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Clinical features and treatment of apoplectic intratumoral hemorrhage of glioma. 胶质瘤瘤内出血的临床特征和治疗方法。
IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-24 DOI: 10.1186/s12883-024-03753-6
Jia-Hua Zhou, Chao Wang, Di Yang, Ying-Xi Wu, Da-Yun Feng, Huaizhou Qin, Ju-Lei Wang, Ming-Hao Wei

Objective: The primary objective of this study was to explore the clinical characteristics of apoplectic intratumoral hemorrhage in gliomas and offer insights for improving the diagnosis and treatment of this disease.

Methods: We analyzed the clinical data of 35 patients with glioma and hemorrhage. There were eight cases of multiple cerebral lobe involvement, and 22 cases involved a single lobe. Twenty-one patients had a preoperative Glasgow Coma Scale (GCS) score of ≥ 9 and had a craniotomy with tumor resection and hematoma evacuation after undergoing preoperative preparation. A total of 14 patients with GCS < 9, including one with thalamic hemorrhage breaking into the ventricles and acute obstructive hydrocephalus, underwent craniotomy for tumor resection after external ventricular drainage (EVD). One patient had combined thrombocytopenia, which was surgically treated after platelet levels were normalized through transfusion. The remaining 12 patients received immediate intervention in the form of craniotomy hematoma evacuation and tumor resection.

Results: We performed subtotal resection on three tumors of thalamic origin and two tumors of corpus callosum origin, but we were able to successfully resect all the tumors in other locations that were gross total resection Pathology results showed that 71.43% of cases accounted for WHO-grade 4 tumors. Among the 21 patients with a GCS score of ≥ 9, two died perioperatively. Fourteen patients had a GCS score < 9, of which eight patients died perioperatively.

Conclusions: Patients with a preoperative GCS score ≥ 9 who underwent subemergency surgery and received aggressive treatment showed a reasonable prognosis. We found their long-term outcomes to be correlated with the pathology findings. On the other hand, patients with a preoperative GCS score < 9 required emergency treatment and had a high perioperative mortality rate.

研究目的本研究的主要目的是探讨胶质瘤瘤内出血的临床特征,为改善该疾病的诊断和治疗提供见解:我们分析了35例胶质瘤合并出血患者的临床资料。方法:我们分析了 35 例胶质瘤合并出血患者的临床资料,其中 8 例为多脑叶受累,22 例为单脑叶受累。21例患者术前格拉斯哥昏迷量表(GCS)评分≥9分,在接受术前准备后进行了开颅手术,切除肿瘤并清除血肿。共有 14 名患者的格拉斯哥昏迷量表(GCS)评分为 9 分:我们对 3 例丘脑起源的肿瘤和 2 例胼胝体起源的肿瘤进行了次全切除,但我们成功切除了其他位置的所有肿瘤,并进行了全切。 病理结果显示,71.43% 的病例为 WHO 4 级肿瘤。在 21 例 GCS 评分≥9 分的患者中,有两人在围手术期死亡。14名患者的GCS评分为结论:术前 GCS 评分≥9 分、接受亚急诊手术和积极治疗的患者预后良好。我们发现他们的长期预后与病理结果相关。另一方面,术前 GCS 评分
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引用次数: 0
Transition to end-of-life care in patients with neurological diseases in an acute hospital ward 急症病房神经系统疾病患者向临终关怀的过渡
IF 2.6 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-22 DOI: 10.1186/s12883-024-03768-z
Gudrun Jonsdottir, Erna Haraldsdottir, Runar Vilhjalmsson, Valgerdur Sigurdardottir, Haukur Hjaltason, Marianne Elisabeth Klinke, Gudny Bergthora Tryggvadottir, Helga Jonsdottir
Transitioning to end-of-life care and thereby changing the focus of treatment directives from life-sustaining treatment to comfort care is important for neurological patients in advanced stages. Late transition to end-of-life care for neurological patients has been described previously. To investigate whether previous treatment directives, primary medical diagnoses, and demographic factors predict the transition to end-of-life care and time to eventual death in patients with neurological diseases in an acute hospital setting. All consecutive health records of patients diagnosed with stroke, amyotrophic lateral sclerosis (ALS), and Parkinson’s disease or other extrapyramidal diseases (PDoed), who died in an acute neurological ward between January 2011 and August 2020 were retrieved retrospectively. Descriptive statistics and multivariate Cox regression were used to examine the timing of treatment directives and death in relation to medical diagnosis, age, gender, and marital status. A total of 271 records were involved in the analysis. Patients in all diagnostic categories had a treatment directive for end-of-life care, with patients with haemorrhagic stroke having the highest (92%) and patients with PDoed the lowest (73%) proportion. Cox regression identified that the likelihood of end-of-life care decision-making was related to advancing age (HR = 1.02, 95% CI: 1.007–1.039, P = 0.005), ischaemic stroke (HR = 1.64, 95% CI: 1.034–2.618, P = 0.036) and haemorrhagic stroke (HR = 2.04, 95% CI: 1.219–3.423, P = 0.007) diagnoses. End-of-life care decision occurred from four to twenty-two days after hospital admission. The time from end-of-life care decision to death was a median of two days. Treatment directives, demographic factors, and diagnostic categories did not increase the likelihood of death following an end-of-life care decision. Results show not only that neurological patients transit late to end-of-life care but that the timeframe of the decision differs between patients with acute neurological diseases and those with progressive neurological diseases, highlighting the particular significance of the short timeframe of patients with the progressive neurological diseases ALS and PDoed. Different trajectories of patients with neurological diseases at end-of-life should be further explored and clinical guidelines expanded to embrace the high diversity in neurological patients.
过渡到临终关怀,从而将治疗指令的重点从维持生命的治疗转变为舒适护理,这对于晚期神经系统患者来说非常重要。神经系统患者晚期向临终关怀过渡的情况此前已有描述。目的:研究在急诊医院环境中,先前的治疗指令、主要医疗诊断和人口统计学因素是否能预测神经系统疾病患者向临终关怀的过渡和最终死亡的时间。研究人员回顾性检索了2011年1月至2020年8月期间在神经科急症病房死亡的脑卒中、肌萎缩性脊髓侧索硬化症(ALS)、帕金森病或其他锥体外系疾病(PDoed)患者的所有连续病历。研究人员使用描述性统计和多变量 Cox 回归分析了治疗指令和死亡时间与医疗诊断、年龄、性别和婚姻状况的关系。共有 271 份记录参与了分析。所有诊断类别的患者都有临终关怀治疗指令,其中出血性中风患者比例最高(92%),而卒中患者比例最低(73%)。Cox 回归发现,临终关怀决策的可能性与年龄增长(HR = 1.02,95% CI:1.007-1.039,P = 0.005)、缺血性卒中(HR = 1.64,95% CI:1.034-2.618,P = 0.036)和出血性卒中(HR = 2.04,95% CI:1.219-3.423,P = 0.007)诊断有关。临终关怀的决定时间为入院后 4 到 22 天。从做出临终关怀决定到死亡的时间中位数为两天。治疗指令、人口学因素和诊断类别并不会增加临终关怀决定后死亡的可能性。研究结果表明,不仅神经系统疾病患者的临终关怀转归较晚,而且急性神经系统疾病患者和进展期神经系统疾病患者做出临终关怀决定的时间框架也不尽相同。应进一步探索神经系统疾病患者临终前的不同轨迹,并扩大临床指南的范围,以涵盖神经系统疾病患者的高度多样性。
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引用次数: 0
Knowledge, attitudes, and practices of caregivers with children diagnosed with epilepsy attending a pediatric outpatient clinic: a descriptive, cross-sectional, questionnaire-based study in Addis Ababa, Ethiopia 埃塞俄比亚亚的斯亚贝巴儿科门诊中被诊断患有癫痫的儿童的看护者的知识、态度和做法:基于问卷的横断面描述性研究
IF 2.6 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-22 DOI: 10.1186/s12883-024-03766-1
Absalat Serawit Negussie, Mansour Fayz Dehan, Samuel Ayalew Mekonnen, Tesfaye Getaneh Zelleke
Caregivers’ knowledge and attitudes influence help-seeking behavior and treatment decisions of patients with epilepsy, which in turn significantly impacts epilepsy care. In Ethiopia, epilepsy is often misunderstood, associated with misconceptions and accompanied by persistent negative attitudes. The objective of this study is to assess the knowledge, attitude, and practice of caregivers of children with epilepsy. We conducted a hospital-based survey at the Yekatit 12 Hospital Pediatric Neurology Clinic, Addis Ababa, Ethiopia, between May and July 2022. We invited caregivers of children with epilepsy taking one or more daily anti-seizure medications to participate. Caregivers were invited to complete a structured questionnaire with guidance from a trained nurse to estimate knowledge and attitudes towards epilepsy and its treatment. Knowledge and attitudes were categorized as “good” and “favorable” (correct answers to ≥ 50% of questions) or “bad” and “unfavorable” (< 50% correct answers), respectively. Attitudes towards standard care versus non-standard (e.g., spiritual) care were also estimated. A total of 120 caregivers completed the questionnaire. Many caregivers were familiar with the term ‘epilepsy’, with more than half (51.7%) having heard or read about it previously. The reported causes of epilepsy varied, with birth injury being the most common cause (44 out of 120 caregivers). Notably, there was association between the caregiver’s gender and their knowledge score, with a p-value = 0.05. Caregivers exposed to information about epilepsy through hearing or reading demonstrated significantly higher levels of knowledge, with a p-value < 0.001. Additionally, knowing someone with epilepsy other than the index child was significantly associated with higher knowledge scores (p-value < 0.001). The study also revealed negative attitudes toward epilepsy: for example, 56.7% of surveyed caregivers believed it is unlikely that a child with epilepsy has normal cognitive abilities and 39.1% believed they should never be allowed to attend regular school. Additionally, a high proportion of caregivers (70%) sought alternative treatments (e.g., spiritual help) alongside standard medical care. A significant knowledge gap was identified among caregivers, revealing prevalent misconceptions and negative attitudes. Improving epilepsy awareness, attitudes, and practices among caregivers will potentially contribute to overall improved quality of life for children with epilepsy.
护理人员的知识和态度会影响癫痫患者的求助行为和治疗决定,进而对癫痫护理产生重大影响。在埃塞俄比亚,癫痫常常被误解,与错误观念相关联,并伴有持续的负面态度。本研究旨在评估癫痫患儿护理人员的知识、态度和做法。2022 年 5 月至 7 月期间,我们在埃塞俄比亚亚的斯亚贝巴的 Yekatit 12 医院儿科神经病学诊所开展了一项医院调查。我们邀请每天服用一种或多种抗癫痫药物的癫痫患儿的护理人员参与调查。我们邀请护理人员在受过培训的护士的指导下填写一份结构化问卷,以了解他们对癫痫及其治疗的知识和态度。知识和态度分别被分为 "好 "和 "赞成"(回答正确率≥50%)或 "坏 "和 "不赞成"(回答正确率<50%)。此外,还对标准护理与非标准护理(如精神护理)的态度进行了估计。共有 120 名护理人员完成了问卷调查。许多护理人员对 "癫痫 "一词并不陌生,半数以上(51.7%)的护理人员以前听说过或读到过这个词。所报告的癫痫病因各不相同,其中产伤是最常见的病因(120 名护理人员中有 44 人)。值得注意的是,护理人员的性别与他们的知识得分之间存在关联,P 值 = 0.05。护理人员通过听力或阅读接触到的癫痫相关信息的知识水平明显更高,p 值小于 0.001。此外,除了患儿外,认识其他癫痫患者也与较高的知识水平有显著关系(p 值 < 0.001)。研究还揭示了对癫痫的消极态度:例如,56.7% 的受访照顾者认为癫痫患儿不可能有正常的认知能力,39.1% 的受访照顾者认为永远都不应该让他们上正规学校。此外,很大比例的照护者(70%)在寻求标准医疗护理的同时,还寻求替代疗法(如精神帮助)。在护理人员中发现了巨大的知识差距,揭示了普遍存在的误解和消极态度。改善护理人员对癫痫的认识、态度和做法将有助于全面提高癫痫患儿的生活质量。
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引用次数: 0
CHANTER syndrome in the context of pain medication: a case report 使用止痛药的 CHANTER 综合征:病例报告
IF 2.6 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-22 DOI: 10.1186/s12883-024-03748-3
Nils Jansen, Leonard Wulff, Moritz Conty, Wolf-Rüdiger Schäbitz, Randolf Klingebiel
CHANTER (Cerebellar Hippocampal and Basal Nuclei Transient Edema with Restricted diffusion) is a recently described syndrome occurring in the context of drug abuse. While clinical findings are rather unspecific (disorientation, unresponsiveness), MR imaging (MRI) discloses a characteristic pattern (restricted diffusion in the basal ganglia and hippocampi, cerebellar oedema and haemorrhage), allowing for timely diagnosis before complications such as cerebellar swelling and herniation do occur. Here we report a case of CHANTER primarily based on imaging findings, as there was no evidence of drug abuse on admission. A 62-year-old Patient was admitted to our hospital after being unresponsive at home. Prehospital intubation was performed, which limited neurological assessment. Under these circumstances no obvious symptoms could be determined, i.e. pupils were isocoric and responsive, and there were no signs of seizures. While the initial CT scan was unremarkable, the subsequent MRI scan showed a distinct imaging pattern: moderately enhancing areas in the basal ganglia and hippocampi with diffusion restriction, accompanied by cerebellar haemorrhage and oedema (Figs. 1 and 2). A comprehensive clinical and laboratory work-up was performed, including drug screening, spinal tap, Holter ECG, echocardiography and EEG. The only conspicuous anamnestic finding was a chronic pain syndrome whose medication had been supplemented with opioids two months previously. The opioid medication was discontinued, which led to a rapid improvement in the patient’s clinical condition without any further measures. The patient was able to leave the intensive care unit and was discharged 10 days after admission without persistent neurological deficits. Familiarity with typical MRI patterns of toxic encephalopathy in patients from high-risk groups, such as drug abusers, is crucial in emergency neuroradiology. In the presence of typical MRI findings, CHANTER syndrome should be included in the differential diagnosis, even if there is no history of drug abuse, to avoid delay in diagnosis and treatment.
CHANTER(小脑海马和基底核短暂水肿伴弥散受限)是最近描述的一种在药物滥用背景下发生的综合征。虽然临床表现没有特异性(定向障碍、反应迟钝),但核磁共振成像(MRI)显示出特征性模式(基底节和海马的弥散受限、小脑水肿和出血),可在小脑肿胀和疝出等并发症发生前及时诊断。由于入院时没有证据表明患者滥用药物,因此我们在此报告一例主要基于影像学检查结果的 CHANTER 病例。一名 62 岁的患者在家中无反应后被送入我院。入院前进行了插管,这限制了神经系统的评估。在这种情况下,无法确定明显的症状,即瞳孔等大且反应灵敏,也没有癫痫发作的迹象。虽然最初的CT扫描没有发现异常,但随后的核磁共振扫描却显示出明显的成像模式:基底节和海马区中度增强,弥散受限,伴有小脑出血和水肿(图1和图2)。对患者进行了全面的临床和实验室检查,包括药物筛查、脊髓穿刺、Holter心电图、超声心动图和脑电图。唯一明显的异常发现是慢性疼痛综合征,两个月前曾使用阿片类药物辅助治疗。停用阿片类药物后,患者的临床状况迅速好转,无需采取任何进一步措施。患者得以离开重症监护室,并在入院 10 天后出院,没有出现持续的神经功能缺损。熟悉吸毒等高危人群患者中毒性脑病的典型磁共振成像模式对急诊神经放射学至关重要。如果出现典型的 MRI 检查结果,即使没有吸毒史,也应将 CHANTER 综合征列入鉴别诊断,以免延误诊断和治疗。
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引用次数: 0
Experiences and preferences of people with stroke and caregivers, around supports provided at the transition from hospital to home: a qualitative descriptive study 脑卒中患者和照护者在从医院到家庭的过渡时期对所提供支持的体验和偏好:定性描述研究
IF 2.6 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-22 DOI: 10.1186/s12883-024-03767-0
Geraldine O’Callaghan, Martin Fahy, Sigrid O’Meara, Sebastian Lindblom, Lena von Koch, Peter Langhorne, Rose Galvin, Frances Horgan
Transitioning home from the structured hospital setting poses challenges for people with stroke (PWS) and their caregivers (CGs), as they navigate through complex uncertainties. There are gaps in our understanding of appropriate support interventions for managing the transition home. In this qualitative study, we explored the perspectives of PWS and their CGs regarding their support experiences and preferences during this period. Between November 2022 and March 2023, and within six months of hospital discharge, audio-recorded, semi-structured interviews were conducted with PWS and CGs. All interviews were transcribed, imported into NVivo software, and analysed using reflexive thematic analysis. Sixteen interviews were conducted, nine with PWS and seven with CGs. Four themes relevant to their collective experiences and preferences were identified: (i) Need for tailored information-sharing, at the right time, and in the right setting; (ii) The importance of emotional support; (iii) Left in limbo, (iv) Inequity of access. Experiences depict issues such as insufficient information-sharing, communication gaps, and fragmented and inequitable care; while a multi-faceted approach is desired to ease anxiety and uncertainty, minimise delays, and optimise recovery and participation during transition. Our findings highlight that regardless of the discharge route, and even with formal support systems in place, PWS and families encounter challenges during the transition period. The experiences of support at this transition and the preferences of PWS and CGs during this important period highlights the need for better care co-ordination, early and ongoing emotional support, and equitable access to tailored services and support. Experiences are likely to be improved by implementing a partnership approach with improved collaboration, including joint goal-setting, between PWS, CGs, healthcare professionals and support organisations.
从结构化的医院环境向家庭过渡对中风患者(PWS)及其照护者(CGs)来说是一项挑战,因为他们要在复杂的不确定因素中摸索前进。我们对管理家庭过渡的适当支持干预措施的理解还存在差距。在这项定性研究中,我们探讨了 PWS 及其照顾者在此期间的支持体验和偏好。2022 年 11 月至 2023 年 3 月期间,在出院后的六个月内,我们对 PWS 和 CG 进行了半结构式录音访谈。所有访谈均已誊写,并导入 NVivo 软件,使用反思性主题分析法进行分析。共进行了 16 次访谈,其中 9 次是与 PWS 进行的,7 次是与 CGs 进行的。确定了与他们的集体经历和偏好相关的四个主题:(i) 需要在适当的时间和适当的环境中分享有针对性的信息;(ii) 情感支持的重要性;(iii) 处于无助状态;(iv) 获取信息的不平等。这些经历反映了信息共享不足、沟通缺失、护理零散且不公平等问题;同时,我们希望采取多方面的方法来缓解焦虑和不确定性,尽量减少延误,并优化过渡期间的康复和参与。我们的研究结果强调,无论出院途径如何,即使有正式的支持系统,PWS 及其家人在过渡时期都会遇到挑战。在这一重要时期,PWS 和 CG 在过渡时期获得支持的经历以及他们的偏好突出表明,需要更好的护理协调、早期和持续的情感支持,以及公平获得量身定制的服务和支持。通过实施伙伴关系方法,改善 PWS、CG、医疗保健专业人员和支持组织之间的合作,包括共同制定目标,可能会改善这些经历。
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引用次数: 0
Primary diffuse large B-cell lymphoma of the central nervous system identified with CSF biomarkers 利用脑脊液生物标记物鉴定中枢神经系统原发性弥漫大B细胞淋巴瘤
IF 2.6 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-22 DOI: 10.1186/s12883-024-03761-6
Valentin Loser, Amandine Segot, Laurence de Leval, Bettina Bisig, Jean-Philippe Brouland, Ekkehard Hewer, Carmen Barcena, Andreas F. Hottinger, Caroline Pot
Diagnosis of primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is challenging and often delayed. MRI imaging, CSF cytology and flow cytometry have a low sensitivity and even brain biopsies can be misleading. We report three cases of PCNSL with various clinical presentation and radiological findings where the diagnosis was suggested by novel CSF biomarkers and subsequently confirmed by brain biopsy or autopsy. Case presentations. The first case is a 79-year-old man with severe neurocognitive dysfunction and static ataxia evolving over 5 months. Brain MRI revealed a nodular ventriculitis. An open brain biopsy was inconclusive. The second case is a 60-year-old woman with progressive sensory symptoms in all four limbs, evolving over 1 year. Brain and spinal MRI revealed asymmetric T2 hyperintensities of the corpus callosum, corona radiata and corticospinal tracts. The third case is a 72-year-old man recently diagnosed with primary vitreoretinal lymphoma of the right eye. A follow-up brain MRI performed 4 months after symptom onset revealed a T2 hyperintense fronto-sagittal lesion, with gadolinium uptake and perilesional edema. In all three cases, CSF flow cytometry and cytology were negative. Mutation analysis on the CSF (either by digital PCR or by next generation sequencing) identified the MYD88 L265P hotspot mutation in all three cases. A B-cell clonality study, performed in case 1 and 2, identified a monoclonal rearrangement of the immunoglobulin light chain lambda (IGL) and kappa (IGK) gene. CSF CXCL-13 and IL-10 levels were high in all three cases, and IL-10/IL-6 ratio was high in two. Diagnosis of PCNSL was later confirmed by autopsy in case 1, and by brain biopsy in case 2 and 3. Taken together, 5 CSF biomarkers (IL-10, IL-10/IL-6 ratio, CXCL13, MYD88 mutation and monoclonal IG gene rearrangements) were strongly indicative of a PCNSL. Using innovative CSF biomarkers can be sensitive and complementary to traditional CSF analysis and brain biopsy in the diagnosis of PCNSL, potentially allowing for earlier diagnosis and treatment.
中枢神经系统原发性弥漫大 B 细胞淋巴瘤(PCNSL)的诊断具有挑战性,而且往往会被延误。核磁共振成像、脑脊液细胞学和流式细胞术的灵敏度较低,甚至脑活检也会产生误导。我们报告了三例具有不同临床表现和放射学发现的 PCNSL 病例,这些病例的诊断是由新型 CSF 生物标记物提示的,随后通过脑活检或尸检得到确诊。病例介绍。第一个病例是一名79岁的男性,患有严重的神经认知功能障碍和静态共济失调,病程超过5个月。脑磁共振成像显示他患有结节性脑室炎。开放性脑活检未得出结论。第二个病例是一名 60 岁的妇女,四肢出现进行性感觉症状,病程超过 1 年。脑和脊髓 MRI 显示胼胝体、放射冠和皮质脊髓束出现不对称 T2 高密度。第三个病例是一名 72 岁的男性,最近被诊断为右眼原发性玻璃体视网膜淋巴瘤。症状出现 4 个月后进行的脑部磁共振成像随访显示,该病灶为 T2 超强前矢状病变,伴有钆摄取和周围水肿。所有三个病例的脑脊液流式细胞术和细胞学检查均为阴性。脑脊液突变分析(通过数字 PCR 或新一代测序)在所有三个病例中都发现了 MYD88 L265P 热点突变。在病例1和2中进行的B细胞克隆性研究发现,免疫球蛋白轻链λ(IGL)和卡帕(IGK)基因发生了单克隆重排。三个病例的 CSF CXCL-13 和 IL-10 水平都很高,其中两个病例的 IL-10/IL-6 比值也很高。后来,病例1通过尸检确诊为PCNSL,病例2和病例3通过脑活检确诊为PCNSL。综合来看,5种CSF生物标记物(IL-10、IL-10/IL-6比值、CXCL13、MYD88突变和单克隆IG基因重排)对PCNSL具有很强的提示作用。在PCNSL的诊断中,使用创新的CSF生物标记物可以提高灵敏度,并与传统的CSF分析和脑活检形成互补,从而有可能实现早期诊断和治疗。
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引用次数: 0
Novel and characteristic radiological features of neurosyphilis: a case series. 神经梅毒的放射学新特征:一个病例系列。
IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-20 DOI: 10.1186/s12883-024-03762-5
Kenji Ohira, Nanako Hashimoto, Daisuke Kanai, Yukio Inoue

Background: Treponema pallidum can invade the central nervous system (CNS) early in its infection, causing neurosyphilis. Neurosyphilis typically presents with meningovasculitis in the acute or subacute phase, while tabes dorsalis and dementia paralytica are classical conditions in the later stages. However, syphilis is often misdiagnosed as other conditions such as tumors or autoimmune diseases including vasculitis and encephalitis, which is why the condition is known as "The Great Mimicker." The increasing incidence of syphilis in recent years emphasizes the importance of early diagnosis and treatment; however, its multiple clinical manifestations impose diagnostic challenges for clinicians because it resembles other diseases. In this case series, we present the impressive manifestations of neurosyphilis through three unique radiological presentations.

Case presentation: Case 1 details optic nerve involvement in an HIV-positive male, where MRI and fundoscopic findings confirmed syphilitic optic neuritis. Case 2 describes a patient in her pregnancy initially suspected of acoustic neuroma on MRI, later diagnosed with syphilitic gumma affecting the inner ear canal. Case 3 is a young male with clinical features mimicking temporal arteritis, ultimately identified as skull osteomyelitis secondarily causing inflammation of the musculus temporalis and meningitis.

Conclusions: These cases underscore the necessity of considering syphilis in differential diagnoses, given the diversity of its clinical presentations. Radiology plays an important role in avoiding unnecessary interventions. The increasing prevalence of recurrent syphilis imposes diagnostic challenges, emphasizing the importance of the early diagnosis and treatment of neurosyphilis by clinicians.

背景:苍白螺旋体可在感染早期侵入中枢神经系统(CNS),导致神经梅毒。神经梅毒在急性或亚急性期通常表现为脑膜血管炎,而在晚期则表现为背痛和麻痹性痴呆。然而,梅毒常常被误诊为其他疾病,如肿瘤或自身免疫性疾病,包括血管炎和脑炎,因此这种疾病被称为 "伟大的模仿者"。近年来,梅毒的发病率不断上升,强调了早期诊断和治疗的重要性;然而,由于梅毒的多种临床表现与其他疾病相似,给临床医生的诊断带来了挑战。在这组病例中,我们通过三种独特的放射学表现,介绍了神经梅毒令人印象深刻的表现:病例 1 详细描述了一名 HIV 阳性男性的视神经受累情况,核磁共振成像和眼底镜检查结果证实了梅毒性视神经炎。病例 2 描述了一名妊娠期患者,核磁共振成像最初怀疑其患有听神经瘤,后来确诊为梅毒性牙龈瘤累及内耳道。病例 3 是一名年轻男性,其临床特征类似颞动脉炎,最终被确定为颅骨骨髓炎,继而引起颞肌炎和脑膜炎:鉴于梅毒临床表现的多样性,这些病例强调了在鉴别诊断中考虑梅毒的必要性。放射学在避免不必要的干预方面发挥着重要作用。复发性梅毒的发病率越来越高,给诊断带来了挑战,强调了临床医生早期诊断和治疗神经梅毒的重要性。
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引用次数: 0
Harlequin syndrome in a patient with probable hemicrania continua and exertional headache - is there a link? a case report. 病例报告:一名可能患有持续性头痛和劳累性头痛的患者的哈勒金综合征--两者之间有联系吗?
IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-17 DOI: 10.1186/s12883-024-03731-y
Markus Miedl, Philipp Baumgartner, Leah Raffaela Disse, Konrad Peter Weber, Heiko Pohl, Susanne Wegener

Background: The harlequin syndrome is a rare disorder of the autonomic nervous system characterized by unilateral diminished flushing and sweating of the face following exposure to heat or physical activity. It results from sympathetic dysfunction and most commonly occurs idiopathically. A secondary development due to an underlying pathology (e.g., carotid artery dissection, tumors) must be excluded at first appearance. There is evidence that the cranial autonomic system is involved in the pathophysiology of trigeminal autonomic headaches like hemicrania continua. Therefore, an overlap in the pathophysiology of harlequin syndrome and trigeminal autonomic headache disorders seems plausible. However, the association of a harlequin syndrome with hemicrania continua was never reported.

Case presentation: This work describes the case of a 42-year-old female patient presenting to our headache unit. The patient reported persisting unilateral headache of the right side of dragging or squeezing character accompanied by trigeminal autonomic symptoms, including lacrimation, nasal congestion, conjunctival injection and Horner's syndrome, and was responsive to treatment with 75mg/d indomethacin. Five months after the initial consultation, the patient noted that the upper right quadrant of her face was pale after jogging. A harlequin syndrome was diagnosed. Further, she developed a short-lasting, bilateral headache of pulsatile character during strenuous exercise consistent with exertional headache. Comprehensive diagnostic evaluations, encompassing cranial and cervical MRI scans, laboratory tests, and biopsies, culminated in the diagnosis of Sjögren's syndrome. This finding suggests that the trigemino-autonomic dysfunction may either be idiopathic or a direct manifestation of Sjögren's syndrome.

Conclusions: This report documents the case of a rare combination of a headache resembling probable hemicrania continua and the harlequin syndrome (and even exertional headache). It illustrates the underlying anatomy of the autonomic nervous system in a clinical context and emphasizes the hypothesis of a pathophysiological link between abnormal sympathetic activity and trigeminal autonomic headaches.

背景:哈奎因综合征是一种罕见的自主神经系统疾病,其特征是在暴露于高温或体力活动后单侧脸部潮红和出汗减少。它由交感神经功能障碍引起,最常见的是特发性。初次出现时必须排除潜在病理(如颈动脉剥离、肿瘤)引起的继发性发展。有证据表明,头颅自律神经系统参与了三叉神经自律神经性头痛(如持续性头痛)的病理生理学。因此,哈雷综合征和三叉神经自律性头痛疾病的病理生理学重叠似乎是可信的。然而,从未有报道称哈雷金综合征与连续性头痛伴发:本文描述的是一名 42 岁女性患者在我院头痛科就诊的病例。患者报告了持续性单侧头痛,右侧为拖曳性或挤压性,伴有三叉神经自主神经症状,包括流泪、鼻塞、结膜注射和霍纳综合征,对 75 毫克/天的吲哚美辛治疗有反应。初诊五个月后,患者注意到慢跑后右上脸部苍白。经诊断,她患上了哈勒普综合征。此外,她在剧烈运动时出现持续时间短的双侧搏动性头痛,与劳累性头痛一致。综合诊断评估包括头颅和颈椎磁共振成像扫描、实验室检查和活组织检查,最终确诊为斯约格伦综合征。这一发现表明,三叉神经-自主神经功能障碍可能是特发性的,也可能是斯约格伦综合征的直接表现:本报告记录了一例罕见的头痛合并症,类似于可能的连续性头痛和哈勒金综合征(甚至是劳累性头痛)。它从临床角度说明了自律神经系统的基本解剖结构,并强调了交感神经活动异常与三叉神经自律神经性头痛之间存在病理生理联系的假设。
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引用次数: 0
Diffusion spectrum imaging in patients with idiopathic normal pressure hydrocephalus: correlation with ventricular enlargement. 特发性正常压力脑积水患者的弥散谱成像:与脑室扩大的相关性。
IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-16 DOI: 10.1186/s12883-024-03741-w
Qian Wu, Wenjie He, Chenyuan Liu, Xiaolin Yang, Jiakuan Chen, Boyan Xu, Xi Zhou, Guodong Huang, Jun Xia

Background: To investigate the association between white matter changes and ventricular expansion in idiopathic normal pressure hydrocephalus (iNPH) based on diffusion spectrum imaging (DSI).

Methods: We included 32 patients with iNPH who underwent DSI using a 3T MRI scanner. The lateral ventricles were manually segmented, and ventricular volumes were measured. Two methods were utilised in the study: manual region-of-interest (ROI) delineation and tract diffusion profile analysis. General fractional anisotropy (GFA) and fractional anisotropy (FA) were extracted in different white matter regions, including the bilateral internal capsule (anterior and posterior limbs) and corpus callosum (body, genu, and splenium) with manual ROI delineation. The 18 main tracts in the brain of each patient were extracted; the diffusion metrics of 100 equidistant nodes on each fibre were calculated, and Spearman's correlation coefficient was used to determine the correlation between diffusion measures and ventricular volume of iNPH patients.

Results: The GFA and FA of all ROI showed no significant correlation with lateral ventricular volume. However, in the tract diffusion profile analysis, lateral ventricular volume was positively correlated with part of the cingulum bundle, left corticospinal tract, and bilateral thalamic radiation posterior, whereas it was negatively correlated with the bilateral cingulum parahippocampal (all p < 0.05).

Conclusions: The effect of ventricular enlargement in iNPH on some white matter fibre tracts around the ventricles was limited and polarizing, and most white matter fibre tract integrity changes were not associated with ventricular enlargement; this reflects that multiple pathological mechanisms may have been combined to cause white matter alterations in iNPH.

背景:基于弥散谱成像(DSI)研究特发性正常压力脑积水(iNPH)患者白质变化与脑室扩张之间的关联:根据弥散谱成像(DSI)研究特发性正常压力脑积水(iNPH)患者白质变化与脑室扩张之间的关系:我们纳入了32名使用3T磁共振成像扫描仪进行DSI检查的特发性正常压力脑积水患者。人工分割侧脑室并测量脑室容积。研究中使用了两种方法:人工兴趣区(ROI)划分和束扩散轮廓分析。通过手动 ROI 划分,提取了不同白质区域的一般分数各向异性(GFA)和分数各向异性(FA),包括双侧内囊(前肢和后肢)和胼胝体(体、属和脾)。提取每位患者大脑中的18条主要纤维束,计算每条纤维上100个等距节点的扩散指标,并利用斯皮尔曼相关系数确定扩散指标与iNPH患者脑室容积的相关性:结果:所有 ROI 的 GFA 和 FA 与侧脑室容积无明显相关性。然而,在束扩散轮廓分析中,侧脑室容积与部分脑室束、左侧皮质脊髓束和双侧丘脑辐射后部呈正相关,而与双侧脑室旁呈负相关(均为 p):iNPH患者脑室扩大对脑室周围部分白质纤维束的影响是有限的,而且是两极化的,大多数白质纤维束的完整性变化与脑室扩大无关;这反映出多种病理机制可能共同导致了iNPH患者白质的改变。
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引用次数: 0
Rheumatoid meningitis in the absence of rheumatoid arthritis: 2 cases. 没有类风湿性关节炎的类风湿性脑膜炎:2 个病例。
IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-07-15 DOI: 10.1186/s12883-024-03751-8
Juan Yang, Lu Liu, Jiahui Peng, Boya Ma, Xiao Yang

Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis (RA) that has been increasingly recognized by neurologists. However, the diversity of its clinical manifestations makes its diagnosis difficult. RM does not have a unified diagnostic standard, and its link with RA needs to be studied further. Here we report two cases of RM without a history of RA. The first patient, an 80-year-old woman, presented with sudden unilateral limb weakness, with brain MR showing abnormal signals in the leptomeningeal of the right frontal parietal. Subarachnoid hemorrhage was excluded after imaging examination, and infectious meningitis was ruled out after cerebrospinal fluid (CSF) examination. The patient was diagnosed as having RM, she had increased levels of CCP and AKA, the markers of RA, but no history of the disease or other clinical manifestations of it. Another case, a 65-year-old man, was hospitalized with Bell's palsy. We found that he had intracranial imaging changes highly consistent with those characteristic of RM during his routine examination. Except for the left peripheral facial palsy, the patient had no other neurological signs or symptoms and no RA history. After a careful physical examination, we found no joint or other manifestations or serological abnormalities consistent with RA (RF, CCP, AKA, etc.). However, after excluding infection meningitis and considering the patient's unique imaging results, we diagnosed him as having RM. We report these two cases as references for clinical diagnosis and treatment of RM, providing a discussion of our rationale.

类风湿性脑膜炎(RM)是类风湿性关节炎(RA)的一种罕见关节外表现,已被越来越多的神经科医生所认识。然而,其临床表现的多样性给诊断带来了困难。RM尚无统一的诊断标准,其与RA的关系也有待进一步研究。在此,我们报告了两例无 RA 病史的 RM 患者。第一例患者是一名80岁的女性,突发单侧肢体无力,脑MR显示右侧额顶叶脑膜异常信号。影像学检查排除了蛛网膜下腔出血,脑脊液(CSF)检查排除了感染性脑膜炎。该患者被诊断为 RM,她的 CCP 和 AKA(RA 的标志物)水平升高,但没有该病的病史或其他临床表现。另一个病例是一名 65 岁的男性,因贝尔麻痹住院。我们在对他进行常规检查时发现,他的颅内影像学改变与RM的特征高度一致。除了左侧外周面部麻痹外,患者没有其他神经系统体征或症状,也没有 RA 病史。经过仔细的体格检查,我们没有发现与 RA 相符的关节或其他表现或血清学异常(RF、CCP、AKA 等)。然而,在排除感染性脑膜炎并考虑到患者独特的影像学结果后,我们诊断他患有RM。我们报告了这两个病例,作为RM临床诊断和治疗的参考,并对我们的理由进行了讨论。
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