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[(Auto)immunity in focal epilepsy: mechanisms of (auto‑)immune-inflammatory epileptogenic neurodegeneration]. [局灶性癫痫的(自身)免疫:(自身)免疫炎症致痫性神经变性的机制]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-02 DOI: 10.1007/s00115-024-01695-5
Nico Melzer, Katharina Weber, Saskia Räuber, Felix Rosenow

Objective: While the neuronal mechanisms of epileptic hyperexcitability (HE) have been studied in detail, recent findings suggest that extraneuronal, mainly immune-mediated inflammatory and vascular mechanisms play an important role in the development and progression of HE in epilepsy and the cognitive and behavioral comorbidities.

Material and methods: Narrative review.

Results: As in autoimmune (limbic) encephalitis (ALE/AIE) or Rasmussen's encephalitis (RE), the primary adaptive and innate immune responses and associated changes in the blood-brain barrier (BBB) and neurovascular unit (NVU) can cause acute cortical hyperexcitability (HE) and the development of hippocampal sclerosis (HS) and other structural cortical lesions with chronic HE. Cortical HE, which is associated with malformation of cortical development (MCD) and low-grade epilepsy-associated tumors (LEAT), for example, can be accompanied by secondary adaptive and innate immune responses and alterations in the BBB and NVU, potentially modulating the ictogenicity and epileptogenicity. These associations illustrate the influence of adaptive and innate immune mechanisms and associated changes in the BBB and NVU on cortical excitability and vice versa, suggesting a dynamic and complex interplay of these factors in the development and progression of epilepsy in general.

Discussion: The described concept of a neuro-immune-vascular interaction in focal epilepsy opens up new possibilities for the pathogenetic understanding and thus also for the selective therapeutic intervention.

目的:虽然癫痫高兴奋性(HE)的神经元机制已被详细研究,但最近的研究结果表明,外神经元,主要是免疫介导的炎症和血管机制在癫痫HE的发生和发展以及认知和行为合并症中起着重要作用:叙述性综述:与自身免疫性(边缘)脑炎(ALE/AIE)或拉斯穆森脑炎(RE)一样,原发性适应性免疫反应和先天性免疫反应以及血脑屏障(BBB)和神经血管单元(NVU)的相关变化可导致急性皮质过度兴奋(HE)以及海马硬化(HS)和其他结构性皮质病变与慢性HE的发展。例如,与皮质发育畸形(MCD)和低度癫痫相关肿瘤(LEAT)有关的皮质高兴奋性可伴有继发性适应性和先天性免疫反应以及 BBB 和 NVU 的改变,从而可能调节致病性和致痫性。这些关联说明了适应性免疫和先天性免疫机制以及 BBB 和 NVU 的相关变化对大脑皮层兴奋性的影响,反之亦然,这表明这些因素在整个癫痫的发生和发展过程中具有动态和复杂的相互作用:讨论:所描述的局灶性癫痫中神经-免疫-血管相互作用的概念为了解病因,从而进行选择性治疗干预提供了新的可能性。
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引用次数: 0
[Medical practice in classical Greece : The neurological and psychiatric case reports of the Hippocratic Corpus]. [古典希腊的医疗实践:希波克拉底文库中的神经和精神病例报告]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 Epub Date: 2024-06-27 DOI: 10.1007/s00115-024-01698-2
Werner A Golder, Waltraud Golder

Objective: Which theoretical and practical competences do the neurological and psychiatric case histories of the Hippocratic Corpus convey?

Material and methods: The 431 Hippocratic case histories have been studied for reports and communication on the diagnostics, treatment and prognosis of single persons and groups of patients suffering from neurological and psychiatric diseases.

Results: In the 7 books of the Hippocratic Epidemics, a total of 128 patients with neurological and psychiatric symptoms are described. Epidemic fever and its variants were the leading predisposing conditions and the main symptoms were delirium, coma, insomnia, headache, speech disorders and convulsions. A number of patients with phrenitis and opisthotonos are also reported. The majority of the sick persons were male, were teenagers or adults and 47 of them are mentioned by name. The patient's information about the course is often just as informative as the doctor's observations. Treatment was limited to physical and dietary measures.

Discussion: The Hippocratic physician diagnosed and attempted to treat a large number of neurological and psychiatric diseases. The often almost continuous observations of the patients led to astonishingly precise predictions of the course and the prospects of recovery. Numerous symptoms described in the case studies, including carphologia and opisthotonus, have entered the neurological vocabulary. The retrospective etiological analysis of the reports leads to the almost explicit identification of neurosyphilis and encephalitis lethargica. The therapeutic measures described by the author were, as the changeable course of the diseases shows, only of limited effectiveness despite a very differentiated application over time, both against the underlying diseases and the neurological and psychiatric complications.

目的希波克拉底文库》中的神经病学和精神病学病例史传达了哪些理论和实践能力?研究了 431 篇希波克拉底病历,以了解单个和群体神经和精神疾病患者的诊断、治疗和预后的报告和交流:在《希波克拉底流行病学》的 7 本书中,共描述了 128 名患有神经和精神症状的病人。流行性热病及其变种是主要的致病条件,主要症状是谵妄、昏迷、失眠、头痛、语言障碍和抽搐。此外,还报告了一些膈肌炎和瞳孔散大的患者。大多数患者为男性、青少年或成年人,其中有 47 人的名字被提及。病人提供的病程信息往往与医生的观察结果同样具有参考价值。治疗仅限于物理和饮食措施:希波克拉底医生诊断并试图治疗大量的神经和精神疾病。对病人的观察往往几乎是持续不断的,从而对病程和康复前景做出了惊人的精确预测。病例研究中描述的许多症状,包括 "呓语"(carphologia)和 "瞳孔缩小"(opisthotonus),都已成为神经病学的词汇。通过对病例报告进行病因学分析,几乎可以明确诊断出神经梅毒和白塞脑炎。作者所描述的治疗措施,正如疾病多变的病程所显示的那样,尽管随着时间的推移,针对潜在疾病以及神经和精神并发症的治疗措施也有很大的不同,但效果有限。
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引用次数: 0
[Good bye, Neurology]. [再见,神经病学]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 DOI: 10.1007/s00115-024-01757-8
Hans J Markowitsch
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引用次数: 0
[Migraine or pseudomigraine, that is the question! : The HaNDL syndrome]. [偏头痛还是假性偏头痛,这是个问题!:HaNDL 综合征]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 Epub Date: 2024-06-03 DOI: 10.1007/s00115-024-01682-w
Jens Schaumberg
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引用次数: 0
Mitteilungen der Deutschen Schlaganfall-Gesellschaft (DSG). 德国中风协会通讯》(DSG)。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 DOI: 10.1007/s00115-024-01748-9
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引用次数: 0
[Pathogen-induced diseases of the spinal cord and adjacent structures]. [由病原体引起的脊髓及邻近结构疾病]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 Epub Date: 2024-09-12 DOI: 10.1007/s00115-024-01743-0
B Bassa, F Hahner, U Meyding-Lamadé

Pathogen-induced myelitis is an inflammatory disease of the spinal cord that can be caused by various pathogens including viruses, bacteria, fungi or parasites. The most frequent viral pathogens include herpes and enteroviruses, while bacterial myelitis can be caused by, e.g., Mycobacterium tuberculosis, Borrelia burgdorferi and Treponema pallidum. Fungi such as Candida and Aspergillus and parasites such as Toxoplasma gondii and schistosomes can also cause myelitis. The main symptom is subacute paraplegia with motor, sensory and autonomic deficits to varying degrees, often accompanied by fever and a general malaise. Following a thorough clinical examination and review of the medical history diagnostic imaging procedures, such as magnetic resonance imaging (MRI) along with cerebrospinal fluid analysis and blood tests that include antibody testing are warranted. The treatment is directed at the cause of the myelitis and mostly with anti-infective agents but for some viral pathogens no specific treatment is available and the only option is a symptomatic treatment. The prognosis is very variable and depends on the etiology and severity of the disease. A fast diagnosis and targeted treatment are crucial to achieve a good outcome.

病原体诱发的脊髓炎是一种脊髓炎症性疾病,可由病毒、细菌、真菌或寄生虫等各种病原体引起。最常见的病毒病原体包括疱疹病毒和肠道病毒,而细菌性脊髓炎可由结核分枝杆菌、布氏杆菌和苍白链球菌等引起。念珠菌和曲霉菌等真菌以及弓形虫和血吸虫等寄生虫也可引起脊髓炎。主要症状是亚急性截瘫,伴有不同程度的运动、感觉和自主神经功能障碍,常伴有发热和全身不适。在进行全面的临床检查和病史回顾后,需要进行诊断性影像学检查,如磁共振成像(MRI)、脑脊液分析和包括抗体检测在内的血液检测。治疗主要针对脊髓炎的病因,大多使用抗感染药物,但对于某些病毒性病原体,目前尚无特效治疗方法,唯一的选择是对症治疗。预后很不稳定,取决于病因和疾病的严重程度。快速诊断和有针对性的治疗是取得良好疗效的关键。
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引用次数: 0
[Peripheral neuroimmunological diseases - Neuropathological insights and clinical perspectives]. [周围神经免疫疾病--神经病理学见解和临床视角]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 Epub Date: 2024-09-20 DOI: 10.1007/s00115-024-01725-2
Sarah Hoffmann, Marie-Therese Holzer, Corinna Preuße, Tobias Ruck, Nikolas Ruffer, Frauke Stascheit, Werner Stenzel

This article deals with peripheral neuroimmunological diseases and briefly outlines the currently most important aspects and treatment developments. Idiopathic inflammatory myopathies have different mechanisms of development, manifestations and prognoses. New classification systems and more specific treatment concepts have been developed. The IIMs include different subgroups. These entities can have specific autoantibodies. Diagnostically, a muscle biopsy is generally desirable for a precise diagnosis and is essential in unclear cases. Primary systemic vasculitides can be divided into different groups based on the predominant pattern of involvement, while secondary vasculitides and single organ vasculitides are also differentiated. Vasculitic myopathy cannot be equated with myositis and a reliable distinction is currently only possible by a muscle biopsy. Treatment concepts should be developed on an interdisciplinary basis. Chronic inflammatory demyelinating polyneuropathy is the most frequent immune-mediated neuropathy and is characterized by a predominant demyelination of the motor and sensory nerves. The disease course runs in phases or is progressive and leads to significant disability and reduction in quality of life, despite current standard treatment. Novel treatment approaches are currently undergoing clinical trials. Myasthenia gravis, with the leading symptom of exercise-induced muscle weakness, is caused by autoantibodies against structures of the neuromuscular endplate. Autoantibody testing is the most important pillar in the diagnosis and is now also increasingly guiding treatment decisions. Overall, peripheral neuroimmunological diseases represent a heterogeneous group. Increasing knowledge of the pathophysiology is the key to numerous developments in diagnostics and treatment, which could lead to far-reaching practical changes in the future.

本文论述了周围神经免疫疾病,并简要概述了当前最重要的方面和治疗进展。特发性炎症性肌病的发病机制、表现和预后各不相同。新的分类系统和更具体的治疗概念已经形成。特发性炎症性肌病包括不同的亚组。这些疾病都有特定的自身抗体。在诊断方面,肌肉活检通常是精确诊断的理想方法,对于不明确的病例来说,肌肉活检是必不可少的。原发性系统性血管炎可根据主要受累模式分为不同的组别,而继发性血管炎和单器官血管炎也有区别。血管炎性肌病不能等同于肌炎,目前只有通过肌肉活检才能进行可靠的区分。应在跨学科的基础上制定治疗方案。慢性炎症性脱髓鞘性多发性神经病是最常见的免疫介导型神经病,其特点是运动神经和感觉神经脱髓鞘为主。该病的病程呈阶段性或进行性发展,会导致严重的残疾和生活质量下降,尽管目前采用了标准治疗方法。新的治疗方法目前正在进行临床试验。肌无力症的主要症状是运动引起的肌无力,是由针对神经肌肉终板结构的自身抗体引起的。自身抗体检测是诊断中最重要的支柱,现在也越来越多地指导治疗决策。总体而言,外周神经免疫疾病是一个异质性的群体。对病理生理学的进一步了解是诊断和治疗领域取得众多进展的关键,这可能会在未来带来意义深远的实际变化。
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引用次数: 0
[Immunological foundations of neurological diseases]. [神经系统疾病的免疫学基础]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-01 Epub Date: 2024-07-02 DOI: 10.1007/s00115-024-01696-4
Hansjörg Schild, Tobias Bopp

Background: Neurodegenerative diseases represent an increasing challenge in ageing societies, as only limited treatment options are currently available.

Objective: New research methods and interdisciplinary interaction of different disciplines have changed the way neurological disorders are viewed and paved the way for the comparatively new field of neuroimmunology, which was established in the early 1980s. Starting from neurological autoimmune diseases, such as multiple sclerosis, knowledge about the involvement of immunological processes in other contexts, such as stroke or traumatic brain injury, has been significantly expanded in recent years.

Material and methods: This review article provides an overview of the role of the immune system and the resulting potential for novel treatment approaches.

Results: The immune system plays a central role in fighting infections but is also able to react to the body's own signals under sterile conditions and cause inflammation and subsequent adaptive immune responses through the release of immune mediators and the recruitment and differentiation of certain immune cell types. This can be beneficial in initiating healing processes; however, chronic inflammatory conditions usually have destructive consequences for the tissue and the organism and must be interrupted.

Conclusion: It is now known that different cells of the immune system play an important role in neurological diseases. Regulatory mechanisms, which are mediated by regulatory T cells or Th2 cells, are usually associated with a good prognosis, whereas inflammatory processes and polarization towards Th1 or Th17 have a destructive character. Novel immunomodulators, which are also increasingly being used in cancer treatment, can now be used in a tissue-specific manner and therefore offer great potential for use in neurological diseases.

背景:神经退行性疾病是老龄化社会面临的一个日益严峻的挑战,因为目前可供选择的治疗方法非常有限:新的研究方法和不同学科间的互动改变了人们对神经系统疾病的看法,并为 20 世纪 80 年代初建立的相对较新的神经免疫学领域铺平了道路。从神经系统自身免疫性疾病(如多发性硬化症)开始,近年来有关免疫过程参与其他情况(如中风或脑外伤)的知识得到了显著扩展:这篇综述文章概述了免疫系统的作用以及由此产生的新型治疗方法的潜力:免疫系统在抗感染方面发挥着核心作用,但在无菌条件下也能对人体自身的信号做出反应,并通过释放免疫介质以及招募和分化某些免疫细胞类型来引起炎症和随后的适应性免疫反应。这对启动愈合过程是有益的;但慢性炎症通常会对组织和机体造成破坏性后果,必须加以阻断:现在人们已经知道,免疫系统的不同细胞在神经系统疾病中扮演着重要角色。由调节性 T 细胞或 Th2 细胞介导的调节机制通常预后良好,而炎症过程和 Th1 或 Th17 极化则具有破坏性。新型免疫调节剂也越来越多地用于癌症治疗,现在可以以组织特异性的方式使用,因此在神经系统疾病方面具有巨大的应用潜力。
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引用次数: 0
[Mentally ill citizens as victims of violation of physical integrity and extermination under the NS regime based on the example of a German city]. [根据德国城市的例子,精神病公民是新国家政权侵犯人身安全和灭绝行为的受害者]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-26 DOI: 10.1007/s00115-024-01745-y
Beatrice Rose, Jörg-Stefan Schulz, Eckhard Sundermann, Paraskevi Mavrogiorgou, Georg Juckel

Background: Even 90 years after the National Socialist seizure of power and the beginning of the darkest chapter of German psychiatric history, examination of the medical historical past retains great relevance as an ongoing medical responsibility. The interventions in the lives of mentally ill people by the National Socialist regime are a firmly established part of medical historical research; however, little is known about how specific regions proceeded with affected citizens.

Objective: The aim of this study was therefore to identify transfer routes of the "euthanasia" transports and places of death of the victims from Bochum as well as their patient characteristics.

Materials and methods: During the timeframe studied, inpatient care for mentally ill patients from the Westphalia region was provided in seven provincial institutions. There was no psychiatric clinic in Bochum at the time, so patients were distributed among these institutions. The investigation was based on the transfer lists of the Westphalian provincial institutions to the killing sites.

Results and conclusion: A total of 366 Bochum citizens affected by "euthanasia" transfers were identified. The transport lists were verified by admission and departure books, death lists, and patient files. The hereditary health files of the city archive were used to examine the relocated Bochum patients to determine whether they were victims of forced sterilization.

背景:即使在国家社会主义夺取政权和德国精神病史上最黑暗篇章开始的 90 年之后,作为一项持续的医学责任,对医学历史的研究仍具有重要意义。国家社会主义政权对精神病患者生活的干预是医学史研究的一个既定部分;然而,人们对特定地区如何处理受影响的公民却知之甚少:因此,本研究旨在确定 "安乐死 "的转运路线、波鸿受害者的死亡地点及其患者特征:在研究期间,威斯特伐利亚地区的精神病患者在七个省级机构接受住院治疗。当时波鸿还没有精神病诊所,因此病人分布在这些机构中。调查以威斯特法伦省精神病院向杀人现场的转院名单为依据:结果和结论:共确认了 366 名受 "安乐死 "转院影响的波鸿市民。转院名单通过入院和出院记录、死亡名单和病人档案进行了核实。市档案馆的遗传健康档案被用来检查被转移的波鸿病人,以确定他们是否是强制绝育的受害者。
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引用次数: 0
[The relevance of pharmacogenetic testing in forensics]. [药物基因检测在法医学中的相关性]。
IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-26 DOI: 10.1007/s00115-024-01746-x
Helmar Weiß, Erikas Vasiliauskas, Volkmar Blendl, Aleh Maksimiuk
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引用次数: 0
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