Revue neurologique最新文献_第6页

Utility of tissue-based assays to elucidate an autoantibody-negative paraneoplastic syndrome: Lessons from a case report 利用基于组织的检测来阐明自身抗体阴性的副肿瘤综合征：来自一个病例报告的经验教训。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-08-13 DOI: 10.1016/j.neurol.2025.07.013

G. Cogan , D. Reguigne , L.D. Do , G. Picard , M. Mongin , B. Degos

引用次数: 0

Climate-related challenges to brain health: A European perspective review 气候对大脑健康的相关挑战：欧洲视角综述。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-08-13 DOI: 10.1016/j.neurol.2025.07.010

J. Reis , A. Buguet , Z. Tulek , A.-M. Landtblom , M.W. Radomski , Ş. Öztürk , M. Pugliatti , U. Kallweit , P.S. Spencer

In this paper, we provide a brief overview on the assessment of the numerous effects of climate change on human health, with particular emphasis on brain health. After reviewing the epidemiological data concerning neuro-psychiatric mortality and morbidity, we will examine the known effects of global warming in a European context. We will consider respectively, neurological diseases (epilepsy, migraine, stroke, sleep, neurodegenerative and neuroinfectious disorders), neurological involvement of climate-change induced health conditions, neuro-psychological aspects (post-traumatic stress and sleep disorders, eco-anxiety) and emerging risks for the brain (toxins and infections). We suggest some underlying mechanisms impacting the brain health. Given the paucity of available data, we call for increased research attention to the adverse effects of climate change. This perspective is intended to support a higher degree of preparedness, the development of improved preventive measures, and the promotion of targeted education for health professionals.

在本文中，我们简要概述了气候变化对人类健康的众多影响的评估，特别强调了大脑健康。在回顾了有关神经精神疾病死亡率和发病率的流行病学数据后，我们将研究全球变暖在欧洲背景下的已知影响。我们将分别考虑神经系统疾病（癫痫、偏头痛、中风、睡眠、神经退行性疾病和神经感染性疾病）、气候变化引起的健康状况的神经系统参与、神经心理方面（创伤后应激和睡眠障碍、生态焦虑）以及对大脑的新风险（毒素和感染）。我们提出了一些影响大脑健康的潜在机制。鉴于现有数据的缺乏，我们呼吁增加对气候变化不利影响的研究关注。这一观点的目的是支持更高程度的准备，制定改进的预防措施，并促进对保健专业人员的有针对性的教育。

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引用次数: 0

Factors predicting seizure control and delivery outcomes in women with epilepsy with planned and unplanned pregnancy 预测癫痫伴计划妊娠和非计划妊娠妇女癫痫发作控制和分娩结局的因素。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-08-08 DOI: 10.1016/j.neurol.2025.07.006

S. Melikova , A. Mammadbayli , A. Guekht

Objective

To determine and ascertain factors influencing seizure control and maternal and neonatal outcomes in women with epilepsy (WWE) with planned versus unplanned pregnancies.

Methods

One hundred twelve pregnant WWE were prospectively evaluated for over an eight-year period. Patients were subsequently evaluated at 3, 6, and 12 months after delivery and then were followed up by a neurologist at least three years after delivery.

Results

The proportion of the patients with increased seizure frequency was higher in focal epilepsy versus generalized epilepsy (P < 0.05). The occurrence of seizures during pregnancy was associated with poor seizure control one year prior to the pregnancy and nonadherence to treatment (P < 0.0001). Structural brain abnormalities were associated with a higher risk of seizures during pregnancy (P = 0.03). Women with seizures during pregnancy (P = 0.0069) and with non-adherence to antiseizure medication (ASM) treatment had a higher rate of cesarean section (P = 0.0069 and P < 0.05, respectively). The rates of adverse delivery outcomes were not higher in women with planned pregnancies compared to women with unplanned pregnancies. However, offspring of mothers with unplanned pregnancies and nonadherence to treatment were at higher risk of an Apgar score at 5 minutes ≤ 7 compared with infants of planned pregnancies (P = 0.02 and P = 0.0001, respectively).

Conclusion

Seizure control prior to pregnancy, epilepsy type, adherence to ASM therapy, and pregnancy planning are potential factors influencing both seizure control and delivery outcomes in WWE. Our findings imply that systematic preconception counseling is crucial to preventing seizure deterioration in pregnancy and reducing the maternal and fetal complications.

目的：确定和确定影响癫痫妇女（WWE）的癫痫发作控制和孕产妇和新生儿结局的因素。方法：对112名怀孕的WWE进行了为期8年的前瞻性评估。患者随后在分娩后3、6和12个月接受评估，然后在分娩后至少3年接受神经科医生的随访。结果：局灶性癫痫发作频率增加的患者比例高于全身性癫痫(p结论：孕前癫痫控制、癫痫类型、坚持ASM治疗和妊娠计划是影响WWE患者癫痫控制和分娩结局的潜在因素。我们的研究结果表明，系统的孕前咨询对预防妊娠期癫痫恶化和减少母胎并发症至关重要。

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引用次数: 0

Hyperoxemia and its impact on in-hospital mortality in intracerebral hemorrhage patients: A retrospective cohort study 高氧血症及其对脑出血患者住院死亡率的影响：一项回顾性队列研究

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-08-06 DOI: 10.1016/j.neurol.2025.07.003

C. Tian , H. Zhou , M. Yuan

Introduction

This study aims to explore the relationship between arterial partial pressure of oxygen (PaO₂) and in-hospital mortality in patients admitted for the first time with intracerebral hemorrhage, with emphasis on the effect of hyperoxia on mortality.

Methods

We screened the MIMIC IV database for 1985 patients with cerebral hemorrhage admitted for the first time between 2008 and 2019. Exclusions included cases with duplicate records, patients with tumors, aneurysms, traumatic cerebral hemorrhage, cerebral hemorrhage due to blood disorders, and cases missing PaO₂ values — 828 patients for final analysis. Based on PaO₂ levels within 24 hours of admission, patients were divided into three groups: high (> 150 mmHg), medium (100–150 mmHg), and low (< 100 mmHg). A logistic mixed-effects regression model was used to analyze the relationship between PaO₂ and hospital mortality.

Results

The overall mortality rate during hospitalization was 37.1%. After adjusting for confounding factors, the mortality risk ratio (OR) of the low PaO₂ group and high PaO₂ group were 1.78 (95%CI: 1.04–3.03, P = 0.034) and 2.09 (95%CI: 1.28–3.42, P = 0.003), respectively. However, no significant associations were found between PaO₂levels and 28-, 60-, or 90-day mortality. Subgroup analysis showed an interaction between in-hospital mortality and PaO₂ level in patients with chronic lung disease (P = 0.002). Sensitivity analysis showed that the relationship still existed after excluding extreme PaO₂ values.

Conclusions

Elevated arterial oxygen tension within the first 24 hours of admission was independently associated with increased in-hospital mortality in patients with intracerebral hemorrhage.

前言：本研究旨在探讨首次入院脑出血患者动脉血氧分压（PaO2）与住院死亡率的关系，重点探讨高氧对死亡率的影响。方法：对2008年至2019年首次入院的1985例脑出血患者进行MIMIC IV数据库筛选。排除重复记录、肿瘤、动脉瘤、外伤性脑出血、血液病所致脑出血、PaO2值缺失等病例，最终分析828例。根据入院24小时内的PaO2水平，将患者分为三组：高（100 ~ 150mmHg）、中（100 ~ 150mmHg）、低(2)和住院死亡率。结果：住院期间总死亡率为37.1%。调整混杂因素后，低PaO2组和高PaO2组的死亡风险比（OR）分别为1.78 （95%CI: 1.04 ~ 3.03， P=0.034）和2.09 （95%CI: 1.28 ~ 3.42， P=0.003）。然而，没有发现pao2水平与28天、60天或90天死亡率之间的显著关联。亚组分析显示慢性肺病患者住院死亡率与PaO2水平存在交互作用（P=0.002）。敏感性分析表明，在排除极端PaO2值后，两者之间的关系仍然存在。结论：入院前24小时内动脉血氧浓度升高与脑出血患者住院死亡率升高独立相关。

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引用次数: 0

Homozygous DNAJB4 deletion revealing myopathy with acute respiratory failure 纯合子DNAJB4缺失显示肌病伴急性呼吸衰竭。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-08-05 DOI: 10.1016/j.neurol.2025.07.004

D.M. Chitimus , C. Adam , C. Cauquil , B. Keren , N. Heming , S. Amthor , D. Annane , G. Nicolas , P. Laforêt , C. Métay , C. Lefeuvre

Homozygous mutations in the DNAJB4 (NM_007034) gene impair HSP40 function, leading to early respiratory failure due to diaphragm involvement and rigid-spine-like characteristics. We describe the case of a 23-year-old male patient who was admitted for acute respiratory failure and motor deficit of the distal upper limbs. Creatine kinase values were elevated (10x upper normal limit), while the pulmonary function tests showed restrictive respiratory syndrome (forced vital capacity at 20% of theoretical values). The deltoid muscle biopsy findings were consistent with myofibrillar myopathy. Genetic analysis by NGS panel sequencing identified a homozygous deletion c.(?_1)_(1014_?)del, p.? (HGVS nomenclature) of the entire DNAJB4 gene, confirmed by qPCR. Both healthy parents exhibited the variant at the heterozygous state. Our results demonstrate that homozygous c.(?1)(1014_?)del, p.? deletion in DNAJB4 leads to a hereditary myopathy, further underscoring the gene's crucial role in muscle maintenance and function.

DNAJB4 （NM_007034）基因的纯合突变损害HSP40功能，导致膈肌受累和脊柱样僵硬特征导致早期呼吸衰竭。我们描述了一个23岁的男性病人谁是入院急性呼吸衰竭和上肢远端运动缺陷。肌酸激酶值升高（正常上限的10倍），而肺功能检查显示限制性呼吸综合征（强迫肺活量为理论值的20%）。三角肌活检结果与肌原纤维肌病一致。遗传分析通过NGS面板测序鉴定出一个纯合缺失c.(?_1)_(1014 ?)del, p.？（HGVS命名法），经qPCR证实。在杂合状态下，健康父母均表现出该变异。我们的结果表明纯合c.(1)(1014_?)del, p.？DNAJB4缺失导致遗传性肌病，进一步强调了该基因在肌肉维持和功能中的关键作用。

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引用次数: 0

Literature review: CAR T-cell therapy as a promising immunotherapeutic approach for medulloblastoma 文献综述：CAR - t细胞疗法是治疗成神经管细胞瘤的一种很有前途的免疫治疗方法。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-08-05 DOI: 10.1016/j.neurol.2025.07.005

C. Eksteen , J. Riedemann , H. Johnson , A.-M. Engelbrecht

Medulloblastoma (MB) accounts for approximately 20–25% of all childhood brain tumours and 63% of intracranial embryonic tumours, with an annual incidence of around 5 cases per million in the paediatric population. This high-grade neuroepithelial tumour of the posterior fossa can develop at any age during childhood, adolescence and even adulthood, often spreading via cerebrospinal fluid. While most MB cases are sporadic, they can be associated with genetic predisposition syndromes. Although these genetic mutations present potential therapeutic targets, the limited number of mutations and few existing therapies aimed at these neoantigens pose significant challenges. Despite aggressive multimodal treatment approaches, approximately 30% of patients ultimately succumb to MB, and survivors frequently face long-term side effects that severely impact their quality of life. MB harbours unique molecular factors, necessitating careful consideration of therapeutic targets such as the blood-brain barrier, tumour microenvironment, and the differing responses of cancer stem cells versus bulk tumour tissue. Conventional treatment typically involves maximal safe resection, risk-adapted chemotherapy, and/or radiation craniospinal irradiation. While there is general agreement on the benefits of chemotherapy for MB patients, adverse side effects remain prevalent, underscoring the need for alternative therapeutic strategies. Given the heterogeneous nature of MBs and the lack of salvage treatment, immunotherapy has emerged as a promising novel treatment avenue. This personalized approach aims to enhance specificity and potentially reduce side effects. Among these innovative methods, adoptive cell therapy, particularly chimeric antigen receptor T (CAR T) cell therapy, shows great promise. This review will explore the potential of CAR T-cell therapies in targeting MB, building on their successful application in other solid tumours.

髓母细胞瘤（MB）约占所有儿童脑肿瘤的20-25%和颅内胚胎肿瘤的63%，在儿科人群中每年的发病率约为百万分之5。后窝高级别神经上皮肿瘤可在儿童、青少年甚至成年的任何年龄发生，常通过脑脊液扩散。虽然大多数MB病例是散发的，但它们可能与遗传易感性综合征有关。尽管这些基因突变提供了潜在的治疗靶点，但针对这些新抗原的突变数量有限，现有的治疗方法也很少，这构成了重大挑战。尽管采用了积极的多模式治疗方法，但大约30%的患者最终死于MB，幸存者经常面临严重影响其生活质量的长期副作用。MB具有独特的分子因子，需要仔细考虑治疗靶点，如血脑屏障、肿瘤微环境以及癌症干细胞与大块肿瘤组织的不同反应。传统治疗通常包括最大限度的安全切除、适应风险的化疗和/或放射颅脊髓照射。虽然人们普遍认同化疗对MB患者的益处，但副作用仍然普遍存在，这强调了寻找替代治疗策略的必要性。鉴于MBs的异质性和缺乏补救性治疗，免疫疗法已成为一种有希望的新治疗途径。这种个性化的方法旨在提高特异性并潜在地减少副作用。在这些创新方法中，过继细胞疗法，特别是嵌合抗原受体T （CAR - T）细胞疗法显示出很大的前景。本文将在CAR - t细胞疗法在其他实体肿瘤中的成功应用的基础上，探讨靶向MB的CAR - t细胞疗法的潜力。

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引用次数: 0

Neurological damage from recreational nitrous oxide use: Two distinct electroclinical profiles in a retrospective cohort 娱乐性氧化亚氮的神经损伤：回顾性队列中两种不同的电临床特征。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-07-25 DOI: 10.1016/j.neurol.2025.07.002

F. Boulin , A.-L. Bédat-Millet , A. Didier-Laurent , F. Louillet , G. Quesney , B. Hébant , B. Sudrié-Arnaud , D. Maltête , M.L. Welter , L. Guyant-Maréchal , L. Zourdani

The recreational nitrous oxide (N₂O) use is increasingly recognized as a cause of serious neurological disorders, particularly among young individuals. This retrospective multicenter study aimed to describe the clinical, biological, and electrophysiological features of 41 patients with neurological impairments linked to recreational N₂O use. Most patients presented myeloneuropathy and motor-dominant, length-dependent, axonal neuropathy involving the lower limbs. Notably, two distinct electroclinical patterns emerged from nerve conduction studies and electromyography: a predominant sensorimotor axonal neuropathy (78.4% of cases) and a pure motor neuropathy (13.5%), both primarily involving the lower limbs. Despite normal serum B12 levels in most cases, elevated homocysteine and methylmalonic acid levels confirmed a functional vitamin B12 deficiency. These findings highlight the characteristic electrophysiological profiles associated with recreational N₂O use and underscore the importance of early detection and targeted management to prevent long-term disability.

娱乐性氧化亚氮（N2O）的使用越来越被认为是导致严重神经系统疾病的原因，尤其是在年轻人中。本回顾性多中心研究旨在描述41例与娱乐性N2O使用相关的神经损伤患者的临床、生物学和电生理特征。大多数患者表现为脊髓神经病变和运动主导型、长度依赖性、轴索神经病变累及下肢。值得注意的是，神经传导研究和肌电图显示了两种不同的电临床模式：主要是感觉运动轴索神经病（78.4%的病例）和纯粹的运动神经病（13.5%），两者主要累及下肢。尽管大多数病例的血清B12水平正常，但同型半胱氨酸和甲基丙二酸水平升高证实了功能性维生素B12缺乏症。这些发现强调了与娱乐性N2O使用相关的特征性电生理特征，并强调了早期发现和有针对性的管理对于预防长期残疾的重要性。

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引用次数: 0

Evaluation of choroidal thickness changes in patients with migraine using optical coherence tomography 光学相干断层成像评价偏头痛患者脉络膜厚度变化。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-07-24 DOI: 10.1016/j.neurol.2025.06.004

M. Rekik , Y. Walha , K.S. Moalla , S. Kammoun , C. Mhiri , M. Dammak , A. Trigui

Introduction

Optical coherence tomography (OCT) has enabled the study of ocular impairments associated with certain neurological pathologies, such as migraine. This common condition represents a risk factor for ocular ischemic complications. We investigated changes in choroidal thickness (CT) in migraine patients with and without aura (MWA and MWoA respectively) compared to healthy controls using OCT and identified factors influencing the occurrence of these anomalies in migraine.

Methods

This was a cross-sectional case-control study including migraine patients and control subjects. All patients and controls underwent a complete ophthalmological examination and CT measurements using a spectral domain-OCT device (DRI OCT Triton; Topcon Corp, Japan). The duration of migraine, the frequency and duration of migraine attacks, the migraine disability assessment (MIDAS) and migraine severity scale (MIGSEV) questionnaire scores were recorded.

Results

One hundred and twenty eyes from 60 patients (60 eyes in the MWoA group and 60 eyes in the MWA group) were included. The control group included 30 age- and gender-matched healthy participants (60 eyes). OCT revealed that CT was significantly reduced in MWoA and MWA groups compared with the control group and in the MWA group compared to MWoA group. The duration of migraine and attacks as well as the disease severity were correlated with CT. Multiregression analysis showed that disease severity was the main determinant of CT.

Conclusion

Our study highlights the significant impact of both types of migraine on choroidal structure. OCT proves to be promising in understanding migraine pathophysiology and potentially serves as a valuable biomarker for diagnosis and monitoring.

光学相干断层扫描（OCT）使得与某些神经系统疾病（如偏头痛）相关的眼损伤的研究成为可能。这种常见情况是眼缺血并发症的危险因素。我们利用OCT研究了有和无先兆偏头痛患者（分别为MWA和MWoA）与健康对照组相比脉络膜厚度（CT）的变化，并确定了影响偏头痛中这些异常发生的因素。方法：这是一项横断面病例对照研究，包括偏头痛患者和对照组。所有患者和对照组均接受了完整的眼科检查和CT测量，使用光谱域OCT设备(DRI OCT Triton；Topcon公司，日本)。记录偏头痛持续时间、偏头痛发作频率和持续时间、偏头痛残疾评估（MIDAS）和偏头痛严重程度量表（MIGSEV）问卷得分。结果：纳入60例患者120只眼（MWoA组60只眼，MWA组60只眼）。对照组包括30名年龄和性别匹配的健康参与者（60只眼睛）。OCT显示MWoA组和MWA组与对照组相比CT明显降低，MWA组与MWoA组相比CT明显降低。偏头痛持续时间、发作时间及病情严重程度与CT相关。多元回归分析显示疾病严重程度是CT的主要决定因素。结论：我们的研究强调了两种类型的偏头痛对脉络膜结构的显著影响。OCT被证明在了解偏头痛病理生理方面是有希望的，并且可能作为诊断和监测的有价值的生物标志物。

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引用次数: 0

Can a virtual reality tool detect minor executive functions impairment in patients with multiple sclerosis? 虚拟现实工具能否检测多发性硬化症患者的轻微执行功能障碍？

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-07-23 DOI: 10.1016/j.neurol.2025.06.009

R. Delsanti , E. Le Mercier , A. Briant , N. Derache , C. Arnaud , P. Branger , G. Defer

Introduction

Traditional neuropsychological tests do not always identify executive disorders in patients with multiple sclerosis (MS), despite their complaints. Many studies suggest that virtual reality (VR) may be useful to evaluate cognitive functions as close as possible to patients’ daily activities. We investigated the validity of a VR tool in the assessment of executive impairment in MS patients.

Methods

Thirty patients with relapsing-remitting multiple sclerosis (RRMS) and 30 matched healthy controls performed a shopping task using the Virtual Action Planning-Supermarket (VAP-S) which simulates a supermarket environment (main measures: total test duration, distance traveled, incorrect actions, number of stops, duration of stops and time to pay) with a familiarization phase followed by a test phase. In addition, neuropsychological tests assessing executive functions were proposed. Inter-group comparisons were conducted using adapted statistical tests and correlation analyses between the VAP-S and the traditional tests results were performed.

Results

No significant difference was observed between the two groups in traditional tests. However, during the VAP-S familiarization phase, RRMS patients performed worse than controls, showing differences in distance traveled (P = 0.042), total test duration (P = 0.021) and number of stops (P = 0.034). In the VAP-S test phase, no difference was found. Correlations were observed between some of the VAP-S scores and traditional tests results in both groups.

Conclusion

A VR tool appears to be more effective than traditional tests in detecting mild executive difficulties in MS patients. We noticed a learning effect during the test phase, suggesting that VR could support cognitive rehabilitation and improve daily living.

传统的神经心理学测试并不总是识别多发性硬化症（MS）患者的执行障碍，尽管他们的抱怨。许多研究表明，虚拟现实（VR）可能有助于评估尽可能接近患者日常活动的认知功能。我们调查了VR工具在评估多发性硬化症患者执行功能障碍中的有效性。方法：30例复发-缓解型多发性硬化症（RRMS）患者和30例匹配的健康对照者使用虚拟行动计划-超市（vv - s）进行购物任务，该虚拟行动计划-超市模拟超市环境（主要测量指标：总测试时间、行进距离、错误行为、停车次数、停车时间和付款时间），并进行熟悉阶段和测试阶段。此外，还提出了评估执行功能的神经心理测试。采用适应性统计检验进行组间比较，并对VAP-S与传统检验结果进行相关性分析。结果：两组在传统试验中差异无统计学意义。然而，在VAP-S熟悉阶段，RRMS患者的表现比对照组差，表现出旅行距离（P=0.042），总测试时间（P=0.021）和停止次数（P=0.034）的差异。在VAP-S测试阶段，没有发现差异。在两组中观察到一些VAP-S评分与传统测试结果之间的相关性。结论：在检测MS患者轻度执行困难方面，VR工具似乎比传统测试更有效。在测试阶段，我们注意到一个学习效果，这表明VR可以支持认知康复和改善日常生活。

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引用次数: 0

Zilucoplan as a fast-acting treatment for severe myasthenic exacerbation: A case series Zilucoplan作为严重肌无力加重的速效治疗：一个病例系列。

IF 2.3 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-07-19 DOI: 10.1016/j.neurol.2025.06.010

V. Fabry , B. Acket , P. Girardie , L. Espagno , O. Cointault , P. Cintas

引用次数: 0