Neurologia最新文献

{"title":"Adaptación transcultural y validación a la población española con daño cerebral adquirido de la Prueba de Sistemas de Evaluación del Equilibrio (BESTest) y sus versiones reducidas (Mini-BESTest y Brief-BESTest)","authors":"M. Fernández-Hontoria ,&nbsp;R.P. Romero-Galisteo ,&nbsp;M. Torres-Lacomba ,&nbsp;C. González-Alted ,&nbsp;A. Megía-García-Carpintero ,&nbsp;C. Lirio-Romero","doi":"10.1016/j.nrl.2025.501929","DOIUrl":"10.1016/j.nrl.2025.501929","url":null,"abstract":"<div><h3>Introduction</h3><div>Balance assessment measures are often validated in the general population or older adults, with limited validation in individuals with neurological impairments. The objective of this study is to culturally adapt and validate the Balance Evaluation Systems Test (BESTest) and its shortened versions, the Mini-BESTest and Brief-BESTest, in a Spanish population with acquired brain injury.</div></div><div><h3>Methods</h3><div>The study was divided into three phases: 1)<!--> <!-->translation and adaptation of the tests; 2)<!--> <!-->pilot testing of the adapted version, and 3)<!--> <!-->assessment of psychometric properties to assess the (reliability and validity). Berg Balance Scale was used as a criterion variable; construct validity was assessed by exploratory factor analysis of all items of each test; and reliability was tested by Cronbach's alpha and intra-class correlation interval.</div></div><div><h3>Results</h3><div>108 subjects in subacute and chronic phases of brain injury participated. Psychometric analysis of the three tests demonstrated good convergent validity, internal consistency, inter-rater agreement (0.998-0.969), and test-retest reliability (0.985-0.989). Convergent validity was observed with the Berg Balance Scale (r<!--> <!-->=<!--> <!-->.901, <em>P</em> <!-->&lt;<!--> <!-->.001; r<!--> <!-->=<!--> <!-->.977, <em>P</em> <!-->&lt;<!--> <!-->.001; r<!--> <!-->=<!--> <!-->.852, <em>P</em> <!-->&lt;<!--> <!-->.001, respectively), as well as other gait and balance scales. No ceiling or floor effects were found in the adapted versions of the BESTest, Mini-BESTest, and Brief-BESTest for the Spanish population with acquired brain injury.</div></div><div><h3>Conclusions</h3><div>All three tests are reliable and valid, with BESTest being the better option for assessing balance in people with acquired brain injury, both in the subacute and chronic phase, as it includes domains that other tools do not assess.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 501929"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412485","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}

{"title":"Comentarios sobre el artículo «Las tasas de mortalidad para la Enfermedad de Parkinson están incrementando en España. Un análisis Edad-Periodo-Cohorte y Joinpoints en las tasas de mortalidad desde 1981 a 2020»","authors":"J. Benito-León ,&nbsp;C.M. Benito-Rodríguez","doi":"10.1016/j.nrl.2024.10.002","DOIUrl":"10.1016/j.nrl.2024.10.002","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 101893"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412026","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}

{"title":"Isolated bilateral facial palsy as the initial presenting manifestation of generalized myasthenia gravis preceding myasthenic crisis","authors":"R. Ghosh ,&nbsp;M. León-Ruiz ,&nbsp;K. Bole ,&nbsp;S. Dubey ,&nbsp;J. Benito-León","doi":"10.1016/j.nrl.2023.11.010","DOIUrl":"10.1016/j.nrl.2023.11.010","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 101894"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412027","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}

{"title":"Recomendaciones para el diagnóstico, tratamiento y seguimiento de la enfermedad de Pompe de inicio tardío","authors":"C. Domínguez-González ,&nbsp;M.Á. Barba Romero ,&nbsp;C. Caballero Eraso ,&nbsp;J. de las Heras ,&nbsp;E. Farrero Muñoz ,&nbsp;Ó. García-Campos ,&nbsp;M. González ,&nbsp;J.M. Grau ,&nbsp;A. Hernández-Voth ,&nbsp;R. Juntas Morales ,&nbsp;J.C. León Hernández ,&nbsp;M. Ley Martos ,&nbsp;D. López-Padilla ,&nbsp;N. Muelas ,&nbsp;A. Nascimento ,&nbsp;M. Olivé ,&nbsp;C. Paradas ,&nbsp;J. Pardo Fernández ,&nbsp;S.I. Pascual ,&nbsp;I. Pitarch ,&nbsp;J. Díaz-Manera","doi":"10.1016/j.nrl.2025.501933","DOIUrl":"10.1016/j.nrl.2025.501933","url":null,"abstract":"<div><div>Pompe disease or glycogenosis type II is a rare disease caused by mutations in the <em>GAA</em> gene that leads to deficiency of the acid alpha-1,4-glucosidase enzyme. As a result of the enzymatic defect, a progressive accumulation of intralysosomal glycogen occurs in various tissues, causing smooth, cardiac and skeletal muscle involvement. When the age of onset of the disease is after the first year of life, it is called late-onset Pompe disease (LOPD). Weakness of the axial and proximal waist muscles and respiratory dysfunction are common manifestations. Enzyme replacement therapy (ERT) has been available for more than 15 years and is the standard treatment. This therapy changes the course of the disease, although the effectiveness of the treatment reduces over time. New enzyme therapies represent new treatment opportunities for patients with LOPD. Here we present updated recommendations from a group of experts in Pompe disease on the diagnosis, treatment and follow-up of LOPD patients, with the aim of providing a guide for the clinical management of the disease.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 501933"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412483","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}

{"title":"Protective effect of adult vaccination on the development of dementias: A systematic review","authors":"E. Mariscal-Lopez ,&nbsp;M. Agredano-Sanchez ,&nbsp;R.M. Lopez-Gigosos ,&nbsp;A. Mariscal ,&nbsp;F. Fariñas-Guerrero ,&nbsp;M. Gutierrez-Bedmar ,&nbsp;M. Guts-Chornoknyzha","doi":"10.1016/j.nrl.2025.501939","DOIUrl":"10.1016/j.nrl.2025.501939","url":null,"abstract":"<div><h3>Introduction</h3><div>Dementias currently impose a significant burden in terms of morbidity, mortality, socio-economic costs, and human suffering. Several studies have indicated that certain infectious diseases may increase the risk of dementia. Additionally, research has suggested that adult vaccines may help to prevent dementia. This systematic review looks into the possible association between adult vaccines and dementia in studies published between 2016 and April 2024.</div></div><div><h3>Development</h3><div>A search was conducted in accordance with the PRISMA 2020 guidelines to assess the potential association between adult vaccines and dementia in adults aged 50 and above. Quality and potential bias were evaluated using the revised Assessing the Methodological Quality of Systematic Reviews (AMSTAR-2) scale, the Newcastle–Ottawa scale, and the Risk-of-bias in Studies of Temporal Trends (ROBITT) tool. Fifteen out of the 16 selected studies showed a high degree of uniformity in terms of vaccinated adults having lower rates of dementia than unvaccinated adults. The observed risk reduction ranged from 4% to 50% (relative risk measures). The influenza vaccine has been the subject of the most extensive research, with 10 of the 16 studies focusing on it. Five studies demonstrated a dose–response relationship, indicating that a higher number of vaccines per patient is associated with a lower risk of dementia. Only one of the selected studies found an association between vaccination of adults and an increased risk of dementia.</div></div><div><h3>Conclusions</h3><div>The studies examined provide evidence supporting the hypothesis that adult vaccination may have a protective effect against the development of dementia. However, further molecular biology and pathophysiology studies are required to elucidate the underlying mechanisms and confirm the plausibility of this hypothesis.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 501939"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412484","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}

{"title":"Alcoholic Wernicke's encephalopathy with cranial neuropathies, atypical neuroimaging, dry beriberi, and Graves’ disease: A novel variant?","authors":"A. Nag ,&nbsp;M. León-Ruiz ,&nbsp;K. Bole ,&nbsp;R. Ghosh ,&nbsp;D. Naga ,&nbsp;J. Benito-León","doi":"10.1016/j.nrl.2024.10.001","DOIUrl":"10.1016/j.nrl.2024.10.001","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 101892"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412025","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}

{"title":"¿Cómo afrontamos los desafíos de la Sociedad Española de Neurología? Plan Estratégico de la Sociedad Española de Neurología","authors":"J.M. Láinez Andrés ,&nbsp;C. Íñiguez Martínez ,&nbsp;J. Porta Etessam ,&nbsp;D. Ezpeleta Echávarri ,&nbsp;M.T. Martínez de Albéniz Zabaleta ,&nbsp;M.M. Bilbao ,&nbsp;F. Escamilla Sevilla ,&nbsp;D.M. Cerdán Santacruz ,&nbsp;D. García Azorín ,&nbsp;J. Camiña Muñiz ,&nbsp;S. Arias Rivas ,&nbsp;S. Gil Navarro ,&nbsp;I.C. Labordena ,&nbsp;M.E. Gil Girbau ,&nbsp;C. Santarrosa Mateo","doi":"10.1016/j.nrl.2025.501934","DOIUrl":"10.1016/j.nrl.2025.501934","url":null,"abstract":"<div><h3>Introduction</h3><div>With the aim of redesigning the role of promoting and fostering the progress of neurology and anticipating the social, scientific, health, and economic context provided by the development of our specialty, the Spanish Society of Neurology (SEN) has decided to formalize its direction in a Strategic Plan, the elements of which are shared in this article.</div></div><div><h3>Methods</h3><div>The development of the Plan has been structured in three phases: internal and external analysis, strategic projection, and formalization of the plan. A qualitative and strategic analysis approach has been incorporated, through surveys, interviews, and participatory sessions with the SEN, with the participation of approximately 500 members and other professionals in the field. The current situation of the SEN and its environment has been explicitly stated, the corporate identity has been defined, and strengths, weaknesses, threats, and opportunities have been analyzed using the SWOT/CAME matrix. Finally, an Action Plan has been developed that identifies strategic pillars, objectives, and actions to be implemented.</div></div><div><h3>Results</h3><div>Five Strategic Pillars have been identified (SEN Image; Service Portfolio; Participation Spaces; Digital Transformation; Results-Oriented Management), comprising a total of 23 strategic objectives. A total of 80 actions are proposed to achieve the Plan's objectives by 2025.</div></div><div><h3>Conclusions</h3><div>The deployment of the Strategic Plan involves having a backbone instrument for the strategic lines that are expected to favor the position of the SEN as a key player within the specialty of neurology in the face of current and future challenges.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 2","pages":"Article 501934"},"PeriodicalIF":3.1,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147412444","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}

{"title":"Dysautonomia after COVID-19 infection: A case report","authors":"C.H. Vera-Cáceres ,&nbsp;M. García-Huguet ,&nbsp;A. Gil de Genover ,&nbsp;S.D. Sagula ,&nbsp;L. Martín Muñóz ,&nbsp;D. López Domínguez","doi":"10.1016/j.nrl.2024.08.004","DOIUrl":"10.1016/j.nrl.2024.08.004","url":null,"abstract":"<div><h3>Introduction</h3><div>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.</div></div><div><h3>Case presentation</h3><div>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.</div><div>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.</div><div>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.</div></div><div><h3>Conclusion</h3><div>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.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 101891"},"PeriodicalIF":3.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886487","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}

{"title":"Epidemiología y clínica de las crisis de ausencias frontales frente a las ausencias primarias","authors":"J.M. Ramos-Fernández ,&nbsp;A. Extraviz Moreno ,&nbsp;N. del Arco Guzman ,&nbsp;R. Calvo Medina ,&nbsp;L. Rodríguez Santos ,&nbsp;P. Navas Sánchez","doi":"10.1016/j.nrl.2023.11.014","DOIUrl":"10.1016/j.nrl.2023.11.014","url":null,"abstract":"<div><h3>Introduction</h3><div>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.</div></div><div><h3>Objective</h3><div>To describe the epidemiology of FAE in childhood and compare the characteristics, clinical progression, and pharmacological response with primary absence epilepsy.</div></div><div><h3>Patients and methods</h3><div>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.</div></div><div><h3>Results</h3><div>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).</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 101904"},"PeriodicalIF":3.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886485","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}

{"title":"Unravelling the temporal evolution of Parkinson's disease mortality in Spanish autonomous communities (1999–2021)","authors":"L. Cayuela ,&nbsp;E. Zapata-Arriaza ,&nbsp;A. de Albóniga-Chindurza ,&nbsp;A. González García ,&nbsp;A. Cayuela","doi":"10.1016/j.nrl.2025.501911","DOIUrl":"10.1016/j.nrl.2025.501911","url":null,"abstract":"<div><h3>Objective</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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.</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"41 1","pages":"Article 501911"},"PeriodicalIF":3.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886480","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}