Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101904

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

Introduction

Absence seizures due to secondary bilateral synchrony may be a manifestation of frontal seizures, indistinguishable from primary absence epilepsy (PAE), with a different prognosis and control. There are few epidemiological studies on frontal absences (FAE) in the group of absence epilepsy (AE) in children.

Objective

To describe the epidemiology of FAE in childhood and compare the characteristics, clinical evolution, and pharmacological response with PAE.

Patients and method

Retrospective study of cases with AE in children under 14 years of age from 2013 to 2022 in a tertiary hospital. Demographic variables, number, duration and type of associated seizures, frontal EEG focality with and without relation to generalized discharge, pharmacological response and neuroimaging were comparatively studied.

Results

94 patients with a median age of 8.6 years (6–10.1 years; 49M/45H) with AE were included. 84% presented exclusively absence seizures. Hyperventilation induced seizures in 94.2%; photoparoxysms present in 5.3%. They showed EEG focus, 63/94 and in 45/94 it was frontal. In 14/94 (14.8%) the frontal focality preceded the critical spike-wave discharge. The bivariate analysis did not show significant differences in age, time until the consultation, psychomotor development/behavior alteration, association of other types of seizures and triggers. The number of absences/days was significantly lower in the EAFs (p = 0.004) and the need for combination therapy was greater in the bivariate (p = 0.005) and multivariate (p = 0.035) analysis.

Conclusions

FAE represents a significant percentage of AE with seizures of identical morphology, age, and duration, although with fewer seizures/day and a worse response to treatment.

继发性双侧同步引起的失神癫痫可能是额叶癫痫的一种表现，与原发性失神癫痫（PAE）难以区分，预后和控制不同。目前关于儿童缺失性癫痫（AE）组额叶缺失（FAE）的流行病学研究较少。目的：描述儿童期FAE的流行病学，并与PAE的特点、临床演变及药理反应进行比较。患者与方法：回顾性分析某三级医院2013 - 2022年14岁以下儿童AE病例。比较研究了人口统计学变量、相关癫痫发作次数、持续时间和类型、与全面性放电相关和不相关的额叶脑电图病灶、药物反应和神经影像学。结果：纳入94例AE患者，中位年龄8.6岁（6-10.1岁；49 M/45 H）。84%表现为完全失神发作。过度通气诱发癫痫发作94.2%；5.3%出现光发现象。他们的脑电图显示病灶，63/94,45/94是额叶。在14/94（14.8%）中，额部聚焦先于临界尖峰波放电。双变量分析未显示年龄、就诊时间、精神运动发展/行为改变、其他类型癫痫发作和触发因素的关联有显著差异。在EAFs中，缺勤天数明显较低（p = 0.004），在双变量（p = 0.005）和多变量（p = 0.035）分析中，联合治疗的需求更大。结论：FAE在AE患者中具有相同形态、年龄和持续时间的癫痫发作中所占比例显著，尽管其每天发作次数较少，对治疗的反应较差。

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引用次数: 0

Fourth cranial nerve neuropathy due to Ecchordosis physaliphora: literature review and case report 第4例颅脑神经病变：文献复习及病例报告。

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101887

Vidal Fernández-Rodríguez, José María Losada Domingo, Ana Moreno-Estébanez, Alba Rebollo Pérez

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

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101897

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。

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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）

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.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

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

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101895

D. López Dominguez , G. Álvarez Bravo

引用次数: 0

Phenotypic and genotypic heterogeneity in a series of cases associated with dopa-responsive dystonia 多巴反应性肌张力障碍一系列病例的表型和基因型异质性

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101900

I.G. Díaz , E.O. Aguilar , G.F. Pajarín , I.C. López , D.A.G. Estevez , B.A. Pensado , Á.S. 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

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101901

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。

{"title":"Association between comorbidities and health-related quality of life in patients with multiple sclerosis","authors":"O. Mirmosayyeb , M. Barzegar , M. Rezaei , S. Houshi , A. Afshari-Safavi , M. Falahatian , N. Nehzat , V. Shaygannejad","doi":"10.1016/j.nrleng.2025.101901","DOIUrl":"10.1016/j.nrleng.2025.101901","url":null,"abstract":"<div><h3>Purpose</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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.</div></div><div><h3>Conclusion</h3><div>Our results show a significant association between comorbidities and HRQOL in MS patients.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 101901"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146015555","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

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

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.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.nrleng.2025.501925","DOIUrl":"10.1016/j.nrleng.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":94155,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 501925"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146015660","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

Dysautonomia after COVID-19 infection: A case report COVID-19感染后自主神经异常1例

Neurologia

Pub Date : 2026-01-01 DOI: 10.1016/j.nrleng.2025.101891

C.H. Vera-Cáceres , M. García-Huguet , A. Gil de Genover , S.D. Sagula , L. Martín Muñóz , D. López Domínguez

Introduction

This case report discusses a case of a patient who experienced acute autonomic dysfunction during the parainfectious phase of COVID-19, attributed to Guillain-Barre Syndrome (GBS). This is the first well-documented case of such an association.

Case presentation

A 64-year-old woman, previously infected with COVID-19, was admitted to the emergency department due to altered mental status. Brain computed tomography (CT) revealed bilateral occipital diffuse hypodensity. Throughout her hospitalization, she exhibited elevated blood pressure necessitating intravenous treatment.

The initial brain MRI revealed T2-weighted image hyperintensity at the parietal and occipital levels, indicative of vasogenic edema. These neuroimaging findings were suggestive of posterior reversible encephalopathy syndrome (PRES). Euvolemic hyponatremia with concurrent low serum osmolality and high urine osmolality and sodium was observed, indicating a syndrome of inappropriate antidiuretic hormone (SIADH). Throughout the patient's stay, her level of consciousness exhibited significant improvement. However, she developed ascending symmetrical limb weakness and progressive loss of reflexes, along with a severe motor deficit and gait disturbance, accompanied by arterial blood pressure fluctuations and other signs of autonomic dysfunction.

Clinical manifestations, neurophysiological findings, and laboratory results were indicative of Guillain-Barre Syndrome (GBS), leading to a conclusive diagnosis of GBS with dysautonomia triggered by a COVID-19 infection.

Conclusion

This case reveals the relevance of diagnosing autonomic dysfunction (including PRES) as the initial manifestation of GBS linked to COVID-19 infection and the importance of early diagnosis to prevent potential complications.

本病例报告讨论了一例患者在COVID-19副感染期经历急性自主神经功能障碍，归因于格林-巴利综合征（GBS）。这是第一个有充分证据证明这种关联的案例。病例介绍一名64岁女性，先前感染了COVID-19，因精神状态改变而入院急诊。脑部电脑断层扫描显示双侧枕部弥漫性低密度。在住院期间，她表现出血压升高，需要静脉注射治疗。最初的脑部MRI显示t2加权图像在顶叶和枕叶水平高，表明血管源性水肿。这些神经影像学结果提示后路可逆性脑病综合征（PRES）。低钠血症并发低血清渗透压和高尿渗透压和钠，提示不适当的抗利尿激素（SIADH）综合征。在住院期间，她的意识水平有了显著的改善。然而，她出现了上升的对称肢体无力和进行性反射丧失，同时伴有严重的运动缺陷和步态障碍，并伴有动脉血压波动和其他自主神经功能障碍的迹象。临床表现、神经生理学结果和实验室结果提示格林-巴利综合征（GBS），最终诊断为由COVID-19感染引发的格林-巴利综合征伴自主神经异常。结论本病例揭示了将自主神经功能障碍（包括PRES）诊断为与COVID-19感染相关的GBS的初始表现的相关性，以及早期诊断对预防潜在并发症的重要性。

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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 : 2025-12-25 DOI: 10.1016/j.nrleng.2025.501931

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

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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引用次数: 0