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The importance of studying the history of neurosciences 研究神经科学史的重要性。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-16 DOI: 10.1016/j.neurol.2025.12.001
G. Fénelon
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引用次数: 0
Intranasal insulin for mild cognitive impairment and Alzheimer's disease: A systematic review and meta-analysis of randomized controlled trials 鼻内胰岛素治疗轻度认知障碍和阿尔茨海默病:随机对照试验的系统回顾和荟萃分析
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-22 DOI: 10.1016/j.neurol.2025.11.005
A.M.P. Silva , O.R. Gonçalves , G.C.N. Tudella , M.V.S. Nascimento , M.A.O.M. Pereira , L.A.O Nantes , J.V.A. Fernandes , D.G. Quiroga , D.V.S. Lima Jr , E.S. Franco , M.B.S. Maia

Background

Central insulin resistance has been implicated in the pathophysiology of Alzheimer's disease (AD), supporting the hypothesis that, in some individuals, AD may represent “type 3 diabetes.” Intranasal insulin has been proposed as a non-invasive approach to enhance brain insulin signaling while minimizing peripheral metabolic effects, but clinical evidence remains inconsistent.

Methods

We conducted a systematic review and meta-analysis of randomized controlled trials (RCTs) comparing intranasal insulin with placebo in patients with mild cognitive impairment (MCI) or mild-to-moderate AD. Primary outcomes included changes in cognitive performance (ADAS-Cog13, CDR-SB, DSRS, delayed recall) and functional ability (ADCS-ADL). Secondary outcomes included cerebrospinal fluid (CSF) biomarkers (Aβ42, total tau, phosphorylated tau) and safety endpoints. Literature searches were performed in PubMed, Embase, and Cochrane Central up to April 2025. Random-effects models were used to pool effect estimates, and risk of bias was assessed using the Cochrane RoB 2 tool.

Results

Five RCTs enrolling 540 participants met the inclusion criteria. Pooled analyses showed no significant differences between intranasal insulin and placebo for ADAS-Cog13 (mean difference: −1.09 [95% CI: −4.89; 2.71]), ADCS-ADL (mean difference 0.06 [−0.33; 0.45]), CDR-SB, DSRS, or delayed recall. No significant effects were observed on CSF Aβ42, total tau, or p-tau181. Gastrointestinal adverse events were more frequent with insulin (risk ratio: 1.57; [95% CI: 1.15; 2.14]), whereas cardiovascular events were less frequent (risk ratio: 0.30 [0.12; 0.79]). No differences were found for other safety outcomes, and discontinuation rates were comparable between groups.

Conclusion

Intranasal insulin was generally well tolerated but did not produce meaningful improvements in cognitive, functional, or biomarker outcomes in patients with MCI or mild-to-moderate AD. Current evidence does not support its routine clinical use, and further trials with standardized dosing, longer follow-up, and biomarker-stratified designs are warranted to clarify its therapeutic potential.
背景:中枢性胰岛素抵抗与阿尔茨海默病(AD)的病理生理有关,这支持了AD在某些个体中可能代表“3型糖尿病”的假设。鼻内胰岛素被认为是一种非侵入性的方法,可以增强脑胰岛素信号,同时最大限度地减少外周代谢影响,但临床证据仍不一致。方法:我们对比较鼻内胰岛素和安慰剂治疗轻度认知障碍(MCI)或轻中度AD患者的随机对照试验(rct)进行了系统回顾和荟萃分析。主要结局包括认知表现(ADAS-Cog13、CDR-SB、DSRS、延迟回忆)和功能能力(ADCS-ADL)的变化。次要终点包括脑脊液(CSF)生物标志物(Aβ42、总tau蛋白、磷酸化tau蛋白)和安全性终点。文献检索在PubMed, Embase和Cochrane Central中进行,直到2025年4月。随机效应模型用于汇总效应估计,并使用Cochrane RoB 2工具评估偏倚风险。结果:5项随机对照试验纳入540名受试者,符合纳入标准。合并分析显示,鼻内胰岛素和安慰剂在ADAS-Cog13(平均差值:-1.09 [95% CI: -4.89; 2.71])、ADCS-ADL(平均差值0.06[-0.33;0.45])、CDR-SB、DSRS或延迟回忆方面无显著差异。未观察到对CSF Aβ42、总tau或p-tau181的显著影响。胰岛素组胃肠道不良事件发生率较高(风险比:1.57;[95% CI: 1.15; 2.14]),而心血管不良事件发生率较低(风险比:0.30[0.12;0.79])。其他安全性结果没有发现差异,停药率在两组之间具有可比性。结论:鼻内胰岛素通常耐受性良好,但对轻度认知损伤或轻度至中度AD患者的认知、功能或生物标志物结局没有显著改善。目前的证据不支持其常规临床应用,需要进一步的标准化剂量、更长时间的随访和生物标志物分层设计的试验来阐明其治疗潜力。
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引用次数: 0
Small vessel disease: Trigger or bystander of late-onset epilepsy of unknown origin? 小血管疾病:病因不明的晚发性癫痫的诱因还是旁观者?
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-22 DOI: 10.1016/j.neurol.2025.10.007
E. Hologne , Y. Chen , S. Moulin , L. Tyvaert
Late-onset epilepsy of unknown origin (LOEU) is a prevalent and disabling condition. Emerging evidence suggests a potential link between LOEU and new-onset dementia. Cerebral small vessel disease (cSVD) is a common pathology and a major risk factor for both stroke and dementia. cSVD has been hypothesized to contribute to the development of LOEU and cognitive decline through blood-brain barrier dysfunction. This review summarizes current data exploring the association between LOEU and cSVD, highlighting conflicting results, probably due to major methodological limitations. Furthermore, in individuals over 60 years of age conditions such as obstructive sleep apnea (OSA), amyloidopathy, and tauopathy are frequently observed and independently associated with both LOEU and cSVD. To date, no robust evidence has established cSVD as a causal factor of LOEU. The complex interplay of these conditions necessitates further investigation to quantify the contribution of each pathology to the development of LOEU. Future studies using rigorous methodologies are required to determine whether cSVD acts as a primary trigger or merely represents a bystander in LOEU.
迟发性不明原因癫痫(LOEU)是一种常见的致残疾病。新出现的证据表明LOEU与新发痴呆之间存在潜在联系。脑血管病(cSVD)是卒中和痴呆的常见病理和主要危险因素。cSVD被假设为通过血脑屏障功能障碍导致LOEU的发展和认知能力下降。这篇综述总结了目前研究LOEU和cSVD之间关系的数据,强调了可能由于主要方法限制而产生的相互矛盾的结果。此外,在60岁以上的个体中,经常观察到阻塞性睡眠呼吸暂停(OSA)、淀粉样变性和tau病等疾病,并与LOEU和cSVD独立相关。迄今为止,没有强有力的证据证明cSVD是LOEU的病因。这些条件的复杂相互作用需要进一步研究,以量化每种病理对LOEU发展的贡献。未来的研究需要使用严格的方法来确定cSVD是LOEU的主要触发因素还是仅仅代表一个旁观者。
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引用次数: 0
Addressing sexual difficulties in Parkinson's disease 解决帕金森病中的性困难。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-10-31 DOI: 10.1016/j.neurol.2025.10.004
E. Oprea
Sexual difficulties are common but under-recognized in Parkinson's disease (PD), significantly affecting quality of life. They include both sexual dysfunction (SD) — a non-motor symptom — and hypersexuality (HS) — an impulse control disorder (ICD). SD often presents as reduced libido, arousal issues, or orgasmic problems, while HS involves compulsive sexual thoughts or behaviors, often linked to dopamine agonists. These opposing symptoms may coexist, adding to diagnostic complexity. Sexual health in PD is influenced by neurological, vascular, endocrine, psychological, and medication-related factors. Despite its impact, sexual difficulties are rarely discussed in clinical settings due to limited time and patient reluctance. A proactive, nonjudgmental approach is essential. This review aims to equip neurologists with practical, time-efficient strategies to identify and manage sexual difficulties in both men and women with PD.
性困难在帕金森病(PD)中很常见,但未得到充分认识,严重影响生活质量。它们包括性功能障碍(SD)——一种非运动症状——和性欲亢进(HS)——一种冲动控制障碍(ICD)。SD通常表现为性欲减退、性唤起问题或性高潮问题,而HS则涉及强迫性的性想法或性行为,通常与多巴胺激动剂有关。这些相反的症状可能同时存在,增加了诊断的复杂性。PD患者的性健康受到神经、血管、内分泌、心理和药物相关因素的影响。尽管它的影响,性困难很少讨论在临床设置由于有限的时间和病人的不情愿。积极主动、不加评判的方法是必不可少的。本综述旨在为神经科医生提供实用的、高效的策略来识别和管理男性和女性PD患者的性障碍。
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引用次数: 0
Gerstmann–Sträussler–Scheinker syndrome neuropathology in a Creutzfeldt–Jakob disease-like phenotype patient caused by a novel 6-OPRI sequence in the PRNP gene 由PRNP基因中一个新的6-OPRI序列引起的克雅氏病样表型患者的Gerstmann-Sträussler-Scheinker综合征神经病理学
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-26 DOI: 10.1016/j.neurol.2025.11.007
M. Sýkora , S. Baranová , E. Parobková , T. Moško , J. Keller , K. Holada , R. Rusina , R. Matěj
Gerstmann–Sträussler–Scheinker syndrome is an extremely rare hereditary human prion disease caused by distinct mutations in the prion protein-encoding gene and is frequently associated with a positive family history. The disease typically presents with progressive cerebellar symptoms such as gaze apraxia with limb ataxia and axial ataxia; thus, the diagnostic process is often challenging due to nonspecific clinical presentation. We present a case of a 73-year-old patient with no family history of dementia and cerebellar symptomatology during the course of rapidly progressing dementia. Owing to the clinical suspicion of prion disease, antemortem analysis of cerebrospinal fluid using a real-time quaking-induced conversion (RT-QuIC) assay was performed, with positive results. Postmortem histopathological examination confirmed a familiar form of human prion disease with concomitant asymptomatic tauopathy. An additional finding was a novel 6 octapeptide repeat insertion mutation in the prion gene. Familiar cases with an increasing number of repeated insertions seem to be associated with a longer overall disease course, milder clinical deterioration and often false-negative RT-QuIC results. The performance of RT-QuIC in inherited prion diseases may vary. Our case, involving a 6 octapeptide repeat insertion mutation, is particularly noteworthy due to the rapidly progressive clinical course and positive RT-QuIC results in both antemortem and postmortem tissue analyses.
Gerstmann-Sträussler-Scheinker综合征是一种极其罕见的遗传性人类朊病毒疾病,由朊病毒蛋白编码基因的明显突变引起,通常与阳性家族史相关。该病典型表现为进行性小脑症状,如伴肢体共济失调和轴向共济失调的凝视失用症;因此,由于非特异性临床表现,诊断过程往往具有挑战性。我们提出一个病例,73岁的患者没有家族病史的痴呆和小脑症状的过程中迅速进展的痴呆。由于临床怀疑是朊病毒疾病,因此采用实时震颤诱导转化(RT-QuIC)法对死前脑脊液进行了分析,结果呈阳性。死后组织病理学检查证实了一种常见的人类朊病毒病,并伴有无症状的牛头病。另一个发现是在朊病毒基因中有一个新的6八肽重复插入突变。重复插入次数增加的熟悉病例似乎与较长的总体病程、较轻的临床恶化和经常假阴性RT-QuIC结果相关。RT-QuIC在遗传性朊病毒疾病中的表现可能有所不同。我们的病例涉及6个八肽重复插入突变,由于其快速进展的临床过程和在死前和死后组织分析中RT-QuIC阳性结果,特别值得注意。
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引用次数: 0
Trends in the real-world management of cluster headache patients treated by subcutaneous sumatriptan and/or oxygen in France – An analysis of the French National Social Security System Open Data over eleven years (2014–2024) 法国皮下舒马曲坦和/或氧气治疗丛集性头痛患者的现实管理趋势——对法国国家社会保障系统11年来(2014-2024年)开放数据的分析。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-16 DOI: 10.1016/j.neurol.2025.11.004
E.K. Van Obberghen , R. Fabre , L. Bailly , M. Lanteri-Minet
The burden of cluster headache (CH) requires better knowledge of management to improve it.

Objectives

To describe changes in the real-world management of CH treated with subcutaneous sumatriptan and/or oxygen in France over the period 2014–2024.

Methods

This is an analysis of two open data databases from the French Social Health Insurance (‘Open Medic’ and ‘Open LPP’), providing an annual estimate from 2014 to 2024 of the delivery of subcutaneous sumatriptan and/or oxygen, the number of beneficiaries of these treatments and their socio-demographic profile.

Results

Annual deliveries of subcutaneous sumatriptan increased from 286,999 boxes in 2014 to 454,275 boxes in 2024 (58.2% increase). Beneficiaries of subcutaneous sumatriptan increased from 13,638 individuals in 2014 to 19,109 individuals in 2024 (40.1% increase). Annual deliveries of package for the weekly use of oxygen therapy equipment increased from 224,143 in 2014 to 790,768 in 2024 (2.5 times more). Beneficiaries of oxygen for CH increased from 7493 individuals in 2014 to 22,346 individuals in 2024 (2 times more). Over the period 2014–2024, the male to female ratio decreased from 2.3/1 to 1.5/1 and from 1.5/1 to 0.8/1 for individuals receiving subcutaneous sumatriptan and individuals receiving oxygen respectively.

Conclusions

The delivery of subcutaneous sumatriptan and oxygen increased from 2014 to 2024, reflecting an improvement in the management of cluster headache in France. Nevertheless, given the one year-prevalence of this disease and the number of people expected to suffer from it, the number of people benefiting from these two treatments in 2024 indicates that there are still unmet needs. This study confirms the increase in the number of women treated for cluster headache observed over the last twenty years.
丛集性头痛的负担需要更好的管理知识来改善它。目的:描述2014-2024年期间法国皮下舒马曲坦和/或氧气治疗CH的实际管理变化。方法:这是对来自法国社会健康保险(“open Medic”和“open LPP”)的两个开放数据库的分析,提供了2014年至2024年皮下注射舒马曲坦和/或氧气的年度估计,这些治疗的受益者人数及其社会人口统计资料。结果:皮下舒马匹坦的年交付量从2014年的286999箱增加到2024年的454275箱(增长58.2%)。皮下舒马曲坦的受益人从2014年的13,638人增加到2024年的19,109人(增加40.1%)。氧气治疗设备每周使用包的年交付量从2014年的224143个增加到2024年的790768个(增加了2.5倍)。用于CH的氧气受益人从2014年的7493人增加到2024年的22346人(增加了2倍)。2014-2024年间,皮下注射舒马匹坦组和吸氧组的男女比例分别从2.3/1下降到1.5/1,从1.5/1下降到0.8/1。结论:2014年至2024年,法国患者皮下舒马匹坦及供氧量增加,反映出集束性头痛的管理有所改善。然而,考虑到这种疾病一年的流行率和预计将患此病的人数,2024年受益于这两种治疗的人数表明,仍有未满足的需求。这项研究证实了在过去二十年中观察到的治疗丛集性头痛的妇女人数的增加。
{"title":"Trends in the real-world management of cluster headache patients treated by subcutaneous sumatriptan and/or oxygen in France – An analysis of the French National Social Security System Open Data over eleven years (2014–2024)","authors":"E.K. Van Obberghen ,&nbsp;R. Fabre ,&nbsp;L. Bailly ,&nbsp;M. Lanteri-Minet","doi":"10.1016/j.neurol.2025.11.004","DOIUrl":"10.1016/j.neurol.2025.11.004","url":null,"abstract":"<div><div>The burden of cluster headache (CH) requires better knowledge of management to improve it.</div></div><div><h3>Objectives</h3><div>To describe changes in the real-world management of CH treated with subcutaneous sumatriptan and/or oxygen in France over the period 2014–2024.</div></div><div><h3>Methods</h3><div>This is an analysis of two open data databases from the French Social Health Insurance (‘Open Medic’ and ‘Open LPP’), providing an annual estimate from 2014 to 2024 of the delivery of subcutaneous sumatriptan and/or oxygen, the number of beneficiaries of these treatments and their socio-demographic profile.</div></div><div><h3>Results</h3><div>Annual deliveries of subcutaneous sumatriptan increased from 286,999 boxes in 2014 to 454,275 boxes in 2024 (58.2% increase). Beneficiaries of subcutaneous sumatriptan increased from 13,638 individuals in 2014 to 19,109 individuals in 2024 (40.1% increase). Annual deliveries of package for the weekly use of oxygen therapy equipment increased from 224,143 in 2014 to 790,768 in 2024 (2.5 times more). Beneficiaries of oxygen for CH increased from 7493 individuals in 2014 to 22,346 individuals in 2024 (2 times more). Over the period 2014–2024, the male to female ratio decreased from 2.3/1 to 1.5/1 and from 1.5/1 to 0.8/1 for individuals receiving subcutaneous sumatriptan and individuals receiving oxygen respectively.</div></div><div><h3>Conclusions</h3><div>The delivery of subcutaneous sumatriptan and oxygen increased from 2014 to 2024, reflecting an improvement in the management of cluster headache in France. Nevertheless, given the one year-prevalence of this disease and the number of people expected to suffer from it, the number of people benefiting from these two treatments in 2024 indicates that there are still unmet needs. This study confirms the increase in the number of women treated for cluster headache observed over the last twenty years.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"182 1","pages":"Pages 75-81"},"PeriodicalIF":2.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145775593","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
Impact of HIV on disability progression in multiple sclerosis: An observational retrospective matched cohort study HIV对多发性硬化症致残进展的影响:一项观察性回顾性匹配队列研究。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-15 DOI: 10.1016/j.neurol.2025.11.001
A. Moulignier , J. Guillaume , V. Pourcher , E. Maillart , E. Januel , C. Papeix

Background

The coexistence of HIV infection and multiple sclerosis (MS) is uncommon, and poorly characterized. Whether HIV-related immune modulation influences MS, progression remains uncertain. Despite theoretical immunological interactions, real-world data are scarce.

Objectives

To compare long-term disability outcomes in persons living with HIV (PLWHIVs) with MS (MS-PLWHIVs) versus matched HIV-negative MS controls (MS-controls).

Methods

This observational retrospective matched cohort study used databases from two French tertiary MS centers (1991–2021). Each MS-PLWHIV was matched with up to five MS-controls for MS subtype (relapsing-remitting or primary-progressive), sex, age at first MS attack (±5 years), and first-to-second attack interval (±5 years). The primary outcome was time to reach sustained EDSS-6 (cane use for  12 months). Time-to-event analyses used Kaplan–Meier estimates and Cox regression adjusted for matching variables.

Results

We identified 16 MS-PLWHIVs and 75 matched MS-controls, with comparable baseline MS characteristics and a median follow-up > 20 years. MS-PLWHIVs received significantly fewer MS disease-modifying therapies (MS-DMTs) (median: 0 [0–1] vs. 2 [1–3.5], P < 0.001), and only 19% were still treated at study end versus 75% of controls. Notwithstanding this treatment gap, MS-PLWHIVs reached EDSS-6 a median of 8 years later than controls (22.8 vs. 14.5 years, P = 0.05). However, the risk of reaching EDSS-6 did not differ significantly (adjusted HR: 1.60 [95% CI: 0.6–4.2], P = 0.6). One-third of patients in each group converted to secondary progressive MS.

Conclusion

Despite fewer MS-DMTs, MS-PLWHIVs did not show worse disability progression and even appeared to progress more slowly. While HIV-related immune modulation could contribute, this remains speculative. These findings, though limited by small sample size and retrospective design, provide rare long-term data on this understudied comorbidity.
背景:HIV感染和多发性硬化症(MS)共存是不常见的,并且缺乏特征。hiv相关的免疫调节是否影响MS的进展仍不确定。尽管存在理论上的免疫相互作用,但实际数据很少。目的:比较艾滋病病毒感染者(PLWHIVs)合并多发性硬化症(MS-PLWHIVs)与匹配的HIV阴性多发性硬化症对照组(MS-对照)的长期残疾结局。方法:这项观察性回顾性匹配队列研究使用了两个法国三级MS中心(1991-2021)的数据库。每个MS- plwhiv与MS亚型(复发缓解型或原发进行性)、性别、首次MS发作年龄(±5年)和首次到第二次发作间隔(±5年)匹配多达5个MS对照。主要终点是达到持续EDSS-6的时间(手杖使用≥12个月)。时间-事件分析使用Kaplan-Meier估计和Cox回归调整匹配变量。结果:我们确定了16例MS- plwhivs和75例匹配的MS对照,具有相似的MS基线特征,中位随访时间为20年。MS- plwhivs接受的MS疾病改善治疗(MS- dmts)明显较少(中位数:0[0-1]对2[1-3.5])。结论:尽管MS- dmts较少,MS- plwhivs并没有表现出更严重的残疾进展,甚至似乎进展更慢。虽然艾滋病毒相关的免疫调节可能有所贡献,但这仍然是推测性的。这些发现,虽然受到小样本量和回顾性设计的限制,但提供了这种未充分研究的合并症的罕见长期数据。
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引用次数: 0
Fertility ratio of functional/dissociative seizures compared to other neuropsychiatric disorders 功能性/解离性癫痫发作与其他神经精神疾病的生育比率。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-15 DOI: 10.1016/j.neurol.2025.11.002
A. Nishi
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引用次数: 0
Assessment of social cognition impairments in patients with amyotrophic lateral sclerosis: How can it be improved? A systematic review 肌萎缩侧索硬化症患者社会认知障碍的评估:如何改善?系统回顾。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-15 DOI: 10.1016/j.neurol.2025.11.003
B. Vovard , J. Faure-de Baets , P. Codron , P. Allain , J. Cassereau

Objectives

The aim of this review was to evaluate the current evidence on which part of social cognition is impaired in amyotrophic lateral sclerosis (ALS) patients among emotion recognition, affective empathy and Theory of Mind (ToM) and to suggest improvements in social cognition testing protocols.

Methods

A systematic review was conducted according to PRISMA guidelines. We included controlled cross-sectional or longitudinal studies published in English before September 1, 2024, that assessed social cognition in non-demented ALS patients. Searches were performed in Medline, Embase, Web of Science, and PsycINFO using specific MeSH terms. Studies using social cognition tests as a primary or secondary outcome were included.

Results

Thirty-four studies were analyzed. Impairments in emotion recognition — especially for negative emotions — were frequently reported, even in cognitively preserved ALS patients. Results for cognitive ToM were mixed and may be confounded by executive dysfunction or test limitations. In contrast, affective ToM deficits were more consistently identified. However, no included study directly assessed affective empathy. Tests used in the reviewed studies were often overly specific and lacked ecological validity, which may explain inconsistent results across domains and weak correlations with imaging or executive function.

Conclusion

Social cognition is increasingly recognized as a key non-motor domain affected in ALS. However, current assessment tools may lack the sensitivity and ecological validity needed to capture real-life deficits. The implementation of dynamic, multimodal assessments such as real-life social interaction tests or tests like the Movie Assessment for Social Cognition (MASC) could improve detection and guide clinical interventions but remain to be validated for ALS patients.
目的:本综述旨在评估肌萎缩侧索硬化症(ALS)患者在情绪识别、情感共情和心理理论(ToM)中哪部分社会认知受损的现有证据,并提出社会认知测试方案的改进建议。方法:根据PRISMA指南进行系统评价。我们纳入了2024年9月1日之前以英文发表的对照横断面或纵向研究,这些研究评估了非痴呆性ALS患者的社会认知。在Medline, Embase, Web of Science和PsycINFO中使用特定的MeSH术语进行搜索。使用社会认知测试作为主要或次要结果的研究被纳入。结果:共分析34项研究。情绪识别障碍——尤其是负面情绪——经常被报道,甚至在认知能力完好的ALS患者中也是如此。认知性汤姆的结果是混杂的,可能与执行功能障碍或测试限制相混淆。相比之下,情感性ToM缺陷的识别更为一致。然而,没有纳入研究直接评估情感共情。所审查的研究中使用的测试通常过于具体,缺乏生态有效性,这可能解释了跨领域结果不一致以及与成像或执行功能的弱相关性。结论:社会认知被越来越多的人认为是ALS患者受影响的关键非运动领域。然而,目前的评估工具可能缺乏捕捉现实生活中的缺陷所需的敏感性和生态有效性。实施动态、多模式的评估,如现实生活中的社会互动测试或社会认知电影评估(MASC)等测试,可以提高检测和指导临床干预,但仍有待于对ALS患者进行验证。
{"title":"Assessment of social cognition impairments in patients with amyotrophic lateral sclerosis: How can it be improved? A systematic review","authors":"B. Vovard ,&nbsp;J. Faure-de Baets ,&nbsp;P. Codron ,&nbsp;P. Allain ,&nbsp;J. Cassereau","doi":"10.1016/j.neurol.2025.11.003","DOIUrl":"10.1016/j.neurol.2025.11.003","url":null,"abstract":"<div><h3>Objectives</h3><div>The aim of this review was to evaluate the current evidence on which part of social cognition is impaired in amyotrophic lateral sclerosis (ALS) patients among emotion recognition, affective empathy and Theory of Mind (ToM) and to suggest improvements in social cognition testing protocols.</div></div><div><h3>Methods</h3><div>A systematic review was conducted according to PRISMA guidelines. We included controlled cross-sectional or longitudinal studies published in English before September 1, 2024, that assessed social cognition in non-demented ALS patients. Searches were performed in Medline, Embase, Web of Science, and PsycINFO using specific MeSH terms. Studies using social cognition tests as a primary or secondary outcome were included.</div></div><div><h3>Results</h3><div>Thirty-four studies were analyzed. Impairments in emotion recognition — especially for negative emotions — were frequently reported, even in cognitively preserved ALS patients. Results for cognitive ToM were mixed and may be confounded by executive dysfunction or test limitations. In contrast, affective ToM deficits were more consistently identified. However, no included study directly assessed affective empathy. Tests used in the reviewed studies were often overly specific and lacked ecological validity, which may explain inconsistent results across domains and weak correlations with imaging or executive function.</div></div><div><h3>Conclusion</h3><div>Social cognition is increasingly recognized as a key non-motor domain affected in ALS. However, current assessment tools may lack the sensitivity and ecological validity needed to capture real-life deficits. The implementation of dynamic, multimodal assessments such as real-life social interaction tests or tests like the Movie Assessment for Social Cognition (MASC) could improve detection and guide clinical interventions but remain to be validated for ALS patients.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"182 1","pages":"Pages 10-25"},"PeriodicalIF":2.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145769061","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
Refining the definition of severe migraine: Evidence from a prospective observational study 改进严重偏头痛的定义:来自前瞻性观察研究的证据。
IF 2.3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-01 Epub Date: 2025-12-22 DOI: 10.1016/j.neurol.2025.12.002
J. Henri , S. Redon , A. Donnet

Introduction

Migraine severity is often assessed by attack frequency, but this single dimension fails to reflect the full burden of disease. We aimed to validate a pragmatic multidimensional definition of severe migraine in a tertiary care setting.

Methods

We conducted an observational study including 96 consecutive adult migraine patients diagnosed according to ICHD-3 criteria at a tertiary pain center (Marseille, January–April 2024). Data collected included migraine days/month, HIT-6, MIGSEV, and HAD scores. Patients were categorized by frequency (< 8 vs. ≥ 8 days/month), HIT-6 (< 60, 60–64,  65), and MIGSEV grades (1–3).

Results

Median monthly frequency was 8 days. Patients with  8 days/month had significantly higher HIT-6 scores (P = 0.044). Among patients with < 8 days/month, both HIT-6  65 and MIGSEV grade 3 identified individuals with greater functional disability and higher depressive symptoms, comparable to those in the high-frequency group. No demographic or comorbidity variables significantly distinguished severe cases.

Discussion

A multidimensional definition of severe migraine is supported: (A)  8 days/month, or (B) < 8 days/month with HIT-6  65 and/or MIGSEV grade 3. This definition integrates frequency, functional impact, and perceived severity, providing a simple and reproducible framework to identify high-burden patients. It may improve preventive treatment decisions, referral to specialized care, and harmonization of research inclusion criteria.
偏头痛的严重程度通常通过发作频率来评估,但这一单一维度无法反映疾病的全部负担。我们的目的是验证三级医疗环境中严重偏头痛的实用多维定义。方法:我们在一家三级疼痛中心(马赛,2024年1月至4月)进行了一项观察性研究,包括96名根据ICHD-3标准诊断的连续成年偏头痛患者。收集的数据包括偏头痛天数/月、HIT-6、MIGSEV和HAD评分。患者按频次分类(结果:中位月频次为8天。≥8天/月的患者HIT-6评分显著高于对照组(P=0.044)。讨论:支持重度偏头痛的多维定义:(A)≥8天/月,或(B)
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引用次数: 0
期刊
Revue neurologique
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