Neurologia最新文献_第3页

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 Epub Date: 2026-01-22 DOI: 10.1016/j.nrleng.2025.101895

D. López Dominguez , G. Álvarez Bravo

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 Epub Date: 2026-01-22 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。

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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 Epub Date: 2026-01-22 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

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 Epub Date: 2026-01-22 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。

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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 Epub Date: 2026-01-22 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

Recommendations for the critical reading of clinical trials on disease-modifying drugs for multiple sclerosis 对多发性硬化症治疗药物临床试验的批判性阅读建议

Neurologia

Pub Date : 2025-11-01 Epub Date: 2025-11-04 DOI: 10.1016/j.nrleng.2025.08.003

A. Rivero-de-Aguilar , M. Mascareñas-García , M. Pérez-Ríos , M.A. Llaneza-Gonzalez , P. Mulero , M. Mendibe , À. Rovira , V. Meca-Lallana , A.J. García-Ruiz , L. Landete , J.R. Vizoso Hermida , P. Carrascal Rueda , A. Ruano-Raviña , M. Puente-Hernandez , L. Varela-Lema

Introduction

In recent years there has been an increase in the number of disease-modifying drugs (DMDs) approved for multiple sclerosis (MS). The evidence of their safety and efficacy has been obtained through several phase III and IV clinical trials. Acquiring the skills for their appraisal is indispensable for clinicians to assess the most pertinent treatment for patients. The objective of this study is to provide guidance in the critical reading of these trials.

Methods

A three-round e-Delphi study was carried out. In the preparatory phase, a multidisciplinary expert panel was established. Panel members were selected based on their scientific credentials and experience, seeking to include people involved in MS diagnosis, treatment and research. A semi-open questionnaire was developed based on key generic and MS-specific methodological instruments identified through a scoping bibliographic search. The experts were required to identify essential aspects for critically appraising clinical trials on DMDs for MS.

Results

The expert panel consisted of nine independent leading Spanish experts with long-standing experience with MS (five neurologists, a neuroradiologist, a pharmacologist, a research methodologist and an MS community representative). The e-Delphi study resulted in consensus recommendations intended to help readers in answering five major questions: “Is the study free of bias?”; “Are the included patients adequate?”; “Are the outcome measures appropriate?”; “Are the results relevant?”; and “Is the study transparent?”.

Conclusion

This study proposes consensus recommendations intended to guide neurologists in the critical reading of phase III and IV clinical trials on DMDs for MS.

近年来，批准用于多发性硬化症（MS）的疾病调节药物（dmd）数量有所增加。其安全性和有效性的证据已经通过几次III期和IV期临床试验获得。获得评估的技能对于临床医生评估患者最相关的治疗是必不可少的。本研究的目的是为这些试验的批判性阅读提供指导。方法采用三轮e-Delphi法。在筹备阶段，设立了一个多学科专家小组。小组成员是根据他们的科学资历和经验选择的，力求包括参与MS诊断、治疗和研究的人员。一份半开放式问卷是基于通过范围界定书目检索确定的关键通用和ms特有的方法学工具开发的。结果专家组由9位具有长期多发性硬化症治疗经验的西班牙独立权威专家组成（5位神经科医生、1位神经放射学家、1位药理学家、1位研究方法学家和1位多发性硬化症社区代表）。e-Delphi研究得出了一致的建议，旨在帮助读者回答五个主要问题：“研究是否没有偏见？”“纳入的病人足够吗？”“结果衡量标准是否合适？”“结果相关吗？”以及“研究是否透明？”本研究提出了共识性建议，旨在指导神经科医生对dmd治疗多发性硬化症的III期和IV期临床试验进行批判性阅读。

{"title":"Recommendations for the critical reading of clinical trials on disease-modifying drugs for multiple sclerosis","authors":"A. Rivero-de-Aguilar , M. Mascareñas-García , M. Pérez-Ríos , M.A. Llaneza-Gonzalez , P. Mulero , M. Mendibe , À. Rovira , V. Meca-Lallana , A.J. García-Ruiz , L. Landete , J.R. Vizoso Hermida , P. Carrascal Rueda , A. Ruano-Raviña , M. Puente-Hernandez , L. Varela-Lema","doi":"10.1016/j.nrleng.2025.08.003","DOIUrl":"10.1016/j.nrleng.2025.08.003","url":null,"abstract":"<div><h3>Introduction</h3><div>In recent years there has been an increase in the number of disease-modifying drugs (DMDs) approved for multiple sclerosis (MS). The evidence of their safety and efficacy has been obtained through several phase III and IV clinical trials. Acquiring the skills for their appraisal is indispensable for clinicians to assess the most pertinent treatment for patients. The objective of this study is to provide guidance in the critical reading of these trials.</div></div><div><h3>Methods</h3><div>A three-round e-Delphi study was carried out. In the preparatory phase, a multidisciplinary expert panel was established. Panel members were selected based on their scientific credentials and experience, seeking to include people involved in MS diagnosis, treatment and research. A semi-open questionnaire was developed based on key generic and MS-specific methodological instruments identified through a scoping bibliographic search. The experts were required to identify essential aspects for critically appraising clinical trials on DMDs for MS.</div></div><div><h3>Results</h3><div>The expert panel consisted of nine independent leading Spanish experts with long-standing experience with MS (five neurologists, a neuroradiologist, a pharmacologist, a research methodologist and an MS community representative). The e-Delphi study resulted in consensus recommendations intended to help readers in answering five major questions: “Is the study free of bias?”; “Are the included patients adequate?”; “Are the outcome measures appropriate?”; “Are the results relevant?”; and “Is the study transparent?”.</div></div><div><h3>Conclusion</h3><div>This study proposes consensus recommendations intended to guide neurologists in the critical reading of phase III and IV clinical trials on DMDs for MS.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"40 9","pages":"Pages 875-883"},"PeriodicalIF":0.0,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145435360","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

Adaptation and norming of the Spanish version of the Attention, Memory and Frontal Abilities Test (AMFAST) for people aged 55 years and older 55岁及以上人群西班牙语版注意、记忆和额叶能力测试（AMFAST）的适应和规范。

Neurologia

Pub Date : 2025-11-01 Epub Date: 2025-10-07 DOI: 10.1016/j.nrleng.2025.10.002

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 analysed using binary logistic regression, and sensitivity (Sen), specificity (Spe), and positive (PPV) and negative (NPV) 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 3 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 = −0.33, 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 Sen = 85.71%, Spe = 71.63%, PPV = 23.08%, and NPV = 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.

简介：筛选试验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岁或以上的人群开发标准数据的注意，记忆和额叶能力筛选测试（AMFAST）。方法：155名年龄在55岁至82岁之间认知健康的参与者使用综合神经心理学电池和AMFAST进行评估。采用二元逻辑回归分析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（Sen）、特异性（Spe）、阳性（PPV）和阴性（NPV）预测值。规范数据采用线性回归控制年龄、性别和教育水平的影响。结果：AMFAST总分与年龄、教育程度相关，与性别无关。以3分及以上低分作为客观认知障碍的判定标准，AMFAST总分与神经心理电池低分数相关（r=- 0.33, p）。结论：AMFAST是一种简单、快速的检测方法，有助于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。

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引用次数: 0

Impact of fatigue on quality of life in adults with multiple sclerosis 疲劳对复发缓解型多发性硬化症患者生活质量的影响。

Neurologia

Pub Date : 2025-11-01 Epub Date: 2025-10-09 DOI: 10.1016/j.nrleng.2025.10.006

R. Piñar-Morales , P.A. Guirado-Ruiz , F.J. Barrero Hernández

Introduction

Fatigue in multiple sclerosis (MS) is defined as the lack of physical and/or mental energy perceived by the individual that interferes with normal activities. It is the most common symptom in MS, present in up to 90% of people with MS. Fatigue along with disability, depression, cognitive impairment, and disease-modifying therapy (DMT) affect quality of life (QoL).

Method

We designed a prospective observational study in patients with MS and DMT of moderate efficacy to assess the association between fatigue and the epidemiological, clinical, and pharmacological aspects that influence in the QoL. We analysed variables related to patients, disability, fatigue (MFIS), clinical and radiological activity, depression (BDI), cognitive impairment (SDMT), and QoL (EQ-5D).

Results

we included 91 people, 65.9% women, mean age 43.9 years. The DMT were: 27.4% interferon-β, 15.38% glatiramer acetate, 9.89% teriflunomide, and 47.25% dimethyl fumarate. The median of the EDSS was 1.5 points. 40.9% have presented fatigue, 36.3% cognitive deterioration and 30.7% of the patients depression.

Conclusions

Patients with fatigue are older, more disabled, have a higher prevalence of depression and worse QoL. Evolution time, relapses, MRI lesion load, and DMTs are not associated with fatigue. Fatigue is a frequent symptom in patients with MS that influences in the QoL, hence the importance of its diagnosis and treatment.

简介：多发性硬化症（MS）的疲劳被定义为个体感觉到的身体和/或精神能量的缺乏，干扰了正常的活动。它是多发性硬化症中最常见的症状，高达90%的多发性硬化症患者存在疲劳、残疾、抑郁、认知障碍和疾病改善治疗（DMT）影响生活质量（QoL）。方法：我们设计了一项前瞻性观察研究，对中度疗效的MS和DMT患者进行研究，以评估疲劳与影响生活质量的流行病学、临床和药理学方面的关系。我们分析了与患者、残疾、疲劳（MFIS）、临床和放射活动、抑郁（BDI）、认知障碍（SDMT）和生活质量（EQ-5D）相关的变量。结果：纳入91例，女性65.9%，平均年龄43.9岁。DMT分别为：干扰素-β 27.4%，醋酸格拉替雷默15.38%，特立氟米特9.89%，富马酸二甲酯47.25%。EDSS的中位数为1.5点。40.9%的患者表现为疲劳，36.3%的患者表现为认知能力下降，30.7%的患者表现为抑郁。结论：疲劳患者年龄大，残疾程度高，抑郁患病率高，生活质量差。发展时间、复发、MRI病变负荷和dmt与疲劳无关。疲劳是影响MS患者生活质量的常见症状，因此其诊断和治疗具有重要意义。

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引用次数: 0

The discriminative linguistic profiles of progressive supranuclear palsy and Parkinson's disease 进行性核上性麻痹和帕金森病的鉴别语言特征

Neurologia

Pub Date : 2025-11-01 Epub Date: 2025-11-04 DOI: 10.1016/j.nrleng.2025.08.001

L. Busteed , A. Horta-Barba , A. Reig , C. García-Sánchez , B. Pascual-Sedano , I. Gich , P. Roy Ciffone , J. Kulisevsky , J. Pagonabarraga

Introduction

Speech and language disturbances are very frequent in progressive supranuclear palsy (PSP). Therefore, they are part of the diagnostic criteria set forth by the International Parkinson and Movement Disorder Society (MDS) for the disease and are considered a core clinical feature. However, more studies are needed to characterize the linguistic profile of PSP, thus being able to assist in the differential diagnosis. Additionally, studies assessing linguistic differences among PSP phenotypes are needed. The objective of this study is to analyze the language alterations presented by patients with PSP, as well as its different phenotypes, and differentiate them from those presented in patients with Parkinson's disease (PD).

Methods

An extensive cognitive and linguistic assessment was administered to 13 PSP patients, 19 PD patients and 19 healthy controls (HC) with similar sociodemographic features. Language assessment included evaluation of: syntactic processing, object naming, and phonetic and semantic fluencies. We included a subgroup of 6 PSP patients, 19 PD patients and 19 HC for further analysis of language. This analysis included, in addition to the general evaluation, the assessment of alternating fluency, comprehension, naming, automatic speech, repetition, object recognition, verbal and written instructions, writing to dictation, and oral expression.

Results

We found greater impairment on phonetic, semantic, and alternating fluencies, following verbal instructions, repetition, syntactic processing and writing (without phonetic paragraphia) in the PSP group compared to patients with PD and HC. Distinguishing linguistic features of PSP with a less marked reduction than the previously mentioned features were automatic speech, fluency of speech, and naming. Language analysis did not distinguish between PSP phenotypes.

Conclusions

Language disturbances distinguish PSP from PD and HC but were not able to discriminate PSP phenotypes.

进行性核上性麻痹（PSP）患者经常出现言语和语言障碍。因此，它们是国际帕金森与运动障碍协会（MDS）为该疾病制定的诊断标准的一部分，被认为是核心临床特征。然而，需要更多的研究来描述PSP的语言特征，从而能够帮助鉴别诊断。此外，还需要研究评估PSP表型之间的语言差异。本研究的目的是分析PSP患者的语言改变及其不同的表型，并将其与帕金森病（PD）患者的语言改变进行区分。方法对13例PSP患者、19例PD患者和19例具有相似社会人口统计学特征的健康对照（HC）进行广泛的认知和语言评估。语言评估包括：句法处理、对象命名、语音和语义流畅性的评估。我们纳入了6名PSP患者，19名PD患者和19名HC患者进行进一步的语言分析。除了一般评价外，该分析还包括对交替流畅性、理解、命名、自动语音、重复、物体识别、口头和书面指示、书面听写和口头表达的评估。结果我们发现，与PD和HC患者相比，PSP组在语音、语义和交替流畅性、遵循口头指示、重复、句法处理和写作（无语音段落）方面的损害更大。与前面提到的特征相比，PSP的显著性语言特征是自动语音、语言流畅性和命名。语言分析没有区分PSP表型。结论语言障碍可区分PSP与PD和HC，但不能区分PSP的表型。

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引用次数: 0

Neuropsychological differential diagnosis of Alzheimer’s disease and Lewy body dementia: A systematic review 阿尔茨海默病和路易体痴呆的神经心理学鉴别诊断：系统综述。

Neurologia

Pub Date : 2025-11-01 Epub Date: 2025-10-06 DOI: 10.1016/j.nrleng.2025.10.001

T. Julio-Ramos , V. Mora-Castelleto , C. Foncea-González , C. Adames-Valencia , I. Cigarroa , C. Méndez-Orellana , D. Toloza-Ramirez

Introduction

Alzheimer's disease (AD) reports heterogeneity of neuropsychological symptoms misleading the differential diagnosis with other forms of dementia, such as dementia with Lewy bodies (DLB). About 50% of DLB patients are misdiagnosed as AD cases. Likewise, the diagnosis of both diseases is mainly based on clinical characteristics. However, differentiating AD of those with DLB based on neuropsychological symptoms and anatomical and functional brain changes remains challenging.

Aim

To establish the main neuropsychological, anatomical, and functional similarities and differences in patients with AD and DLB.

Methods

The present study followed the PRISMA guidelines and included studies from the PubMed, Scopus, and Web of Sciences databases, published between January 2000 and July 2022.

Results

41 articles were included in this systematic review for critical analysis. Our results suggest that the cognitive key domains to consider in the differential diagnosis are memory, executive function, attention, visuospatial/visuoconstructive skills, and verbal fluency (both semantic and phonological). The stage and severity of both diseases would be essential for differential diagnosis. On the other hand, the anatomical and functional changes suggest a similar atrophy pattern between AD and DLB in the frontal, parietal, temporal, hippocampal, and precuneus regions.

Conclusion

The differential diagnosis between AD and DLB is challenging in clinical practice. Therefore, our results suggest exploring cognitive linguistic markers along with correlating these markers with anatomical and functional brain changes.

导读：阿尔茨海默病（AD）报告了神经心理症状的异质性，误导了与其他形式的痴呆（如路易体痴呆（DLB））的鉴别诊断。约50%的DLB患者被误诊为AD病例。同样，这两种疾病的诊断主要基于临床特征。然而，根据神经心理症状和大脑解剖和功能变化来区分DLB患者的AD仍然具有挑战性。目的：探讨AD和DLB患者主要神经心理、解剖学和功能上的异同。方法：本研究遵循PRISMA指南，纳入了2000年1月至2022年7月期间发表的PubMed、Scopus和Web of Sciences数据库中的研究。结果：本系统综述纳入41篇文章进行批判性分析。我们的研究结果表明，在鉴别诊断中需要考虑的认知关键领域是记忆、执行功能、注意力、视觉空间/视觉构建技能和语言流畅性（包括语义和语音）。两种疾病的分期和严重程度对于鉴别诊断至关重要。另一方面，解剖和功能变化表明AD和DLB在额叶、顶叶、颞叶、海马和楔前叶区域有相似的萎缩模式。结论：AD与DLB的鉴别诊断在临床实践中具有挑战性。因此，我们的研究结果建议探索认知语言标记，并将这些标记与大脑的解剖和功能变化联系起来。

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