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Quantitative spinal cord imaging: Early ALS diagnosis and monitoring of disease progression. 脊髓定量成像:早期 ALS 诊断和疾病进展监测。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-14 DOI: 10.1016/j.neurol.2024.10.005
M Khamaysa, M El Mendili, V Marchand, G Querin, P-F Pradat

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons in the cortex, brainstem, and spinal cord. This degeneration leads to muscular weakness, progressively impairing motor functions and ultimately resulting in respiratory failure. The clinical, genetic, and pathological heterogeneity of ALS, combined with the absence of reliable biomarkers, significantly challenge the efficacy of therapeutic trials. Despite these hurdles, neuroimaging, and particularly spinal cord imaging, has emerged as a promising tool. It provides insights into the involvement of both upper and lower motor neurons. Quantitative spinal imaging has the potential to facilitate early diagnosis, enable accurate monitoring of disease progression, and refine the design of clinical trials. In this review, we explore the utility of spinal cord imaging within the broader context of developing spinal imaging biomarkers in ALS. We focus on a both diagnostic and prognostic biomarker in ALS, highlighting its pivotal role in elucidating the disease's underlying pathology. We also discuss the existing limitations and future avenues for research, aiming to bridge the translational gap between academic research and its application in clinical practice and therapeutic trials.

肌萎缩侧索硬化症(ALS)是一种致命的神经退行性疾病,其特征是大脑皮层、脑干和脊髓中的运动神经元逐渐退化。这种退化导致肌肉无力,逐渐损害运动功能,最终导致呼吸衰竭。渐冻人症在临床、遗传和病理上的异质性,再加上缺乏可靠的生物标志物,给治疗试验的疗效带来了巨大挑战。尽管存在这些障碍,神经成像,尤其是脊髓成像,已成为一种很有前途的工具。它可以深入了解上下运动神经元的参与情况。脊髓定量成像具有促进早期诊断、准确监测疾病进展和完善临床试验设计的潜力。在本综述中,我们将在开发 ALS 脊柱成像生物标记物的大背景下探讨脊髓成像的效用。我们将重点放在 ALS 的诊断和预后生物标志物上,强调其在阐明该疾病潜在病理方面的关键作用。我们还讨论了现有的局限性和未来的研究途径,旨在弥合学术研究与临床实践和治疗试验应用之间的转化差距。
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引用次数: 0
Adding corticosteroids to galcanezumab in medication overuse headache: A three-arm head-to-head prospective observational cohort study. 在治疗药物滥用性头痛时,在加坎儿珠单抗中加入皮质类固醇:三臂头对头前瞻性观察队列研究。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-06 DOI: 10.1016/j.neurol.2024.10.003
S Braca, R De Simone, A Stornaiuolo, G Cretella, A Miele, C V Russo

Background: Medication overuse headache (MOH) is a condition where pain relief medications cause chronic headaches due to excessive use. Recent advancements highlight the effectiveness of preventive treatments like anti-CGRP monoclonal antibodies. Current strategies combine medication withdrawal and preventive treatments, with corticosteroids traditionally used to ease withdrawal symptoms.

Methods: This is a prospective three-arm observational cohort study comparing the effectiveness and safety of galcanezumab alone, galcanezumab plus prednisone and prednisone alone for the treatment of MOH. We enrolled 75 patients. Prednisone was administered at an initial dose of 50mg daily, and then tapered off over 28days. Duration of follow-up was 3months.

Results: All treatments proved effective (P<0.001). We found a significant reduction of mean monthly days with headache in the galcanezumab plus prednisone group (baseline: 25, IQR: 20-30; after 3months: 7, IQR: 5-10), in the galcanezumab group (baseline: 25, IQR: 20-30; after 3months: 10, IQR: 5-14) and in the Prednisone group (baseline: 25, IQR: 20-28; after 3months: median: 15 days, IQR: 8-22days). Patients treated with prednisone reported a higher incidence of side effects (P=0.002).

Conclusion: Our study indicates that both galcanezumab and prednisone decrease the frequency of headaches in patients with MOH. The combined usage of these treatments showed the highest reduction in mean monthly headache days. However, treatment with prednisone determined a significant rate of adverse events, therefore we suggest its use only in unresponsive patients. In all other patients galcanezumab appears to be a safe and effective option.

背景:药物过度使用性头痛(MOH)是一种因过度使用止痛药物而导致慢性头痛的病症。最近的研究进展突显了抗 CGRP 单克隆抗体等预防性治疗的有效性。目前的策略是将停药和预防性治疗结合起来,传统上使用皮质类固醇来缓解停药症状:这是一项前瞻性三臂观察性队列研究,比较了单用加奈珠单抗、加奈珠单抗加泼尼松和单用泼尼松治疗MOH的有效性和安全性。我们共招募了75名患者。泼尼松的初始剂量为每天50毫克,然后在28天内逐渐减量。随访时间为 3 个月:结果:所有治疗方法均有效(PC结论:所有治疗方法均有效:我们的研究表明,加康珠单抗和泼尼松都能降低MOH患者的头痛频率。联合使用这两种疗法后,每月平均头痛天数的减少幅度最大。然而,使用泼尼松治疗会产生大量不良反应,因此我们建议仅在无反应的患者中使用。对于所有其他患者,加康珠单抗似乎是一种安全有效的选择。
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引用次数: 0
Reprint of: Scientific statement from the French neurovascular and cardiac societies for improved detection of atrial fibrillation after ischaemic stroke and transient ischaemic attack. 重印本:法国神经血管和心脏学会关于改进缺血性中风和短暂性缺血发作后心房颤动检测的科学声明。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-06 DOI: 10.1016/j.neurol.2024.10.001
Nicolas Gaillard, Jean-Claude Deharo, Laurent Suissa, Pascal Defaye, Igor Sibon, Christophe Leclercq, Sonia Alamowitch, Céline Guidoux, Ariel Cohen

Atrial fibrillation (AF) is the primary cause of ischaemic stroke and transient ischaemic attack (TIA). AF is associated with a high risk of recurrence, which can be reduced using optimal prevention strategies, mainly anticoagulant therapy. The availability of effective prophylaxis justifies the need for a significant, coordinated and thorough transdisciplinary effort to screen for AF associated with stroke. A recent French national survey, initiated and supported by the Société française neurovasculaire (SFNV) and the Société française de cardiologie (SFC), revealed many shortcomings, such as the absence or inadequacy of telemetry equipment in more than half of stroke units, insufficient and highly variable access to monitoring tools, delays in performing screening tests, heterogeneous access to advanced or connected ambulatory monitoring techniques, and a lack of dedicated human resources. The present scientific document has been prepared on the initiative of the SFNV and the SFC with the aim of helping to address the current shortcomings and gaps, to promote efficient and cost-effective AF detection, and to improve and, where possible, homogenize the quality of practice in AF screening among stroke units and outpatient post-stroke care networks. The working group, composed of cardiologists and vascular neurologists who are experts in the field and are nominated by their peers, reviewed the literature to propose statements, which were discussed in successive cycles, and maintained, either by consensus or by vote, as appropriate. The text was then submitted to the SFNV and SFC board members for review. This scientific statement document argues for the widespread development of patient pathways to enable the most efficient AF screening after stroke. This assessment should be carried out by a multidisciplinary team, including expert cardiologists and vascular neurologists.

心房颤动(房颤)是缺血性中风和短暂性脑缺血发作(TIA)的主要原因。心房颤动具有很高的复发风险,而采用最佳预防策略(主要是抗凝疗法)可以降低复发风险。有效的预防措施的可用性证明,有必要开展一项重要、协调和彻底的跨学科工作,筛查与中风相关的房颤。最近,由法国神经血管协会(SFNV)和法国心脏病协会(SFC)发起并支持的一项法国全国性调查显示了许多不足之处,如超过半数的中风科室没有遥测设备或遥测设备不足、监测工具不足且使用情况参差不齐、筛查测试延迟、先进或联网的非卧床监测技术使用情况参差不齐,以及缺乏专门的人力资源。本科学文件由国家卒中筛查中心(SFNV)和国家急性心肌梗死筛查中心(SFC)共同编写,旨在帮助解决目前存在的不足和差距,促进高效、经济的心房颤动检测,提高并尽可能统一卒中单元和卒中后门诊护理网络的心房颤动筛查实践质量。工作组由该领域的心脏病专家和血管神经科专家组成,他们由同行提名,通过查阅文献提出声明,并在连续的周期内对声明进行讨论,根据情况以协商一致或投票的方式维持声明。声明文本随后提交给 SFNV 和 SFC 董事会成员审阅。本科学声明文件主张广泛制定患者路径,以便在卒中后进行最有效的房颤筛查。该评估应由包括心脏病专家和血管神经学家在内的多学科团队进行。
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引用次数: 0
The immunology underlying CNS autoantibody diseases 中枢神经系统自身抗体疾病的免疫学基础。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.07.002
J. Cleaver , B. Ceronie , C. Strippel , A. Handel , S.R. Irani
The past two decades have seen a considerable paradigm shift in the way autoimmune CNS disorders are considered, diagnosed, and treated; largely due to the discovery of novel autoantibodies directed at neuroglial surface or intracellular targets. This approach has enabled multiple bona fide CNS autoantibody-associated diseases to thoroughly infiltrate the sphere of clinical neurology, facilitating advances in patient outcomes. This review focusses on the fundamental immunological concepts behind CNS autoantibody-associated diseases. First, we briefly review the broad phenotypic profiles of these conditions. Next, we explore concepts around immune checkpoints and the related B cell lineage. Thirdly, the sources of autoantibody production are discussed alongside triggers of tolerance failure, including neoplasms, infections and iatrogenic therapies. Penultimately, the role of T cells and leucocyte trafficking into the CNS are reviewed. Finally, biological insights from responses to targeted immunotherapies in different CNS autoantibody-associated diseases are summarised. The continued and rapid expansion of the CNS autoantibody-associated field holds promise for further improved diagnostic and therapeutic paradigms, ultimately leading to further improvements in patient outcomes.
在过去二十年中,自身免疫性中枢神经系统疾病的考虑、诊断和治疗方式发生了巨大的范式转变,这主要归功于针对神经胶质细胞表面或细胞内靶点的新型自身抗体的发现。这种方法使多种真正的中枢神经系统自身抗体相关疾病彻底渗入临床神经病学领域,促进了患者治疗效果的改善。本综述重点探讨中枢神经系统自身抗体相关疾病背后的基本免疫学概念。首先,我们简要回顾了这些疾病的广泛表型特征。其次,我们探讨了免疫检查点和相关 B 细胞系的概念。第三,讨论自身抗体产生的来源以及耐受失败的诱因,包括肿瘤、感染和先天性疗法。最后,回顾了 T 细胞的作用和白细胞向中枢神经系统的迁移。最后,总结了不同中枢神经系统自身抗体相关疾病对靶向免疫疗法反应的生物学启示。中枢神经系统自身抗体相关领域的持续快速发展为进一步改进诊断和治疗范例带来了希望,最终将进一步改善患者的预后。
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引用次数: 0
Predicting the future of autoimmune encephalitides 预测自身免疫性脑炎的未来。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.08.003
M. Guasp , J. Dalmau
The concept that many neurologic and psychiatric disorders of unknown cause are immune-mediated has evolved fast during the past 20 years. The main contribution to the expansion of this field has been the discovery of antibodies that attack neuronal or glial cell-surface proteins or receptors, directly modifying their structure and function. These antibodies facilitate the diagnosis and prompt treatment of patients who often improve with immunotherapy. The identification of this group of diseases, collectively named “autoimmune encephalitides”, was preceded by many years of investigations on other autoimmune CNS disorders in which the antibodies are against intracellular proteins, occur more frequently with cancer, and associate with cytotoxic T-cell responses that are less responsive to immunotherapy. Here, we first trace the recent history of the autoimmune encephalitides and address how to assess the clinical value and implement in our practice the rapid pace of autoantibody discovery. In addition, we review recent developments in the post-acute stage of the two main autoimmune encephalitides (NMDAR and LGI1) focusing on symptoms that are frequently overlooked or missed, and therefore undertreated. Because a better understanding of the pathophysiology of these diseases relies on animal models, we examine currently available studies, recognizing the existing needs for better and all-inclusive neuro-immunobiological models. Finally, we assess the status of biomarkers of disease outcome, clinical scales, current treatment strategies, and emerging therapies including CAR T-cell technology. Altogether, this overview is intended to identify gaps of knowledge and provide suggestions for improvement and insights for future research.
在过去的 20 年中,许多原因不明的神经和精神疾病都是由免疫介导的这一概念得到了快速发展。这一领域的发展主要得益于抗体的发现,这些抗体可以攻击神经元或神经胶质细胞表面蛋白或受体,直接改变它们的结构和功能。这些抗体为诊断和及时治疗患者提供了便利,这些患者往往在接受免疫疗法后病情有所好转。在发现这一类疾病(统称为 "自身免疫性脑炎")之前,我们已对其他自身免疫性中枢神经系统疾病进行了多年研究,这些疾病的抗体针对细胞内蛋白,更常与癌症同时发生,并与对免疫疗法反应较弱的细胞毒性T细胞反应相关联。在此,我们首先回顾了自身免疫性脑病的近代史,并探讨了如何评估自身抗体的临床价值,以及如何在实践中快速应用自身抗体的发现。此外,我们还回顾了两种主要自身免疫性脑炎(NMDAR 和 LGI1)急性期后阶段的最新进展,重点关注那些经常被忽视或遗漏、因而治疗不足的症状。由于更好地了解这些疾病的病理生理学有赖于动物模型,我们审视了现有的研究,认识到目前需要更好的、包罗万象的神经免疫生物学模型。最后,我们评估了疾病结局生物标志物、临床量表、当前治疗策略以及包括 CAR T 细胞技术在内的新兴疗法的现状。总之,本综述旨在找出知识差距,为未来研究提供改进建议和见解。
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引用次数: 0
Soluble biomarkers for immune checkpoint inhibitor-related encephalitis: A mini-review 免疫检查点抑制剂相关脑炎的可溶性生物标记物:微型综述。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.08.007
A. Farina , M. Villagrán-García , B. Joubert
Immune checkpoint inhibitors lead to effective antitumour responses but also to immune-related adverse events (irAEs), which affect the nervous system in 1–5% of patients. Encephalitis is the most frequent central nervous system irAE and is clinically relevant due to its high severity and mortality. Early diagnosis is crucial but is hampered by the broad list of alternative diagnoses, the lack of established diagnostic criteria, and the need of extensive diagnostic procedures (e.g., spinal tap, brain MRI) alongside expert neurological evaluation. Additionally, the response to corticosteroids is inconsistent, and the management of corticosteroid-refractory patients remains poorly defined. This mini-review discusses the role of various soluble biomarkers in the diagnosis, prognostication, and management of ICI-encephalitis. Neural antibodies, which are well-established biomarkers of autoimmune and paraneoplastic encephalitis, are found in only a subset of ICI-encephalitis, in which they can aid to establish the diagnosis. The most prevalent are paraneoplastic neurological syndromes (PNS)-associated antibodies, which are found almost exclusively in focal ICI-encephalitis syndromes and are associated with poor outcomes, possibly due to predominantly cytotoxic T cell involvement leading to irreversible neuronal loss. Beside antibodies, serum brain injury biomarkers such as NfL and S100B are elevated in ICI-encephalitis and, even if non-specific, may be useful as a routine test to quickly identify patients in whom neurological evaluation and second-level diagnostic procedures should be prioritized. Additionally, higher serum and CSF NfL levels have been associated with lack of treatment response in ICI-encephalitis, suggesting they may have a prognostic role. Among cytokines, elevated interleukin 6 (IL6) levels have been observed in serum and/or CSF samples of some patients with ICI-encephalitis, but the role of IL6 as a biomarker for response to IL6-directed therapies requires further investigation. Likewise, the value of other biomarkers, including T cells markers and HLA haplotypes, still needs to be evaluated in large cohorts. Overall, neural antibodies are important diagnostic and prognostic biomarkers in ICI-encephalitis, and other soluble biomarkers, especially NfL, deserve further investigation since they have a promising application in clinical practice.
免疫检查点抑制剂能产生有效的抗肿瘤反应,但也会导致免疫相关不良事件(irAEs),其中1%-5%的患者会影响神经系统。脑炎是最常见的中枢神经系统 irAE,因其严重性和死亡率高而具有临床意义。早期诊断至关重要,但由于可供选择的诊断较多、缺乏既定的诊断标准、需要进行广泛的诊断程序(如脊髓穿刺、脑核磁共振成像)以及专业的神经系统评估,早期诊断受到了阻碍。此外,患者对皮质类固醇的反应并不一致,对皮质类固醇难治性患者的处理方法也不明确。这篇微型综述讨论了各种可溶性生物标志物在 ICI脑炎的诊断、预后和管理中的作用。神经抗体是自身免疫性脑炎和副肿瘤性脑炎的公认生物标志物,但仅在一部分 ICI 脑炎中发现神经抗体,它们有助于确定诊断。最常见的是副肿瘤性神经综合征(PNS)相关抗体,这种抗体几乎只出现在局灶性 ICI 脑炎综合征中,而且与不良预后有关,这可能是由于细胞毒性 T 细胞的主要参与导致了不可逆的神经元损失。除抗体外,血清脑损伤生物标志物(如 NfL 和 S100B)在 ICI脑炎中也会升高,即使是非特异性的,也可作为常规检测,用于快速识别应优先进行神经系统评估和二级诊断程序的患者。此外,较高的血清和脑脊液 NfL 水平与 ICI脑炎患者缺乏治疗反应有关,这表明它们可能具有预后作用。在细胞因子中,一些 ICI 脑炎患者的血清和/或 CSF 样本中观察到白细胞介素 6(IL6)水平升高,但 IL6 作为 IL6 引导疗法反应的生物标志物的作用还需要进一步研究。同样,其他生物标记物的价值,包括T细胞标记物和HLA单倍型,仍需在大样本中进行评估。总之,神经抗体是 ICI 脑炎重要的诊断和预后生物标志物,其他可溶性生物标志物,尤其是 NfL,值得进一步研究,因为它们在临床实践中具有广阔的应用前景。
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引用次数: 0
Antibodies in immune-mediated peripheral neuropathies. Where are we in 2024? 免疫介导的周围神经病中的抗体。2024 年我们在哪里?
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.09.002
J.-C. Antoine
Over the past 30 years, about 20 antibodies have been identified in immune-mediated neuropathies, recognizing membrane or intracellular proteins or glycolipids of neuron and Schwann cells. This article reviews the different methods used for their detection, what we know about their pathogenic role, how they have helped identify several disorders, and how they are essential for diagnosis. Despite sustained efforts, some immune-mediated disorders still lack identified autoantibodies, notably the classical form of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. The reasons for this are discussed. The article also tries to determine potential future developments in antibody research, particularly the use of omic approaches and the search for other types of biomarkers beyond diagnostic ones, such as those that can identify patients who will respond to a given treatment.
过去 30 年中,在免疫介导的神经病中发现了约 20 种抗体,它们能识别神经元和许旺细胞的膜蛋白或细胞内蛋白或糖脂。本文回顾了用于检测这些抗体的不同方法、我们对其致病作用的了解、它们如何帮助确定几种疾病以及它们如何对诊断至关重要。尽管做出了不懈的努力,但一些免疫介导的疾病仍然缺乏已确定的自身抗体,特别是经典形式的格林-巴利综合征和慢性炎症性脱髓鞘性多发性神经病。文章讨论了造成这种情况的原因。文章还试图确定抗体研究未来的潜在发展方向,特别是使用欧姆方法和寻找诊断方法以外的其他类型生物标记物,如能识别对特定治疗方法有反应的患者的生物标记物。
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引用次数: 0
Mechanisms of immune tolerance breakdown in paraneoplastic neurological syndromes 副肿瘤性神经综合征的免疫耐受破坏机制。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.08.002
E. Peter , P. Dumez , J. Honnorat , V. Desestret
Paraneoplastic neurological syndromes (PNS) are rare autoimmune disorders triggered by the presence of a cancer. The autoimmunity is herein directed against proteins expressed both in the tumor and in the nervous system, namely the onconeural antigens, against which are directed specific autoantibodies, each of them characterizing a neurological syndrome. The mechanisms of the immune tolerance breakdown in PNS leading to the production of specific autoantibodies directed against the nervous system and leading to the immune attack begins to be explained. Each syndrome is associated with a specific histo-molecular subtype of tumor suggesting a link between the PNS genesis and oncogenesis. The expression of the onconeural antigen by these tumors is insufficient to explain the immune tolerance breakdown. In some PNS tumors, alterations of the antigen have been identified: mutations, gene copy number variation and overexpression of transcript and protein. But in others PNS, no such molecular alterations of the onconeural antigens have been demonstrated. In these cases, other mechanisms of neoantigen generation that may be involved remain to be deciphered. Cancer outcomes of PNS tumors are also characterized by the high frequency of lymph node metastasis at diagnosis. At the primary tumor site, the antitumor immune reaction seems to be particularly intense and characterized by a prominence of B-cell and Ig-secreting plasma cells that may generate the autoantibody secretion. The immune control mechanisms leading to such organization of the immune attack are not known to date. Renewed research efforts are thus needed to better understand the mechanism of immune tolerance breakdown in each PNS and determine potential targets to meet the therapeutic challenges posed by these rare disorders.
副肿瘤性神经综合征(PNS)是由癌症引发的罕见自身免疫性疾病。自身免疫针对的是肿瘤和神经系统中都表达的蛋白质,即肿瘤抗原,针对这些抗原产生特异性自身抗体,每种抗体都是一种神经综合征的特征。我们开始解释 PNS 免疫耐受破坏导致产生针对神经系统的特异性自身抗体并引发免疫攻击的机制。每种综合征都与特定的组织分子亚型肿瘤有关,这表明 PNS 的发生与肿瘤发生之间存在联系。这些肿瘤表达的肿瘤抗原不足以解释免疫耐受的崩溃。在一些 PNS 肿瘤中,已发现抗原发生了改变:突变、基因拷贝数变异以及转录本和蛋白质的过度表达。但在其他一些 PNS 中,则未发现肿瘤抗原有此类分子改变。在这些病例中,可能涉及新抗原生成的其他机制仍有待破译。PNS 肿瘤的癌症结局还以诊断时淋巴结转移的高频率为特征。在原发肿瘤部位,抗肿瘤免疫反应似乎特别强烈,其特点是 B 细胞和分泌 Ig 的浆细胞突出,可能产生自身抗体分泌。迄今为止,导致这种免疫攻击组织的免疫控制机制尚不清楚。因此,需要重新开展研究工作,以更好地了解每种 PNS 免疫耐受破坏的机制,并确定潜在的靶点,以应对这些罕见疾病带来的治疗挑战。
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引用次数: 0
What's new in NMOSD and MOGAD? NMOSD 和 MOGAD 有哪些新内容?
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.08.004
R. Marignier
In this mini-review, we focus on novelties in the field of neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD). We first describe the proposed criteria for MOGAD and evaluate their impact and potential limitations, with a highlight on the subgroups of patients tested MOG-antibody positive only in the cerebrospinal fluid. We then propose a brief state of the art on the current knowledge on the so-call “double seronegative” NMOSD group, regarding nosology, clinical, biological and imaging features and the unmet need in this field. The last part is dedicating to the present and future of acute treatment in NMSOD and MOGAD.
在这篇微型综述中,我们将重点关注神经脊髓炎视网膜频谱疾病(NMOSD)和髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)领域的新进展。我们首先介绍了 MOGAD 的拟议标准,并评估了这些标准的影响和潜在局限性,重点关注仅在脑脊液中检测出 MOG 抗体阳性的亚组患者。然后,我们简要介绍了目前对所谓 "双血清阴性 "NMOSD 群体的认识,包括其病理学、临床、生物学和影像学特征,以及该领域尚未满足的需求。最后一部分是关于 NMSOD 和 MOGAD 急性治疗的现状和未来。
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引用次数: 0
History and novelties in autoimmune encephalitis and paraneoplastic neurological syndromes 自身免疫性脑炎和副肿瘤性神经综合征的历史和新进展
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-01 DOI: 10.1016/j.neurol.2024.10.004
J. Honnorat
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引用次数: 0
期刊
Revue neurologique
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