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Shared neural signatures in Functional Neurological Disorder and Chronic Pain: a multimodal narrative review. 功能性神经障碍和慢性疼痛的共享神经特征:多模态叙事回顾。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-07-13 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001032
Siddarth Kannan, Kajal Patel, Daniela Di Basilio, Antonia Kirkby, Manoj Sivan, Anthony Jones, Rajiv Mohanraj, Abhijit Das

Background: Functional neurological disorder (FND) frequently co-exists with chronic pain (CP), notably nociceptive and nociplastic (primary) pain disorders. The considerable overlap implies shared underlying mechanisms because of their similar clinical and epidemiological profiles. Although standard neuroimaging and electrophysiological tests typically show normal results in both FND and primary pain disorders, recent advancements in neuroimaging techniques have begun identifying neural biomarkers common to both conditions, though these findings remain preliminary and require further exploration.

Method: We performed a detailed literature review of studies investigating neural activity in FND and chronic pain using electroencephalogram, magneto-encephalography, functional MRI, positron emission tomography and single photon emission computed tomography. Given the diverse nature of the reviewed studies, the synthesis is presented narratively.

Results: Despite methodological differences, convergent data suggest disrupted neural networks across both FND and CP. Common findings include (1) hyperactivation of sensorimotor networks, (2) altered activity within the default mode network-a critical region for self-referential thought-and (3) dysfunction in emotional processing regions, notably the anterior cingulate cortex and insula. Thalamocortical dysrhythmia was identified as a potential unifying concept, characterised by abnormal theta and beta oscillations that enhance pain perception in CP and trigger functional symptoms in FND. Both conditions also exhibit reduced alpha oscillations, likely amplifying sensory sensitivity and emotional responsiveness.

Conclusion: This review highlights shared neural abnormalities (Triple Network model) and introduces thalamocortical dysrhythmia as a novel explanatory framework linking FND and CP. Future research should target populations with coexisting disorders, potentially paving the way for innovative treatments, including hypnosis and neuromodulation/neurofeedback.

背景:功能性神经障碍(FND)经常与慢性疼痛(CP)共存,特别是伤害性和伤害性(原发性)疼痛障碍。由于其相似的临床和流行病学概况,相当大的重叠意味着共享的潜在机制。虽然标准的神经成像和电生理测试通常在FND和原发性疼痛疾病中显示正常结果,但最近神经成像技术的进步已经开始识别这两种疾病共同的神经生物标志物,尽管这些发现仍处于初步阶段,需要进一步探索。方法:采用脑电图、脑磁图、功能磁共振成像、正电子发射断层扫描和单光子发射计算机断层扫描等方法,对FND和慢性疼痛的神经活动进行了详细的文献回顾。鉴于所审查的研究的多样性,综合呈现叙述。结果:尽管方法上存在差异,但聚合数据表明FND和CP的神经网络都受到了破坏。共同的发现包括:(1)感觉运动网络的过度激活,(2)默认模式网络(自我参照思维的关键区域)的活动改变,(3)情绪处理区域的功能障碍,尤其是前扣带皮层和脑岛。丘脑皮质节律异常被认为是一个潜在的统一概念,其特征是异常的θ波和β波振荡增强CP的痛觉,并引发FND的功能性症状。这两种情况都表现出减少的α振荡,可能会放大感官敏感性和情绪反应。结论:这篇综述强调了共同的神经异常(三重网络模型),并将丘脑皮质节律异常作为一种新的解释框架,将FND和CP联系起来。未来的研究应该针对共存疾病的人群,为创新治疗铺平道路,包括催眠和神经调节/神经反馈。
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引用次数: 0
Transcranial magnetic stimulation as a primer for rapid improvement in functional neurological disorder: a case series. 经颅磁刺激作为快速改善功能性神经障碍的引物:一个病例系列。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-07-07 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001102
Benjamin Scrivener, Harry Jordan, Neil Anderson, Tony Zhang, P Alan Barber, Cathy Stinear

Background: Functional weakness is common, and the prognosis can be poor without treatment. Transcranial magnetic stimulation (TMS) and specialist physiotherapy have each been trialled separately as interventions for functional weakness. We tested a novel approach for treating functional weakness and gait disorder using TMS as a primer before specialist physiotherapy.

Methods: Single-pulse TMS, therapeutic education and limb pressure feedback were used as a primer for immediate specialist physiotherapy. TMS-primed physiotherapy was used for the first time in three consecutive patients with functional limb weakness and immobility.

Results: Two women and one man (aged 30-55 years) with severe functional limb weakness such that they were unable to stand or walk independently, with symptom duration between 3 weeks and 7 years, were studied. All three had a rapid return of voluntary limb movement and achieved unassisted walking within a few hours of a single TMS-primed physiotherapy session. Treatment was well-tolerated, and outcomes were sustained at follow-up.

Conclusions: These cases provide preliminary evidence supporting the efficacy of this approach, which may be further developed with future research. They also illustrate a practical approach for treating a clinically challenging population with severe functional weakness.

背景:功能衰弱是一种常见的疾病,如果不及时治疗,预后很差。经颅磁刺激(TMS)和专业物理治疗分别作为功能虚弱的干预措施进行了试验。我们测试了一种治疗功能无力和步态障碍的新方法,在专业物理治疗之前使用经颅磁刺激作为引物。方法:采用单脉冲经颅磁刺激、治疗教育和肢体压力反馈作为立即专科物理治疗的引子。首次采用tms启动物理治疗连续3例功能性肢体无力和活动不全患者。结果:研究了两名女性和一名男性(年龄30-55岁),他们患有严重的功能性肢体无力,无法独立站立或行走,症状持续时间在3周到7年之间。这三个人都能迅速恢复自主肢体活动,并在单次经颅磁刺激物理治疗的几个小时内实现了独立行走。治疗耐受性良好,随访结果持续。结论:这些病例为该方法的有效性提供了初步的证据,可以在未来的研究中进一步发展。他们也说明了一种实用的方法来治疗具有严重功能虚弱的临床挑战性人群。
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引用次数: 0
Effect of anticoagulation on the age distribution of thrombi in stroke patients with non-valvular atrial fibrillation. 抗凝治疗对卒中合并非瓣膜性房颤患者血栓年龄分布的影响。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-07-03 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2024-000954
Sophie Schumann, Jens Jürgen Schwarze, Silvio Brandt, Korinna Jöhrens, Olaf Dirsch, Karim Ibrahim, Bernhard Rosengarten

Objective: Atrial fibrillation (AF) is an important risk factor for cerebral stroke. We studied whether anticoagulation affected histological age aspects of thrombi retrieved from patients with AF-related stroke.

Methods: In this monocentric study, AF patients according to criteria (Trial of Org 10 172 in Acute Stroke Treatment) with occlusion of the middle cerebral artery were prospectively and consecutively included. They were assigned to three groups: anticoagulation naïve, adequately anticoagulated, and with paused anticoagulation. In addition to patient characteristics and stroke workup, extracted thrombi were histologically classified into different age categories according to their cellular to fibrotic transition.

Results: A total of 244 patients were studied, from which 136 (58 females; 78±9 years) were drug naïve, 34 (15 females; 78±8 years) had paused anticoagulation, and 74 (29 females; 79±9 years) were adequately anticoagulated. Groups did not differ regarding stroke severity at admission (modified Rankin Score, mRS: median, IQR: 5 (1); 5 (0.75); 5 (1), respectively; National Institutes of Health Stroke Scale (NIHSS): median, IQR: 16 (8); 16 (8); 16 (7), respectively). Due to thrombectomy, median scores declined in all groups without differences between groups (mRS: 3.5 (4); 4 (4); 4 (4); NIHSS: 5 (16); 11 (31); 7 (18)). With a small but significant effect (p=0.043), thrombus age differed between the groups due to significantly younger thrombi in the paused medication group as compared with the adequately anticoagulated patients.

Conclusions: Thrombus age distribution seems not to be affected by anticoagulation. The younger thrombi in patients with paused anticoagulation possibly point to a rebound effect needing further investigations.

目的:心房颤动(AF)是脑卒中的重要危险因素。我们研究抗凝是否影响心房颤动相关卒中患者取血栓的组织学年龄方面。方法:在这项单中心研究中,前瞻性和连续性纳入符合标准的大脑中动脉闭塞的房颤患者(急性卒中治疗试验Org 10172)。他们被分为三组:抗凝naïve,充分抗凝和暂停抗凝。除了患者特征和卒中检查外,提取的血栓根据其细胞向纤维化的转变在组织学上被划分为不同的年龄类别。结果:共纳入244例患者,其中136例(女性58例;78±9岁)为药物naïve, 34例(女性15例;78±8年)停止抗凝,74例(女性29例;79±9年)。各组入院时卒中严重程度无差异(修正Rankin评分,mRS:中位数,IQR: 5 (1);5 (0.75);5 (1);美国国立卫生研究院卒中量表(NIHSS):中位数,IQR: 16 (8);16 (8);16(7))。由于取栓,各组中位评分均下降,组间无差异(mRS: 3.5 (4);4 (4);4 (4);Nihss: 5 (16);11 (31);7(18))。两组之间血栓形成年龄的差异虽小但效果显著(p=0.043),这是由于停药组的血栓明显比充分抗凝的患者年轻。结论:抗凝治疗似乎不影响血栓年龄分布。在暂停抗凝治疗的患者中,较年轻的血栓可能指向反弹效应,需要进一步的研究。
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引用次数: 0
Disease-modifying therapy during pregnancy and postpartum relapse activity in women with multiple sclerosis undergoing assisted reproductive technology treatment: a nationwide cohort study. 接受辅助生殖技术治疗的多发性硬化症妇女孕期疾病改善治疗和产后复发活动:一项全国性队列研究
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-07-03 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001092
Lene von Kappelgaard, Elisabeth Framke, Ditte Vassard, Anja Pinborg, Juan Enrique Schwarze, Meritxell Sabidó, Melinda Magyari

Background: Evidence is scarce concerning the impact on postpartum relapse activity of disease-modifying therapy (DMT) use during pregnancy after assisted reproductive technology (ART) treatment. We investigated relapse activity before pregnancy, during pregnancy and 3 and 12 months postpartum overall and according to DMT exposure during pregnancy.

Methods: Women with relapsing-remitting multiple sclerosis (MS) from the Danish MS Registry who gave birth after ART from 1995 to 2018 were eligible for inclusion. Annualised relapse rate (ARR) before pregnancy, during pregnancy and postpartum was evaluated using a negative binomial regression model with relapse count as the dependent variable overall and according to DMT exposure during pregnancy. Logistic regression was used to identify predictors of being relapse-free 12 months postpartum.

Results: A total of 111 women, median age 32 years (IQR: 29-35), were included. Overall, ARR (95% CI) was 0.14 (0.08 to 0.24) before pregnancy, 0.13 (0.07 to 0.25) during pregnancy and 0.33 (0.17 to 0.61) 3 months postpartum; rate ratio difference between before pregnancy and postpartum was 2.42 (1.03 to 5.67), and between during pregnancy and postpartum was 2.46 (1.18 to 5.13). Age ≥35 years and no DMT exposure before pregnancy were predictors of being relapse-free 12 months postpartum.

Conclusions: Relapse activity was more than two times higher 3 months postpartum than before and during pregnancy. Stratified for DMT exposure during pregnancy, rate ratios were similar and did not reach statistical significance. Postpartum relapse activity more than doubled from both low ARR (unexposed to DMT during pregnancy) and higher ARR (exposed to DMT during pregnancy) pre-pregnancy.

背景:关于辅助生殖技术(ART)治疗后妊娠期间使用疾病修饰疗法(DMT)对产后复发活动的影响的证据很少。我们调查了怀孕前,怀孕期间和产后3个月和12个月的复发活动,并根据怀孕期间的DMT暴露。方法:1995年至2018年期间在丹麦MS登记处接受抗逆转录病毒治疗后分娩的复发-缓解型多发性硬化症(MS)妇女符合纳入条件。使用负二项回归模型评估孕前、孕期和产后的年化复发率(ARR),复发率作为总体因变量,并根据妊娠期间的DMT暴露。采用Logistic回归确定产后12个月无复发的预测因素。结果:共纳入111例女性,中位年龄32岁(IQR: 29-35)。总体而言,孕前ARR (95% CI)为0.14(0.08 ~ 0.24),孕期为0.13(0.07 ~ 0.25),产后3个月为0.33 (0.17 ~ 0.61);孕前与产后的比率差为2.42(1.03 ~ 5.67),孕期与产后的比率差为2.46(1.18 ~ 5.13)。年龄≥35岁和孕前未接触DMT是产后12个月无复发的预测因素。结论:产后3个月复发率比孕前和孕期高2倍以上。对怀孕期间的DMT暴露进行分层,比率相似,未达到统计学意义。产后复发活动比低ARR(怀孕期间未暴露于DMT)和高ARR(怀孕期间暴露于DMT)怀孕前增加了一倍以上。
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引用次数: 0
Measuring neurological severity and complexity in acute setting: the modified Neurological Impairment Scale. 测量急性神经系统的严重程度和复杂性:改良的神经损伤量表。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-06-26 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001107
Alessandro Padovani, Irene Mattioli, Tiziana Comunale, Nicola Zoppi, Cinzia Zatti, Enis Guso, Marcello Catania, Andrea Morotti, Chiara Agosti, Stefano Gipponi, Lynne Turner-Stokes, Andrea Pilotto

Abstract:

Background: Given the increasing diversity among neurological patients, standardised protocols are essential for evaluating the severity and complexity of the variety of conditions. The aim of the present work was to standardise the assessment of the severity and complexity of neurological impairment in an acute setting by using a modified version of the Neurological Impairment Scale (mNIS).

Methods: Consecutively hospitalised neurological inpatients underwent a multidimensional standardised assessment of multimorbidity, frailty, functional dependency and neurological impairment using mNIS and other validated scales. Inter-rater reliability of the mNIS total and subscores was evaluated. Construct validity was assessed separately in patients with cerebrovascular disease, performing correlations between corresponding subscores of mNIS, original NIS and National Institutes of Health Stroke Scale. mNIS Complexity Index (mNIS-CI) for neurological severity was used to classify patients into subtle, mild, moderate and severe impairment.

Results: 1081 neurological patients admitted to a neurological ward from the emergency setting were enrolled. The inter-rater reliability was remarkable for mNIS total and subscores (intraclass correlation coefficient 0.90, 95% CI 0.82 to 0.95). The mNIS showed strong construct validity for total and subscores compared with other clinical scales (r 0.47-0.97, p<0.001) and 52.7% of patients scored at least one in one of the four newly listed items. The stratification of patients according to mNIS-CI exhibited high construct validity, distinguishing the extent of impairment and involved domains.

Conclusions: The mNIS is valuable for measuring neurological severity and complexity in acute inpatients and holds significant potential for application in different settings.

摘要:背景:鉴于神经系统患者的多样性日益增加,标准化的方案对于评估各种疾病的严重性和复杂性至关重要。本研究的目的是通过使用改良版的神经损伤量表(mNIS)对急性环境中神经损伤的严重程度和复杂性进行标准化评估。方法:对连续住院的神经系统住院患者采用mNIS及其他经验证的量表对其多病、虚弱、功能依赖和神经功能损害进行多维标准化评估。评估了mNIS总分和分值的评分间信度。在脑血管疾病患者中单独评估结构效度,将mNIS、原始NIS和美国国立卫生研究院卒中量表的相应亚分进行相关性分析。神经系统严重程度的mNIS复杂性指数(mNIS- ci)将患者分为轻度、轻度、中度和重度。结果:1081名急诊科神经病房的患者入组。mNIS总分和分值的组间信度显著(组内相关系数0.90,95% CI 0.82 ~ 0.95)。与其他临床量表相比,mNIS在总分和亚分上显示出较强的结构效度(r = 0.47-0.97)。结论:mNIS在衡量急性住院患者神经系统严重程度和复杂性方面具有重要价值,在不同的环境中具有显著的应用潜力。
{"title":"Measuring neurological severity and complexity in acute setting: the modified Neurological Impairment Scale.","authors":"Alessandro Padovani, Irene Mattioli, Tiziana Comunale, Nicola Zoppi, Cinzia Zatti, Enis Guso, Marcello Catania, Andrea Morotti, Chiara Agosti, Stefano Gipponi, Lynne Turner-Stokes, Andrea Pilotto","doi":"10.1136/bmjno-2025-001107","DOIUrl":"10.1136/bmjno-2025-001107","url":null,"abstract":"<p><strong>Abstract: </strong></p><p><strong>Background: </strong>Given the increasing diversity among neurological patients, standardised protocols are essential for evaluating the severity and complexity of the variety of conditions. The aim of the present work was to standardise the assessment of the severity and complexity of neurological impairment in an acute setting by using a modified version of the Neurological Impairment Scale (mNIS).</p><p><strong>Methods: </strong>Consecutively hospitalised neurological inpatients underwent a multidimensional standardised assessment of multimorbidity, frailty, functional dependency and neurological impairment using mNIS and other validated scales. Inter-rater reliability of the mNIS total and subscores was evaluated. Construct validity was assessed separately in patients with cerebrovascular disease, performing correlations between corresponding subscores of mNIS, original NIS and National Institutes of Health Stroke Scale. mNIS Complexity Index (mNIS-CI) for neurological severity was used to classify patients into subtle, mild, moderate and severe impairment.</p><p><strong>Results: </strong>1081 neurological patients admitted to a neurological ward from the emergency setting were enrolled. The inter-rater reliability was remarkable for mNIS total and subscores (intraclass correlation coefficient 0.90, 95% CI 0.82 to 0.95). The mNIS showed strong construct validity for total and subscores compared with other clinical scales (r 0.47-0.97, p<0.001) and 52.7% of patients scored at least one in one of the four newly listed items. The stratification of patients according to mNIS-CI exhibited high construct validity, distinguishing the extent of impairment and involved domains.</p><p><strong>Conclusions: </strong>The mNIS is valuable for measuring neurological severity and complexity in acute inpatients and holds significant potential for application in different settings.</p>","PeriodicalId":52754,"journal":{"name":"BMJ Neurology Open","volume":"7 1","pages":"e001107"},"PeriodicalIF":2.1,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12207106/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144531032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Contemporary positive signs of functional limb weakness in post-acute sequelae of SARS-CoV-2: an exploratory analysis of their utility in diagnosis and follow-up. SARS-CoV-2急性后后遗症中功能性肢体无力的当代阳性体征:在诊断和随访中的探索性分析
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-06-26 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2024-000995
Masayuki Ohira, Takashi Osada, Hiroaki Kimura, Terunori Sano, Masaki Takao

Background: Sequelae of the acute phase of coronavirus disease-19, termed long COVID, are characterised by numerous indicators, including neurological symptoms. Functional neurological disorder (FND) can occur with or without various structural diseases. No previous study has examined the relationship between long COVID and FND, with positive signs for FND. This study confirmed positive signs of functional limb weakness (hereafter positive signs) in patients with long COVID.

Methods: This was an observational, retrospective, single-centre study at an outpatient clinic conducted from 1 June 2021 to 31 May 2024. We collected patients' clinical data, including positive signs. The primary outcome was the prevalence of positive signs. Patients with positive signs were followed up over 2 months, and subjective patient perceptions of symptomatic improvements and changes in positive signs were analysed.

Results: Overall, 502 were diagnosed with long COVID, and 100 assessed patients had positive signs. Female sex, time of infection after 2022, comorbidity of psychiatric diseases, fatigue, headache and muscle weakness were statistically significant in patients with positive signs compared with those in patients without positive signs. 89 patients (41 with positive signs and 48 without positive signs) were followed up, and 28 (68.3%) with positive signs and 33 (68.8%) without positive signs reported improvements. Positive signs disappeared in patients with symptomatic improvements but not in patients without symptomatic improvements (p=0.0001).

Conclusions: Positive signs were found in over one-third of patients (33.9%) who were investigated in this study. Some positive signs disappeared concurrently with their symptomatic improvement.

背景:冠状病毒病-19急性期的后遗症,称为长冠状病毒,具有许多指标的特征,包括神经系统症状。功能性神经障碍(FND)可伴有或不伴有各种结构疾病。之前没有研究调查过长COVID和FND之间的关系,FND有积极的迹象。本研究证实长COVID患者存在功能性肢体无力的阳性迹象(以下简称阳性迹象)。方法:这是一项观察性、回顾性、单中心研究,于2021年6月1日至2024年5月31日在一家门诊诊所进行。我们收集了患者的临床资料,包括积极的迹象。主要结果是阳性症状的普遍性。阳性体征患者随访2个月以上,分析患者对症状改善和阳性体征变化的主观感知。结果:确诊长冠502例,阳性体征100例。阳性体征患者与无阳性体征患者相比,女性、2022年后感染时间、精神疾病合并症、疲劳、头痛、肌无力等差异均有统计学意义。89例患者(阳性症状41例,无阳性症状48例)随访,阳性症状改善28例(68.3%),无阳性症状改善33例(68.8%)。症状改善的患者阳性体征消失,无症状改善的患者阳性体征消失(p=0.0001)。结论:在本研究中,超过三分之一(33.9%)的患者出现阳性体征。一些阳性体征在症状改善的同时消失。
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引用次数: 0
Epidemiology and patient characteristics of the US myasthenia gravis population: real-world evidence from a large insurance claims database. 美国重症肌无力人群的流行病学和患者特征:来自大型保险索赔数据库的真实世界证据。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-06-23 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001076
Lesley-Ann Miller-Wilson, Joel Arackal, Yuriy Edwards, Jennifer Schwinn, Kristen Elizabeth Rockstein, Brett Venker, Richard J Nowak

Background: The epidemiology of myasthenia gravis (MG), a rare autoimmune disease, is not completely understood. This retrospective claims analysis assessed the epidemiologic rates of MG in the USA and the characteristics of the incident and prevalent MG populations.

Methods: Patients with MG were identified using Inovalon closed claims data; those with ≥2 International Classification of Diseases, 10th Revision (ICD-10) codes for MG from 2016 to 2019, separated by ≥30 days, were eligible. Patients in the prevalent cohort had continuous insurance coverage from January to December 2019. Patients in the incident cohort had continuous insurance coverage from January 2018 to December 2019 and no MG claims before 2019. Incidence and prevalence rates were adjusted using US census data. Treatment utilisation was assessed in both cohorts and grouped by provider type. Exacerbations were identified using ICD-10 code G70.01; codes occurring ≤30 days following a previous exacerbation code were considered as part of a single event.

Results: The incident cohort included 1442 patients (adjusted incidence: 4.3 per 100000); the prevalent cohort included 14 373 patients (adjusted prevalence: 35.7 per 100 000). In 2019, approximately two-thirds of patients in both cohorts (incident, 67.7%; prevalent, 61.6%) received MG treatments, most commonly acetylcholinesterase inhibitors, followed by oral steroids. Among incident patients with ≥1 year of continuous follow-up (n=1204), 32.1% (n=387) experienced ≥1 exacerbation, with 50% (n=194) having ≥2 exacerbations and 41% (n=159) having an exacerbation as the index MG event.

Conclusions: Estimates of MG incidence and prevalence were comparable to findings from two recent US studies. Acetylcholinesterase inhibitors and corticosteroids remain widely used. Exacerbations occurred in >30% of incident patients with ≥1 year of continuous follow-up, including 13% who had a relapse as their index event. These results highlight the need for earlier detection and use of more effective therapies in the treatment paradigm to achieve sustained disease control.

背景:重症肌无力(MG)是一种罕见的自身免疫性疾病,其流行病学尚未完全了解。本回顾性索赔分析评估了美国MG的流行病学发生率以及发病率和流行人群的特征。方法:使用Inovalon封闭索赔数据对MG患者进行识别;2016 - 2019年《国际疾病分类第十版(ICD-10)》MG编码≥2条,间隔≥30天者入选。流行队列中的患者从2019年1月至12月连续投保。事件队列中的患者在2018年1月至2019年12月期间连续投保,并且在2019年之前没有MG索赔。发病率和患病率使用美国人口普查数据进行调整。在两个队列中评估治疗利用情况,并按提供者类型分组。使用ICD-10代码G70.01识别加重;在前一次加重后≤30天发生的症状被视为单一事件的一部分。结果:事件队列纳入1442例患者(调整后的发病率:4.3 / 100000);流行队列包括14373例患者(调整患病率:35.7 / 100000)。在2019年,两个队列中约有三分之二的患者(事件,67.7%;(61.6%)接受MG治疗,最常见的是乙酰胆碱酯酶抑制剂,其次是口服类固醇。在连续随访≥1年的事件患者(n=1204)中,32.1% (n=387)的急性发作≥1次,其中50% (n=194)的急性发作≥2次,41% (n=159)的急性发作为MG事件的指标。结论:MG发病率和流行率的估计值与美国最近两项研究的结果相当。乙酰胆碱酯酶抑制剂和皮质类固醇仍被广泛使用。在连续随访≥1年的事件患者中,有30%的患者出现加重,其中13%的患者以复发为主要事件。这些结果强调需要在治疗范式中更早发现和使用更有效的治疗方法,以实现持续的疾病控制。
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引用次数: 0
Retraction: Novel IRF2BPL gene mutation manifesting as a broad spectrum of neurological disorders: a case report. 撤回:新型IRF2BPL基因突变表现为广泛的神经系统疾病:一个病例报告。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-06-19 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2023-000459ret

[This retracts the article DOI: 10.1136/bmjno-2023-000459.].

[本文撤回文章DOI: 10.1136/bmjno-2023-000459.]。
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引用次数: 0
Integrating clinical predictors and glial fibrillary acidic protein in prediction models for the prehospital identification of intracerebral haemorrhage in suspected stroke. 整合临床预测因子和胶质纤维酸性蛋白在院前诊断疑似脑卒中脑出血预测模型中的应用。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-06-19 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001160
Mohammed Almubayyidh, David A Jenkins, Edoardo Gaude, Adrian R Parry-Jones

Abstract:

Introduction: Accurate and timely differentiation of intracerebral haemorrhage (ICH) from other suspected stroke cases is crucial in prehospital settings, where early blood pressure reduction in the ambulance can improve outcomes. This study aims to assess whether combining clinical predictors and glial fibrillary acidic protein (GFAP) in prediction models can effectively distinguish ICH from other suspected stroke cases.

Methods: Data were derived from the Testing for Identification Markers of Stroke trial, a prospective diagnostic accuracy study. Suspected stroke patients within 6 hours of symptom onset were included. Clinical predictors were selected based on known associations with ICH, and a predefined GFAP cut-off of 290 pg/mL was applied. Logistic regression was used to assess the performance of clinical predictors and GFAP, individually and in combination. Internal validation and optimism correction were performed via bootstrapping, and comparisons of the area under the curve (AUC) were conducted using DeLong's test.

Results: We included 209 suspected stroke cases, of which 5% were finally diagnosed with ICH. Clinical predictors alone achieved an optimism-corrected AUC of 0.74 (95% CI 0.60 to 0.88), while GFAP alone resulted in an optimism-corrected AUC of 0.83 (95% CI 0.69 to 0.99). Combining clinical predictors with GFAP significantly enhanced the AUC, yielding an optimism-corrected value of 0.90 (95% CI 0.79 to 0.98). This combined model also demonstrated high predictive accuracy, with an optimism-corrected sensitivity of 60% (95% CI 29.0% to 90.0%) and a specificity of 98% (95% CI 96.1% to 100.0%).

Conclusions: Combining clinical predictors with GFAP shows promise for the prehospital identification of ICH to support transport decision-making and potentially initiate treatment while en route for these patients. Prospective validation using portable point-of-care devices is required to confirm the utility of this approach in the prehospital setting.

摘要:引言:准确和及时地区分脑出血(ICH)与其他疑似中风病例在院前环境中至关重要,在救护车中早期降低血压可以改善预后。本研究旨在评估在预测模型中结合临床预测因子和胶质纤维酸性蛋白(GFAP)是否能有效区分脑出血与其他疑似脑卒中病例。方法:数据来源于卒中识别标记物测试试验,这是一项前瞻性诊断准确性研究。纳入症状出现6小时内的疑似脑卒中患者。根据已知的与脑出血的关联选择临床预测因子,并采用预定义的GFAP临界值290 pg/mL。采用Logistic回归单独或联合评估临床预测指标和GFAP的表现。采用bootstrapping进行内部验证和乐观校正,采用DeLong’s test比较曲线下面积(AUC)。结果:我们纳入了209例疑似脑卒中病例,其中5%最终诊断为脑出血。单独的临床预测获得了0.74的乐观校正AUC (95% CI 0.60 - 0.88),而单独的GFAP导致了0.83的乐观校正AUC (95% CI 0.69 - 0.99)。结合临床预测因子和GFAP显著提高了AUC,得到了0.90的乐观校正值(95% CI 0.79 - 0.98)。该联合模型也显示出较高的预测准确性,乐观校正后的灵敏度为60% (95% CI 29.0%至90.0%),特异性为98% (95% CI 96.1%至100.0%)。结论:将临床预测指标与GFAP相结合,有望在院前识别脑出血,以支持转运决策,并可能在途中为这些患者启动治疗。需要使用便携式护理点设备进行前瞻性验证,以确认该方法在院前环境中的实用性。
{"title":"Integrating clinical predictors and glial fibrillary acidic protein in prediction models for the prehospital identification of intracerebral haemorrhage in suspected stroke.","authors":"Mohammed Almubayyidh, David A Jenkins, Edoardo Gaude, Adrian R Parry-Jones","doi":"10.1136/bmjno-2025-001160","DOIUrl":"10.1136/bmjno-2025-001160","url":null,"abstract":"<p><strong>Abstract: </strong></p><p><strong>Introduction: </strong>Accurate and timely differentiation of intracerebral haemorrhage (ICH) from other suspected stroke cases is crucial in prehospital settings, where early blood pressure reduction in the ambulance can improve outcomes. This study aims to assess whether combining clinical predictors and glial fibrillary acidic protein (GFAP) in prediction models can effectively distinguish ICH from other suspected stroke cases.</p><p><strong>Methods: </strong>Data were derived from the Testing for Identification Markers of Stroke trial, a prospective diagnostic accuracy study. Suspected stroke patients within 6 hours of symptom onset were included. Clinical predictors were selected based on known associations with ICH, and a predefined GFAP cut-off of 290 pg/mL was applied. Logistic regression was used to assess the performance of clinical predictors and GFAP, individually and in combination. Internal validation and optimism correction were performed via bootstrapping, and comparisons of the area under the curve (AUC) were conducted using DeLong's test.</p><p><strong>Results: </strong>We included 209 suspected stroke cases, of which 5% were finally diagnosed with ICH. Clinical predictors alone achieved an optimism-corrected AUC of 0.74 (95% CI 0.60 to 0.88), while GFAP alone resulted in an optimism-corrected AUC of 0.83 (95% CI 0.69 to 0.99). Combining clinical predictors with GFAP significantly enhanced the AUC, yielding an optimism-corrected value of 0.90 (95% CI 0.79 to 0.98). This combined model also demonstrated high predictive accuracy, with an optimism-corrected sensitivity of 60% (95% CI 29.0% to 90.0%) and a specificity of 98% (95% CI 96.1% to 100.0%).</p><p><strong>Conclusions: </strong>Combining clinical predictors with GFAP shows promise for the prehospital identification of ICH to support transport decision-making and potentially initiate treatment while en route for these patients. Prospective validation using portable point-of-care devices is required to confirm the utility of this approach in the prehospital setting.</p>","PeriodicalId":52754,"journal":{"name":"BMJ Neurology Open","volume":"7 1","pages":"e001160"},"PeriodicalIF":2.1,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12182047/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Time to endovascular thrombectomy for acute ischaemic stroke in language other than English (LOTE) communities in South-Western Sydney. 悉尼西南部非英语(LOTE)社区急性缺血性卒中的血管内血栓切除术时间。
IF 2.1 Q3 CLINICAL NEUROLOGY Pub Date : 2025-06-19 eCollection Date: 2025-01-01 DOI: 10.1136/bmjno-2025-001114
Vivek Pidugun, Daniel Wardman, Peter Thomas, Helen Badge, Jing Ji, Chushuang Chen, Timmy Pham, Andrew K Cheung, Nathan Manning, Christopher Blair, Cecilia Cappelen-Smith, Longting Lin, Suzanne J Hodgkinson, Alan McDougall, Mark Parsons, Dennis Cordato

Background: Access to endovascular thrombectomy (EVT) for acute ischaemic stroke (AIS) and the outcome disparities in culturally and linguistically diverse (CALD) populations are understudied. South-Western Sydney (SWS), characterised by high prevalence of CALD populations, provides an ideal setting to explore these disparities. This study aimed to assess whether being born in a country where English is not the primary language ('language other than English'; LOTE) affects access to EVT and outcomes for acute ischaemic stroke while also identifying demographic risk factors influencing stroke severity and outcomes.

Methods: A retrospective cohort study was conducted on consecutive patients with AIS who underwent EVT at Liverpool Hospital, Sydney, from 2018 to 2023. Participants were categorised based on country-of-origin Australia/New Zealand/United Kingdom or LOTE. Primary outcomes included time metrics-onset-to-arrival (OTA) and arrival-to-puncture (ATP)-and 3-month modified Rankin Score (mRS). Statistical analyses included multivariate logistic regression to evaluate predictors of functional outcomes.

Results: Of 911 EVT referrals, 721 patients were included. LOTE patients (50.3%) were more likely to have diabetes mellitus (30% vs 16%; p<0.001) and presented with higher stroke severity (median National Institutes of Health Stroke Scale (NIHSS) 17 vs 14; p<0.001). They also experienced shorter ATP (158 vs 174 min; p=0.006). Patients requiring interpreters equally exhibited shorter ATP times (152 vs 170 min; p=0.01) and higher stroke severity. There was no significant disadvantage hailing from the LOTE group in OTA times and in any time metrics for subgroup analyses stratified by primary presenter status. However, LOTE patients had poorer 3-month outcomes (mRS ≤2: 46.5% vs 55.4%; p=0.021), which was associated with higher baseline NIHSS and diabetes in adjusted analyses.

Conclusion: LOTE patients in SWS undergoing EVT were not disadvantaged in hospital time metrics. Poorer 3-month functional outcomes in LOTE patients highlight the need for targeted strategies addressing risk factors, such as diabetes mellitus.

背景:急性缺血性卒中(AIS)的血管内血栓切除术(EVT)以及文化和语言多样性(CALD)人群的结果差异尚未得到充分研究。西南悉尼(SWS)的特点是CALD人群的高患病率,为探索这些差异提供了理想的环境。这项研究旨在评估是否出生在英语不是主要语言的国家(“英语以外的语言”;LOTE)影响急性缺血性卒中的EVT获取和预后,同时也确定影响卒中严重程度和预后的人口统计学危险因素。方法:对2018年至2023年在悉尼利物浦医院连续接受EVT治疗的AIS患者进行回顾性队列研究。参与者根据原产国澳大利亚/新西兰/英国或LOTE进行分类。主要结局包括时间指标——从发病到到达(OTA)和到达到穿刺(ATP),以及3个月的修正Rankin评分(mRS)。统计分析包括多变量逻辑回归来评估功能结局的预测因素。结果:在911例EVT转诊中,纳入721例患者。LOTE患者(50.3%)更容易患糖尿病(30% vs 16%;结论:在接受EVT治疗的SWS患者中,LOTE患者在住院时间指标上并不处于劣势。LOTE患者3个月的功能预后较差,这表明需要针对糖尿病等危险因素采取有针对性的策略。
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引用次数: 0
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BMJ Neurology Open
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