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Multiple sclerosis mortality trends by sex in Chile, 1997–2019 按性别划分的多发性硬化症死亡率趋势:智利1997-2019。
Pub Date : 2025-11-01 DOI: 10.1016/j.nrleng.2025.10.003
J. Arriagada Opazo , V. Farah González , M. González Delgadillo , L. Núñez-Franz , C. Morales Garcia , P. Jiménez Rodríguez

Background

An increasing trend has been observed in the prevalence of Multiple Sclerosis (MS) globally. It is estimated that a total of 2.8 million people live with MS worldwide. In 2008, Chile started a pilot program for the Modifying Therapy of MS, and its clinical practice guideline.

Aim

This study aims to analyse the trend of the MS mortality rate in Chile from 1997 to 2019 disaggregated by sex and latitude.

Methods

MS mortality data (ICD-10, G35) from 1997−2019 were extracted from the Department of Health Statistical Information and population data from the National Institute of Statistics. Standardized mortality rates (SMRs) by age were calculated. The trend of the SMR was analysed using Joinpoint and with RiskDiff the effect of the risk of dying, the structure, and the size of the population on the crude mortality rate was evaluated.

Results

The trend of the TME has been decreasing significantly since 2005. When disaggregating by sex the decrease was not significant. According to latitude, the north and center south have a significant decreasing trend; in the south, the decreasing trend is not significant. There is an increase of 25% in the crude mortality rate due to the change in the structure of the population and a decrease of 25.46% is attributed to the risk of dying.

Conclusion

The SMR due to MS in Chile during 1997−2019 has decreased, in addition, a decreasing SMR is evidenced only in the northern zone. The crude rate has increased as a consequence of changes in the population, however, the risk of dying has decreased, especially in men.
背景:全球多发性硬化症(MS)患病率呈上升趋势。据估计,全球共有280万人患有多发性硬化症。2008年,智利启动了多发性硬化症改良疗法的试点项目及其临床实践指南。目的:本研究旨在分析智利1997 - 2019年按性别和纬度分类的多发性硬化症死亡率趋势。方法:从卫生统计信息部和国家统计局的人口数据中提取1997-2019年MS死亡率数据(ICD-10, G35)。按年龄计算标准化死亡率(SMRs)。采用Joinpoint和RiskDiff分析了死亡风险、人口结构和人口规模对粗死亡率的影响。结果:自2005年以来,TME呈明显下降趋势。当按性别分类时,下降不显著。从纬度上看,北部和中部南部有明显的下降趋势;在南方,下降趋势不明显。由于人口结构的变化,粗死亡率增加了25%,死亡风险降低了25.46%。结论:1997-2019年智利因多发性硬化症引起的SMR呈下降趋势,且仅在北部地区有所下降。由于人口的变化,粗死亡率有所上升,然而,死亡的风险却有所下降,特别是在男性中。
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引用次数: 0
Case series of epilepsy with eyelid myoclonia 癫痫伴眼睑肌阵挛病例分析。
Pub Date : 2025-11-01 DOI: 10.1016/j.nrleng.2025.10.005
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 generalised 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 specialised 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 generalised epilepsy. Stressful situations were the most reported precipitant. 93.75% of the patients initially presented palpebral myoclonic seizures and after several years presented generalised 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 subsidised 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.
简介: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。
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引用次数: 0
Primary headaches as the reason for consultation in Primary Care: a prospective study 原发性头痛作为初级保健咨询的原因:一项前瞻性研究。
Pub Date : 2025-11-01 DOI: 10.1016/j.nrleng.2025.10.004
I. Rivera , N. Fontanillas , L. Cadelo , J. Pascual

Objectives

To prospectively study the rate of consultation, diagnoses, burden and historical management patterns of those patients attending due to primary headache in Primary Care.

Patients and methods

We included 200 patients with primary headache in a semi-urban Health Center. To study the consultation impact, 100 patients were consecutively recruited in one primary care quota. For every patient we obtained demographic data, comorbidities, rate of consultation, absenteeism, impact in quality of life (MIDAS and HIT-6 scales) and previous patterns of referral and treatment.

Results

A total of 2.2% of the demand consultations were due to primary headache. Migraine (60%) was the most frequent reason, followed by tension-type headache (38.5%) and trigeminal autonomic headaches (1.5%). In 58% of cases the diagnosis was incomplete or incorrect. Between 33% (MIDAS) and 50.8% (HIT-6) of cases showed at least a moderate decrease in their quality of life, which was higher for migraine (40.8% and 72.5%, respectively). There was absenteeism in 12% of patients the last year (in 87.5% of cases due to migraine). More than half (58%) of cases had been referred to Neurology and preventive treatments had been initiated in Primary Care in only 5.5% of patients.

Conclusions

Primary headaches, and mainly migraine, are a frequent reason for consultation in Primary Care. Even though migraine induces a significant absenteeism and damages quality of life, there is a clear room for improvement in their management. Primary headaches should be included in the health plans of Primary Care.
目的:前瞻性研究初级保健中因原发性头痛就诊患者的问诊率、诊断率、负担和历史管理模式。患者和方法:我们纳入了一家半城市卫生中心的200例原发性头痛患者。为了研究咨询的影响,在一个初级保健配额中连续招募了100名患者。我们获得了每位患者的人口统计数据、合并症、就诊率、缺勤率、对生活质量的影响(MIDAS和HIT-6量表)以及以前的转诊和治疗模式。结果:因原发性头痛就诊的占2.2%。偏头痛(60%)是最常见的原因,其次是紧张性头痛(38.5%)和三叉神经性头痛(1.5%)。在58%的病例中,诊断不完整或不正确。33% (MIDAS)至50.8% (HIT-6)的患者表现出至少中度的生活质量下降,偏头痛患者的这一比例更高(分别为40.8%和72.5%)。去年有12%的患者缺勤(其中87.5%的病例是由于偏头痛)。超过一半(58%)的病例被转介到神经科,只有5.5%的患者在初级保健中开始了预防性治疗。结论:原发性头痛,主要是偏头痛,是初级保健咨询的常见原因。尽管偏头痛会导致严重的缺勤并损害生活质量,但在治疗上仍有明显的改进空间。原发性头痛应列入初级保健保健计划。
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引用次数: 0
Evaluation of autonomic dysfunction in hereditary transthyretin amyloidosis 遗传性甲状腺转蛋白淀粉样变性患者自主神经功能障碍的评价
Pub Date : 2025-11-01 DOI: 10.1016/j.nrleng.2025.08.002
F. Martínez-Valle , C. Casasnovas-Pons , M. Romero-Acebal , L. Galán-Dávila

Background

Autonomic dysfunction is a common manifestation of hereditary transthyretin (ATTRv) amyloidosis that frequently appears early and has a major impact on disease severity, survival, and quality of life. Early detection of autonomic symptoms is paramount to avoid delayed diagnosis or misdiagnosis of ATTRv amyloidosis and thereby achieve timely interventions with novel therapies. We thus require a better understanding and recognition of the warning signs of autonomic nervous system involvement in patients with ATTRv amyloidosis.

Development

According to the literature and the expertise of key opinion leaders on ATTRv amyloidosis, early signs and symptoms of autonomic dysfunction often include orthostatic hypotension, gastrointestinal disturbances, and cardiac and sudomotor sympathetic denervation. Assessment methods for a rapid and accurate diagnostic thus comprise blood pressure monitoring, nutritional status evaluation, cardiac imaging techniques, and pathological and neurophysiological tests assessing small fiber function. The COMPASS-31 and Norfolk QOL-DN tests allow assessment of the severity of autonomic dysfunction and its impact on quality of life, respectively.

Conclusion

The screening strategy for autonomic dysfunction should involve diverse neurological and non-neurological tests to encompass the wide variety of potential manifestations of autonomic dysfunction in ATTRv amyloidosis. Further studies addressing the correlations among different tests and their prognostic value in different TTR variants are required to establish a single standardized method or the minimum set of tests needed to assess autonomic dysfunction in ATTRv amyloidosis patients.
背景:自主神经功能障碍是遗传性甲状腺转甲状腺素(ATTRv)淀粉样变的常见表现,经常出现在早期,对疾病严重程度、生存和生活质量有重要影响。自主神经症状的早期发现对于避免ATTRv淀粉样变的延迟诊断或误诊至关重要,从而及时采用新疗法进行干预。因此,我们需要更好地理解和识别自主神经系统受累的警告信号与ATTRv淀粉样变患者。根据有关ATTRv淀粉样变的文献和主要意见领袖的专业知识,自主神经功能障碍的早期体征和症状通常包括直立性低血压、胃肠道紊乱、心脏和压迫性交感神经去支配。因此,快速准确诊断的评估方法包括血压监测、营养状况评估、心脏成像技术以及评估小纤维功能的病理和神经生理测试。COMPASS-31和Norfolk QOL-DN测试分别用于评估自主神经功能障碍的严重程度及其对生活质量的影响。结论自主神经功能障碍的筛查策略应包括多种神经和非神经检查,以涵盖ATTRv淀粉样变性自主神经功能障碍的各种潜在表现。需要进一步研究不同测试之间的相关性及其在不同TTR变异中的预后价值,以建立单一的标准化方法或最少的测试集来评估ATTRv淀粉样变患者的自主神经功能障碍。
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引用次数: 0
Influence of dehydration on collateral circulation and clinical outcome after endovascular therapy in patients with acute ischemic stroke 急性缺血性脑卒中患者血管内治疗后脱水对侧支循环的影响及临床预后。
Pub Date : 2025-11-01 DOI: 10.1016/j.nrleng.2025.10.007
M. Guasch-Jiménez , P. Camps-Renom , C. Toscano-Prat , D. Guisado-Alonso , A. Martínez-Domeño , L. Prats-Sánchez , A. Ramos-Pachón , I. Fernández-Cadenas , J.P. Martínez-González , I. Fernández-Pérez , C. Avellaneda-Gómez , E. de Celis-Ruiz , J. Rodríguez-Pardo , M. del Mar Freijo , A. Luna , F. Moniche , B. Pardo-Galiana , J. Ortega-Quintanilla , J.F. Arenillas , E. Cortijo , J. Martí-Fàbregas

Introduction

Dehydration in patients with acute ischemic stroke (AIS) is associated with poor outcome. Our aim is to investigate whether dehydration is associated with collateral circulation (CC) and functional outcome in patients with AIS treated with mechanical thrombectomy (MT).

Methods

Prospective multicenter study of patients with anterior circulation AIS treated with MT (January 2020–June 2021). Dehydration was assessed with two formulas: plasma osmolarity and urea/creatinine (U/C) ratio. CC was quantified with an automated software (Brainomix Ltd.) on baseline computed tomography angiography. The primary outcome was the association between CC (expressed numerically as the percentage of change between hemispheres) and dehydration (osmolarity ≥ 295 mmol/L, U/C ≥ 80). Secondary outcomes included disability at discharge and at three months of follow-up, assessed using the modified Rankin scale (poor outcome: 3–6). Multivariable logistic and ordinal regression analyses were performed.

Results

Two hundred sixty patients were included. 65.8% were dehydrated according to osmolarity and 2.9% according to U/C. There was no association between CC score and dehydration [71% in dehydrated vs 73% in non-dehydrated; P = .875]. 64.3% of dehydrated patients vs. 46.7% of non-dehydrated patients had a poor outcome at discharge (P = .026). In multivariable logistic regression analysis, dehydration was an independent predictor of poor outcome at discharge (OR 2.50; P = .011) and at three months of follow-up (OR 2.27; P = .046).

Conclusions

Dehydration on admission is associated with poor outcome in patients with AIS treated with MT, but it is not related to CC.
急性缺血性卒中(AIS)患者脱水与不良预后相关。我们的目的是研究机械取栓(MT)治疗AIS患者的脱水是否与侧支循环(CC)和功能结局相关。方法:前瞻性多中心研究,前循环AIS患者接受MT治疗(2020年1月- 2021年6月)。脱水评估采用血浆渗透压和尿素/肌酐(U/C)比两种公式。在基线计算机断层血管造影上,使用自动化软件(Brainomix Ltd.)对CC进行量化。主要结局是CC(以半球间变化百分比表示)与脱水(渗透压≥295mmol/L, U/C≥80)之间的关系。次要结局包括出院时和随访3个月时的残疾,使用改进的Rankin量表进行评估(差结局:3-6)。进行了多变量逻辑回归和有序回归分析。结果:共纳入260例患者。渗透压脱水率为65.8%,U/C脱水率为2.9%。CC评分与脱水无关联[脱水71% vs非脱水73%;p = 0.875]。64.3%的脱水患者和46.7%的非脱水患者出院时预后较差(p = 0.026)。在多变量logistic回归分析中,脱水是出院时不良预后的独立预测因子(OR 2.50, p = 0.011)和随访3个月时(OR 2.27, p = 0.046)。结论:入院时脱水与接受MT治疗的AIS患者预后不良相关,但与CC无关。
{"title":"Influence of dehydration on collateral circulation and clinical outcome after endovascular therapy in patients with acute ischemic stroke","authors":"M. Guasch-Jiménez ,&nbsp;P. Camps-Renom ,&nbsp;C. Toscano-Prat ,&nbsp;D. Guisado-Alonso ,&nbsp;A. Martínez-Domeño ,&nbsp;L. Prats-Sánchez ,&nbsp;A. Ramos-Pachón ,&nbsp;I. Fernández-Cadenas ,&nbsp;J.P. Martínez-González ,&nbsp;I. Fernández-Pérez ,&nbsp;C. Avellaneda-Gómez ,&nbsp;E. de Celis-Ruiz ,&nbsp;J. Rodríguez-Pardo ,&nbsp;M. del Mar Freijo ,&nbsp;A. Luna ,&nbsp;F. Moniche ,&nbsp;B. Pardo-Galiana ,&nbsp;J. Ortega-Quintanilla ,&nbsp;J.F. Arenillas ,&nbsp;E. Cortijo ,&nbsp;J. Martí-Fàbregas","doi":"10.1016/j.nrleng.2025.10.007","DOIUrl":"10.1016/j.nrleng.2025.10.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Dehydration in patients with acute ischemic stroke (AIS) is associated with poor outcome. Our aim is to investigate whether dehydration is associated with collateral circulation (CC) and functional outcome in patients with AIS treated with mechanical thrombectomy (MT).</div></div><div><h3>Methods</h3><div>Prospective multicenter study of patients with anterior circulation AIS treated with MT (January 2020–June 2021). Dehydration was assessed with two formulas: plasma osmolarity and urea/creatinine (U/C) ratio. CC was quantified with an automated software (Brainomix Ltd.) on baseline computed tomography angiography. The primary outcome was the association between CC (expressed numerically as the percentage of change between hemispheres) and dehydration (osmolarity ≥ 295 mmol/L, U/C ≥ 80). Secondary outcomes included disability at discharge and at three months of follow-up, assessed using the modified Rankin scale (poor outcome: 3–6). Multivariable logistic and ordinal regression analyses were performed.</div></div><div><h3>Results</h3><div>Two hundred sixty patients were included. 65.8% were dehydrated according to osmolarity and 2.9% according to U/C. There was no association between CC score and dehydration [71% in dehydrated vs 73% in non-dehydrated; <em>P</em> = .875]. 64.3% of dehydrated patients vs. 46.7% of non-dehydrated patients had a poor outcome at discharge (<em>P</em> = .026). In multivariable logistic regression analysis, dehydration was an independent predictor of poor outcome at discharge (OR 2.50; <em>P</em> = .011) and at three months of follow-up (OR 2.27; <em>P</em> = .046).</div></div><div><h3>Conclusions</h3><div>Dehydration on admission is associated with poor outcome in patients with AIS treated with MT, but it is not related to CC.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"40 9","pages":"Pages 822-829"},"PeriodicalIF":0.0,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145282377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Healthcare impact of electroencephalography performed during clinical neurophysiology on-call duty in a third-level hospital 某三级医院临床神经生理学随叫随到期间脑电图对医疗保健的影响。
Pub Date : 2025-10-01 DOI: 10.1016/j.nrleng.2025.09.006
F. Vázquez-Sánchez , M.C. Lloria-Gil , B. García-López , O. Pérez-Gil , A. Saponaro-González , E. Rivas-Navas , A.I. Gómez-Menéndez

Introduction

The electroencephalogram (EEG) is a useful tool in the diagnosis of pathologies such as non-convulsive status epilepticus (NCSE) or brain death (BD), cardiac arrest (CA), and status epilepticus (SE) treatment monitoring. In addition, it provides irreplaceable information depending on the time it is performed, as is the case with the diagnosis of epilepsy after a first epileptic seizure (ES) or to differentiate these from non-epileptic paroxysmal events (NEPE). Its usefulness is maintained outside the usual working day, but it is not available in many centers.

Subjects and methods

Retrospective observational study based on the clinical history of 86 patients who underwent an on-call EEG (ocEEG) at our hospital during 2020.

Results

One hundred two records requested by Intensive Care (41.1%), Neurology (37.3%), Pediatrics (17.6%) or other services (4%) were made. Suspected NCSE represented 56.7%, followed by treatment monitoring in EE (21.6%). The ME accounted for only 6.9% of the total. The ocEEG avoided potential iatrogenesis in a 56.3% of cases with therapeutic implications, allowed to treat 27.58% of patients who would have remained without treatment until the conventional EEG. An increase in the level of care was required in only 22,2% of all cases. The ocEEG was anticipated a mean of 31.6 hours to the next conventional EEG that would have been available.

Conclusions

The availability of ocEEG is beneficial in terms of diagnosis, therapy, and hospital management, advancing decision-making and avoiding iatrogenesis. Its availability should become widespread.
脑电图(EEG)是诊断非惊厥性癫痫持续状态(NCSE)或脑死亡(BD)、心脏骤停(CA)和癫痫持续状态(SE)治疗监测等病理的有用工具。此外,根据执行时间的不同,它提供了不可替代的信息,例如首次癫痫发作(ES)后的癫痫诊断或将其与非癫痫性发作事件(NEPE)区分开来。它在正常工作日之外仍然有用,但在许多中心无法使用。对象和方法:基于2020年在我院接受随叫随到EEG (ocEEG)检查的86例患者的临床病史,进行回顾性观察性研究。结果:重症监护科(41.1%)、神经科(37.3%)、儿科(17.6%)及其他科(4%)共查询记录102份。疑似NCSE占56.7%,其次是EE治疗监测(21.6%)。ME仅占总额的6.9%。在56.3%的具有治疗意义的病例中,脑电图避免了潜在的医源性,允许治疗27.58%的患者,这些患者在常规脑电图之前不会接受治疗。只有22.2%的病例需要提高护理水平。预计下一次常规脑电图的平均时间为31.6小时。结论:脑电图的可用性在诊断、治疗和医院管理,促进决策和避免医源性方面都是有益的。它的可用性应该变得广泛。
{"title":"Healthcare impact of electroencephalography performed during clinical neurophysiology on-call duty in a third-level hospital","authors":"F. Vázquez-Sánchez ,&nbsp;M.C. Lloria-Gil ,&nbsp;B. García-López ,&nbsp;O. Pérez-Gil ,&nbsp;A. Saponaro-González ,&nbsp;E. Rivas-Navas ,&nbsp;A.I. Gómez-Menéndez","doi":"10.1016/j.nrleng.2025.09.006","DOIUrl":"10.1016/j.nrleng.2025.09.006","url":null,"abstract":"<div><h3>Introduction</h3><div>The electroencephalogram (EEG) is a useful tool in the diagnosis of pathologies such as non-convulsive status epilepticus (NCSE) or brain death (BD), cardiac arrest (CA), and status epilepticus (SE) treatment monitoring. In addition, it provides irreplaceable information depending on the time it is performed, as is the case with the diagnosis of epilepsy after a first epileptic seizure (ES) or to differentiate these from non-epileptic paroxysmal events (NEPE). Its usefulness is maintained outside the usual working day, but it is not available in many centers.</div></div><div><h3>Subjects and methods</h3><div>Retrospective observational study based on the clinical history of 86 patients who underwent an on-call EEG (ocEEG) at our hospital during 2020.</div></div><div><h3>Results</h3><div>One hundred two records requested by Intensive Care (41.1%), Neurology (37.3%), Pediatrics (17.6%) or other services (4%) were made. Suspected NCSE represented 56.7%, followed by treatment monitoring in EE (21.6%). The ME accounted for only 6.9% of the total. The ocEEG avoided potential iatrogenesis in a 56.3% of cases with therapeutic implications, allowed to treat 27.58% of patients who would have remained without treatment until the conventional EEG. An increase in the level of care was required in only 22,2% of all cases. The ocEEG was anticipated a mean of 31.6 hours to the next conventional EEG that would have been available.</div></div><div><h3>Conclusions</h3><div>The availability of ocEEG is beneficial in terms of diagnosis, therapy, and hospital management, advancing decision-making and avoiding iatrogenesis. Its availability should become widespread.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"40 8","pages":"Pages 746-752"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145008626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sensory neuronopathy in a patient with anti-FGFR3 antibodies and lung adenocarcinoma, coincidence or causality? 抗fgfr3抗体患者的感觉神经病变与肺腺癌,是巧合还是因果关系?
Pub Date : 2025-10-01 DOI: 10.1016/j.nrleng.2025.09.001
L. Fernández-Llarena, A. Moreno-Estébanez, A. González-Eizaguirre, A. Jauregi-Barrutia
{"title":"Sensory neuronopathy in a patient with anti-FGFR3 antibodies and lung adenocarcinoma, coincidence or causality?","authors":"L. Fernández-Llarena,&nbsp;A. Moreno-Estébanez,&nbsp;A. González-Eizaguirre,&nbsp;A. Jauregi-Barrutia","doi":"10.1016/j.nrleng.2025.09.001","DOIUrl":"10.1016/j.nrleng.2025.09.001","url":null,"abstract":"","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"40 8","pages":"Pages 807-810"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145016971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A single institution series of intravascular lymphoma: Neurological manifestations and neuroimaging findings 单一机构的一系列血管内淋巴瘤:神经学表现和神经影像学发现
Pub Date : 2025-10-01 DOI: 10.1016/j.nrleng.2025.09.008
J. Corroza , C. Alburquerque , L. Martínez-Martínez , I. Gastón , L. Torné , M.C. Gil-Alzueta , J. Oteiza , T. Cabada , M.C. Viguria , A. Panizo , M.E. Erro

Background

Intravascular lymphoma (IVL) is a diffuse large B-cell lymphoma characterized by occlusion of small vessels of organs by malignant cells with special tropism for the nervous system. Our purpose has been to describe a series of IVL diagnosed in our center and to compare the results with those of the literature.

Methods

A retrospective review of the clinical, imaging, and pathological data of consecutive patients with IVL in the last 20 years was performed. Pathology department database was the source of patient identification.

Results

We identified six cases (three males and three females), with a median age at diagnosis of 54.64 (interquartile range (IR) 51.93–59.67). Five of them (83.3%) debuted with neurological manifestations. The median time to diagnosis was 3.77 months. On cranial magnetic resonance imaging, the findings consisted of multiple white matter and corpus callosum lesions (some of them with enhancement or hemorrhagic foci) and multifocal infarcts with hemorrhagic transformation. One patient displayed leptomeningeal branch aneurysms. A proton emission tomography with fluorodeoxyglucose (PET-FDG) was performed in five patients showing areas of elevated metabolism in all of them. Four patients underwent targeted biopsy, which allowed the diagnosis of IVL to be established in three. Four of the five patients who received treatment remained in complete remission with a median progression-free survival of 69.62 months (IR 49.11–162.82).

Conclusions

IVL begins frequently with neurological manifestations, which must alert the neurologist to be aware of this entity. PET-FDG helps to identify biopsy-accessible lesions and facilitate early diagnosis of IVL.
血管性淋巴瘤(IVL)是一种弥漫性大b细胞淋巴瘤,其特征是恶性细胞阻塞器官的小血管,并对神经系统有特殊的倾向。我们的目的是描述本中心诊断的一系列IVL,并将结果与文献进行比较。方法回顾性分析近20年来连续发生的IVL患者的临床、影像学和病理资料。病理科数据库是患者身份识别的来源。结果6例患者(男3例,女3例),诊断时中位年龄54.64岁(四分位间距(IR) 51.93 ~ 59.67)。其中5例(83.3%)首次出现神经系统症状。中位诊断时间为3.77个月。颅磁共振成像显示多发性白质和胼胝体病变(其中一些有强化或出血性灶)和多灶性梗死伴出血性转化。1例患者显示脑脊膜分支动脉瘤。对5例患者进行了氟脱氧葡萄糖质子发射断层扫描(PET-FDG),显示所有患者的代谢区域均升高。4例患者行靶向活检,其中3例诊断为IVL。接受治疗的5名患者中有4名保持完全缓解,中位无进展生存期为69.62个月(IR 49.11-162.82)。结论sivl常以神经系统表现开始,必须提醒神经科医生注意这一实体。PET-FDG有助于识别活检可及的病变,促进IVL的早期诊断。
{"title":"A single institution series of intravascular lymphoma: Neurological manifestations and neuroimaging findings","authors":"J. Corroza ,&nbsp;C. Alburquerque ,&nbsp;L. Martínez-Martínez ,&nbsp;I. Gastón ,&nbsp;L. Torné ,&nbsp;M.C. Gil-Alzueta ,&nbsp;J. Oteiza ,&nbsp;T. Cabada ,&nbsp;M.C. Viguria ,&nbsp;A. Panizo ,&nbsp;M.E. Erro","doi":"10.1016/j.nrleng.2025.09.008","DOIUrl":"10.1016/j.nrleng.2025.09.008","url":null,"abstract":"<div><h3>Background</h3><div>Intravascular lymphoma (IVL) is a diffuse large B-cell lymphoma characterized by occlusion of small vessels of organs by malignant cells with special tropism for the nervous system. Our purpose has been to describe a series of IVL diagnosed in our center and to compare the results with those of the literature.</div></div><div><h3>Methods</h3><div>A retrospective review of the clinical, imaging, and pathological data of consecutive patients with IVL in the last 20 years was performed. Pathology department database was the source of patient identification.</div></div><div><h3>Results</h3><div>We identified six cases (three males and three females), with a median age at diagnosis of 54.64 (interquartile range (IR) 51.93–59.67). Five of them (83.3%) debuted with neurological manifestations. The median time to diagnosis was 3.77 months. On cranial magnetic resonance imaging, the findings consisted of multiple white matter and corpus callosum lesions (some of them with enhancement or hemorrhagic foci) and multifocal infarcts with hemorrhagic transformation. One patient displayed leptomeningeal branch aneurysms. A proton emission tomography with fluorodeoxyglucose (PET-FDG) was performed in five patients showing areas of elevated metabolism in all of them. Four patients underwent targeted biopsy, which allowed the diagnosis of IVL to be established in three. Four of the five patients who received treatment remained in complete remission with a median progression-free survival of 69.62 months (IR 49.11–162.82).</div></div><div><h3>Conclusions</h3><div>IVL begins frequently with neurological manifestations, which must alert the neurologist to be aware of this entity. PET-FDG helps to identify biopsy-accessible lesions and facilitate early diagnosis of IVL.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"40 8","pages":"Pages 768-776"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145247993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Immune-mediated necrotizing myopathy: an emerging disorder 免疫介导的坏死性肌病:一种新兴疾病。
Pub Date : 2025-10-01 DOI: 10.1016/j.nrleng.2025.09.004
S. Portela-Sánchez , I. Catalina , S. López Muñoz , A. Lozano Ros , A. Sánchez-Soblechero , E. Sola Vendrell , P. Sánchez-Mateos , J.L. Muñoz-Blanco

Objectives

To describe the main features and the clinical management of a cohort of patients with immune-mediated necrotizing myopathy (IMNM).

Methods

We conducted an observational, monocentric, retrospective study of IMNM patients diagnosed in the Neuromuscular Unit of a tertiary hospital in Madrid (Spain) between 2013 and 2021.

Results

Sixteen IMNM cases were diagnosed, with a median age of 71.5 years (range 36−80), nine of whom (56.3%) were female. Thirteen (81.3%) patients had previously been treated with statins. The time from symptom onset to diagnosis was less than six months in eleven (68.8%) patients and the most common clinical symptoms were proximal weakness and myalgia. The only myositis specific autoantibodies detected were anti-3-hydroxy-3-methyl-coenzyme A reductase in thirteen patients. The treatment strategy was based on prednisone, although a combination with other immunosuppressive drugs was required in eight (50%) patients.

Conclusions

There has been an increase in the diagnosis of immune-mediated necrotizing myopathies in the last few years. The anti-HMGCR antibodies were the only detected in this sample showing their key role in the diagnosis. Early recognition of the disease facilitate to start treatment as soon as possible, which should be based on the initial response to corticosteroids and usually requires a combination of several drugs.
目的:描述一组免疫介导坏死性肌病(IMNM)患者的主要特征和临床处理。方法:我们对2013年至2021年间在马德里(西班牙)一家三级医院神经肌肉科诊断的IMNM患者进行了一项观察性、单中心、回顾性研究。结果:16例IMNM确诊病例,中位年龄71.5岁(36 ~ 80岁),其中9例为女性(56.3%)。13例(81.3%)患者曾接受过他汀类药物治疗。11例(68.8%)患者从症状出现到诊断的时间少于6个月,最常见的临床症状为近端无力和肌痛。13例肌炎患者检测到的唯一特异性自身抗体是抗3-羟基-3-甲基辅酶A还原酶。治疗策略以强的松为基础,尽管有8例(50%)患者需要联合使用其他免疫抑制药物。结论:近年来,免疫介导的坏死性肌病的诊断有所增加。抗hmgcr抗体是该样本中唯一检测到的显示其在诊断中的关键作用的抗体。早期识别疾病有助于尽早开始治疗,治疗应以对皮质类固醇的初始反应为基础,通常需要多种药物联合使用。
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引用次数: 0
Palliative Care in Parkinson's Disease and other Movement Disorders. Recommendations and protocol of a multidisciplinary group of experts 帕金森病和其他运动障碍的姑息治疗。多学科专家组的建议和方案。
Pub Date : 2025-10-01 DOI: 10.1016/j.nrleng.2025.09.002
M. Álvarez Saúco , I. Legarda Ramírez , S. Martí Martínez , F. Carrillo García , B. González García , J. Fernández Bueno , R. García-Ramos , D. Santos-García

Introduction

One of the current challenges in Parkinson's disease (PD) and other movement disorders (MD) is how and when to apply palliative care. Aware of the scarce training and implementation of this type of approach, we propose some consensual recommendations for palliative care (PC) in order to improve the quality of life of patients and their environment.

Material and methods

After a first phase of needs analysis through a survey carried out on Spanish neurologists and a review of the literature, we describe recommendations for action structured in: palliative care models, selection of the target population, when, where and how to implement the PC.

Results

Models of neuropalliative care are reviewed, advocating for the role of the neurologist as a driving force. The members of the multidisciplinary team are described, as well as the main clinical markers and tools that help the clinician to decide which patients have greater palliative needs; sender and receiver are defined and it is detailed in what evolutionary moment and how to proceed when sending the patient to palliative care. A scheme of steps to follow in any PC protocol, whether basic or specialized, is provided, emphasizing the framework in the shared planning of care and the comprehensive approach.

Conclusions

It would be desirable to integrate the PC in the management of PD and other MD and to validate models of neuropalliative care in our environment, analyzing their usefulness through the use of indicators, in order to improve the care and quality of life of our patients.
目前帕金森病(PD)和其他运动障碍(MD)面临的挑战之一是如何以及何时应用姑息治疗。意识到这种方法缺乏培训和实施,我们提出了一些关于姑息治疗(PC)的共识建议,以改善患者的生活质量和他们的环境。材料和方法:通过对西班牙神经科医生的调查和文献回顾进行第一阶段的需求分析后,我们描述了以下方面的行动建议:姑息治疗模式,目标人群的选择,何时,何地以及如何实施PC。结果:对神经姑息治疗的模式进行了回顾,倡导神经科医生作为驱动力的作用。描述了多学科团队的成员,以及帮助临床医生决定哪些患者有更大的姑息治疗需求的主要临床标志和工具;定义了发送者和接收者,并详细说明了将患者送到姑息治疗时的进化时刻和如何进行。提供了任何个人护理协议(无论是基本的还是专门的)应遵循的步骤方案,强调了共享护理规划和综合方法的框架。结论:将神经姑息治疗整合到PD和其他MD的治疗中,并在我们的环境中验证神经姑息治疗模型,通过使用指标分析其实用性,以改善我们患者的护理和生活质量。
{"title":"Palliative Care in Parkinson's Disease and other Movement Disorders. Recommendations and protocol of a multidisciplinary group of experts","authors":"M. Álvarez Saúco ,&nbsp;I. Legarda Ramírez ,&nbsp;S. Martí Martínez ,&nbsp;F. Carrillo García ,&nbsp;B. González García ,&nbsp;J. Fernández Bueno ,&nbsp;R. García-Ramos ,&nbsp;D. Santos-García","doi":"10.1016/j.nrleng.2025.09.002","DOIUrl":"10.1016/j.nrleng.2025.09.002","url":null,"abstract":"<div><h3>Introduction</h3><div>One of the current challenges in Parkinson's disease (PD) and other movement disorders (MD) is how and when to apply palliative care. Aware of the scarce training and implementation of this type of approach, we propose some consensual recommendations for palliative care (PC) in order to improve the quality of life of patients and their environment.</div></div><div><h3>Material and methods</h3><div>After a first phase of needs analysis through a survey carried out on Spanish neurologists and a review of the literature, we describe recommendations for action structured in: palliative care models, selection of the target population, when, where and how to implement the PC.</div></div><div><h3>Results</h3><div>Models of neuropalliative care are reviewed, advocating for the role of the neurologist as a driving force. The members of the multidisciplinary team are described, as well as the main clinical markers and tools that help the clinician to decide which patients have greater palliative needs; sender and receiver are defined and it is detailed in what evolutionary moment and how to proceed when sending the patient to palliative care. A scheme of steps to follow in any PC protocol, whether basic or specialized, is provided, emphasizing the framework in the shared planning of care and the comprehensive approach.</div></div><div><h3>Conclusions</h3><div>It would be desirable to integrate the PC in the management of PD and other MD and to validate models of neuropalliative care in our environment, analyzing their usefulness through the use of indicators, in order to improve the care and quality of life of our patients.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"40 8","pages":"Pages 717-728"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145008679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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Neurologia
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