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Transient ischemic attack care pathways in stroke units: Findings from a French nationwide survey 卒中单位的短暂性脑缺血发作护理途径:来自法国全国调查的结果。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.09.008
D. Sablot , S. Rivas Lamelo , P. Renou , N. Nasr , P. Lavallee , C. Plantard , K. Blanc-Lasserre , V. Domigo , I. Sibon , Y. Béjot , C. Cordonnier , S. Alamowitch

Introduction

Transient ischemic attacks (TIAs) are associated with a high risk of early ischemic stroke. Timely and organized care is essential to prevent recurrence, as recommended by national guidelines. This survey aimed to describe current TIA management practices in French Stroke Units (SUs) and identify gaps relative to national and international recommendations.

Method

A declarative survey was sent by email to 139 French SU managers. Six reminders were sent to non-responders and partial responders. A link to the survey was also available on the French Neurovascular Society website from March 18 to November 1, 2023.

Results

A total of 123 SUs (88.5%) responded. A TIA clinic was identified in 15 SU (12% of respondents). In the other 108 SUs, no specific written procedure (63%), or a written procedure was applied at the SU (32%), and the corresponding healthcare territory (5%). The median time from admission to extra- and intra-cervical vessel imaging was 6 hours (IQR: 3–24), but in 25% of SUs, it was not provided in the first 24 hours after hospitalization. The median times to transthoracic echocardiogram and transesophageal echocardiogram were 4 days (IQR: 2–7) and 7 days (IQR: 4–14), respectively.

Conclusions

This study shows that dedicated TIA clinics are uncommon in France, but they are associated with faster diagnostic work-ups and shorter hospital stays. Expanding such structured care models within SUs could enhance the timeliness, consistency, and quality of TIA management nationwide, ultimately reducing the risk of recurrent stroke.
简介:短暂性脑缺血发作(tia)与早期缺血性卒中的高风险相关。按照国家指南的建议,及时和有组织的护理对于预防复发至关重要。本调查旨在描述法国卒中单位(SUs)当前TIA管理实践,并确定与国家和国际建议相关的差距。方法:对139名法国大学管理人员进行电子邮件调查。6个提醒发送给无反应者和部分反应者。从2023年3月18日至11月1日,该调查的链接也可以在法国神经血管学会的网站上找到。结果:123名SUs(88.5%)有应答。在15个州立大学(占应答者的12%)中确定了TIA诊所。在其他108个单位中,没有特定的书面程序(63%),或者在单位(32%)和相应的医疗保健区域(5%)应用书面程序。从入院到颈外和颈内血管成像的中位时间为6小时(IQR: 3-24),但在25%的SUs中,在住院后的前24小时没有提供。经胸超声心动图和经食管超声心动图的中位时间分别为4天(IQR: 2-7)和7天(IQR: 4-14)。结论:这项研究表明,专门的TIA诊所在法国并不常见,但它们与更快的诊断检查和更短的住院时间有关。在SUs内扩展这种结构化护理模式可以提高全国TIA管理的及时性、一致性和质量,最终降低卒中复发的风险。
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引用次数: 0
Surgical outcome of cerebral amyloid angiopathy-related cerebral hemorrhage–A multicenter comparative study 脑淀粉样血管病相关脑出血的手术疗效:多中心比较研究。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.09.006
K. Chikh , J. Burel , A. Nikiema , H. Bulteau , D. Maltete , D. Wallon , E. Gerardin , R. Aboukais , T. Gaberel , S. Derrey , L. Grangeon

Background

Surgery for lobar intracerebral hemorrhages (ICH) associated with cerebral amyloid angiopathy (CAA) is believed to carry a high risk of postoperative rebleeding. The diagnosis of CAA is increasing with an aging population and external validation of the Edinburgh criteria on computed tomography (CT) scans. The aim of this study was to assess the postoperative risk of CAA-related ICH compared to non-CAA-related ICH.

Methods

We included patients admitted between 2008 and 2022 for spontaneous lobar ICH who underwent surgery at three university hospitals. A single-blinded neuroradiologist analyzed the Edinburgh criteria on the initial CT scan before surgery and assessed rebleeding on a repeat CT scan performed within 48 hours after surgery. Patients were classified into the “CAA group” according to the Edinburgh or Boston criteria, and into the “non-CAA group” if they had another cause of ICH.

Results

A total of 140 patients were included, with 23 in the CAA group, 93 in the non-CAA group, and 24 in the undetermined group. The postoperative rebleeding rate at 24–48 hours did not differ significantly between groups (13% in the CAA group vs. 15% in the non-CAA group, P > 0.99). The overall rate of rebleeding associated with clinical deterioration did not differ between groups (9% in the CAA group vs. 6% in the non-CAA group, P = 0.66). The overall mortality rate during the acute phase did not significantly differ between groups (4% in the CAA group vs. 12% in the non-CAA group, P = 0.46). The modified Rankin scale score three months after discharge ranged from 0 to 3 for 63% of CAA patients compared to 53% of non-CAA patients, with no significant difference (P = 0.59).

Conclusion

We did not find a significant difference in the postoperative rebleeding rate after ICH associated with CAA compared to other causes.
背景:大叶性脑出血(ICH)合并脑淀粉样血管病(CAA)的手术被认为具有术后再出血的高风险。随着人口老龄化和计算机断层扫描(CT)的爱丁堡标准的外部验证,CAA的诊断正在增加。本研究的目的是评估caa相关性脑出血与非caa相关性脑出血的术后风险。方法:我们纳入了2008年至2022年间在三所大学医院接受手术的自发性大叶性脑出血患者。一名单盲神经放射学家分析了术前初次CT扫描的爱丁堡标准,并评估了术后48小时内重复CT扫描的再出血情况。根据爱丁堡或波士顿标准将患者分为“CAA组”,如果患者有其他原因导致脑出血,则分为“非CAA组”。结果:共纳入140例患者,其中CAA组23例,非CAA组93例,待定组24例。术后24-48h再出血率组间无显著差异(CAA组13% vs.非CAA组15%,P < 0.99)。与临床恶化相关的总再出血率在两组之间没有差异(CAA组为9%,非CAA组为6%,P=0.66)。急性期的总死亡率在两组间无显著差异(CAA组为4%,非CAA组为12%,P=0.46)。63%的CAA患者与53%的非CAA患者在出院后3个月的改良Rankin量表评分范围为0 ~ 3分,差异无统计学意义(P=0.59)。结论:与其他原因相比,我们没有发现与CAA相关的脑出血术后再出血率有显著差异。
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引用次数: 0
Meige syndrome, a cranio-cervical dystonia with a unique clinical phenotype 梅格综合征,颅颈肌张力障碍具有独特的临床表型
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.10.001
M.-H. Marion , J.M. Flowers , L.A. Hicklin

Background

The eponym “Meige syndrome” and the term “cranio-cervical dystonia” have been used inter-changeably in the literature and recently the validity of this eponym has been debated.

Objectives

To study the uniqueness of the term “Meige syndrome”, from a large cohort of patients with cranio-cervical dystonia.

Methods

We describe the 15 years follow-up of a large cohort of 50 patients with Meige syndrome, seen in a neurology movement disorder clinic and in a multidisciplinary neuro-ear-nose-throat (ENT) clinic. Following the original description of Henry Meige (1866–1940), we have included patients with blepharospasm (BSP) at onset and anatomical spread of dystonia to the jaw, mouth floor and neck, to describe a clinical phenotype of Meige syndrome among the cranio-cervical dystonias.

Results

We report 2 groups: group 1, including 12 patients with cranial dystonia, with BSP spreading around the nose, the mouth, the masticatory muscles with opening or closing jaw dystonia; group 2, including 38 patients with cranio-cervical dystonia, with BSP spreading further to the mouth floor muscles and to the neck including cervical and laryngeal muscles. Among them, 20/38 patients had a clinical presentation dominated by ENT sphere symptoms. In 18/38 patients, the clinical picture is dominated by an anterocollis posture, progressing in the 7 most elderly patients to an extreme posture of chin on sternum.

Conclusion

The eponym Meige syndrome should be reserved for a particular group of dystonic patients, unique in its clinical midline spasm phenotype, and in its rostro-caudal progression, starting with a BSP and resulting in a clinical spectrum with a continuum from cranial to cranio-cervical dystonia. Stormy dystonic episodes, often precipitated by stress, are important to recognise and can be a therapeutic challenge.
“Meige综合征”和“颅颈肌张力障碍”这两个词在文献中被交替使用,最近这个词的有效性一直存在争议。目的从大量颅颈肌张力障碍患者中研究“梅格综合征”一词的独特性。方法:我们对50例Meige综合征患者进行了15年的随访,这些患者分别在神经运动障碍门诊和多学科神经-耳鼻喉科(ENT)门诊就诊。根据Henry Meige(1866-1940)的原始描述,我们纳入了发病时眼睑痉挛(BSP)和肌张力障碍向下颌、口底和颈部解剖扩散的患者,以描述Meige综合征在颅颈肌张力障碍中的临床表型。结果报告2组:1组12例颅骨肌张力障碍患者,BSP在鼻、口、咀嚼肌周围扩散,伴开合颌肌张力障碍;第2组,38例颅颈肌张力障碍患者,BSP进一步扩散至口底肌和颈部,包括颈、喉肌。其中20/38的患者临床表现以耳鼻喉球症状为主。在18/38的患者中,临床表现以前结肠姿势为主,在7例老年患者中进展到下巴在胸骨上的极端姿势。结论Meige综合征应该保留给一组特殊的肌张力障碍患者,其独特的临床中线痉挛表型和其前-尾侧进展,从BSP开始,导致从颅肌到颅颈肌张力障碍的连续临床谱。风暴性张力障碍发作通常是由压力引起的,认识到这一点很重要,也可能是治疗上的挑战。
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引用次数: 0
Disease modifying treatment of radiologically isolated syndrome: A systematic review of the use, efficacy, effectiveness, and safety 放射孤立综合征的疾病改善治疗:使用、疗效、有效性和安全性的系统回顾。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.09.005
D. Ripsman , H. Tremlett , A. Alzahrani , N Makhani

Background

Radiologically isolated syndrome (RIS) is characterized by incidental brain lesions suggestive of demyelination without symptoms of multiple sclerosis (MS). We systematically assessed the use, benefits, and adverse effects of DMTs for RIS.

Methods

MEDLINE, EMBASE, and Web of Science were searched to identify English language studies including individuals with RIS treated with a DMT. Extracted data included patient characteristics, clinical progression, and adverse events. We conducted a meta-analysis using inverse probability weighting. Risk of bias (RoB) assessments used Cochrane's RoB-2 tool and Newcastle-Ottawa cohort study scale.

Results

A total of 1012 abstracts were screened: 20 studies were included consisting of 2 RCTs, 12 observational cohort studies, and 6 case reports. A total of 1401 individuals with RIS were included; 291 (21%) received a DMT. The two RCTs randomized people with RIS to teriflunomide or dimethyl fumarate versus placebo and followed patients for at least 96 weeks. In all other studies, follow-up ranged from 2 months to 18 years; only 3 studies exceeded 5 years. DMT treatment was associated with a lower risk of a clinical demyelinating event (4 studies with different DMTs, adjusted hazard ratio = 0.37 95% confidence interval [CI]: 0.15–0.95, high certainty). There was a higher rate of adverse events in DMT treated patients with RIS versus placebo (risk ratio = 1.44, 95% CI: 1.09–1.90, moderate certainty). RoB was low for both RCTs, but high for 83% (10/12) of cohort studies.

Conclusions

DMTs reduced the risk of a clinical demyelinating event in individuals with RIS, albeit with more adverse events compared to placebo. However, no literature addressed longer-term benefits/adverse effects.
背景:放射学孤立综合征(RIS)的特征是偶然的脑损伤提示脱髓鞘,没有多发性硬化症(MS)的症状。我们系统地评估了dmt治疗RIS的使用、益处和不良反应。方法:检索MEDLINE、EMBASE和Web of Science以确定英语语言研究,包括接受DMT治疗的RIS患者。提取的数据包括患者特征、临床进展和不良事件。我们使用逆概率加权进行了荟萃分析。偏倚风险(RoB)评估采用Cochrane的RoB-2工具和Newcastle-Ottawa队列研究量表。结果:共筛选1012篇摘要,纳入20项研究,包括2项随机对照试验、12项观察性队列研究和6例病例报告。共纳入1401例RIS患者;291例(21%)接受了DMT。这两项随机对照试验将RIS患者随机分为特立氟米特或富马酸二甲酯与安慰剂,并对患者进行至少96周的随访。在所有其他研究中,随访时间从2个月到18年不等;只有3项研究超过了5年。DMT治疗与临床脱髓鞘事件的风险较低相关(4项采用不同DMT治疗的研究,校正风险比=0.37 95%可信区间[CI]: 0.15-0.95,高确定性)。与安慰剂相比,接受DMT治疗的RIS患者的不良事件发生率更高(风险比=1.44,95% CI: 1.09-1.90,中等确定性)。两项随机对照试验的RoB都较低,但83%(10/12)的队列研究的RoB较高。结论:dmt降低了RIS患者临床脱髓鞘事件的风险,尽管与安慰剂相比有更多的不良事件。然而,没有文献涉及长期益处/副作用。
{"title":"Disease modifying treatment of radiologically isolated syndrome: A systematic review of the use, efficacy, effectiveness, and safety","authors":"D. Ripsman ,&nbsp;H. Tremlett ,&nbsp;A. Alzahrani ,&nbsp;N Makhani","doi":"10.1016/j.neurol.2025.09.005","DOIUrl":"10.1016/j.neurol.2025.09.005","url":null,"abstract":"<div><h3>Background</h3><div>Radiologically isolated syndrome (RIS) is characterized by incidental brain lesions suggestive of demyelination without symptoms of multiple sclerosis (MS). We systematically assessed the use, benefits, and adverse effects of DMTs for RIS.</div></div><div><h3>Methods</h3><div>MEDLINE, EMBASE, and Web of Science were searched to identify English language studies including individuals with RIS treated with a DMT. Extracted data included patient characteristics, clinical progression, and adverse events. We conducted a meta-analysis using inverse probability weighting. Risk of bias (RoB) assessments used Cochrane's RoB-2 tool and Newcastle-Ottawa cohort study scale.</div></div><div><h3>Results</h3><div>A total of 1012 abstracts were screened: 20 studies were included consisting of 2 RCTs, 12 observational cohort studies, and 6 case reports. A total of 1401 individuals with RIS were included; 291 (21%) received a DMT. The two RCTs randomized people with RIS to teriflunomide or dimethyl fumarate versus placebo and followed patients for at least 96<!--> <!-->weeks. In all other studies, follow-up ranged from 2<!--> <!-->months to 18<!--> <!-->years; only 3 studies exceeded 5<!--> <!-->years. DMT treatment was associated with a lower risk of a clinical demyelinating event (4 studies with different DMTs, adjusted hazard ratio<!--> <!-->=<!--> <!-->0.37 95% confidence interval [CI]: 0.15–0.95, high certainty). There was a higher rate of adverse events in DMT treated patients with RIS versus placebo (risk ratio<!--> <!-->=<!--> <!-->1.44, 95% CI: 1.09–1.90, moderate certainty). RoB was low for both RCTs, but high for 83% (10/12) of cohort studies.</div></div><div><h3>Conclusions</h3><div>DMTs reduced the risk of a clinical demyelinating event in individuals with RIS, albeit with more adverse events compared to placebo. However, no literature addressed longer-term benefits/adverse effects.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 955-969"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308956","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Neural correlates of foreign accent syndrome: Is white matter the key to the mystery? 外国口音综合征的神经关联:白质是解开谜团的关键吗?
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.08.003
G. Costentin , L. Deheinzelin , L. Zourdani , O. Ozkul , A. Triquenot , E. Massardier , D. Maltete , D. Wallon , A. Morin

Background

Foreign accent syndrome is a rare entity with neural underpinnings that are not yet fully understood. Cases of neurogenic foreign accent syndrome due to right hemisphere lesion are very uncommon. We investigated the cortical and white matter lesions in a patient to better understand this syndrome.

Case report

A 39-year-old French woman had a right middle cerebral artery stroke. This French native speaker was now perceived as speaking with an English accent, without signs of dysarthria or aphasia. Lesions involved right insula, pre-central gyrus and surrounding white matter tracts. After transformation in MNI-152 coordinate system, we compared the lesions in our patient with Tractotron software, a white matter tract tool providing a probability of fiber disconnection.

Discussion

Speech evaluation suggested a disruption in the speech output motor system. We found lesions within this system, in anterior frontal and insular cortices, in accordance with previous descriptions. Besides structural grey matter damage, white matter also exhibited damage related to the stroke, particularly the frontal aslant tract. Very few studies have focused on the specific role of this tract in speech motor programming, but a role in speech production and stuttering have been described. Some cases of foreign accent syndrome appeared consecutively to right hemisphere lesions. There is growing evidence suggesting that right hemisphere lesions might lead to subclinical alteration of prosody contributing to the emergence of foreign accent syndrome.

Conclusion

This study emphasizes the importance of analyzing subcortical areas and their associated tracts, which could play a crucial role in better understanding symptoms and anatomical correlation.
背景:外国口音综合征是一种罕见的实体,其神经基础尚未完全了解。右半球病变引起的神经源性外国口音综合征是非常罕见的。我们研究了患者的皮质和白质病变,以更好地了解这种综合征。病例报告:一名39岁的法国女性右脑中动脉中风。这位以法语为母语的人现在被认为说话带有英语口音,没有构音障碍或失语症的迹象。病变包括右脑岛、中央前回和周围白质束。在MNI-152坐标系下转换后,我们将患者的病变与Tractotron软件进行比较,这是一种提供纤维断开概率的白质束工具。讨论:语音评估提示语音输出运动系统的中断。我们在这个系统中发现了病变,在前额叶和岛叶皮层,与之前的描述一致。除了结构性灰质损伤外,白质也表现出与中风有关的损伤,尤其是额斜束。很少有研究关注这一通道在言语运动编程中的具体作用,但在言语产生和口吃中的作用已被描述。部分病例伴右半球病变出现外国口音综合征。越来越多的证据表明,右半球病变可能导致亚临床的韵律改变,从而导致外国口音综合征的出现。结论:本研究强调了分析皮质下区及其相关束的重要性,这对更好地理解症状和解剖相关性具有重要作用。
{"title":"Neural correlates of foreign accent syndrome: Is white matter the key to the mystery?","authors":"G. Costentin ,&nbsp;L. Deheinzelin ,&nbsp;L. Zourdani ,&nbsp;O. Ozkul ,&nbsp;A. Triquenot ,&nbsp;E. Massardier ,&nbsp;D. Maltete ,&nbsp;D. Wallon ,&nbsp;A. Morin","doi":"10.1016/j.neurol.2025.08.003","DOIUrl":"10.1016/j.neurol.2025.08.003","url":null,"abstract":"<div><h3>Background</h3><div>Foreign accent syndrome is a rare entity with neural underpinnings that are not yet fully understood. Cases of neurogenic foreign accent syndrome due to right hemisphere lesion are very uncommon. We investigated the cortical and white matter lesions in a patient to better understand this syndrome.</div></div><div><h3>Case report</h3><div>A 39-year-old French woman had a right middle cerebral artery stroke. This French native speaker was now perceived as speaking with an English accent, without signs of dysarthria or aphasia. Lesions involved right insula, pre-central gyrus and surrounding white matter tracts. After transformation in MNI-152 coordinate system, we compared the lesions in our patient with Tractotron software, a white matter tract tool providing a probability of fiber disconnection.</div></div><div><h3>Discussion</h3><div>Speech evaluation suggested a disruption in the speech output motor system. We found lesions within this system, in anterior frontal and insular cortices, in accordance with previous descriptions. Besides structural grey matter damage, white matter also exhibited damage related to the stroke, particularly the frontal aslant tract. Very few studies have focused on the specific role of this tract in speech motor programming, but a role in speech production and stuttering have been described. Some cases of foreign accent syndrome appeared consecutively to right hemisphere lesions. There is growing evidence suggesting that right hemisphere lesions might lead to subclinical alteration of prosody contributing to the emergence of foreign accent syndrome.</div></div><div><h3>Conclusion</h3><div>This study emphasizes the importance of analyzing subcortical areas and their associated tracts, which could play a crucial role in better understanding symptoms and anatomical correlation.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 970-974"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145318490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acute Hippocampal encephalopathy in heavy cannabis user: An emerging syndrome? 重度大麻使用者的急性海马脑病:一种新出现的综合征?
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.10.003
L. Lebrun , V. Binkiewicz , M. Reiff , P. Kerschen
{"title":"Acute Hippocampal encephalopathy in heavy cannabis user: An emerging syndrome?","authors":"L. Lebrun ,&nbsp;V. Binkiewicz ,&nbsp;M. Reiff ,&nbsp;P. Kerschen","doi":"10.1016/j.neurol.2025.10.003","DOIUrl":"10.1016/j.neurol.2025.10.003","url":null,"abstract":"","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 1031-1033"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145471815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Short-term follow-up of anti-MAG neuropathy patients given bendamustine plus rituximab combination therapy 苯达莫司汀联合利妥昔单抗治疗抗mag神经病变患者的短期随访
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.09.009
N. Bezou , P. Morel , B. Hivert , E. Diab , P. Merle , C. Tard

Introduction

Anti-myelin-associated glycoprotein (anti-MAG) IgM antibodies provoke demyelinating neuropathies, which can be particularly disabling. Conventionally, patients with anti-MAG neuropathies were treated with rituximab alone. This study aimed to evaluate the clinical and electrophysiological efficacy of bendamustine-rituximab (BR) combination therapy for anti-MAG neuropathy patients.

Methods

We retrospectively consulted the medical records of 11 patients whose diagnosis of anti-MAG neuropathy was retained and who were given BR combination therapy at university and community hospitals in northern France between 2017 and 2022. We collected clinical scores and electrophysiological data before BR therapy and at six to eleven months after treatment end (mean = 8.40 months).

Results

After BR therapy, six of eleven patients (54%) showed an improvement in their overall neuropathy limitations scale (ONLS) score as did five of eleven (45%) in their peripheral neuropathy disability (PND) score, but these results did not reach statistical significance. For the six patients who had a more complete clinical revaluation based on the neuropathy impairment score (NIS), five improved (P = 0.021). An improvement in the sensory NIS was observed in three of six patients (50%), while the motor NIS remained stable. Patients improving their PND score seemed to start from a higher baseline value. Five of eight (62%) patients with electrodiagnostic control data met Lunn and Nobile-Orazio's electrophysiological improvement criteria. The higher the baseline PND score, the less likely was improvement of electrodiagnostic parameters. No link between duration of disease progression before treatment and clinical response was found.

Conclusion

This multicenter cohort of anti-MAG patients given BR combination therapy is, to our knowledge, the largest published to date. Although BR therapy seems to improve the clinical and electrophysiological outcome of anti-MAG patients, precise evaluation scales are nevertheless needed to highlight improvement. Sensitive scales such as the NIS should be used routinely for monitoring anti-MAG patients. Prospective studies with a larger number of patients would be useful to confirm the efficacy of BR therapy in these patients.
抗髓鞘相关糖蛋白(抗mag) IgM抗体可引起脱髓鞘性神经病变,这可能特别致残。传统上,抗mag神经病变患者单独使用利妥昔单抗治疗。本研究旨在评价苯达莫司汀-利妥昔单抗(BR)联合治疗抗mag神经病变患者的临床和电生理疗效。方法回顾性查阅2017年至2022年在法国北部大学和社区医院接受BR联合治疗的11例抗mag神经病变患者的病历。我们收集了BR治疗前和治疗结束后6 - 11个月(平均8.40个月)的临床评分和电生理数据。结果BR治疗后,11例患者中有6例(54%)的总神经病变限制量表(ONLS)评分改善,11例患者中有5例(45%)的周围神经病变功能障碍(PND)评分改善,但这些结果均无统计学意义。在基于神经病变损害评分(NIS)进行更完整临床重估的6例患者中,5例患者改善(P = 0.021)。6例患者中有3例(50%)感觉NIS得到改善,而运动NIS保持稳定。改善PND评分的患者似乎从较高的基线值开始。有电诊断对照数据的8例患者中有5例(62%)符合Lunn和Nobile-Orazio的电生理改善标准。基线PND评分越高,电诊断参数改善的可能性越小。未发现治疗前疾病进展时间与临床反应之间的联系。据我们所知,这项针对接受BR联合治疗的抗mag患者的多中心队列研究是迄今为止发表的规模最大的研究。尽管BR治疗似乎改善了抗mag患者的临床和电生理结果,但仍需要精确的评估量表来突出改善。应常规使用NIS等敏感量表监测抗mag患者。更多患者的前瞻性研究将有助于确认BR治疗对这些患者的疗效。
{"title":"Short-term follow-up of anti-MAG neuropathy patients given bendamustine plus rituximab combination therapy","authors":"N. Bezou ,&nbsp;P. Morel ,&nbsp;B. Hivert ,&nbsp;E. Diab ,&nbsp;P. Merle ,&nbsp;C. Tard","doi":"10.1016/j.neurol.2025.09.009","DOIUrl":"10.1016/j.neurol.2025.09.009","url":null,"abstract":"<div><h3>Introduction</h3><div>Anti-myelin-associated glycoprotein (anti-MAG) IgM antibodies provoke demyelinating neuropathies, which can be particularly disabling. Conventionally, patients with anti-MAG neuropathies were treated with rituximab alone. This study aimed to evaluate the clinical and electrophysiological efficacy of bendamustine-rituximab (BR) combination therapy for anti-MAG neuropathy patients.</div></div><div><h3>Methods</h3><div>We retrospectively consulted the medical records of 11 patients whose diagnosis of anti-MAG neuropathy was retained and who were given BR combination therapy at university and community hospitals in northern France between 2017 and 2022. We collected clinical scores and electrophysiological data before BR therapy and at six to eleven months after treatment end (mean<!--> <!-->=<!--> <!-->8.40<!--> <!-->months).</div></div><div><h3>Results</h3><div>After BR therapy, six of eleven patients (54%) showed an improvement in their overall neuropathy limitations scale (ONLS) score as did five of eleven (45%) in their peripheral neuropathy disability (PND) score, but these results did not reach statistical significance. For the six patients who had a more complete clinical revaluation based on the neuropathy impairment score (NIS), five improved (<em>P</em> <!-->=<!--> <!-->0.021). An improvement in the sensory NIS was observed in three of six patients (50%), while the motor NIS remained stable. Patients improving their PND score seemed to start from a higher baseline value. Five of eight (62%) patients with electrodiagnostic control data met <em>Lunn and Nobile-Orazio's</em> electrophysiological improvement criteria. The higher the baseline PND score, the less likely was improvement of electrodiagnostic parameters. No link between duration of disease progression before treatment and clinical response was found.</div></div><div><h3>Conclusion</h3><div>This multicenter cohort of anti-MAG patients given BR combination therapy is, to our knowledge, the largest published to date. Although BR therapy seems to improve the clinical and electrophysiological outcome of anti-MAG patients, precise evaluation scales are nevertheless needed to highlight improvement. Sensitive scales such as the NIS should be used routinely for monitoring anti-MAG patients. Prospective studies with a larger number of patients would be useful to confirm the efficacy of BR therapy in these patients.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 1008-1014"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145712268","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Predictive factors of epilepsy in a cohort of brain arteriovenous malformation patients with a 5-year follow-up 脑动静脉畸形患者5年随访中癫痫的预测因素。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.09.007
B.O. Pirlog , M. Porché , M. Kyheng , J. Labreuche , A. Taleb , E. Dubus , E. Houdart , M. Mazighi

Background and aims

Data on factors associated with new-onset epilepsy in the follow-up of patients with brain arteriovenous malformations (BAVM) are scarce. We aimed to characterize the baseline patient and BAVM characteristics related to epilepsy and the predictive factors of new-onset epilepsy in BAVM patients during their follow-up.

Patients and methods

We retrospectively analyzed 200 BAVM patients treated between 2000 and 2023. We used univariate and multivariate analyses to assess the association between baseline characteristics and seizures. The risk of developing seizures during follow-up was estimated using nonparametric survival analysis for interval-censored data.

Results

In the present cohort, 90/200 patients (45%) had epilepsy at baseline. Patients with epilepsy were predominantly men (71.1%), and alcohol users (18.8%), and presented with BAVM located more often in the frontal lobe (55.6%) as compared to patients without epilepsy (40%, 7.4%, 23.6% respectively). Male gender was associated with higher prevalence of seizures at inclusion (OR 3.81 [95%CI, 1.77; 8.24]), while headaches (OR 0.19 [95% CI, 0.09; 0.41]), focal deficit (OR 0.22 [0.09; 0.49]) and BAVM occipital localization (OR 0.16 [95% CI, 0.05; 0.47]) remained associated with lower prevalence of epilepsy at inclusion. Among patients without epilepsy at baseline, 20.6% had new-onset epilepsy after five years. Baseline predictors of seizure occurrence included ruptured BAVM (HR 3.77 [95%CI 1.50; 9.44]) and surgery (HR 7.75, 95%CI, [2.04; 29.45]).

Conclusions

For patients with newly diagnosed BAVM, male gender, and frontal topography were baseline characteristics associated with a higher risk of epilepsy. Among patients without epilepsy, ruptured BAVM and surgery at baseline were predictors of epilepsy at five years follow-up.
背景与目的:脑动静脉畸形(BAVM)患者随访中与新发癫痫相关因素的数据很少。我们的目的是在随访期间描述基线患者和与癫痫相关的BAVM特征以及BAVM患者新发癫痫的预测因素。患者和方法:我们回顾性分析了2000年至2023年间治疗的200例BAVM患者。我们使用单变量和多变量分析来评估基线特征与癫痫发作之间的关系。随访期间癫痫发作的风险使用间隔剔除数据的非参数生存分析进行估计。结果:在本队列中,90/200例患者(45%)在基线时患有癫痫。癫痫患者主要为男性(71.1%)和酗酒者(18.8%),与非癫痫患者(分别为40%、7.4%和23.6%)相比,BAVM更常位于额叶(55.6%)。男性与纳入时较高的癫痫患病率相关(OR 3.81 [95%CI, 1.77; 8.24]),而头痛(OR 0.19 [95%CI, 0.09; 0.41])、局灶性缺陷(OR 0.22[0.09; 0.49])和BAVM枕部定位(OR 0.16 [95%CI, 0.05; 0.47])仍与纳入时较低的癫痫患病率相关。在基线时无癫痫的患者中,20.6%在5年后新发癫痫。癫痫发作的基线预测因素包括脑脊髓炎破裂(HR 3.77 [95%CI 1.50; 9.44])和手术(HR 7.75, 95%CI[2.04; 29.45])。结论:对于新诊断的BAVM患者,男性性别和额叶地形是与癫痫高风险相关的基线特征。在没有癫痫的患者中,bam破裂和基线手术是5年随访时癫痫的预测因素。
{"title":"Predictive factors of epilepsy in a cohort of brain arteriovenous malformation patients with a 5-year follow-up","authors":"B.O. Pirlog ,&nbsp;M. Porché ,&nbsp;M. Kyheng ,&nbsp;J. Labreuche ,&nbsp;A. Taleb ,&nbsp;E. Dubus ,&nbsp;E. Houdart ,&nbsp;M. Mazighi","doi":"10.1016/j.neurol.2025.09.007","DOIUrl":"10.1016/j.neurol.2025.09.007","url":null,"abstract":"<div><h3>Background and aims</h3><div>Data on factors associated with new-onset epilepsy in the follow-up of patients with brain arteriovenous malformations (BAVM) are scarce. We aimed to characterize the baseline patient and BAVM characteristics related to epilepsy and the predictive factors of new-onset epilepsy in BAVM patients during their follow-up.</div></div><div><h3>Patients and methods</h3><div>We retrospectively analyzed 200 BAVM patients treated between 2000 and 2023. We used univariate and multivariate analyses to assess the association between baseline characteristics and seizures. The risk of developing seizures during follow-up was estimated using nonparametric survival analysis for interval-censored data.</div></div><div><h3>Results</h3><div>In the present cohort, 90/200 patients (45%) had epilepsy at baseline. Patients with epilepsy were predominantly men (71.1%), and alcohol users (18.8%), and presented with BAVM located more often in the frontal lobe (55.6%) as compared to patients without epilepsy (40%, 7.4%, 23.6% respectively). Male gender was associated with higher prevalence of seizures at inclusion (OR 3.81 [95%CI, 1.77; 8.24]), while headaches (OR 0.19 [95% CI, 0.09; 0.41]), focal deficit (OR 0.22 [0.09; 0.49]) and BAVM occipital localization (OR 0.16 [95% CI, 0.05; 0.47]) remained associated with lower prevalence of epilepsy at inclusion. Among patients without epilepsy at baseline, 20.6% had new-onset epilepsy after five years. Baseline predictors of seizure occurrence included ruptured BAVM (HR 3.77 [95%CI 1.50; 9.44]) and surgery (HR 7.75, 95%CI, [2.04; 29.45]).</div></div><div><h3>Conclusions</h3><div>For patients with newly diagnosed BAVM, male gender, and frontal topography were baseline characteristics associated with a higher risk of epilepsy. Among patients without epilepsy, ruptured BAVM and surgery at baseline were predictors of epilepsy at five years follow-up.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 991-997"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313491","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Lecanemab in France: The Times They Are a-Changin’? 法国的勒卡内马:他们正在改变的时代?
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.10.002
N. Villain , V. Planche , A. Garnier-Crussard , D. Wallon
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引用次数: 0
Chronic meningoencephalitis due to enterovirus A71 complicating rituximab therapy 肠病毒A71引起的慢性脑膜脑炎合并利妥昔单抗治疗。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.neurol.2025.09.003
A. David , M. Claudé , G. Martin de Frémont , G. Trédez , C. Henry

Background

Rituximab and other anti-CD20 therapies are increasingly used in the treatment of autoimmune and hematologic disorders. These treatments are associated with persistent immune impairment, potentially leading to severe infections. We describe a resolutive case of proven chronic enterovirus A71 (EV A71) meningoencephalitis complicating rituximab maintenance therapy for non-Hodgkin lymphoma.

Methods

This article combines an original case report and a literature review of cases of enteroviral meningoencephalitis complicating rituximab treatment.

Results

A 38-year-old man was treated with rituximab and chemotherapy for a mantle cell lymphoma. During maintenance treatment with rituximab, he developed a “hand-foot-mouth disease”, and one month later severe neurological deterioration including quadriparesis and major neurocognitive disorder leading to a diagnosis of chronic enteroviral meningoencephalitis. A treatment associating monthly intravenous immunoglobulins (IVIg) and fluoxetine was initiated two months after neurological symptoms onset, resulting in dramatic clinical improvement within six months. A brief literature review shows that a treatment with high-dose IVIg often results in clinical improvement. Fluoxetine was added in recent reports based on in vitro evidence of anti-viral activity against enteroviruses.

Discussion

Enteroviral infection should be evoked in patients treated with rituximab presenting with an encephalitic symptomatology, and restoring humoral immunity with high-dose IVIg might improve their condition.
背景:利妥昔单抗和其他抗cd20疗法越来越多地用于自身免疫性疾病和血液系统疾病的治疗。这些治疗与持续的免疫损伤有关,可能导致严重的感染。我们描述了一个确诊的慢性肠病毒A71 (EV A71)脑膜脑炎合并利妥昔单抗维持治疗非霍奇金淋巴瘤的决定性病例。方法:本文结合一例原始病例报告和文献复习肠病毒性脑膜脑炎合并利妥昔单抗治疗的病例。结果:一名38岁的男性接受了利妥昔单抗和化疗治疗套细胞淋巴瘤。在利妥昔单抗维持治疗期间,他出现了“手足口病”,一个月后,严重的神经系统恶化,包括四肢瘫和严重的神经认知障碍,导致诊断为慢性肠病毒脑膜脑炎。在神经系统症状出现两个月后开始每月静脉注射免疫球蛋白(IVIg)和氟西汀治疗,6个月内临床显著改善。一篇简短的文献综述显示,高剂量IVIg治疗通常会导致临床改善。根据对肠道病毒的体外抗病毒活性证据,在最近的报告中添加了氟西汀。讨论:在接受利妥昔单抗治疗的患者出现脑病症状时应引起肠病毒感染,使用大剂量IVIg恢复体液免疫可能改善其病情。
{"title":"Chronic meningoencephalitis due to enterovirus A71 complicating rituximab therapy","authors":"A. David ,&nbsp;M. Claudé ,&nbsp;G. Martin de Frémont ,&nbsp;G. Trédez ,&nbsp;C. Henry","doi":"10.1016/j.neurol.2025.09.003","DOIUrl":"10.1016/j.neurol.2025.09.003","url":null,"abstract":"<div><h3>Background</h3><div>Rituximab and other anti-CD20 therapies are increasingly used in the treatment of autoimmune and hematologic disorders. These treatments are associated with persistent immune impairment, potentially leading to severe infections. We describe a resolutive case of proven chronic enterovirus A71 (EV A71) meningoencephalitis complicating rituximab maintenance therapy for non-Hodgkin lymphoma.</div></div><div><h3>Methods</h3><div>This article combines an original case report and a literature review of cases of enteroviral meningoencephalitis complicating rituximab treatment.</div></div><div><h3>Results</h3><div>A 38-year-old man was treated with rituximab and chemotherapy for a mantle cell lymphoma. During maintenance treatment with rituximab, he developed a “hand-foot-mouth disease”, and one month later severe neurological deterioration including quadriparesis and major neurocognitive disorder leading to a diagnosis of chronic enteroviral meningoencephalitis. A treatment associating monthly intravenous immunoglobulins (IVIg) and fluoxetine was initiated two months after neurological symptoms onset, resulting in dramatic clinical improvement within six months. A brief literature review shows that a treatment with high-dose IVIg often results in clinical improvement. Fluoxetine was added in recent reports based on in vitro evidence of anti-viral activity against enteroviruses.</div></div><div><h3>Discussion</h3><div>Enteroviral infection should be evoked in patients treated with rituximab presenting with an encephalitic symptomatology, and restoring humoral immunity with high-dose IVIg might improve their condition.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 975-980"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200877","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Revue neurologique
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