxidants that affect the pathogenetic links of this disease. The new Russian antiepileptic drug fenosanoic acid (Dibufelon) has an anticonvulsant effect due to the inhibition of lipid peroxidation of brain cells and the reconstruction of cell membranes. This mechanism of action also paves the way for an increase in the effectiveness of co-administered drugs with phenosanoic acid. The effect of the drug on the cognitive and mental functions of patients, the regression of asthenia phenomena is also observed. The clinical efficacy and safety of the combination of this drug with other antiepileptic drugs in epilepsy has been demonstrated in experimental and clinical studies.

癫痫是世界上研究最多的慢性神经系统疾病之一。尽管癫痫研究取得了重大进展,但一些患者仍会反复发作。已经证明氧化应激在癫痫中起着重要作用。靶向氧化应激可能提供一种新的治疗干预,以减轻癫痫发生以及癫痫患者的认知和精神障碍。在寻找治疗癫痫的新策略中,一个有希望的方向是联合使用抗癫痫药物和抗氧化剂来影响这种疾病的发病环节。俄罗斯新型抗癫痫药物苯醚酸(双苯非龙)由于抑制脑细胞脂质过氧化和细胞膜重建而具有抗惊厥作用。这种作用机制也为增加与酚酸共同施用药物的有效性铺平了道路。观察该药对患者认知和心理功能的影响,并观察其对衰弱现象的消退。该药与其他抗癫痫药物联用治疗癫痫的临床疗效和安全性已在实验和临床研究中得到证实。
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引用次数: 0
[Features of the reward-based decision-making in patients with alcohol use disorders]. [酒精使用障碍患者基于奖励的决策特征]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro202312302137
S A Galkin, N A Bokhan
Foreign studies increasingly emphasize the role of reward-based cognitive decision-making and its contribution to alcohol and drug abuse. Unfortunately, such studies are extremely few in Russia. Given the importance of the insufficiency of cognitive functions, primarily the decision-making process based on reward, among the risk factors of addictive pathology, increasing the severity of alcoholism, reducing the effectiveness of its therapy and rehabilitation, research in this direction is extremely relevant. This review summarizes and systematizes current data on the features of the cognitive decision-making process based on reward in patients with alcohol dependence. An analysis of the literature has shown that reward-based decision making is an important component of addictive behavior. Patients with alcohol dependence, as well as people, who abuse alcohol, demonstrate clear impairment of this cognitive function. Thus, the relative value of diagnosing disorders in decision-making in modern psychiatry is beyond doubt. It is recommended to include the above tests in the diagnostic complex of a psychiatrist, along with standard psychometric tools.
国外的研究越来越强调基于奖励的认知决策的作用及其对酗酒和吸毒的影响。不幸的是,这样的研究在俄罗斯非常少。鉴于认知功能不足(主要是基于奖励的决策过程)在成瘾病理危险因素中的重要性,增加了酒精中毒的严重程度,降低了其治疗和康复的有效性,因此该方向的研究非常相关。这篇综述总结并系统化了目前关于酒精依赖患者基于奖励的认知决策过程特征的数据。对文献的分析表明,基于奖励的决策是成瘾行为的重要组成部分。酒精依赖患者以及滥用酒精的人都表现出这种认知功能的明显损害。因此,诊断决策障碍在现代精神病学中的相对价值是毋庸置疑的。建议在精神病医生的诊断复合体中包括上述测试,以及标准的心理测量工具。
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引用次数: 1
[Virtual reality technology as a promising direction in neurorehabilitation]. [虚拟现实技术作为神经康复的一个有前途的方向]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro2023123011131
N S Bofanova, A Yu Tychkov, Ya A Khanfar, R V Zolotarev

Nervous system damage affects more than a billion people worldwide and is one of the leading causes of cognitive impairment. An urgent issue in modern medicine is the neurorehabilitation of this particular group of patients. The purpose of this article is to search for new approaches to achieve more effective recovery of cognitive functions, precisely by using virtual reality technology as a promising direction in neurorehabilitation. It has been shown that neurobiological effects of virtual reality have a positive effect on the plasticity of neurons, improve cognitive functions and positively affect the psychoemotional state. A case of the positive impact of being in the virtual environment «Outer Space» in a female patient with cognitive impairment and chronic pain is presented.

神经系统损伤影响全球超过10亿人,是导致认知障碍的主要原因之一。现代医学的一个紧迫问题是这一特殊患者群体的神经康复。本文的目的是寻找新的方法来实现更有效的恢复认知功能,正是利用虚拟现实技术作为神经康复的一个有前途的方向。研究表明,虚拟现实的神经生物学效应对神经元的可塑性有积极的影响,改善认知功能,并对心理情绪状态产生积极的影响。一个案例的积极影响是在虚拟环境«外太空»的女性患者认知障碍和慢性疼痛提出。
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引用次数: 0
[FIRES Syndrome in a Preschool Child]. [学龄前儿童的火灾综合症]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro2023123021132
N V Skripchenko, E Y Gorelik, E S Egorova, A A Vilnits, E Y Skripchenko, V B Voitenkov, N V Marchenko, A I Konev

Severe epileptic syndromes of childhood are an urgent problem for pediatric neurologists and neuroresuscitators. The article presents a clinical observation of FIRES syndrome in a pediatric patient, which is a form of severe drug-resistant epilepsy in children of preschool and school age, the development of which is caused by hyperthermia, probably associated with herpesvirus (human herpesvirus type 6) infection. The features of the progressive course and the difficulties of diagnostic search are reflected. An empirical approach to etiotropic therapy is described, since the disease manifested itself with respiratory manifestations and fever. The tactic of pathogenetic treatment is described, in which drugs of polyfunctional action, such as Cytoflavin, have a priority, many years of experience in the use of which allows the authors to recommend it as a starting intensive therapy.

儿童严重的癫痫综合征是儿科神经科医生和神经复苏员迫切需要解决的问题。本文介绍了一名儿科患者的临床观察,该患者是学龄前和学龄儿童严重耐药癫痫的一种形式,其发展是由高温引起的,可能与疱疹病毒(人类疱疹病毒6型)感染有关。反映了病程进展的特点和诊断查找的困难。一种经验的方法,以病因治疗描述,因为疾病本身表现为呼吸症状和发烧。本文描述了发病治疗的策略,其中具有多种功能作用的药物,如细胞黄素,具有优先地位,多年的使用经验使作者推荐将其作为开始的强化治疗。
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引用次数: 0
[Improving the effectiveness of pharmacotherapy in comorbid patients with chronic cerebral ischemia on an outpatient basis]. [在门诊基础上提高慢性脑缺血合并症患者药物治疗的有效性]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro202312303151
T L Vizilo, E G Arefieva

Objective: To study the efficacy and safety of sequential therapy with Mexidol (500 mg 1 time/day for 14 days intravenously) and Mexidol FORTE 250 (Mexidol FORTE 250 for 250 mg 3 times/day, 60 days) in patients with chronic cerebral ischemia (CCI) on an outpatient basis.

Material and methods: The open comparative study included 56 patients aged 46-74 years, age - 60.5+7.9 years. In all patients, the diagnosis of CCI was confirmed by clinical and neuroimaging methods. Patients of group 1 (n=28) received basic therapy and Mexidol, group 2 (n=28) received only basic therapy.

Results: Against the background of therapy in patients of group 1, there was a statistically significant improvement in the state of cognitive functions, a decrease in the severity of symptoms of depression and anxiety, manifestations of asthenia. The treatment was characterized by good tolerability, absence of adverse events and cases of drug interactions.

Conclusion: Sequential therapy with Mexidol and Mexidol FORTE 250 drugs provides relief of the main clinical manifestations of CCI, is characterized by good tolerability and safety.

目的:研究门诊慢性脑缺血(CCI)患者顺序治疗墨西哥idol (500 mg 1次/天,静脉注射14天)和墨西哥idol FORTE 250(墨西哥idol FORTE 250, 250 mg 3次/天,60天)的疗效和安全性。材料与方法:开放性比较研究纳入56例患者,年龄46 ~ 74岁,年龄- 60.5+7.9岁。所有患者均通过临床和神经影像学方法确诊CCI。组1 (n=28)患者同时接受基础治疗和Mexidol治疗,组2 (n=28)患者仅接受基础治疗。结果:在治疗背景下,1组患者认知功能状态改善,抑郁、焦虑症状严重程度减轻,乏力表现明显,均有统计学意义。治疗的特点是良好的耐受性,没有不良事件和药物相互作用的情况下。结论:美西多和美西多FORTE 250药物序贯治疗可缓解CCI的主要临床表现,具有良好的耐受性和安全性。
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引用次数: 1
[Modern principles of therapy for patients with spinal muscular atrophy]. 【脊髓性肌萎缩症的现代治疗原则】。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro202312303134
N S Bofanova, A R Eliseeva, V S Onchina

Spinal muscular atrophy (SMA) is a common cause of childhood mortality among hereditary diseases of the central nervous system, which are caused by the processes of degeneration and death of motor neurons of the anterior horns of the spinal cord. An urgent issue of modern neurology is pathogenetic therapy for this group of patients, the purpose of which is to increase the level of motoneuron survival protein. We performed a search on current methods of treating SMA in Web of Science, Scopus, PubMed, Embase by the keywords: spinal muscular atrophy, neuromuscular diseases, pathogenetic therapy. Significant progress has been made in the treatment of SMA over the past 7 years. A major advance is the introduction of disease-modifying therapies using SMN2 splicing modulation or gene replacement therapy. At the moment, there are 3 FDA-approved drugs for pathogenetic therapy: Nusinersen, Risdiplam, Zolgensma. The article compares the drugs, evaluates their safety and effectiveness according to the available literature. Modern drugs for the pathogenetic therapy of SMA are highly effective and reduce the mortality rate. The results of clinical trials predict the emergence of new modern drugs. This suggests a favorable prognosis for the treatment of patients with SMA.

脊髓性肌萎缩症(SMA)是由脊髓前角运动神经元的退化和死亡引起的中枢神经系统遗传性疾病中儿童死亡的常见原因。现代神经病学的一个紧迫问题是对这类患者进行病理治疗,其目的是提高运动神经元存活蛋白的水平。我们在Web of Science, Scopus, PubMed, Embase中检索了目前治疗SMA的方法,关键词:脊髓性肌萎缩症,神经肌肉疾病,病理治疗。在过去7年中,SMA的治疗取得了重大进展。一个主要的进展是引入了使用SMN2剪接调节或基因替代疗法的疾病修饰疗法。目前,fda批准了3种用于病理治疗的药物:Nusinersen, Risdiplam, Zolgensma。本文根据现有文献对两种药物进行比较,评价其安全性和有效性。现代药物对SMA的病理治疗效果显著,降低了死亡率。临床试验的结果预示着新的现代药物的出现。这表明治疗SMA患者预后良好。
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引用次数: 0
[A clinical case of multiple sclerosis with an episode of schizophrenia-like syndrome]. [多发性硬化症伴精神分裂症样综合征发作的临床病例]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro2023123041120
U Sh Kuzmina, A V Tukhvatullin, K Z Bakhtiyarova, M A Kutlubaev, O V Lyutov, T R Gizatullin

The article presents a clinical observation of a schizophrenia-like disorder in a patient with multiple sclerosis (MS). The patient had highly active MS with a relapsing course, the diagnosis was made based on the McDonald 2017 criteria. During the course of a demyelinating disease of the nervous system, the patient developed an episode of psychotic disorders with symptoms of mutism, hallucinations, delusions and impaired thinking, which was quickly stopped in stationary conditions. This case is of particular interest to neurologists and psychiatrists, since psychotic disorders occur in MS patients and cause difficulties in diagnosis and treatment.

本文介绍了一个临床观察精神分裂症样障碍患者多发性硬化症(MS)。患者为高活性MS伴复发病程,根据McDonald 2017标准进行诊断。在神经系统脱髓鞘疾病的过程中,患者出现了一段精神障碍,症状包括失语、幻觉、妄想和思维受损,在固定条件下很快停止。这个病例是神经学家和精神病学家特别感兴趣的,因为精神障碍发生在多发性硬化症患者身上,给诊断和治疗带来了困难。
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引用次数: 0
[A role of neurosteroids in the pathogenesis of psychiatric disorders]. [神经类固醇在精神疾病发病机制中的作用]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro202312304131
D S Petelin, S P Bairamova, R V Akhapkin, N V Kudryashov, O Yu Sorokina, S A Semin, B A Volel

Despite the proven importance of neurosteroids in many physiological processes, their role in the pathogenesis of the most of psychiatric disorders remains relatively understudied. This article reviews the current clinical evidence on the effects of neurosteroids on the formation and treatment of anxiety disorder, depression, bipolar disorder, and schizophrenia. In particular, the article points out the ambivalent nature of the effects of neurosteroids on GABAA- and other receptors. We are especially interested in the anxiolytic and anxiogenic effects of some neurosteroids, the antidepressant effect of allopregnanolone in treating postpartum and other forms of depression, and the nature of short- and long-term mechanisms of antidepressant effects of neurosteroids of different types. The currently unproven hypothesis about the effect of changes in the level of neurosteroids on the course of bipolar disorder is also discussed, with an analysis of the scientific evidence on the development of schizophrenic symptomatology in relation to changing neurosteroid levels in the context of positive and cognitive symptoms.

尽管神经类固醇在许多生理过程中的重要性已得到证实,但它们在大多数精神疾病发病机制中的作用仍相对缺乏研究。本文综述了目前关于神经类固醇在焦虑症、抑郁症、双相情感障碍和精神分裂症的形成和治疗中的作用的临床证据。特别是,这篇文章指出了神经类固醇对GABAA-和其他受体影响的矛盾性质。我们特别感兴趣的是一些神经类固醇的抗焦虑和致焦虑作用,异孕酮在治疗产后抑郁症和其他形式抑郁症中的抗抑郁作用,以及不同类型神经类固醇抗抑郁作用的短期和长期机制的本质。本文还讨论了目前尚未证实的关于神经类固醇水平变化对双相情感障碍病程影响的假设,并分析了在阳性和认知症状背景下神经类固醇水平变化与精神分裂症症状学发展的科学证据。
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引用次数: 1
[Ethnic features of multipathology in elderly and senile patients of the Republic of Sakha suffering from chronic brain ischemia]. [萨哈共和国老年和老年慢性脑缺血患者的多病理民族特征]。
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.17116/jnevro202312307150
P I Kudrina, A N Bogolepova, A L Ariev
OBJECTIVETo study ethnic characteristics of multipathology in elderly and senile patients with chronic cerebral ischemia living in the Republic of Sakha (Yakutia).MATERIAL AND METHODSThe study included 522 inpatients, aged 60 to 89 years, who were divided into subgroups depending on the stage of chronic cerebral ischemia, ethnicity (Evens, Yakuts and Russians) and age (elderly and senile).RESULTSIn addition to vascular cerebral pathology, comorbidities were identified in patients of older age groups. At the same time, polymorbidity was less pronounced in the Evens, the indigenous inhabitants of the northern regions of Yakutia, than in the Yakuts and representatives of the non-indigenous population - Russians.CONCLUSIONThe relatively rare occurrence of comorbid pathologies in Evens is presumably associated with greater adaptation to the extreme climatic conditions of the North.
目的:探讨萨哈(雅库特)地区老年慢性脑缺血患者的多病理民族特征。材料与方法:研究纳入522例住院患者,年龄60 ~ 89岁,根据慢性脑缺血分期、民族(伊文人、雅库特人和俄罗斯人)和年龄(老年和老年)分为亚组。结果:除了血管性脑病理外,在老年患者中还发现了合并症。与此同时,在雅库特北部地区的土著居民埃文人身上,多病发病率比在雅库特人和非土著人口的代表- -俄罗斯人身上更不明显。结论:Evens相对罕见的共病可能与对北方极端气候条件的更大适应有关。
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引用次数: 0
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Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
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