Neurologia最新文献

英文中文

Super-refractory status epilepticus during a seronegative immune-mediated relapse after herpes simplex virus 1 encephalitis. 单纯疱疹病毒1型脑炎后血清阴性免疫介导复发期间的超难治性癫痫持续状态。

Neurologia

Pub Date : 2026-03-23 DOI: 10.1016/j.nrleng.2026.501926

Iago García Díaz, Esperanza Ortegón Aguilar, María Recuerda Núñez, Gloria Rivera Rubiales

引用次数: 0

Reply to "Assessment of the diagnostic utility of the electroencephalogram in pediatric emergencies": the importance of not overlooking psychogenic nonepileptic seizures in the paediatric population. 回复“评估脑电图在儿科急症中的诊断效用”：在儿科人群中不忽视心因性非癫痫性发作的重要性。

Neurologia

Pub Date : 2026-03-23 DOI: 10.1016/j.nrleng.2026.501927

Moisés León-Ruiz, Julián Benito-León, Carlos Castañeda-Cabrero

引用次数: 0

Protective effect of adult vaccination on the development of dementias: A systematic review 成人疫苗接种对痴呆发展的保护作用：一项系统综述。

Neurologia

Pub Date : 2026-03-01 Epub Date: 2026-02-27 DOI: 10.1016/j.nrleng.2026.501939

E. Mariscal-Lopez , M. Agredano-Sanchez , R.M. Lopez-Gigosos , A. Mariscal , F. Fariñas-Guerrero , M. Gutierrez-Bedmar , M. Guts-Chornoknyzha

Introduction

Dementias currently impose a significant burden in terms of morbidity, mortality, socio-economic costs, and human suffering. Several studies have indicated that certain infectious diseases may increase the risk of dementia. Additionally, research has suggested that adult vaccines may help to prevent dementia. This systematic review looks into the possible association between adult vaccines and dementia in studies published between 2016 and April 2024.

Development

A search was conducted in accordance with the PRISMA 2020 guidelines to assess the potential association between adult vaccines and dementia in adults aged 50 and above. Quality and potential bias were evaluated using the revised Assessing the Methodological Quality of Systematic Reviews (AMSTAR-2) scale, the Newcastle–Ottawa scale, and the Risk-of-bias in Studies of Temporal Trends (ROBITT) tool. Fifteen out of the 16 selected studies showed a high degree of uniformity in terms of vaccinated adults having lower rates of dementia than unvaccinated adults. The observed risk reduction ranged from 4% to 50% (relative risk measures). The influenza vaccine has been the subject of the most extensive research, with 10 of the 16 studies focusing on it. Five studies demonstrated a dose–response relationship, indicating that a higher number of vaccines per patient is associated with a lower risk of dementia. Only one of the selected studies found an association between vaccination of adults and an increased risk of dementia.

Conclusions

The studies examined provide evidence supporting the hypothesis that adult vaccination may have a protective effect against the development of dementia. However, further molecular biology and pathophysiology studies are required to elucidate the underlying mechanisms and confirm the plausibility of this hypothesis.

导言：痴呆症目前在发病率、死亡率、社会经济成本和人类痛苦方面造成了重大负担。几项研究表明，某些传染性疾病可能会增加患痴呆症的风险。此外，研究表明，成人疫苗可能有助于预防痴呆症。本系统综述探讨了2016年至2024年4月期间发表的成人疫苗与痴呆之间可能存在的关联。进展：根据PRISMA 2020指南进行了一项研究，以评估成人疫苗与50岁及以上成人痴呆之间的潜在关联。使用修订后的评估系统评价方法学质量（AMSTAR-2）量表、纽卡斯尔-渥太华量表和时间趋势研究的偏倚风险（ROBITT）工具对质量和潜在偏倚进行评估。在16项选定的研究中，有15项研究显示，接种疫苗的成年人患痴呆症的比例低于未接种疫苗的成年人，这一结果高度一致。观察到的风险降低范围从4%到50%（相对风险测量）。流感疫苗一直是最广泛研究的主题，16项研究中有10项关注它。五项研究证明了剂量-反应关系，表明每位患者接种的疫苗数量越多，患痴呆症的风险就越低。在选定的研究中，只有一项发现成人接种疫苗与痴呆风险增加之间存在关联。结论：所检查的研究提供证据支持成人疫苗接种可能对痴呆的发展有保护作用的假设。然而，需要进一步的分子生物学和病理生理学研究来阐明潜在的机制并证实这一假设的合理性。

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引用次数: 0

Comments on the article “Mortality rates for Parkinson’s disease are increasing in Spain. An age-period-cohort and joinpoint analysis of mortality rates from 1981 to 2020” 对文章“帕金森氏症的死亡率在西班牙正在上升”的评论。1981年至2020年死亡率的年龄期队列和连接点分析。”

Neurologia

Pub Date : 2026-03-01 Epub Date: 2025-12-24 DOI: 10.1016/j.nrleng.2025.101893

Julián Benito-León , Carla Mª Benito-Rodríguez

引用次数: 0

Transcultural adaptation and validity to Spanish population with acquired brain injury of the Balance Evaluation Systems Test (BESTest) and their reduced versions (Mini-BESTest and Brief-BESTest) 平衡评估系统测试（BESTest）及其简化版本（Mini-BESTest和Brief-BESTest）对西班牙获得性脑损伤人群的跨文化适应和有效性。

Neurologia

Pub Date : 2026-03-01 Epub Date: 2025-12-24 DOI: 10.1016/j.nrleng.2025.501929

M. Fernández-Hontoria , R.P. Romero-Galisteo , M. Torres-Lacomba , C. González-Alted , Á. Megía-García-Carpintero , C. Lirio-Romero

Introduction

Balance assessment measures are often validated in the general population or older adults, but rarely in individuals with neurological impairment. This study reports the transcultural adaption and validation of the Balance Evaluation Systems Test (BESTest) and its short forms, the Mini-BESTest and Brief-BESTest, in a Spanish population with acquired brain injury (ABI).

Methods

The study was conducted in 3 stages: 1) translation and adaptation of the tests, 2) pilot test of the adapted version, and 3) assessment of psychometric properties (reliability and validity). The Berg Balance Scale was used as the criterion variable; construct validity was assessed by exploratory factor analysis of all items of each test; and reliability was tested by calculating Cronbach's alpha and the intra-class correlation coefficient.

Results

A total of 108 patients with subacute and chronic ABI participated in the study. Psychometric analysis of the 3 tests demonstrated good convergent validity, internal consistency, inter-rater reliability (0.998−0.969), and test-retest reliability (0.985−0.989). Convergent validity was observed with the Berg Balance Scale (r = 0.901, P < .001; r = 0.977, P < .001; r = 0.852, P < .001, respectively), as well as other gait and balance scales. No ceiling or floor effects were found in the adapted versions of the BESTest, Mini-BESTest, and Brief-BESTest for the Spanish population with ABI.

Conclusions

All 3 tests are reliable and valid, with BESTest being the best option for assessing balance in people with ABI, both in the subacute and chronic phase, as it includes domains that other tools do not assess.

简介：平衡评估措施通常在一般人群或老年人中得到验证，但很少在神经损伤患者中得到验证。本研究报告了平衡评估系统测试（BESTest）及其简短形式Mini-BESTest和Brief-BESTest在西班牙获得性脑损伤（ABI）人群中的跨文化适应和验证。方法：本研究分3个阶段进行：1)译文和改编，2)改编版本的先导测试，3)心理测量特性评估（信度和效度）。采用Berg平衡量表作为标准变量；构念效度采用探索性因子分析对各测试项进行评估；通过计算Cronbach’s alpha和类内相关系数来检验信度。结果：共有108例亚急性和慢性ABI患者参与研究。3个测试的心理测量分析显示，具有良好的收敛效度、内部一致性、量表间信度（0.998 ~ 0.969）、重测信度（0.985 ~ 0.989）。Berg平衡量表的收敛效度（r = 0.901， P）结论：所有3个测试都是可靠和有效的，BESTest是评估ABI患者亚急性期和慢性期平衡的最佳选择，因为它包括其他工具无法评估的领域。

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引用次数: 0

Recommendations for the diagnosis, treatment, and follow-up of late-onset Pompe disease 迟发性庞贝病的诊断、治疗和随访建议。

Neurologia

Pub Date : 2026-03-01 Epub Date: 2025-12-24 DOI: 10.1016/j.nrleng.2025.501933

C. Domínguez-González , M.Á. Barba Romero , C. Caballero Eraso , J. de las Heras , E. Farrero Muñoz , Ó. García-Campos , M. González , J.M. Grau , A. Hernández-Voth , R. Juntas Morales , J.C. León Hernández , M. Ley Martos , D. López-Padilla , N. Muelas , A. Nascimento , M. Olivé , C. Paradas , J. Pardo Fernández , S.I. Pascual , I. Pitarch , J. Díaz-Manera

Pompe disease or glycogenosis type II is a rare disease caused by mutations in the GAA gene that leads to deficiency of the acid alpha-1,4-glucosidase enzyme. As a result of the enzymatic defect, a progressive accumulation of intralysosomal glycogen occurs in various tissues, causing smooth, cardiac and skeletal muscle involvement. When the age of onset of the disease is after the first year of life, it is called late-onset Pompe disease (LOPD). Weakness of the axial and proximal waist muscles and respiratory dysfunction are common manifestations. Enzyme replacement therapy (ERT) has been available for more than 15 years and is the standard treatment. This therapy changes the course of the disease, although the effectiveness of the treatment reduces over time. New enzyme therapies represent new treatment opportunities for patients with LOPD. Here we present updated recommendations from a group of experts in Pompe disease on the diagnosis, treatment and follow-up of LOPD patients, with the aim of providing a guide for the clinical management of the disease.

庞贝病或II型糖原病是由GAA基因突变引起的一种罕见疾病，导致酸性α -1,4-葡萄糖苷酶缺乏。由于酶缺陷，溶酶体内糖原进行性积累发生在各种组织中，导致平滑肌、心肌和骨骼肌受累。当发病年龄在一岁以后时，称为迟发性庞贝病（LOPD）。腰轴肌和近端肌无力和呼吸功能障碍是常见的表现。酶替代疗法（ERT）已经有超过15年的历史，是标准的治疗方法。这种疗法改变了疾病的进程，尽管治疗的有效性会随着时间的推移而降低。新的酶疗法为LOPD患者提供了新的治疗机会。在此，我们介绍一组庞贝病专家对LOPD患者的诊断、治疗和随访的最新建议，旨在为该疾病的临床管理提供指导。

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引用次数: 0

How do we face the challenges of the Spanish Society of Neurology? Strategic Plan of the Spanish Society of Neurology 我们如何面对西班牙神经学会的挑战？西班牙神经病学学会战略计划。

Neurologia

Pub Date : 2026-03-01 Epub Date: 2025-12-24 DOI: 10.1016/j.nrleng.2025.501934

J.M. Láinez Andrés , C. Íñiguez Martínez , J. Porta Etessam , D. Ezpeleta Echávarri , M.T. Martínez de Albéniz Zabaleta , M.M. Bilbao , F. Escamilla Sevilla , D.M. Cerdán Santacruz , D. García Azorín , J. Carmiña Muñiz , S. Arias Rivas , S. Gil Navarro , I.C. Labordena , M.E. Gil Girbau , C. Santarrosa Mateo

Introduction

With the aim of redesigning the role of promoting and fostering the progress of neurology and anticipating the social, scientific, health, and economic context provided by the development of our specialty, the Spanish Society of Neurology has decided to formalise its direction in a Strategic Plan, the elements of which are shared in this article.

Methods

The development of the Plan has been structured in 3 phases: internal and external analysis, strategic projection, and formalisation of the plan. A qualitative and strategic analysis approach has been incorporated, through surveys, interviews, and participatory sessions with the SEN, with the participation of approximately 500 members and other professionals in the field. The current situation of the SEN and its environment has been explicitly stated, the corporate identity has been defined, and strengths, weaknesses, threats, and opportunities have been analysed using the SWOT/CAME matrix. Finally, an Action Plan has been developed that identifies strategic pillars, objectives, and actions to be implemented.

Results

Five Strategic Pillars have been identified (the SEN’s Image; Service Portfolio; Participatory Spaces; Digital Transformation; Results-Oriented Management), comprising a total of 23 strategic objectives. A total of 80 actions are proposed to achieve the Plan’s objectives by 2025.

Conclusions

The deployment of the Strategic Plan involves having a backbone instrument for the strategic lines that are expected to favour the position of the SEN as a key player within the specialty of neurology in the face of current and future challenges.

前言：为了重新设计促进和促进神经学进步的角色，并预测我们专业发展所提供的社会、科学、健康和经济背景，西班牙神经学学会决定在战略计划中正式确定其方向，本文将分享其要素。方法：规划的制定分为三个阶段：内部和外部分析、战略规划和规划的正式制定。在大约500名成员和该领域其他专业人员的参与下，通过调查、访谈和与环境局局长的参与性会议，采用了定性和战略分析方法。SEN及其环境的现状已经明确陈述，企业形象已经定义，优势，劣势，威胁和机会已经使用SWOT/CAME矩阵进行了分析。最后，制定了一项行动计划，确定了战略支柱、目标和要实施的行动。结果：确定了五个战略支柱（环境局的形象、服务组合、参与式空间、数字化转型、结果导向管理），共包括23个战略目标。为到2025年实现该计划的目标，共提出了80项行动。结论：战略计划的部署涉及为战略线提供一个骨干工具，预计将有利于SEN在面对当前和未来挑战时作为神经病学专业的关键参与者的地位。

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引用次数: 0

Daytime dysfunction and SUDEP risk: Exploring the role of sleep and heart rate variability in epilepsy 白天功能障碍和猝死风险：探索睡眠和心率变异性在癫痫中的作用。

Neurologia

Pub Date : 2026-03-01 Epub Date: 2026-02-27 DOI: 10.1016/j.nrleng.2026.501940

Z. Shao , Z. Hu , R. Tang , X. Wang , W. Peng

Objective

This study aimed to investigate the interrelationship between sleep disturbances, heart rate variability (HRV), and the risk of sudden unexpected death in epilepsy (SUDEP), with a focus on identifying novel risk factors related to sleep and autonomic function.

Methods

Patients with epilepsy undergoing overnight video-electroencephalographic monitoring were recruited between December 2020 and June 2022. Seizure-related characteristics were collected. Subjective and objective sleep quality were assessed using the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), duration of the first and longest cycle of the whole night's sleep (cycle 1 duration), sleep latency, and number of arousals from lights-off to the start of cycle 1 (AR number). Autonomic function was evaluated using HRV parameters, including root mean square of successive differences (RMSSD) and the percentage of NN50 intervals (PNN50), during non-rapid eye movement (NREM) sleep stages 2 and 3. The risk of SUDEP was measured using the 7-item SUDEP inventory (SUDEP-7).

Results

A total of 127 patients with epilepsy were included in the study. Patients with a cycle 1 duration of less than 60 min had significantly higher PSQI scores (t test, P < .05) compared to those with a cycle 1 duration of 60 min or more, consistent with poorer subjective sleep quality. Daytime dysfunction (reflected in the 7th domain of the PSQI) and RMSSD and PNN50 during NREM sleep stage 3 (one of the HRV time domain parameters) were both independent risk factors for SUDEP. Although no positive mediation effect of HRV on daytime dysfunction and SUDEP risk was observed, an inverse relationship between RMSSD during NREM stage 3 and both outcomes was identified.

Conclusion

Patients with epilepsy (and especially those with generalised tonic–clonic seizures) exhibiting daytime dysfunction should be prioritised for SUDEP risk screening. Further research should explore additional factors beyond HRV and potential interventions targeting sleep and autonomic function in patients with epilepsy.

目的：本研究旨在探讨睡眠障碍、心率变异性（HRV）和癫痫猝死（SUDEP）风险之间的相互关系，重点发现与睡眠和自主神经功能相关的新危险因素。方法：招募2020年12月至2022年6月期间接受夜间视频脑电图监测的癫痫患者。收集癫痫相关特征。采用匹兹堡睡眠质量指数（PSQI）、Epworth嗜睡量表（ESS）、整晚睡眠的第一个和最长周期的持续时间（周期1持续时间）、睡眠潜伏期和从关灯到周期1开始的唤醒次数（AR数）来评估主观和客观睡眠质量。在非快速眼动（NREM）睡眠阶段2和3，使用HRV参数，包括连续差的均方根（RMSSD）和NN50间隔的百分比（PNN50）来评估自主神经功能。采用7项SUDEP量表（SUDEP-7）测量发生SUDEP的风险。结果：共纳入127例癫痫患者。第1周期持续时间小于60min的患者PSQI评分明显较高（t检验，p）。结论：表现出白天功能障碍的癫痫患者（尤其是全身性强直-阵挛性发作患者）应优先进行SUDEP风险筛查。进一步的研究应该探索心率变异以外的其他因素，以及针对癫痫患者睡眠和自主神经功能的潜在干预措施。

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引用次数: 0

Alcoholic Wernicke's encephalopathy with cranial neuropathies, atypical neuroimaging, dry beriberi, and Graves’ disease: A novel variant? 酒精性韦尼克脑病伴颅神经病变、非典型神经影像学、干性脚气和格雷夫斯病：一种新的变异？

Neurologia

Pub Date : 2026-03-01 Epub Date: 2026-02-27 DOI: 10.1016/j.nrleng.2026.101892

A. Nag , M. León-Ruiz , K. Bole , R. Ghosh , D. Naga , J. Benito-León

引用次数: 0

Motor band sign in 18F-FDG PET/CT studies: a biomarker of degenerative upper motor neuron disease? A study of three cases and literature review 18F-FDG PET/CT研究中的运动带征：退行性上运动神经元疾病的生物标志物？三个案例的研究及文献综述。

Neurologia

Pub Date : 2026-03-01 Epub Date: 2025-12-25 DOI: 10.1016/j.nrleng.2025.501931

Ruiz-Ortiz M. , Esteban-Pérez J. , Gómez-Grande A. , Martínez-Albero E. , Benito-León J.

Introduction

Motor neuron diseases (MND) encompass conditions like amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS), marked by progressive degeneration of upper and/or lower motor neurons. The identification of specific biomarkers is crucial to reduce diagnostic delays.

Methods

This study presents three clinical cases evaluated at the Hospital Universitario 12 de Octubre, where the motor band sign on brain 18 F-FDG PET/CT aided the diagnosis of MND. The studies were conducted using a SIEMENS Biograph True Point 6, with a review of relevant literature.

Results

In all three patients, PET/CT revealed hypometabolism in the prerolandic region, indicative of the motor band sign, contributing to the diagnosis of PLS or ALS.

Discussion

The motor band sign on 18F-FDG PET/CT emerges as a potential marker of upper motor neuron involvement, though the heterogeneity of MNDs and variability across studies call for further research to establish its specificity and sensitivity.

Conclusion

The motor band sign on 18F-FDG PET/CT is a promising biomarker for MNDs, although further studies are required to confirm its diagnostic validity.

运动神经元疾病（MND）包括肌萎缩性侧索硬化症（ALS）和原发性侧索硬化症（PLS），其特征是上部和/或下部运动神经元进行性变性。识别特定的生物标志物对于减少诊断延误至关重要。方法：本研究报告了10月12日在Universitario医院评估的3例临床病例，其中脑18f - fdg PET/CT运动带征象辅助诊断MND。研究使用西门子传记真实点6进行，并回顾了相关文献。结果：3例患者PET/CT均显示前皮质区代谢低下，提示运动带征，有助于PLS或ALS的诊断。讨论：18 F-FDG PET/CT上的运动带征象作为上运动神经元受累的潜在标志，尽管mnd的异质性和不同研究的可变性需要进一步研究以确定其特异性和敏感性。结论：18 F-FDG PET/CT上的运动带征是一种很有前景的mnd生物标志物，尽管需要进一步的研究来证实其诊断有效性。

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