• 文章类型: Journal Article
    背景:伴有可逆性脾病变(MERS)的轻度脑炎/脑病是一种临床放射学综合征,其特征是在磁共振(MR)上出现短暂的中枢神经系统症状和call体(SCC)的可逆性病变。我们报告了一例罕见的成人发作的MERS,并回顾了现有文献。
    方法:我们描述了一例具有罕见症状和体征的成人发作MERS,并对包括四名以上成人发作(>14岁)MERS患者的病例系列进行了系统评价。从2000年1月到2022年12月。我们总结了临床,实验室,成像和治疗数据。
    结果:我们纳入了7项符合条件的研究,共51例成人发作的MERS患者。大多数患者(88%)的神经系统表现先于前驱症状,主要是发烧(78%)。头痛是最常见的症状(50%),其次是癫痫(22%)和意识障碍(22%)。一半的患者出现脑脊液炎症改变,所以定义脑炎病例。诊所康复,除两名在急性期需要呼吸机支持的严重意识障碍患者外,所有患者均可获得。MR显示92%的患者在SCC中出现孤立性病变,而8%的患者也表现出关节外病变,随访影像时,所有病变均得到缓解或改善.
    结论:MERS是脑炎/脑病的一种形式,具有广泛的中枢神经系统表现,通常有轻微的症状,比如头痛,这可能会导致一些案件被忽视,大多数患者预后良好。
    BACKGROUND: Mild encephalitis/encephalopathy with reversible splenial lesion (MERS) is a clinical-radiological syndrome characterized by transient central nervous system symptoms and a reversible lesion in the splenium of the corpus callosum (SCC) on magnetic resonance (MR). We reported a case of adult-onset MERS with uncommon presentation and reviewed the existing literature.
    METHODS: We described a case of adult-onset MERS with uncommon symptoms and signs and performed a systematic review of case series including more than four patients of adult-onset (> 14 years old) MERS, from January 2000 to December 2022. We summarized the clinical, laboratory, imaging and therapy data.
    RESULTS: We included seven eligible studies for a total of 51 adult-onset MERS patients. Neurological manifestations were preceded by prodromal symptoms in most of the patients (88%), mainly with fever (78%). Headache was the most common symptom (50%), followed by seizures (22%) and disturbance of consciousness (22%). Inflammatory changes on cerebrospinal fluid were present in a half of patient, so defining encephalitis cases. Clinal recovery, was achieved in all patients but two with severe disturbance of consciousness who required ventilator support in the acute phase. MR showed isolated lesion in the SCC in 92% of patients, while 8% of patients showed also extracallosal lesions, all the lesions resolved or improved on follow-up imaging.
    CONCLUSIONS: MERS is a form of encephalitis/encephalopathy with a broad range of central nervous system manifestation, often with mild symptoms, such as headache alone, that can lead to overlooked some cases, with an excellent prognosis in most patient.
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  • 文章类型: Case Reports
    最近,已经报道了由抗N-甲基-D-天冬氨酸受体(抗NMDAR)和抗髓鞘少突胶质细胞糖蛋白(MOG)抗体引起的重叠脑炎病例,其临床特征逐渐清晰。急性期治疗通常包括使用类固醇,尽管一些研究表明类固醇是有效的,其功效程度尚未得到充分探讨。
    我们介绍了一例25岁的男性,患有抗NMDAR和抗MOG抗体重叠脑炎,在类固醇治疗后表现出相当大的改善。为了更深入地了解类固醇在治疗这种情况中的功效,我们对急性期接受类固醇治疗的抗NMDAR和抗MOG抗体双阳性脑炎病例进行了文献综述.对13例病例进行了分析,包括我们医院确诊的新病例.所有患者在急性期接受类固醇治疗后均表现出改善。十个病人没有任何后遗症,其中9人在急性期表现出快速或重大反应。相比之下,三名患者经历了后遗症(轻度认知能力下降,视力障碍,和记忆障碍,分别),他们在急性期对类固醇的反应缓慢或有限。5例患者出现复发,一名患者在类固醇逐渐减少的过程中,以及另外两名停止类固醇治疗后的患者。
    类固醇治疗在抗NMDAR和抗MOG抗体重叠脑炎的急性期可有效。在急性期接受类固醇治疗的患者可能会有积极的预后。
    UNASSIGNED: Recently, cases of overlapping encephalitis caused by anti-N-methyl-D-aspartate receptor (anti-NMDAR) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been reported, and their clinical characteristics are gradually becoming clear. Acute-phase treatment typically involves the use of steroids, and although some studies have suggested that steroids can be effective, the extent of their efficacy has not yet been fully explored.
    UNASSIGNED: We present the case of a 25-year-old man with anti-NMDAR and anti-MOG antibody overlapping encephalitis who showed considerable improvement after steroid treatment. To gain a deeper understanding of the efficacy of steroids in managing this condition, we conducted a literature review of cases of anti-NMDAR and anti-MOG antibody double-positive encephalitis that were treated with steroids during the acute phase. Thirteen cases were analyzed, including a new case diagnosed at our hospital. All patients showed improvement after receiving steroid treatment in the acute phase. Ten patients did not have any sequelae, and nine of them showed a rapid or major response during the acute phase. In contrast, three patients experienced sequelae (mild cognitive decline, visual impairment, and memory impairment, respectively), with their response to steroids in the acute phase being slow or limited. Relapses occurred in five patients, in one patient during steroid tapering, and in another two patients after cessation of steroids.
    UNASSIGNED: Steroid therapy can be effective in the acute stage of anti-NMDAR and anti-MOG antibody overlapping encephalitis. A positive prognosis may be expected in patients who experience substantial improvement with steroid therapy during the acute phase.
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  • 文章类型: Journal Article
    脑膜炎是脑膜的感染,覆盖大脑的结缔组织膜,它最常见的影响软脑膜。临床上,脑膜炎可能会出现发烧,颈部僵硬度,精神状态改变,头痛,呕吐,和神经缺陷。脑炎是一种脑部感染,通常表现为发烧,精神状态改变,神经功能缺损,和癫痫。脑膜炎和脑炎是严重的疾病,也可以共存,高发病率和死亡率,因此需要及时诊断和治疗。影像学在这些疾病的临床管理中起着重要作用,尤其是磁共振成像.用于排除模仿并评估并发症的存在。这篇综述的目的是描述最常见的脑膜炎和脑炎的影像学表现。
    Meningitis is the infection of the meninges, which are connective tissue membranes covering the brain, and it most commonly affects the leptomeninges. Clinically, meningitis may present with fever, neck stiffness, altered mental status, headache, vomiting, and neurological deficits. Encephalitis is an infection of the brain, which usually presents with fever, altered mental status, neurological deficits, and seizure. Meningitis and encephalitis are serious conditions which could also coexist, with high morbidity and mortality, thus requiring prompt diagnosis and treatment. Imaging plays an important role in the clinical management of these conditions, especially Magnetic Resonance Imaging. It is indicated to exclude mimics and evaluate the presence of complications. The aim of this review is to depict imaging findings of the most common meningitis and encephalitis.
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  • 文章类型: Journal Article
    由冠状病毒SARS-CoV-2引起的COVID-19大流行揭示了许多问题和医学发现,特别是,关于病毒对中枢神经系统(CNS)和周围神经系统(PNS)的影响。本文是一篇叙述性综述,深入探讨了COVID-19与NS之间的复杂相互作用。因此,本文阐述了由病毒引起的广泛的神经表现和神经退行性疾病。它仔细考虑了SARS-CoV-2到达NS的路线,包括嗅觉系统,当然,血行途径,在讨论病毒的神经发病机制的直接和间接机制时,也包括在内。除了像中风这样的神经病,脑炎,格林-巴利综合征,帕金森病,和多发性硬化症,重点领域也是诊断的挑战,治疗,以及在大流行期间对这些疾病的管理。审查还审查了用于预防这些疾病的战略和干预方法,以及与介导COVID-19引起的神经系统作用有关的ACE2受体。这个详细的概述,将研究结果与案例数据相结合,旨在应对这一流行病挑战,以期将来更好的患者护理和结果。
    The COVID-19 pandemic caused by the coronavirus SARS-CoV-2 revealed a huge number of problems as well as discoveries in medicine, notably, regarding the effects of the virus on the central nervous system (CNS) and peripheral nervous system (PNS). This paper is a narrative review that takes a deep dive into the complex interactions between COVID-19 and the NS. Therefore, this paper explains the broad range of neurological manifestations and neurodegenerative diseases caused by the virus. It carefully considers the routes through which SARS-CoV-2 reaches the NS, including the olfactory system and of course, the hematogenous route, which are also covered when discussing the virus\'s direct and indirect mechanisms of neuropathogenesis. Besides neurological pathologies such as stroke, encephalitis, Guillain-Barré syndrome, Parkinson\'s disease, and multiple sclerosis, the focus area is also given to the challenges of making diagnosis, treatment, and management of these conditions during the pandemic. The review also examines the strategic and interventional approaches utilized to prevent these disorders, as well as the ACE2 receptors implicated in the mediation of neurological effects caused by COVID-19. This detailed overview, which combines research outputs with case data, is directed at tackling this pandemic challenge, with a view toward better patient care and outcomes in the future.
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  • 文章类型: Case Reports
    非结核分枝杆菌(NTM)是颅内感染的极为罕见的病原体。其中,罗得西亚分枝杆菌是一种更不常见的病原体。在本文中,我们报告了第一例由罗得西亚分枝杆菌引起的人类中枢神经系统(CNS)感染病例,使用利奈唑胺具有特定的影像学表现和对治疗的良好反应,克拉霉素,还有米诺环素.通过全面的多模式方法促进了诊断,结合多位点成像,通过下一代测序(NGS)进行脑脊液分析,和有针对性的基因检测。此外,本文提供了在其他已记录的可归因于NTM的CNS感染病例中观察到的临床特征的推导,并基于对当前文献的回顾.我们的经验为了解与NTM相关的中枢神经系统病理的全谱提供了证据,并强调了多学科诊断过程在颅内感染的非典型表现中的重要性。
    Nontuberculous mycobacteria (NTM) are exceedingly rare etiological agents of intracranial infections. Among them, Mycobacterium rhodesiae stands out as an even less common pathogen. In this paper, we report the first documented case of a central nervous system (CNS) infection in humans caused by Mycobacterium rhodesiae, which has specific imaging findings and good response to the therapy by using Linezolid, Clarithromycin, and Minocycline. The diagnosis was facilitated by a comprehensive multimodal approach, incorporating multisite imaging, cerebrospinal fluid analysis via next-generation sequencing (NGS), and targeted genetic testing. Furthermore, this paper provides a derivation of the clinical characteristics observed in other documented instances of CNS infections attributable to NTM and based on a review of the current literature. Our experience contributes to the evidence that is needed to understand the full spectrum of NTM-related CNS pathologies and underscores the importance of a multidisciplinary diagnostic process in atypical presentations of intracranial infections.
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  • 文章类型: Case Reports
    我们描述了一个年轻女孩的抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的挑战性案例。尽管持续数月的意识下降与持续抗体的存在,她最终在5年的随访期内表现出显著的改善.此外,我们对抗NMDAR脑炎的相关文献进行了简要回顾,特别关注抗NMDAR抗体。我们的发现增强了对抗NMDAR脑炎的临床理解,并为临床医生提供了有价值的见解。
    We described a challenging case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in a young girl. Despite enduring months of reduced consciousness with ongoing antibody presence, she ultimately exhibited remarkable improvement within a 5-year follow-up period. Additionally, we conducted a concise review of relevant literature on anti-NMDAR encephalitis, with a specific focus on anti-NMDAR antibodies. Our findings enhance the clinical comprehension of anti-NMDAR encephalitis and offer valuable insights to clinicians for its management.
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  • 文章类型: Journal Article
    N-甲基-d-天冬氨酸受体脑炎(NMDARE)的神经精神症状已导致一些人寻求电惊厥治疗(ECT)的经验性试验。范围审查确定了39例诊断为NMDARE的患者正在接受ECT。分别,对21例患者进行回顾性队列研究.在范围审查中,49%的患者和19%的回顾性队列中,临床改善归因于ECT;免疫疗法的时机是一个混杂因素。据报道,范围审查中28%的患者和回顾性审查中38%的患者在ECT后的临床病程恶化。目前没有足够的数据支持ECT在NMDARE中的有益效果。
    Neuropsychiatric symptoms in N-methyl-d-aspartate receptor encephalitis (NMDARE) have led some to pursue empiric trials of electroconvulsive therapy (ECT). A scoping review identified 39 patients diagnosed with NMDARE undergoing ECT. Separately, a retrospective cohort was reviewed to characterize 21 patients. Clinical improvement was attributed to ECT in 49% of patients in the scoping review and 19% of patients in the retrospective cohort; timing of immunotherapies was a confounding factor. Worsening of clinical course following ECT was reported in 28% of patients in the scoping review and 38% of patient in the retrospective review. There is currently insufficient data supporting a beneficial effect of ECT in NMDARE.
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  • 文章类型: Journal Article
    背景:副肿瘤性脑炎(PE)是睾丸癌(TC)患者的一种罕见但重要的并发症。鉴于缺乏关于这一主题的全面文献,我们的审查旨在巩固现有知识,并为诊断提供循证建议,预后,以及在TC背景下的PE管理。
    方法:遵守PRISMA指南,从1950年到2024年4月,使用PubMed进行了系统的文献综述。搜索的重点是将TC确定为PE的主要病因的文章。采用混合方法评估工具和牛津循证医学中心的证据工具来评估研究质量,并进行了专题分析,以确定趋势和模式。
    结果:在确定的91篇文章中,29符合纳入标准,包括5个回顾性图表评论,3个案例系列,22例报告。研究结果表明,在肿瘤检测之前,PE症状可以在TC的任何阶段出现,治疗期间,甚至治疗后几年。一个值得注意的观察是在超声成像中频繁忽视微观睾丸肿瘤,导致诊断延迟。在TC的背景下,PE的结果是多种多样的,反映了所包括研究的异质性。
    结论:PE,虽然罕见,对于出现神经精神症状的TC患者,这是一个关键的考虑因素。早期识别和适当的诊断检查,包括考虑显微肿瘤,对于及时干预和改善患者预后至关重要。
    BACKGROUND: Paraneoplastic encephalitis (PE) represents a rare but significant complication in patients with testicular cancer (TC). Given the paucity of comprehensive literature on this topic, our review seeks to consolidate current knowledge and provide evidence-based recommendations for the diagnosis, prognosis, and management of PE in the context of TC.
    METHODS: In adherence to PRISMA guidelines, a systematic literature review was conducted from 1950 to April 2024 using PubMed. The search focused on articles where TC was identified as the primary etiology of PE. The Mixed Methods Appraisal Tool and the Oxford Centre for Evidence-Based Medicine\'s levels of evidence tool were employed for assessing study quality, and a thematic analysis was conducted to identify trends and patterns.
    RESULTS: Out of 91 articles identified, 29 met the inclusion criteria, encompassing 5 retrospective chart reviews, 3 case series, and 22 case reports. Findings indicate that PE symptoms can manifest at any stage of TC-before tumor detection, during treatment, or even years posttreatment. A notable observation was the frequent oversight of microscopic testicular tumors in ultrasound imaging, leading to diagnostic delays. The outcomes of PE in the context of TC were diverse, reflecting the heterogeneity of the studies included.
    CONCLUSIONS: PE, although rare, is a critical consideration in patients with TC presenting with neuropsychiatric symptoms. Early recognition and appropriate diagnostic workup, including consideration for microscopic neoplasms, are essential for timely intervention and improved patient outcomes.
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  • 文章类型: Journal Article
    自身免疫性脑炎(AIE)是一种快速、以神经系统炎症为特征的进行性神经系统疾病。虽然Graus标准是最著名的AIE诊断标准,治疗前必须考虑其他符合Graus标准的鉴别诊断.这篇叙述性综述讨论了类似AIE的最常见病因。我们建议在确认AIE诊断之前,常规排除符合Graus标准的模仿者。我们回顾了28项研究,包括356例患者。最初的主要诊断是AIE,然后是副肿瘤性边缘叶脑炎和抗N-甲基-D-天冬氨酸受体脑炎。只有194名患者符合可能的Graus标准。总人口中最常见的疾病是痴呆症,其他神经退行性疾病,精神和功能性神经系统疾病。AIE常被误诊,导致不必要的治疗。尽管公布了Graus标准,模仿这种情况的医疗案例正在出版。通过详细的病史可以排除许多进入AIE鉴别诊断的神经系统疾病。神经系统检查,实验室分析,和其他调查,包括脑脊液和脑磁共振成像。然而,一些鉴别诊断符合可能的Graus标准,一些人同时有抗神经神经元抗体,被认为是真正的模仿者。AIE诊断怀疑主要是临床,但明确的诊断需要各种诊断工具。
    Autoimmune encephalitis (AIE) is a rapid, progressive neurological disorder characterized by nervous system inflammation. While the Graus criteria are the best known criteria for AIE diagnosis, other differential diagnoses meeting the Graus criteria must be considered before management. This narrative review discusses the most common etiologies that resemble AIE. We suggest routine exclusion of mimickers meeting the Graus criteria before confirming an AIE diagnosis. We reviewed 28 studies including 356 patients. The main initial diagnosis was AIE, then paraneoplastic limbic encephalitis and anti-N-methyl-D-aspartate receptor encephalitis. Only 194 patients met the possible Graus criteria. The most frequent conditions among the total population were dementia, other neurodegenerative diseases, and psychiatric and functional neurological disorders. AIE is often misdiagnosed, leading to unnecessary treatment. Despite publication of the Graus criteria, medical cases mimicking this condition are being published. Many neurological diseases entering the differential diagnosis of AIE could be excluded through a detailed history, neurological examination, laboratory analysis, and other investigations, including cerebrospinal fluid and brain magnetic resonance imaging. However, some differential diagnoses complied with the possible Graus criteria, with some having concurrent antineuronal antibodies, which were considered true mimickers. AIE diagnosis suspicion is primarily clinical, but a definitive diagnosis requires various diagnostic tools.
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  • 文章类型: Case Reports
    背景:严重发热伴血小板减少综合征(SFTS)是一种主要通过蜱叮咬传播的自然局灶性疾病,病原体是SFTS病毒(SFTSV)。SFTS可以迅速发展为严重疾病,多器官衰竭(MOF)表现,如休克,呼吸衰竭,弥散性血管内凝血(DIC)和死亡,但是很少报道SFTS患者出现中枢神经系统(CNS)症状,并且口周区域和四肢持续不自主的抖动。
    方法:一名69岁女性发热,口周区域和四肢持续不自主抖动,经脑脊液(CSF)和外周血鉴定为SFTSV的宏基因组下一代测序(mNGS)后,被诊断为SFTS,出现CNS症状。患者在疾病过程中出现了细胞因子风暴和MOF,在积极的抗病毒治疗之后,糖皮质激素,和丙种球蛋白治疗,她的临床症状有所改善,她的实验室指标恢复正常,她的预后很好.
    结论:这个案例给了我们深刻的认识,当中枢神经系统症状类似于病毒性脑炎合并血小板减少和白细胞减少的患者在临床中遇到时,有必要考虑SFTS涉及CNS的可能性。应进行CSF和血液中SFTSV核酸的检测(mNGS或聚合酶链反应(PCR)),尤其是危重病人,应给予相应的治疗。
    BACKGROUND: Severe fever with thrombocytopenia syndrome (SFTS) is a natural focal disease transmitted mainly by tick bites, and the causative agent is SFTS virus (SFTSV). SFTS can rapidly progress to severe disease, with multiple-organ failure (MOF) manifestations such as shock, respiratory failure, disseminated intravascular coagulation (DIC) and death, but cases of SFTS patients with central nervous system (CNS) symptoms onset and marked persistent involuntary shaking of the perioral area and limbs have rarely been reported.
    METHODS: A 69-year-old woman with fever and persistent involuntary shaking of the perioral area and limbs was diagnosed with SFTS with CNS symptom onset after metagenomic next-generation sequencing (mNGS) of cerebrospinal fluid (CSF) and peripheral blood identified SFTSV. The patient developed a cytokine storm and MOF during the course of the disease, and after aggressive antiviral, glucocorticoid, and gamma globulin treatments, her clinical symptoms improved, her laboratory indices returned to normal, and she had a good prognosis.
    CONCLUSIONS: This case gives us great insight that when patients with CNS symptoms similar to those of viral encephalitis combined with thrombocytopenia and leukopenia are encountered in the clinic, it is necessary to consider the possibility of SFTS involving the CNS. Testing for SFTSV nucleic acid in CSF and blood (mNGS or polymerase chain reaction (PCR)) should be carried out, especially in critically ill patients, and treatment should be given accordingly.
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