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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。
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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 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 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 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。
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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。
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引用次数: 0
Late-onset epilepsy as a prodromal symptom 迟发性癫痫作为前驱症状
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-01 DOI: 10.1016/j.neurol.2025.07.007
S. Dupont
Late-onset epilepsy (LOE) of unknown etiology accounts for 15–30% of all LOE cases. A critical question is whether these unexplained seizures represent a prodromal manifestation of an underlying neurological disorder — most notably, stroke or Alzheimer's disease (AD). Growing evidence suggests that seizures may be an early sign of subclinical cerebrovascular disease, serving as a warning signal for future stroke, or an early symptom of a neurodegenerative disorder, particularly AD, reflecting initial pathological changes such as amyloid-β and tau deposition. An alternative hypothesis proposes that shared risk factors — especially cardiovascular ones — underlie all three conditions: LOE, stroke, and AD. As a result, it is recommended that patients with LOE of unknown etiology undergo comprehensive cardiovascular evaluation and receive appropriate management of any identified risk factors. However, it remains unclear whether this approach is sufficient to prevent future strokes. Cognitive assessment is also essential in these patients. In this context, prodromal seizures may provide an opportunity to identify individuals suitable for early, targeted interventions aimed at slowing neurodegeneration.
病因不明的晚发性癫痫(LOE)占所有LOE病例的15-30%。一个关键的问题是,这些无法解释的癫痫发作是否代表一种潜在神经系统疾病的前驱表现——最明显的是中风或阿尔茨海默病(AD)。越来越多的证据表明,癫痫发作可能是亚临床脑血管疾病的早期征兆,是未来中风的警告信号,或者是神经退行性疾病的早期症状,特别是阿尔茨海默病,反映了淀粉样蛋白β和tau沉积等初始病理变化。另一种假说认为,共同的风险因素——尤其是心血管风险因素——构成了这三种疾病的基础:爱情、中风和阿尔茨海默病。因此,建议病因不明的LOE患者进行全面的心血管评估,并对任何确定的危险因素进行适当的管理。然而,目前尚不清楚这种方法是否足以预防未来的中风。在这些患者中,认知评估也是必不可少的。在这种情况下,前驱癫痫发作可能提供了一个机会,以确定个人适合早期,有针对性的干预,旨在减缓神经变性。
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引用次数: 0
In vivo molecular imaging of brain tissue pathology in presymptomatic multiple sclerosis 症状前多发性硬化症脑组织病理的体内分子成像
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-01 DOI: 10.1016/j.neurol.2025.07.015
V.A.G. Ricigliano , B. Stankoff
The presence of focal lesions suggestive of multiple sclerosis (MS) seen with magnetic resonance imaging (MRI) in asymptomatic individuals defines the radiologically isolated syndrome (RIS). Pathologically, tissue changes in RIS subjects recapitulate, although being less extensive and pronounced, those observed in definite MS, with an amount of innate immune cell activation, myelin loss, gliosis, metabolic modifications, and structural damage affecting the neuro-axonal compartment. Conventional MRI, however, is not able to capture these biological abnormalities at the cellular and molecular level. Positron emission tomography (PET) with specific radiolabeled compounds could fill this gap, identifying in vivo and with higher granularity the processes underling the abnormal MRI signals. This review presents data obtained with PET in RIS individuals and discusses the potential application of this technique in exploring the different pathophysiologic changes going on in the RIS brain, with technical considerations on radiotracers already tested in MS or newly developed. By breaking up the study of complex tissular changes into single biological phenomena, the use of PET in RIS could encourage early interventions to selectively target each one of them, with potential consequences on the clinical conversion to MS.
无症状个体在磁共振成像(MRI)中出现提示多发性硬化症(MS)的局灶性病变定义为放射隔离综合征(RIS)。病理上,RIS患者的组织变化虽然不像明确的MS患者那样广泛和明显,但仍有一定数量的先天免疫细胞激活、髓磷脂丢失、胶质增生、代谢改变和影响神经轴突室的结构损伤。然而,传统的MRI不能在细胞和分子水平上捕捉这些生物学异常。具有特定放射性标记化合物的正电子发射断层扫描(PET)可以填补这一空白,在体内以更高的粒度识别异常MRI信号下的过程。这篇综述介绍了用PET在RIS个体中获得的数据,并讨论了该技术在探索RIS大脑中发生的不同病理生理变化方面的潜在应用,以及已经在MS中测试或新开发的放射性示踪剂的技术考虑。通过将复杂组织变化的研究分解为单一的生物学现象,PET在RIS中的应用可以鼓励早期干预,选择性地针对其中的每一种,从而对临床向MS的转化产生潜在的影响。
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引用次数: 0
Presymptomatic multiple sclerosis: Insights from the Radiologically Isolated Syndrome 症状前多发性硬化症:来自影像学孤立综合征的见解。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-01 DOI: 10.1016/j.neurol.2025.06.013
C. Lebrun-Frenay , M. Cohen , D.T. Okuda
Radiologically isolated syndrome (RIS) represents the initial phase of multiple sclerosis (MS) and is identified incidentally in asymptomatic individuals who display typical brain or spinal cord lesions indicative of autoimmune inflammatory demyelination. The 2023 RIS criteria enhance diagnostic precision against imaging mimics by requiring one T2-weighted hyperintense lesion in two of four specified locations (periventricular, juxtacortical/cortical, infratentorial, spinal cord) alongside at least two of the following: a spinal cord lesion, CSF-restricted oligoclonal bands (OCB), or a new T2/gadolinium-enhancing lesion on MRI at any point, defining dissemination in time (DIT). After confirming the diagnosis, established risk factors for transition to clinical MS need to be assessed. Key factors include a younger age, male sex, the presence of infratentorial, spinal cord, or gadolinium-enhancing lesions, and CSF-restricted OCBs or increased kappa-free light chains. Two randomized trials showed the efficacy of two oral disease-modifying therapies in delaying the first clinical event in RIS. However, as some individuals remain asymptomatic, it's crucial to identify suitable candidates to balance treatment benefits with potential risks. Reviewing each RIS case with an MS expert team is advisable for better care and monitoring. The updated 2024 McDonald criteria classify RIS patients with additional features, such as positive CSF and susceptibility MRI biomarkers, as having preclinical MS.
放射分离综合征(RIS)代表多发性硬化症(MS)的初始阶段,偶然发现于表现出典型的自身免疫性炎症性脱髓鞘的脑或脊髓病变的无症状个体。2023年RIS标准通过要求在四个指定位置(脑室周围、皮质旁/皮质、幕下、脊髓)中的两个位置出现一个T2加权高信号病变,以及以下至少两个:脊髓病变、csf限制性低克隆带(OCB),或在任何点MRI上出现新的T2/钆增强病变,从而提高了对成像模拟的诊断精度(DIT)。确诊后,需要评估已确定的向临床多发性硬化症过渡的危险因素。关键因素包括年龄较小、男性、幕下、脊髓或钆增强病变的存在、csf受限的ocb或无kappa轻链增加。两项随机试验显示两种口腔疾病改善疗法在延迟RIS患者首次临床事件方面的疗效。然而,由于有些人仍然无症状,因此确定合适的候选人以平衡治疗益处与潜在风险至关重要。建议与多发性硬化症专家小组一起审查每个RIS病例,以获得更好的护理和监测。更新的2024年McDonald标准将RIS患者分类为具有其他特征的患者,如CSF阳性和易感性MRI生物标志物,为临床前MS。
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引用次数: 0
期刊
Revue neurologique
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