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Setting benchmark for ischemic stroke treated endovascularly: A systematic review and meta-analysis 设定缺血性脑卒中血管内治疗的基准:一项系统回顾和荟萃分析。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-02-05 DOI: 10.1016/j.neurol.2025.12.009
G. De Rubeis , S. Fabiano , L. Bertaccini , M. Mangiardi , F.R. Pezzella , S. Anticoli , L. Saba , E. Pampana

Objective

To develop standardized benchmarks for evaluating endovascular treatment of ischemic stroke by analyzing treatment efficacy and safety. This allows the comparison of new techniques and devices with solid standards and quality control.

Materials and methods

We conducted a systematic review and meta-analysis of randomized controlled trials from the MEDLINE, OVID, and Cochrane databases from January 2015 (MRCLEAN trial published date) to June 2025, focusing on predefined clinical outcomes. After applying the inclusion and exclusion criteria, 35 studies were included from the initial search of 1949 (1.8%). The benchmarks selected were modified treatment for ischemic cerebral infarction (mTICI)  2b, modified Rankin Scale (mRS)  2, symptomatic hemorrhagic transformation rate, and death rate. According to concept of confidence of interval (CI) 95%, everything that falls outside the border is defined as statistically significant.

Results

The I2 rages from 61.6% to 93.86%. The mTICI  2b rate was 75.5% (95% CI: 75.7 to 81.5), the mRS  2 was 43.5% (95% CI: 40.0 to 47.2), with a symptomatic hemorrhagic transformation rate of 5.4% (95% CI: 4.5 to 6.4), and a death rate of 18.4% (95% CI: 16.2 to 20.8) at three months. Subgroup analysis suggest trend in favour of higher mTICI  2b rate intra-arterial thrombolysis + MT vs. MT. The mRS  2 rate was lower in the large core trial and higher in the intra-arterial thrombolysis + MT.

Conclusion

The derived benchmarks served as reference standards for assessing new endovascular treatments and quality control. This methodology enhances the quality of evidence, aids in clinical decision-making, and fosters advancements in treatment technologies.
目的:通过分析缺血性脑卒中血管内治疗的疗效和安全性,建立评价血管内治疗的标准化标准。这使得新技术和设备与坚实的标准和质量控制的比较。材料和方法:我们对MEDLINE、OVID和Cochrane数据库中2015年1月(MRCLEAN试验发布日期)至2025年6月的随机对照试验进行了系统回顾和荟萃分析,重点关注预定的临床结果。应用纳入和排除标准后,从1949年的初始检索中纳入了35项研究(1.8%)。选择的基准是缺血性脑梗死改良治疗(mTICI)≥2b、改良Rankin量表(mRS)≤2、症状性出血转化率和死亡率。根据区间置信区间(CI) 95%的概念,所有落在边界之外的东西都被定义为统计显著。结果:I2在61.6% ~ 93.86%之间。mTICI≥2b率为75.5% (95% CI: 75.7 ~ 81.5), mRS≤2率为43.5% (95% CI: 40.0 ~ 47.2), 3个月时症状性出血转化率为5.4% (95% CI: 4.5 ~ 6.4),死亡率为18.4% (95% CI: 16.2 ~ 20.8)。亚组分析显示动脉溶栓+MT组的mTICI≥2b率高于MT组。大型核心试验中mRS≤2率较低,动脉溶栓+MT组较高。结论:导出的标准可作为评价新型血管内治疗方法和质量控制的参考标准。这种方法提高了证据的质量,有助于临床决策,并促进了治疗技术的进步。
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引用次数: 0
Long-interval dosing of anti-CD20 therapy: Toward affordable treatment for multiple sclerosis 抗cd20治疗的长时间间隔剂量:迈向可负担的多发性硬化症治疗。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-01-16 DOI: 10.1016/j.neurol.2025.12.004
C. Boutiere , R. Saunier , A. Rico , M. Perriguey , S. Demortiere , F. Hilezian , A. Maarouf , M. Roudot , J. Pelletier , B. Audoin
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引用次数: 0
Reliability and validity of the Vietnamese version of the Montreal Cognitive Assessment in Parkinson's disease 越南版蒙特利尔认知评估帕金森病的信度和效度。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-02-03 DOI: 10.1016/j.neurol.2026.01.003
H.T. Tran , M.B.T. Huynh

Background and objectives

The Montreal Cognitive Assessment has been recommended by the Movement Disorder Society (MDS) for screening cognitive disorders in Parkinson's disease (PD), although validations of language variants are required. This study aimed to determine the reliability and validity of the Vietnamese Montreal Cognitive Assessment (MoCA-V) in Vietnamese PD patients.

Methods

PD patients from a movement disorder clinic at a tertiary hospital in Vietnam were recruited. Participants underwent the MoCA-V and Mini-Mental State Examination (MMSE) assessments. Patients were classified into three cognitive groups: dementia, mild cognitive impairment (MCI), and normal cognition (NC), based on the MDS diagnostic criteria. The reliability of the MoCA-V was determined through internal consistency and test-retest reliability. Validity was assessed through concurrent validity, convergent validity, and criterion validity. Exploratory analysis of diagnostic accuracy was performed as a secondary objective.

Results

Among 40 PD patients (dementia: 37.5%, MCI: 52.5%, NC: 10.0%), the MoCA-V demonstrated good internal consistency (Cronbach's alpha = 0.83; 95% confidence interval [0.74–0.90]) and high test-retest reliability (total score's intraclass correlation coefficient = 0.84 [0.69–0.92]; P < 0.001). The total score showed strong correlations with the patient's cognitive status (Kendall's tau-b = 0.63; P < 0.001) and the MMSE total score (Spearman's rho = 0.86; P < 0.001), indicating high concurrent and convergent validity. The scale's criterion validity in discriminating cognitive impairment (including dementia and MCI) from NC and dementia from non-dementia was high (area under the curve [AUC] = 0.98) and moderate (AUC = 0.88), respectively. Notably, the MoCA-V demonstrated superior sensitivity compared to the MMSE in differentiating cognitive impairment from NC.

Conclusions

The MoCA-V is a reliable and valid instrument for cognitive assessment in Vietnamese PD patients across all PD stages, with superior sensitivity to the MMSE for detection of cognitive impairment.
背景和目的:蒙特利尔认知评估已被运动障碍协会(MDS)推荐用于筛查帕金森病(PD)的认知障碍,尽管需要对语言变异进行验证。本研究旨在确定越南蒙特利尔认知评估(MoCA-V)在越南PD患者中的信度和效度。方法:选取越南某三级医院运动障碍门诊PD患者为研究对象。参与者接受MoCA-V和简易精神状态检查(MMSE)评估。根据MDS的诊断标准,将患者分为痴呆、轻度认知障碍(MCI)和正常认知(NC)三组。通过内部一致性和重测信度来确定MoCA-V的信度。效度通过并发效度、收敛效度和标准效度来评估。诊断准确性的探索性分析作为次要目标进行。结果:在40例PD患者中(痴呆:37.5%,MCI: 52.5%, NC: 10.0%), MoCA-V表现出良好的内部一致性(Cronbach's alpha=0.83; 95%可信区间[0.74-0.90])和较高的重测信度(总分的类内相关系数=0.84[0.69-0.92])。结论:MoCA-V是越南PD患者各阶段认知评估的可靠有效工具,对MMSE检测认知障碍具有较高的敏感性。
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引用次数: 0
Deep brain stimulation in patients with mixed movement disorders linked to ADCY5 ADCY5相关混合性运动障碍患者的深部脑刺激
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2025-12-26 DOI: 10.1016/j.neurol.2025.12.003
E. Retailleau , N. Dorison , G. Poulen , A. Roubertie , O. Trouillard , J. Baik , E. Conabady , M.-C. François-Heude , E. Chauvet-Piat , M.-A. Spitz , C. Ravelli , P. Vayssière , C. Nilles , C. Desjardins , C. Dubacq , E. Roze

Introduction

Pathogenic variants in ADCY5 cause mixed hyperkinetic movement disorders (MxMD-ADCY5) that can be occasionally refractory to medical treatment. While deep brain stimulation of the globus pallidus internus (GPi-DBS) has been previously used, knowledge on its indication, efficacy and safety is poor and mainly based on anecdotal reports and short case series.

Methods

We retrospectively reviewed clinical, genetic, therapeutic, and surgical data from patients with ADCY5-related movement disorders who underwent GPi-DBS, operated in two French expert centres or previously published.

Results

We obtained data from 23 patients for analysis. There were two distinct indications for GPi-DBS. The first group consisted of patients with long-standing, pharmacoresistant hyperkinetic movements. The second group included patients with acute motor exacerbations – fulfilling criteria for status dystonicus – and conceptually aligned with the recently proposed framework of severe acute motor exacerbation (SAME). Overall, GPi-DBS was safe and led to mild-to-moderate improvement of the motor condition in the two groups of patients.

Conclusion

GPi-DBS can be considered as a relevant therapeutic option for MxMD-ADCY5 in case of pharmacological treatment failure both in patients with chronic motor impairment and those with paroxysmal exacerbations meeting criteria for SAME. Given the response to DBS in the two groups of patients, it is plausible that MxMD-ADCY5 reflects basal ganglia dysfunction mediated by dysregulated cAMP signalling, and that DBS acts by restoring homeostatic inhibitory control in these circuits.
ADCY5的致病变异可引起混合性多动运动障碍(MxMD-ADCY5),有时药物治疗难以治愈。虽然以前曾使用过深部脑刺激内苍白球(GPi-DBS),但对其适应症、疗效和安全性的了解很少,主要基于轶事报道和短病例系列。方法:我们回顾性地回顾了adcy5相关运动障碍患者的临床、遗传、治疗和手术资料,这些患者接受了GPi-DBS,在两个法国专家中心手术或先前发表过。结果:我们获得了23例患者的资料进行分析。GPi-DBS有两个明显的适应症。第一组由长期耐药的多动运动患者组成。第二组包括急性运动加重患者——满足肌张力障碍状态的标准——并在概念上与最近提出的严重急性运动加重框架(SAME)一致。总体而言,GPi-DBS是安全的,并导致两组患者的运动状况轻度至中度改善。结论:无论是慢性运动障碍患者还是符合SAME标准的发作性加重患者,在药物治疗失败的情况下,GPi-DBS均可作为MxMD-ADCY5的相关治疗选择。考虑到两组患者对DBS的反应,MxMD-ADCY5可能反映了cAMP信号失调介导的基底神经节功能障碍,DBS通过恢复这些回路中的稳态抑制控制起作用。
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引用次数: 0
Visual misperceptions in favour of living beings in Parkinson's disease: A psychophysical study 帕金森氏症患者的视觉错觉:一项心理物理学研究。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-01-15 DOI: 10.1016/j.neurol.2025.12.005
L. Cleret de Langavant , A. Mouton , V. de Gardelle , S. Kouider , A.-C. Bachoud-Lévi , G. Fénelon , P. Remy

Background

Parkinson's disease (PD) patients often report seeing persons or animals rather than objects but this phenomenon remains poorly understood. Here, we use three experimental tasks to confirm such observation and to explore its cognitive mechanisms.

Method

Fourteen PD patients with visual hallucinations (PD-VH), 14 PD patients without visual hallucinations (PD-NVH) and 14 controls with similar cognitive performance were tested using ambiguous stimuli. Ambiguous stimuli were morphs in which visual features from faces and flowers were melted together (Experiments 1 and 2) and a black and white picture where a Dalmatian dog was hidden (Experiment 3). In Experiment 1, participants categorised ambiguous stimuli either as face or flower. In Experiment 2, they were shown an ambiguous stimulus, then a mask, and finally two ambiguous stimuli, one of which was identical to the first stimulus. In this discrimination task, participants chose which of the two last stimuli had been presented before. In Experiment 3, participants guessed the items hidden in the picture. We assessed group differences for categorisation with logistic modelling and computed sensitivity index and criterion psychophysical measures in Experiments 1 and 2. The ratio of living beings identified in the Dalmatian dog task was compared across groups.

Results

In the categorisation task, the PD-VH group tended to use a smaller proportion of visual features (point of subjective equality [PSE] = 41.5%) to label a stimulus as Face compared to PD-NVH (51%) and controls (56.2%). In the discrimination task, criterion c was lower in the PD-VH group compared to controls (c: –0.16 vs. 0.27; P = 0.005). In the Dalmatian dog task, the PD-VH group reported seeing livings beings more frequently than controls (P = 0.040). A bias towards living beings was confirmed in the PD-VH group in the three tasks, and a bias toward non-living beings was measured in controls in the discrimination task.

Interpretation

Observing that controls exhibited bias toward non-living beings in the discrimination task, we suggest that impaired top-down control over perception processes explains the bias toward living beings in PD-VH visual misperceptions.
背景:帕金森病(PD)患者经常报告看到人或动物而不是物体,但这种现象尚不清楚。在这里,我们使用三个实验任务来证实这一观察并探索其认知机制。方法:采用模糊刺激对14例PD伴视幻觉(PD- vh)、14例PD无视幻觉(PD- nvh)和14例认知表现相似的对照组进行测试。在模糊刺激中,人脸和花朵的视觉特征被融合在一起(实验1和2),以及一张隐藏着一只斑点狗的黑白图片(实验3)。在实验1中,参与者将模棱两可的刺激分类为脸或花。在实验二中,他们先看到一个模棱两可的刺激,然后是一个面具,最后是两个模棱两可的刺激,其中一个与第一个刺激相同。在这个辨别任务中,参与者选择最后两个刺激物中的哪一个在之前被呈现过。在实验3中,参与者猜测隐藏在图片中的物品。在实验1和实验2中,我们使用逻辑模型和计算敏感性指数以及标准心理物理测量来评估分组差异。在达尔马提亚狗任务中识别的生物的比例在各组之间进行了比较。结果:在分类任务中,PD-VH组倾向于使用较小比例的视觉特征(主观平等点[PSE]=41.5%)将刺激标记为面部,而PD-NVH组(51%)和对照组(56.2%)。在辨别任务中,PD-VH组的c标准低于对照组(c: -0.16 vs. 0.27; P=0.005)。在斑点狗任务中,PD-VH组比对照组更频繁地报告看到生物(P=0.040)。PD-VH组在三个任务中证实了对生物的偏见,而对照组在歧视任务中测量了对非生物的偏见。解释:观察到控制组在辨别任务中表现出对非生物的偏见,我们认为自上而下对知觉过程的控制受损解释了PD-VH视觉误解中对生物的偏见。
{"title":"Visual misperceptions in favour of living beings in Parkinson's disease: A psychophysical study","authors":"L. Cleret de Langavant ,&nbsp;A. Mouton ,&nbsp;V. de Gardelle ,&nbsp;S. Kouider ,&nbsp;A.-C. Bachoud-Lévi ,&nbsp;G. Fénelon ,&nbsp;P. Remy","doi":"10.1016/j.neurol.2025.12.005","DOIUrl":"10.1016/j.neurol.2025.12.005","url":null,"abstract":"<div><h3>Background</h3><div>Parkinson's disease (PD) patients often report seeing persons or animals rather than objects but this phenomenon remains poorly understood. Here, we use three experimental tasks to confirm such observation and to explore its cognitive mechanisms.</div></div><div><h3>Method</h3><div>Fourteen PD patients with visual hallucinations (PD-VH), 14 PD patients without visual hallucinations (PD-NVH) and 14 controls with similar cognitive performance were tested using ambiguous stimuli. Ambiguous stimuli were morphs in which visual features from faces and flowers were melted together (Experiments 1 and 2) and a black and white picture where a Dalmatian dog was hidden (Experiment 3). In Experiment 1, participants categorised ambiguous stimuli either as face or flower. In Experiment 2, they were shown an ambiguous stimulus, then a mask, and finally two ambiguous stimuli, one of which was identical to the first stimulus. In this discrimination task, participants chose which of the two last stimuli had been presented before. In Experiment 3, participants guessed the items hidden in the picture. We assessed group differences for categorisation with logistic modelling and computed sensitivity index and criterion psychophysical measures in Experiments 1 and 2. The ratio of living beings identified in the Dalmatian dog task was compared across groups.</div></div><div><h3>Results</h3><div>In the categorisation task, the PD-VH group tended to use a smaller proportion of visual features (point of subjective equality [PSE]<!--> <!-->=<!--> <!-->41.5%) to label a stimulus as Face compared to PD-NVH (51%) and controls (56.2%). In the discrimination task, criterion <em>c</em> was lower in the PD-VH group compared to controls (<em>c</em>: –0.16 vs. 0.27; <em>P</em> <!-->=<!--> <!-->0.005). In the Dalmatian dog task, the PD-VH group reported seeing livings beings more frequently than controls (<em>P</em> <!-->=<!--> <!-->0.040). A bias towards living beings was confirmed in the PD-VH group in the three tasks, and a bias toward non-living beings was measured in controls in the discrimination task.</div></div><div><h3>Interpretation</h3><div>Observing that controls exhibited bias toward non-living beings in the discrimination task, we suggest that impaired top-down control over perception processes explains the bias toward living beings in PD-VH visual misperceptions.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"182 3","pages":"Pages 144-154"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145990676","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
EPIGAME a vignette-based study of management practices of medication overuse in migraine, among French neurologists EPIGAME是法国神经科医生对偏头痛药物过度使用管理实践的一项基于小插曲的研究
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-01-27 DOI: 10.1016/j.neurol.2026.01.002
C. Lucas , A. Revol , F. Faist , C. Defawe , M. Lanteri Minet

Background

Medication overuse headache (MOH) is a common and disabling condition associated with the excessive use of acute headache treatments. This study aimed to investigate MOH management by neurologists in France.

Methods

A vignette-based study was conducted among 107 neurologists (60 from private practice, 47 from hospitals or both hospital and private practice) in France. Participants evaluated four randomized clinical vignettes simulating real-life MOH cases and selected from five treatment strategies: (1) drug withdrawal and immediate initiation of preventive treatment, (2) initiation of preventive treatment without drug withdrawal, (3) initiation of preventive treatment with titration followed by drug withdrawal, (4) drug withdrawal alone, and (5) referral to a specialized centre.

Results

The most common strategies of MOH management involved combining drug withdrawal with preventive treatment: 44.1% of cases received preventive treatment with titration followed by drug withdrawal, and 42.5% received immediate preventive treatment alongside drug withdrawal. Most neurologists (82.6%) recommended outpatient care, while hospital-based neurologists were more likely to suggest inpatient treatment. A progressive drug withdrawal protocol was preferred by 73.4%, with the goal of limiting drug use to fewer than 8 days per month in 81.3% of cases. Preventive treatments were most commonly antidepressants (52.2%), followed by beta-blockers (24.4%) and antiepileptics (14.9%). Educational advice, non-pharmacological treatments, and psychological care were frequently recommended as complementary approaches. No significant associations were found between clinical parameters (e.g., drug type, comorbidities) and treatment choices.

Conclusions

This study with an innovative approach provides valuable insight into the current management practices for MOH among neurologists in France, revealing alignment with established guidelines outlined by “La Société française d’études des migraines et céphalées (SFEMC)” guidelines, being withdrawal coupled with preventative treatment.
药物过度使用性头痛(MOH)是一种与过度使用急性头痛治疗相关的常见致残性疾病。本研究旨在调查法国神经科医生对MOH的管理情况。方法对法国107名神经科医生(60名来自私人诊所,47名来自医院或医院和私人诊所)进行了一项基于小问卷的研究。参与者评估了四个模拟现实生活中卫生部病例的随机临床小场景,并从五种治疗策略中进行了选择:(1)停药并立即开始预防性治疗,(2)开始预防性治疗而不停药,(3)开始预防性治疗并滴定后停药,(4)单独停药,以及(5)转诊到专门中心。结果MOH管理最常见的策略是停药与预防治疗相结合,44.1%的病例采取预防性治疗加滴定后停药,42.5%的病例在停药的同时立即进行预防治疗。大多数神经科医生(82.6%)推荐门诊治疗,而以医院为基础的神经科医生更有可能建议住院治疗。73.4%的人倾向于渐进式停药方案,81.3%的病例的目标是将药物使用限制在每月少于8天。预防治疗最常见的是抗抑郁药(52.2%),其次是-受体阻滞剂(24.4%)和抗癫痫药(14.9%)。教育咨询、非药物治疗和心理护理经常被推荐作为补充方法。临床参数(如药物类型、合并症)与治疗选择之间未发现显著关联。该研究以一种创新的方法为法国神经科医生对卫生部目前的管理实践提供了有价值的见解,揭示了与“La societacran(SFEMC)”指南所概述的既定指导方针的一致性,即戒断与预防性治疗相结合。
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引用次数: 0
Predictors of quality of life and social participation in myotonic dystrophy type 1 1型强直性肌营养不良患者生活质量和社会参与的预测因素。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2025-12-29 DOI: 10.1016/j.neurol.2025.11.008
J.-B. Davion , C. Tard , L. Fragoso , A. Wilu-Wilu , L. Defebvre , X. Delbeuck

Introduction

Myotonic dystrophy type 1 (DM1) is a genetic multisystemic disorder, affecting the muscles but also the brain and other organs, and impacting quality of life (QoL). Most of previous studies focused on health-related QoL and its predictors, which might restrict the possibility to observe the consequences of more general factors on QoL.

Methods

We studied QoL from a more global point of view in adult non-congenital DM1 patients included in the DM-VASCOG cohort using the World Health Organization Quality of Life Brief Version (WHOQOL-BREF) questionnaire, which discriminates four domains (physical health, psychological state, social relationships, and environment). Social participation was also evaluated using a questionnaire designed to assess the frequency of and the degree of satisfaction with the patient's involvement in social activities. Associations of these questionnaires with demographic, DM1-related, neuropsychological and behavioral measures were analyzed.

Results

Among our 122 DM1 patients, lower scores for the physical QoL were observed compared to other dimensions (P < 0.001). QoL predictors were different among the physical (motor function, fatigue), psychological (anxiety, depression), social (education, depression) and environmental (anxiety, depression, fatigue) dimensions. Frequency of social participation in DM1 patients was associated with executive functions (Stroop test), while satisfaction with social participation was associated with depression and fatigue.

Conclusion

The different dimensions of QoL and social participation in adult DM1 are associated with different modifiable factors. The effect on QoL of interventions focusing on these factors should be studied in future DM1 trials.
1型肌强直性营养不良(DM1)是一种遗传性多系统疾病,不仅影响肌肉,还影响大脑和其他器官,并影响生活质量(QoL)。先前的大多数研究都集中在与健康相关的生活质量及其预测因素上,这可能限制了观察更一般因素对生活质量影响的可能性。方法:我们使用世界卫生组织生活质量简要版(WHOQOL-BREF)问卷,从更全面的角度研究DM-VASCOG队列中成人非先天性DM1患者的生活质量,该问卷区分了四个领域(身体健康、心理状态、社会关系和环境)。社会参与也通过一份问卷来评估患者参与社会活动的频率和满意度。分析这些问卷与人口学、dm1相关、神经心理学和行为测量的相关性。结果:在122例DM1患者中,身体生活质量得分低于其他维度(p结论:成人DM1患者生活质量和社会参与的不同维度与不同的可调节因素相关。关注这些因素的干预措施对生活质量的影响应在未来的DM1试验中进行研究。
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引用次数: 0
Immune checkpoint inhibitor – associated optic neuropathy: Two illustrative cases and therapeutic implications 免疫检查点抑制剂相关视神经病变:两个说明性病例及其治疗意义。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-01-22 DOI: 10.1016/j.neurol.2025.12.007
B. Manzenza Matuwanga , A. Maarouf , C. Boutiere , J. Pelletier , N. Stolowy , S. Demortiere
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引用次数: 0
Leukodystrophy-like phenotype in early-onset neuropsychiatric systemic lupus erythematosus: Case series and systematic review of the literature 早发性神经精神系统性红斑狼疮的白质营养不良样表型:病例系列和文献系统回顾。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-02-03 DOI: 10.1016/j.neurol.2026.01.004
I.B. Santiago , A.A. da Silva , I.L.P. Nóbrega , M.M.F. de Melo , J.M.C. Neto , L.P. Amadei , A.V. Catunda , P.C.A. de Assis Pereira Matos , D.G.F. Távora , M.S. Pitombeira , C.E.M. Rodrigues

Background

To analyze the clinical, radiological, therapeutic, and clinical outcomes of the leukodystrophy-like phenotype in neuropsychiatric systemic lupus erythematosus (NPSLE).

Methods

We conducted this systematic review following the Preferred Report Items for Systematic Review and Meta-analysis (PRISMA). We searched Pubmed, Embase, and Web of Science databases for articles published until May 31, 2025, using the terms “systemic lupus erythematosus” AND (“diffuse white matter lesions” OR “leukoencephalopathy” OR “leukodystrophies”). Additionally, we report five patients with leukodystrophy-like phenotype in early-onset NPSLE.

Results

Thirty-three cases were reviewed. The mean age was 36.9 ± 14.9 years, and 28 (84.8%) were female patients. A previous diagnosis of SLE was present in 66% of cases. The main neurological symptoms included headache (33.3%), seizures (15.1%), and consciousness disturbances (15.1%). Among the 17 patients with cerebrospinal fluid (CSF) abnormalities, elevated protein levels were observed in 11 (40.7%) cases, and pleocytosis in 6 (22.2%). MRI findings were reported in 31 patients, typically showing cerebral white matter lesions characterized by hyperintense areas with T2-weighted or fluid-attenuated inversion recovery (FLAIR) sequences. Most patients were treated with high-dose corticosteroid pulse therapy (22/32), while others received cyclophosphamide pulses (18/32), therapeutic plasma exchange (PLEX) (4/32), or rituximab (6/32). Overall, the therapeutic response was satisfactory, with clinical improvement in 23 out of 33 patients.

Conclusion

In light of the severe clinical presentation of the leukodystrophy-like phenotype in NPSLE, early diagnosis and aggressive treatment are crucial for successful outcomes, as suggested by our review, which reported clinical improvement in 70% of patients. Future prospective studies are needed to confirm these findings.
背景:分析神经精神系统性红斑狼疮(NPSLE)的白质营养不良样表型的临床、放射学、治疗和临床结果。方法:我们按照系统评价和荟萃分析的首选报告项目(PRISMA)进行了本系统评价。我们检索了Pubmed、Embase和Web of Science数据库,检索了2025年5月31日前发表的文章,检索词为“系统性红斑狼疮”和(“弥漫性白质病变”或“白质脑病”或“白质营养不良”)。此外,我们报告了5例早发性NPSLE患者的白质营养不良样表型。结果:回顾了33例病例。平均年龄36.9±14.9岁,女性28例(84.8%)。66%的病例有SLE的既往诊断。主要神经系统症状包括头痛(33.3%)、癫痫发作(15.1%)和意识障碍(15.1%)。17例脑脊液异常患者中,蛋白水平升高11例(40.7%),细胞增多6例(22.2%)。报告了31例患者的MRI结果,典型表现为脑白质病变,其特征是t2加权或液体衰减反转恢复(FLAIR)序列的高信号区。大多数患者接受大剂量皮质类固醇脉冲治疗(22/32),而其他患者接受环磷酰胺脉冲治疗(18/32),治疗性血浆置换(PLEX)(4/32)或利妥昔单抗(6/32)。总体而言,治疗反应令人满意,33例患者中有23例临床改善。结论:鉴于NPSLE中白质营养不良样表型的严重临床表现,早期诊断和积极治疗对于成功的结果至关重要,正如我们的综述所提示的那样,70%的患者的临床改善。需要进一步的前瞻性研究来证实这些发现。
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引用次数: 0
Michel Dumas (1934–2025): From the neurology ward to global health 米歇尔·杜马(1934-2025):从神经内科病房到全球卫生
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-03-09 DOI: 10.1016/j.neurol.2026.02.153
P.-M. Preux , A.G. Diop , P. Couratier
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引用次数: 0
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Revue neurologique
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