Neurologia最新文献_第2页

Epidemiología y clínica de las crisis de ausencias frontales frente a las ausencias primarias 初级缺勤危机与初级缺勤危机的流行病学和临床

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-09-08 DOI: 10.1016/j.nrl.2023.11.014

J.M. Ramos-Fernández , A. Extraviz Moreno , N. del Arco Guzman , R. Calvo Medina , L. Rodríguez Santos , P. Navas Sánchez

Introduction

Absence seizures due to secondary bilateral synchrony may represent a manifestation of frontal seizures, indistinguishable from primary absence epilepsy, but with different prognosis and management. There are limited epidemiological studies on frontal absences epilepsy (FAE) within childhood absence epilepsy.

Objective

To describe the epidemiology of FAE in childhood and compare the characteristics, clinical progression, and pharmacological response with primary absence epilepsy.

Patients and methods

A retrospective study was conducted on children under 14 years diagnosed with absence epilepsy between 2013 and 2022 at a tertiary hospital. Demographic data, number, duration and types of associated seizures, presence of frontal EEG focality with or without relation to generalised discharges, pharmacological response, and neuroimaging findings were comparatively analysed.

Results

A total of 94 patients with absence epilepsy were included, with a median age of 8.6 years (range: 6-10.1 years; 49 females/45 males). Of these, 84% presented exclusively with absence seizures. Hyperventilation induced seizures in 94.2%, while photoparoxysmal responses occurred in 5.3%. EEG focal activity was observed in 63/94 patients, and in 45/94, it was frontal. In 14/94 cases (14.8%), frontal focal activity preceded the generalised spike-wave discharge. Bivariate analysis showed no significant differences in age, time to consultation, psychomotor development or behavioural alterations, association with other seizure types, or seizure triggers. However, the number of absences per day was significantly lower in FAE patients (p = 0.004), and the need for combination therapy was higher in both bivariate (p = 0.005) and multivariate analyses (p = 0.035).

Conclusions

FAE represents a substantial subset of absence epilepsy with seizures identical in morphology, age at onset, and duration, but characterised by fewer seizures per day and poorer treatment response.

继发性双侧同步引起的失神癫痫可能是额部癫痫的一种表现，与原发性失神癫痫难以区分，但预后和处理方法不同。关于儿童缺失性癫痫中额叶缺失性癫痫（FAE）的流行病学研究有限。目的探讨儿童期FAE的流行病学特点，并将其与原发性缺失性癫痫的临床进展及药理反应进行比较。患者与方法回顾性研究某三级医院2013 ~ 2022年14岁以下诊断为缺失性癫痫的患儿。比较分析了人口统计学数据、相关癫痫发作的次数、持续时间和类型、额叶脑电图灶灶的存在与全身放电、药物反应和神经影像学结果的关系。结果本组共纳入94例缺失性癫痫患者，中位年龄8.6岁（6 ~ 10.1岁），女性49例，男性45例。其中，84%只表现为失神发作。过度通气引起的癫痫发作占94.2%，而发生光性发作反应的占5.3%。脑电图局灶性活动63/94例，45/94例位于额叶。在14/94例（14.8%）病例中，额叶局灶活动先于广泛的尖峰波放电。双变量分析显示，在年龄、就诊时间、精神运动发展或行为改变、与其他癫痫类型的关联或癫痫触发因素方面无显著差异。然而，FAE患者每天缺勤的次数明显较低（p = 0.004），双变量分析（p = 0.005）和多变量分析（p = 0.035）中，联合治疗的需求都较高。结论fae代表了缺失癫痫的一个重要子集，其发作在形态、发病年龄和持续时间上相同，但其特征是每天发作次数较少，治疗反应较差。

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引用次数: 0

Unravelling the temporal evolution of Parkinson's disease mortality in Spanish autonomous communities (1999–2021) 揭示西班牙自治区帕金森病死亡率的时间演变（1999-2021）

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-07-24 DOI: 10.1016/j.nrl.2025.501911

L. Cayuela , E. Zapata-Arriaza , A. de Albóniga-Chindurza , A. González García , A. Cayuela

Objective

This observational retrospective study aimed to analyse the mortality trends of Parkinson's disease (PD) in Spain from 1999 to 2021, by autonomous community and sex.

Methods

The study collected PD mortality data from 1999 to 2021 from the National Statistics Institute of Spain. Age-standardised mortality rates were analysed by sex and age group, using joinpoint analysis to identify significant trends.

Results

Over the study period, 72 907 deaths due to PD were recorded in Spain, with 51% occurring in men and 49% in women. Both men and women showed a consistent upward trend in PD mortality rates at the national level, with annual increases of 2.1% for men and 2.0% for women. Joinpoint analysis revealed significant changes in trends within specific regions. Among men, rates in Catalonia stabilised after an initial increase, whereas Madrid showed a significant increase after a period of stability. Conversely, La Rioja exhibited a notable decrease after a period of increase. Similar patterns were observed for women, with certain regions showing stable rates while others displayed an upward trend. Cantabria, Galicia, and Valencia stabilised after an initial significant increase, Madrid showed a significant increase after a period of stability, and Andalusia demonstrated an acceleration after an initial increase.

Conclusions

Our findings underscore the significance of monitoring PD mortality trends and their varying impact across different regions and sexes. This valuable information can play a crucial role in shaping future healthcare policies and designing effective prevention strategies.

目的：本观察性回顾性研究旨在分析1999年至2021年西班牙帕金森病（PD）死亡率趋势，按自治区和性别分列。方法收集西班牙国家统计局1999年至2021年PD死亡率数据。按性别和年龄组分析了年龄标准化死亡率，采用联结点分析确定了重要趋势。结果在研究期间，西班牙共有72907人死于帕金森病，其中51%为男性，49%为女性。在全国范围内，男性和女性PD死亡率均呈现持续上升趋势，男性每年增长2.1%，女性每年增长2.0%。联合点分析揭示了特定区域内的显著变化趋势。在男性中，加泰罗尼亚的发病率在最初的上升后趋于稳定，而马德里在一段时间的稳定后出现了显著的增长。相反，拉里奥哈在一段时间的增长后出现了显著的下降。在女性身上也观察到了类似的模式，某些地区的发病率保持稳定，而其他地区则呈现上升趋势。坎塔布里亚、加利西亚和瓦伦西亚在最初的显著增长后趋于稳定，马德里在一段时间的稳定后出现了显著增长，安达卢西亚在最初的增长后出现了加速。结论我们的研究结果强调了监测PD死亡率趋势的重要性及其在不同地区和性别之间的不同影响。这些有价值的信息可以在制定未来的医疗保健政策和设计有效的预防战略方面发挥关键作用。

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引用次数: 0

Heterogeneidad fenotípica y genotípica de una serie de casos con mutaciones asociadas a distonía que responde a la dopa 与兴奋剂反应肌张力障碍相关的一系列病例的表型和基因异质性

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-08-21 DOI: 10.1016/j.nrl.2023.09.003

I. García Díaz , E. Ortegón Aguilar , G. Fernández Pajarín , I. Cabo López , D. Apolinar García Estevez , B. Ares Pensado , Á. Sesar Ignacio

Introduction

Dopa-responsive dystonia (DRD) includes a group of neurological disorders that manifest with lower limb dystonia and an excellent response to levodopa. Parkinsonism is commonly associated with DRD, and many cases of GCH1 gene mutations without dystonia but with evidence of nigrostriatal pathway degeneration, indistinguishable to idiopathic Parkinson's disease (PD), have been recently described. This phenotypic variability has been observed even among members of the same family.

Method

Clinical and genetic description of 14 patients from 4 different families with DRD associated mutations.

Results

The results showed GCH1 mutations in three of the four families, while one family had a mutation in the SPR gene. Within patients with GCH1 mutations, two showed DRD symptoms, five had PD phenotype, and four were asymptomatic. The only patient with SPR mutation clinically displayed symptoms of dystonia and parkinsonism. DaTscan® revealed degeneration of the nigroestriatal pathway in two PD patients. Furthermore, phenotypic variability of the same mutation was observed in two families.

Conclusions

DRD associated mutations have a heterogeneous clinical expression GCH1 gene mutations can be manifested with PD phenotype along with the degeneration of the nigroestiatal pathway. Therefore, GCH1 gene testing might be useful in cases of PD suggestive of genetic origin or with family history of dystonia.

多巴反应性肌张力障碍（DRD）包括一组神经系统疾病，表现为下肢肌张力障碍和对左旋多巴的良好反应。帕金森病通常与DRD相关，许多GCH1基因突变没有肌张力障碍，但有黑质纹状体通路变性的证据，与特发性帕金森病（PD）难以区分，最近已被描述。甚至在同一家族的成员中也观察到这种表型变异。方法对来自4个不同家族的14例DRD相关突变患者进行临床和遗传描述。结果4个家族中有3个家族存在GCH1突变，1个家族存在SPR基因突变。GCH1突变患者中，2例出现DRD症状，5例出现PD表型，4例无症状。唯一的SPR突变患者临床表现为肌张力障碍和帕金森病。DaTscan®显示两名PD患者的黑质estrial通路变性。此外，在两个家族中观察到相同突变的表型变异性。结论drd相关突变具有异质性临床表达，GCH1基因突变可表现为PD表型，并伴有黑质通道变性。因此，GCH1基因检测可能对提示遗传起源的PD或有肌张力障碍家族史的PD有用。

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引用次数: 0

Association between comorbidities and health-related quality of life in patients with multiple sclerosis 多发性硬化症患者合并症与健康相关生活质量的关系

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-09-09 DOI: 10.1016/j.nrl.2024.03.002

O. Mirmosayyeb , M. Barzegar , M. Rezaei , S. Houshi , A. Afshari-Safavi , M. Falahatian , N. Nehzat , V. Shaygannejad

Purpose

Multiple sclerosis (MS) is a debilitating disease accompanied by physical and mental comorbidities. Little is known about the relation between different comorbidities and health-related quality of life (HRQOL) in people with MS (pwMS). Therefore, we designed this study to assess the association between comorbidities and HRQOL.

Methods

In this cross-sectional study, of 976 pwMS attending the MS clinic of Kashani Hospital in Isfahan, Iran were assessed. The data on comorbidity were extracted from patients’ medical records. The 36-Item Short Form Survey (SF-36) was used to measure HRQOL. Firstly, the association between each comorbidity and HRQOL was assessed. Then, the comorbidities were categorized into physical, psychiatric, and autoimmune, and the association of each comorbidity group with HRQOL was evaluated.

Results

The mean (SD) age and disease duration were 37.58 (9.22) and 7.41 (5.24); most of them were female (82.8%) and had a relapsing course (77.1%). The most common comorbidity was migraine (13.6%), followed by hypothyroidism (13.5%), obsessive–compulsive disorder (OCD) (13.5%), and anemia (11.5%). There was a significant association between the physical component score (PCS) of HRQOL and reduced epilepsy, coronary artery disease, eye diseases, OCD, major depressive disorder (MDD), and borderline personality disorder. Regarding mental component (MCS), ovarian failure, polycystic ovary syndrome, OCD, and MDD had an association with low MCS. After categorization, both physical and psychiatric comorbidities were related to less PCS and MCS score. However, no significant association between autoimmune comorbidities and HRQOL was found.

Conclusion

Our results show a significant association between comorbidities and HRQOL in MS patients.

目的多发性硬化症（MS）是一种伴有身体和精神合并症的衰弱性疾病。对于多发性硬化症（pwMS）患者不同合并症与健康相关生活质量（HRQOL）之间的关系知之甚少。因此，我们设计了这项研究来评估合并症与HRQOL之间的关系。方法对在伊朗伊斯法罕Kashani医院MS门诊就诊的976例pwMS患者进行横断面研究。合并症数据从患者的医疗记录中提取。采用36项短表问卷（SF-36）测量HRQOL。首先，评估各合并症与HRQOL的关系。然后，将合并症分为身体、精神和自身免疫，并评估每个合并症组与HRQOL的关系。结果平均（SD）年龄为37.58岁（9.22岁），病程为7.41岁（5.24岁）；其中以女性居多（82.8%），有复发期（77.1%）。最常见的合并症是偏头痛（13.6%），其次是甲状腺功能减退（13.5%）、强迫症（13.5%）和贫血（11.5%）。HRQOL生理成分评分（PCS）与癫痫、冠心病、眼病、强迫症、重度抑郁障碍（MDD）、边缘型人格障碍发生率显著相关。关于精神成分（MCS），卵巢功能衰竭、多囊卵巢综合征、强迫症和重度抑郁症与MCS低有关。经分类，躯体和精神合并症均与PCS和MCS评分降低有关。然而，没有发现自身免疫合并症与HRQOL之间的显著关联。结论MS患者合并症与HRQOL之间存在显著相关性。

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引用次数: 0

Impact of a perioperative protocol for Parkinson's disease patients on use of contraindicated drugs 帕金森病患者围手术期方案对禁忌症药物使用的影响

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-08-21 DOI: 10.1016/j.nrl.2023.11.011

P. Salgado-Cámara , B. De la Casa-Fages , A. Sánchez-Soblechero , O. Mateo Sierra , M.L. Martín-Barbero , F. Grandas

Introduction

During surgical admissions, the use of antidopaminergic drugs is associated with increased morbimortality and hospital stay in patients with Parkinson's disease (PD) and other parkinsonisms. We implemented a protocol to ensure adequate perioperative pharmacological management in our center, including educational sessions and complementary tools, such as an electronic Pharmacological Alert and a Patient Information Sheet.

Objective

The objective of this study was to analyze the changes in the prescription of contraindicated medications to PD or parkinsonism patients admitted for surgery, in the three years following implementation of the protocol, and to establish the compliance with the protocol among the medical staff involved in their hospital care over the same period.

Methods

This is an observational, analytical, prospective study with a before–after design.

Results

Prescription of contraindicated drugs decreased significantly over the study period (from 57.8% to 20.6%; p < 0.001). Patients without an activated Pharmacological Alert had a 13.31 times higher risk of being prescribed contraindicated drugs than patients with such an alert (p < 0.001). Neurologists and neurosurgeons used the protocol tools more than Emergency Department surgeons. There was very high compliance with the protocol in admissions for deep brain stimulation and related surgeries.

Conclusions

The protocol reduced the prescription of contraindicated drugs to PD and parkinsonism patients during their surgical admissions in our center. The Pharmacological Alert and Neurology clinics played a crucial role.

在手术入院期间，抗多巴胺能药物的使用与帕金森病（PD）和其他帕金森病患者的死亡率和住院时间增加有关。我们实施了一项方案，以确保我们中心有足够的围手术期药理学管理，包括教育会议和补充工具，如电子药理学警报和患者信息表。目的本研究的目的是分析PD或帕金森病患者手术后三年内禁忌症药物处方的变化，并确定同一时期参与医院护理的医务人员对该方案的依从性。方法本研究为观察性、分析性、前瞻性研究，采用事前-事后设计。结果研究期间禁忌症药物的处方率明显下降（从57.8%降至20.6%,p < 0.001）。未激活药理学警报的患者处方禁忌症药物的风险比具有此类警报的患者高13.31倍（p < 0.001）。神经科医生和神经外科医生比急诊科医生更多地使用协议工具。脑深部刺激及相关手术的住院患者对该方案的依从性非常高。结论该方案减少了PD和帕金森患者在我中心手术住院期间的禁忌症药物处方。药理学警报和神经病学诊所发挥了至关重要的作用。

{"title":"Impact of a perioperative protocol for Parkinson's disease patients on use of contraindicated drugs","authors":"P. Salgado-Cámara , B. De la Casa-Fages , A. Sánchez-Soblechero , O. Mateo Sierra , M.L. Martín-Barbero , F. Grandas","doi":"10.1016/j.nrl.2023.11.011","DOIUrl":"10.1016/j.nrl.2023.11.011","url":null,"abstract":"<div><h3>Introduction</h3><div>During surgical admissions, the use of antidopaminergic drugs is associated with increased morbimortality and hospital stay in patients with Parkinson's disease (PD) and other parkinsonisms. We implemented a protocol to ensure adequate perioperative pharmacological management in our center, including educational sessions and complementary tools, such as an electronic Pharmacological Alert and a Patient Information Sheet.</div></div><div><h3>Objective</h3><div>The objective of this study was to analyze the changes in the prescription of contraindicated medications to PD or parkinsonism patients admitted for surgery, in the three years following implementation of the protocol, and to establish the compliance with the protocol among the medical staff involved in their hospital care over the same period.</div></div><div><h3>Methods</h3><div>This is an observational, analytical, prospective study with a before–after design.</div></div><div><h3>Results</h3><div>Prescription of contraindicated drugs decreased significantly over the study period (from 57.8% to 20.6%; <em>p</em>  <0.001). Neurologists and neurosurgeons used the protocol tools more than Emergency Department surgeons. There was very high compliance with the protocol in admissions for deep brain stimulation and related surgeries.</div></div><div><h3>Conclusions</h3><div>The protocol reduced the prescription of contraindicated drugs to PD and parkinsonism patients during their surgical admissions in our center. The Pharmacological Alert and Neurology clinics played a crucial role.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 101897"},"PeriodicalIF":3.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886406","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

Sleep disturbances in patients with myasthenia gravis: A cross-sectional study 重症肌无力患者的睡眠障碍：一项横断面研究

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-10-07 DOI: 10.1016/j.nrl.2025.501925

J. Yan , K. Choi , P. Fu , J. Lin , M. Gui , Y. Li , L. Luo , Z. Hu , B. Bu , Z. Li

Objective

To explore sleep dysfunction in clinically stable patients with myasthenia gravis (MG) and to identify sleep disturbances and uncover their associated risk factors.

Methods

A cross-sectional study was conducted, involving the recruitment of 306 patients with MG from three MG centers. Participants completed an online self-report questionnaire covering demographic variables, clinical characteristics, and assessments using the Pittsburgh Sleep Quality Index (PSQI) scale, STOP-Bang scale, Myasthenia Gravis Quality of Life 15 (MG-QOL 15) scale, Patient Health Questionnaire (PHQ-9), and Self-Rating Anxiety Scale (SAS), to evaluate sleep quality among patients with MG.

Results

Approximately 68% of patients with MG presented sleep disturbances (PSQI ≥6). Univariate analysis revealed that age, lower education level (≤12 years), being single, late disease onset (>55 years old), generalized subtype, myasthenia crisis, positivity for AChR antibodies, thymoma, thymectomy, and type B thymoma were risk factors for sleep dysfunction in patients with MG. Within the sleep disturbances group, 51% of patients scored ≥3 on the STOP-Bang scale, indicating a higher risk of obstructive sleep apnea. PSQI global scores showed significant linear correlations with MG-QOL 15, STOP-Bang, PHQ-9, and SAS scores (P < .001). Multivariate analysis revealed that sex, marital status, STOP-Bang score, SAS score, and MG-QOL 15 score were correlated with the PSQI score.

Conclusion

Sleep disturbances are prevalent among patients with MG, even in clinically stable cases. Psychological factors such as anxiety and health-related quality of life warrant increased attention in the management of these patients.

目的探讨临床稳定型重症肌无力（MG）患者的睡眠障碍，识别睡眠障碍并揭示其相关危险因素。方法采用横断面研究，从3个MG中心招募306例MG患者。参与者完成了一份在线自我报告问卷，包括人口统计变量、临床特征，并使用匹兹堡睡眠质量指数（PSQI）量表、STOP-Bang量表、重症肌无力生活质量15 （MG- qol 15）量表、患者健康问卷（PHQ-9）和自评焦虑量表（SAS）进行评估，以评估重症肌无力患者的睡眠质量。结果约68%的MG患者出现睡眠障碍（PSQI≥6）。单因素分析显示，年龄、低文化程度（≤12年）、单身、发病晚（55岁）、广义亚型、重症肌无力危象、AChR抗体阳性、胸腺瘤、胸腺切除术、B型胸腺瘤是MG患者睡眠障碍的危险因素。在睡眠障碍组中，51%的患者STOP-Bang评分≥3分，表明阻塞性睡眠呼吸暂停的风险较高。PSQI整体评分与MG-QOL 15、STOP-Bang、PHQ-9和SAS评分呈显著的线性相关（P < .001）。多因素分析显示，性别、婚姻状况、STOP-Bang评分、SAS评分、MG-QOL 15评分与PSQI评分相关。结论睡眠障碍在MG患者中普遍存在，即使在临床稳定的病例中也是如此。心理因素，如焦虑和健康相关的生活质量，需要在这些患者的管理中增加关注。

{"title":"Sleep disturbances in patients with myasthenia gravis: A cross-sectional study","authors":"J. Yan , K. Choi , P. Fu , J. Lin , M. Gui , Y. Li , L. Luo , Z. Hu , B. Bu , Z. Li","doi":"10.1016/j.nrl.2025.501925","DOIUrl":"10.1016/j.nrl.2025.501925","url":null,"abstract":"<div><h3>Objective</h3><div>To explore sleep dysfunction in clinically stable patients with myasthenia gravis (MG) and to identify sleep disturbances and uncover their associated risk factors.</div></div><div><h3>Methods</h3><div>A cross-sectional study was conducted, involving the recruitment of 306 patients with MG from three MG centers. Participants completed an online self-report questionnaire covering demographic variables, clinical characteristics, and assessments using the Pittsburgh Sleep Quality Index (PSQI) scale, STOP-Bang scale, Myasthenia Gravis Quality of Life 15 (MG-QOL 15) scale, Patient Health Questionnaire (PHQ-9), and Self-Rating Anxiety Scale (SAS), to evaluate sleep quality among patients with MG.</div></div><div><h3>Results</h3><div>Approximately 68% of patients with MG presented sleep disturbances (PSQI ≥6). Univariate analysis revealed that age, lower education level (≤12 years), being single, late disease onset (>55 years old), generalized subtype, myasthenia crisis, positivity for AChR antibodies, thymoma, thymectomy, and type B thymoma were risk factors for sleep dysfunction in patients with MG. Within the sleep disturbances group, 51% of patients scored ≥3 on the STOP-Bang scale, indicating a higher risk of obstructive sleep apnea. PSQI global scores showed significant linear correlations with MG-QOL 15, STOP-Bang, PHQ-9, and SAS scores (<em>P</em>  <!-->.001). Multivariate analysis revealed that sex, marital status, STOP-Bang score, SAS score, and MG-QOL 15 score were correlated with the PSQI score.</div></div><div><h3>Conclusion</h3><div>Sleep disturbances are prevalent among patients with MG, even in clinically stable cases. Psychological factors such as anxiety and health-related quality of life warrant increased attention in the management of these patients.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 501925"},"PeriodicalIF":3.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886483","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

Brueghel syndrome: A missing entity. Case report 勃鲁盖尔综合症：缺失的实体。病例报告

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-03-19 DOI: 10.1016/j.nrl.2025.02.001

D. López Dominguez , G. Álvarez Bravo

引用次数: 0

Neuropatía del VI nervio craneal debido a ecchordosis physaliphora: revisión de la literatura a propósito de un caso 因体脂性棘球蚴病引起的VI颅神经病变：一个病例的文献综述

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2026-01-01 Epub Date: 2025-02-17 DOI: 10.1016/j.nrl.2023.07.003

V. Fernández-Rodríguez, J.M. Losada Domingo, A. Moreno-Estébanez, A. Rebollo Pérez

引用次数: 0

Adaptación y baremación de la versión española del Attention, Memory, and Frontal Abilities Screening Test (AMFAST) en población mayor de 55 años 针对55岁以上人群的注意力、记忆和正面能力筛查测试（AMFAST）西班牙版的调整和校准

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2025-11-01 Epub Date: 2024-11-25 DOI: 10.1016/j.nrl.2023.03.010

J. Oltra-Cucarella , B. Bonete-López , E. Sitges-Maciá , C. Iñesta , M. Sánchez San-Segundo , M. Berbegal Bernabeu , M. Tomé Fernández , A. Zaragoza-Martí , B. Freilich , J.A. Hurtado Sánchez

Introduction

Screening tests are useful to identify cognitive impairments during aging. However, they need to assess different cognitive abilities and be easily accessible to researchers and clinicians. The objective of this work is to develop normative data for the population 55 years of age or older for the Attention, Memory, and Frontal Abilities Screening Test (AMFAST).

Method

One-hundred and fifty-five cognitively healthy participants between 55 and 82 years old were assessed both with a comprehensive neuropsychological battery and the AMFAST. The ability of the AMFAST to identify objective cognitive impairment in the neuropsychological assessment was analyzed using binary logistic regression, and sensitivity, specificity, and positive and negative predictive values were calculated. Normative data were developed using linear regression, controlling for the effects of age, gender, and educational level.

Results

The AMFAST total score was statistically associated with age and education, but not with sex. Using four or more low scores as the criterion for objective cognitive impairment, the AMFAST total score was associated with the number of low scores on the neuropsychological battery (r = −.33, p < .001), as well as with objective cognitive impairment (OR = 0.95, 95% CI: 0.92–0.98, p = .003). A total score lower than 74 was associated with sensitivity = 85.71%, specificity = 71.63%, positive predictive value = 23.08%, and negative predictive value = 98.06%.

Conclusions

As a simple and quick test, the AMFAST could help identify early objective cognitive impairment. Normative data of the Spanish adaptation of the AMFAST for its use in clinical and research are provided.

筛选测试对识别衰老过程中的认知障碍是有用的。然而，它们需要评估不同的认知能力，并便于研究人员和临床医生使用。这项工作的目的是为55岁或以上的人群制定注意、记忆和额叶能力筛选测试（AMFAST）的规范数据。方法：155名年龄在55岁到82岁之间认知健康的参与者通过综合神经心理学测试和AMFAST进行评估。采用二元logistic回归分析AMFAST在神经心理学评估中识别客观认知障碍的能力，并计算敏感性、特异性、阳性预测值和阴性预测值。使用线性回归开发规范数据，控制年龄，性别和教育水平的影响。结果AMFAST总分与年龄、受教育程度相关，与性别无关。以4个及以上低分作为客观认知障碍的标准，AMFAST总分与神经心理电池低分数相关（r =−）。33, p < .001)，以及客观认知障碍（OR = 0.95, 95% CI: 0.92-0.98, p = .003）。总分低于74分的敏感性为85.71%，特异性为71.63%，阳性预测值为23.08%，阴性预测值为98.06%。结论AMFAST是一种简单、快速的检测方法，可以早期发现客观认知障碍。提供了西班牙适应AMFAST用于临床和研究的规范性数据。

{"title":"Adaptación y baremación de la versión española del Attention, Memory, and Frontal Abilities Screening Test (AMFAST) en población mayor de 55 años","authors":"J. Oltra-Cucarella , B. Bonete-López , E. Sitges-Maciá , C. Iñesta , M. Sánchez San-Segundo , M. Berbegal Bernabeu , M. Tomé Fernández , A. Zaragoza-Martí , B. Freilich , J.A. Hurtado Sánchez","doi":"10.1016/j.nrl.2023.03.010","DOIUrl":"10.1016/j.nrl.2023.03.010","url":null,"abstract":"<div><h3>Introduction</h3><div>Screening tests are useful to identify cognitive impairments during aging. However, they need to assess different cognitive abilities and be easily accessible to researchers and clinicians. The objective of this work is to develop normative data for the population 55 years of age or older for the Attention, Memory, and Frontal Abilities Screening Test (AMFAST).</div></div><div><h3>Method</h3><div>One-hundred and fifty-five cognitively healthy participants between 55 and 82 years old were assessed both with a comprehensive neuropsychological battery and the AMFAST. The ability of the AMFAST to identify objective cognitive impairment in the neuropsychological assessment was analyzed using binary logistic regression, and sensitivity, specificity, and positive and negative predictive values were calculated. Normative data were developed using linear regression, controlling for the effects of age, gender, and educational level.</div></div><div><h3>Results</h3><div>The AMFAST total score was statistically associated with age and education, but not with sex. Using four or more low scores as the criterion for objective cognitive impairment, the AMFAST total score was associated with the number of low scores on the neuropsychological battery (<em>r</em>  <.001), as well as with objective cognitive impairment (OR=0.95, 95% CI: 0.92–0.98, <em>p</em>          <!-->98.06%.</div></div><div><h3>Conclusions</h3><div>As a simple and quick test, the AMFAST could help identify early objective cognitive impairment. Normative data of the Spanish adaptation of the AMFAST for its use in clinical and research are provided.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 9","pages":"Pages 813-821"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145425495","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

Serie de casos de epilepsia con mioclonías palpebrales 伴有脑瘫肌阵挛的癫痫系列

IF 3.1 4区医学 Q2 CLINICAL NEUROLOGY

Neurologia

Pub Date : 2025-11-01 Epub Date: 2024-11-19 DOI: 10.1016/j.nrl.2023.04.009

M. Henao-Pérez , D.C. López-Medina , A. Arboleda-Ramírez , N.L. Patiño-Jaramillo , J.F. Zapata-Berruecos , R. Andrade

Introduction

Epilepsy with palpebral myoclonus is an idiopathic generalized childhood epilepsy, which is underdiagnosed because it is confused with tics or behavioral actions.

Patients and methods

Quantitative, observational, descriptive of 16 patients with epilepsy and myoclonic eyelids treated at an institution specialized in neurology, between the years 2017 and 2022. Clinical histories and videoelectroencephalograms were evaluated.

Results

Of 16 patients, 11 were women (68.8%), the median age was 17.5 years (IQR 12.5). The first diagnosis most frequently received by patients was idiopathic generalized epilepsy. Stressful situations were the most reported precipitant. 93.75% of the patients initially presented palpebral myoclonic seizures and after several years presented generalized tonic-clonic seizures. The time elapsed between the first seizure and the diagnosis varied between 1 and more than 40 years, being greater among patients with a subsidized health system. Men had a high frequency of family history of epilepsy in the first and second degrees of consanguinity, had a longer delay in diagnosis and reported greater drug resistance. In both women and men, the posterior regions (occipital, temporoccipital and parietoccipital) presented a greater focal epileptiform activity. The photoparoxysmal response occurred with greater predominance in women, with the Waltz type iii and iv responses being the most frequent.

Conclusion

The importance of suspecting this pathology before palpebral myoclonus in children should be highlighted, guaranteeing quality and timely management.

癫痫伴睑肌阵挛是一种特发性全身性儿童癫痫，由于与抽搐或行为动作相混淆而未得到充分诊断。患者和方法对2017年至2022年间在一家神经病学专业机构接受治疗的16例癫痫和肌阵挛性眼睑患者进行定量、观察和描述性研究。评估临床病史和脑电图。结果16例患者中，女性11例（68.8%），中位年龄17.5岁（IQR 12.5）。患者最常接受的第一个诊断是特发性全身性癫痫。有压力的情况是报告中最危险的。93.75%的患者最初表现为睑肌阵挛性发作，几年后出现全身性强直阵挛性发作。从第一次发作到诊断之间的时间从1年到40多年不等，在享受补贴医疗系统的患者中时间更长。男性一、二级血缘家族癫痫家族史发生率高，诊断延误时间长，耐药程度高。在女性和男性中，后侧区域（枕部、颞枕部和顶枕部）表现出更大的局灶性癫痫样活动。光阵发性反应在女性中更占优势，华尔兹iii型和iv型反应最常见。结论应重视患儿睑肌睫状体病变诊断，保证质量，及时处理。

{"title":"Serie de casos de epilepsia con mioclonías palpebrales","authors":"M. Henao-Pérez , D.C. López-Medina , A. Arboleda-Ramírez , N.L. Patiño-Jaramillo , J.F. Zapata-Berruecos , R. Andrade","doi":"10.1016/j.nrl.2023.04.009","DOIUrl":"10.1016/j.nrl.2023.04.009","url":null,"abstract":"<div><h3>Introduction</h3><div>Epilepsy with palpebral myoclonus is an idiopathic generalized childhood epilepsy, which is underdiagnosed because it is confused with tics or behavioral actions.</div></div><div><h3>Patients and methods</h3><div>Quantitative, observational, descriptive of 16 patients with epilepsy and myoclonic eyelids treated at an institution specialized in neurology, between the years 2017 and 2022. Clinical histories and videoelectroencephalograms were evaluated.</div></div><div><h3>Results</h3><div>Of 16 patients, 11 were women (68.8%), the median age was 17.5 years (IQR 12.5). The first diagnosis most frequently received by patients was idiopathic generalized epilepsy. Stressful situations were the most reported precipitant. 93.75% of the patients initially presented palpebral myoclonic seizures and after several years presented generalized tonic-clonic seizures. The time elapsed between the first seizure and the diagnosis varied between 1 and more than 40 years, being greater among patients with a subsidized health system. Men had a high frequency of family history of epilepsy in the first and second degrees of consanguinity, had a longer delay in diagnosis and reported greater drug resistance. In both women and men, the posterior regions (occipital, temporoccipital and parietoccipital) presented a greater focal epileptiform activity. The photoparoxysmal response occurred with greater predominance in women, with the Waltz type <span>iii</span> and <span>iv</span> responses being the most frequent.</div></div><div><h3>Conclusion</h3><div>The importance of suspecting this pathology before palpebral myoclonus in children should be highlighted, guaranteeing quality and timely management.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 9","pages":"Pages 855-863"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145425464","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