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Estimation of initial stroke severity in hospital databases using NIHSS score from population-based stroke registries 基于人群卒中登记的NIHSS评分对医院数据库中初始卒中严重程度的估计。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2026-01-23 DOI: 10.1016/j.neurol.2025.10.005
N. Minier , V. Olié , M. Consigny , J. Coadic , E. Nowak , Y. Béjot , S. Timsit , A. Gabet

Background

Clinical severity assessed by the National Institutes of Health Stroke Scale (NIHSS) score is not available in national hospital database.

Methods

Data from the French National Health Data System (SNDS) in the region of the Brest Stroke Registry (BSR), were used to calculate a probability of death among hospitalized ischemic strokes (IS) and intracerebral hemorrhages (ICH), relying on multivariable logistic regressions using available stroke/patient characteristics. Data from the BSR, have been used to derive equations putting in relation initial NIHSS and probability of death. These equations were used to estimate initial NIHSS based on probability of death. Data from the Dijon Stroke Registry were used to assess the concordance between predicted and observed initial stroke severity levels outside of the geographical area of its training dataset.

Results

In the years 2012–2019, the BSR reported 5883 IS and 816 ICH among people aged 16 or above, while 5623 IS and 787 ICH could be identified in the SNDS. Ten-day mortality was found to be the best proxy for initial stroke severity. In the Dijon Stroke Registry, among 1,254 IS, our algorithm predicted 53.0% events of minor severity (initial NIHSS  4), 38.6% events of intermediate severity (5–20), and 8.5% events of high severity (≥ 21), compared to known prevalence of 53.0%, 38.0%, and 9.0%, respectively. No reliable predictions could be made for ICH.

Conclusion

The possible estimation of prevalence of initial stroke severity levels with satisfying performances in healthcare database is likely to improve epidemiological surveillance of this disease at national and local level.
背景:美国国立卫生研究院卒中量表(NIHSS)评估的临床严重程度在国家医院数据库中没有。方法:使用来自法国国家卫生数据系统(SNDS) Brest卒中登记处(BSR)区域的数据,依靠多变量logistic回归,利用可用的卒中/患者特征计算住院缺血性卒中(IS)和脑出血(ICH)患者的死亡概率。来自BSR的数据已被用于推导初始NIHSS与死亡概率的关系方程。这些方程用于根据死亡概率估计初始NIHSS。来自第戎卒中登记处的数据用于评估其训练数据集地理区域以外预测和观察到的初始卒中严重程度之间的一致性。结果:2012-2019年,BSR在16岁及以上人群中报告了5883例IS和816例ICH,而SNDS中可识别出5623例IS和787例ICH。十天死亡率被发现是最初中风严重程度的最佳指标。在第戎卒中登记处,在1254例IS中,我们的算法预测了53.0%的轻度严重事件(初始NIHSS≤4),38.6%的中度严重事件(5-20)和8.5%的高度严重事件(≥21),而已知患病率分别为53.0%,38.0%和9.0%。无法对非ICH作出可靠的预测。结论:在卫生保健数据库中对卒中初始严重程度的估计,可能会提高国家和地方对该病的流行病学监测水平。
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引用次数: 0
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 Epub Date: 2025-11-04 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
First-bite syndrome following triptan intake: A report of three cases 曲坦类药物摄入后的初咬综合征:附3例报告。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 Epub Date: 2025-09-29 DOI: 10.1016/j.neurol.2025.09.002
V. Fayolle , N. Moreau , S. Redon , G. Demarquay
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引用次数: 0
Diagnosis of Alzheimer's disease: Recommendations from the French Federation of Memory Clinics 阿尔茨海默病的诊断:来自法国记忆诊所联合会的建议。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-12-01 Epub Date: 2025-09-30 DOI: 10.1016/j.neurol.2025.09.004
J. Dumurgier , B. Défontaines , K. Gallouj , B. Garcin , A. Garnier-Crussard , J. Lagarde , J.-M. Pauly , A. Rollin Sillaire , I. Rouch-Leroyer , M. Sarazin , M. Verny , D. Wallon
Alzheimer's disease (AD) is the most common neurodegenerative disorder and the leading cause of major neurocognitive disorder in older adults. Its diagnosis has evolved from clinical to clinico-biological criteria, integrating biomarkers such as beta-amyloid and phosphorylated tau in cerebrospinal fluid or specific positron emission tomography (PET) imaging. Recent therapeutic advances, including anti-amyloid immunotherapies, highlight the need for early and accurate diagnosis. Clinical presentation is heterogeneous and may include amnestic or non-amnestic forms. Diagnosis should be suspected in patients with progressive cognitive decline and confirmed through neuropsychological assessment and biomarker testing. Blood-based biomarkers are promising but not yet validated for routine use. Magnetic resonance imaging (MRI) is recommended for all patients with recent cognitive decline. The role of general practitioners in early detection is critical. These recommendations, developed by the French federation of memory clinics, provide guidance on diagnosis stages, biomarker indications, first-line assessments, referral criteria, and communication of diagnosis. They aim to standardize clinical practice and support timely, individualized care.
阿尔茨海默病(AD)是最常见的神经退行性疾病,也是老年人主要神经认知障碍的主要原因。它的诊断已经从临床发展到临床生物学标准,整合了生物标志物,如脑脊液中的β -淀粉样蛋白和磷酸化tau蛋白或特定的正电子发射断层扫描(PET)成像。最近的治疗进展,包括抗淀粉样蛋白免疫疗法,强调了早期和准确诊断的必要性。临床表现是异质的,可能包括遗忘或非遗忘形式。进行性认知衰退患者应怀疑诊断,并通过神经心理学评估和生物标志物检测予以证实。基于血液的生物标记物很有前景,但尚未被证实可用于常规使用。对于近期认知能力下降的所有患者,建议使用磁共振成像(MRI)。全科医生在早期发现方面的作用至关重要。这些建议由法国记忆诊所联合会制定,为诊断阶段、生物标志物适应症、一线评估、转诊标准和诊断沟通提供指导。他们的目标是规范临床实践,支持及时、个性化的护理。
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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 Epub Date: 2025-10-15 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 Epub Date: 2025-10-25 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 Epub Date: 2025-10-15 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。
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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 Epub Date: 2025-10-17 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。
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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 Epub Date: 2025-11-06 DOI: 10.1016/j.neurol.2025.10.003
L. Lebrun , V. Binkiewicz , M. Reiff , P. Kerschen
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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 Epub Date: 2025-10-25 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}
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Revue neurologique
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