European Neurology最新文献

{"title":"Combined Olfactory Testing and Substantia Nigra Hyperechogenicity for Diagnostic Differentiation of Parkinson's Disease.","authors":"Jiří Kozel, Eva Augste, Patricie Michalčová, Dana Šalounová, Anežka Chalánková, Petra Bártová, István Szegedi, David Školoudík","doi":"10.1159/000552191","DOIUrl":"10.1159/000552191","url":null,"abstract":"<p><strong>Introduction: </strong>Olfactory impairment and substantia nigra (SN) hyperechogenicity are well-recognized features of Parkinson's disease (PD). Their complementary diagnostic value and demographic influences warrant further clarification. This study aimed to assess the relationship and combined diagnostic value of SN hyperechogenicity and olfactory dysfunction in patients with clinically diagnosed PD.</p><p><strong>Methods: </strong>We conducted a cross-sectional study including 89 PD patients and 113 healthy controls. Olfactory function was evaluated using the 16-item Sniffin' Sticks test, and SN echogenicity was measured by transcranial sonography. Participants were stratified by age (≤60 and >60 years). The diagnostic performance of each marker, individually and in combination, was analyzed.</p><p><strong>Results: </strong>PD patients showed significantly lower olfactory scores and higher SN echogenicity than controls (both p < 0.001). Both markers were significantly more prevalent in PD. In controls, olfactory function declined with age (r = -0.489, p < 0.001), whereas SN echogenicity remained stable. No overall correlation was observed between the two markers in PD. Sex differences in olfaction appeared in controls but not in PD. Combining hyposmia and SN hyperechogenicity yielded 83.1% sensitivity, 95.6% specificity, and 90.1% accuracy. In participants aged ≤60 years, higher specificity and positive predictive value were observed, although this subgroup included a relatively small number of patients.</p><p><strong>Conclusion: </strong>Olfactory dysfunction and SN hyperechogenicity are significantly more prevalent in PD than in controls. When used together, these markers provide complementary diagnostic information and high specificity for diagnostic differentiation between PD and healthy controls. Age-related effects on olfactory function highlight the importance of demographic adjustment when interpreting olfactory test results.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"154-164"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148053527","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":"Historical and Clinical Analysis of a Case of Progressive Muscular Atrophy (1853-1871).","authors":"Emmanuel Drouin, Yann Pereon","doi":"10.1159/000550658","DOIUrl":"10.1159/000550658","url":null,"abstract":"<p><strong>Background: </strong>Progressive muscular atrophy (PMA) emerged in the mid-19th century as a distinct clinical entity within the evolving field of French neurology, notably through the work of François Amilcar Aran, Duchenne de Boulogne, and later Jean-Martin Charcot. During this period, uncertainties persisted regarding its nosological status, pathophysiology, and relationship to amyotrophic lateral sclerosis (ALS). Longitudinal clinical observations from this era remain rare but are essential for understanding both the natural history of motor neuron diseases and the historical construction of neurological knowledge.</p><p><strong>Summary: </strong>This article presents a historical and clinical analysis of a unique case of PMA observed for over nearly 2 decades (1853-1871) in Parisian hospitals. The case concerns Auguste-Joseph Bellinghen, whose condition was first documented in an unpublished handwritten manuscript in 1853 and later published with photographic illustrations in 1871. Through a comparative analysis of these two observations, the study traces the slow, asymmetrical, and irreversible progression of muscular atrophy, marked by early fasciculations, the absence of sensory disturbances, and eventual severe motor disability. The case is examined within its institutional, nosological, and therapeutic contexts, highlighting hospital circulation, the role of medical interns, and the empirical treatments of the time, including electrotherapy and thermal baths. Reinterpreted in light of contemporary neurology, this historical observation likely corresponds to a spinal-onset motor neuron disease closely related to ALS. Beyond its clinical significance, the case illustrates the transition from descriptive clinical medicine to anatomoclinical correlation and contributes to the historiography of neurology by illuminating how individual patient trajectories shaped medical knowledge in the 19th century.</p><p><strong>Key messages: </strong>(1) Long-term historical clinical observations provide valuable insights into the natural history of PMA and motor neuron diseases. (2) The Bellinghen case illustrates the evolution of neurological semiology, particularly the early recognition of fasciculations and asymmetrical muscle wasting. (3) This case highlights the transition from Aran's initial clinical description of PMA to Charcot's anatomopathological framework linking PMA to ALS. (4) Historical medical archives offer not only scientific data but also a window into the social consequences of chronic neurological disease in the 19th century. (5) Integrating historical and clinical analysis enriches contemporary understanding of motor neuron disease nosology and medical memory.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"130-137"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147484999","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":"Digital Color Vision Testing Reveals Widespread Cone Dysfunction in Multiple Sclerosis Independent of Optic Neuritis.","authors":"Yehuda Warszawer, Aviva Gal, Terrace Waggoner, Yael Nissan, Anat Achiron","doi":"10.1159/000549814","DOIUrl":"10.1159/000549814","url":null,"abstract":"<p><p><p>Introduction: We aimed to investigate the prevalence and pattern of color vision deficiency (CVD) in relapsing-remitting multiple sclerosis (RRMS) patients, with or without a history of optic neuritis (ON), and to assess its potential as a marker of subclinical optic pathway dysfunction.</p><p><strong>Methods: </strong>Color vision was assessed in a large cohort of 345 RRMS patients using the Waggoner Computerized Color Vision Test, screening for protan, deutan, tritan, and composite color deficiencies, with severity grading based on score thresholds. Statistical differences between eyes with a history of ON, fellow eyes without ON, and eyes from MS patients with no history of ON were analyzed using multivariable logistic regression, controlled for age, gender, disease duration, Expanded Disability Status Scale, and treatment status.</p><p><strong>Results: </strong>Out of the 676 eyes analyzed, CVD was observed in 76.0% of ON-affected eyes, 65.1% of fellow eyes without ON, and 62.3% of eyes from patients without ON. Multivariable logistic regression showed ON-affected eyes had significantly higher odds of severe CVD compared to fellow eyes (OR = 2.48, p = 0.020), which in turn had higher odds of moderate CVD compared to the no-ON group (OR = 2.27, p = 0.010). Conversely, the no-ON group had higher odds of mild CVD compared to fellow eyes without ON (OR = 2.01, p = 0.003). Composite CVD was the most frequent type (57.7%), with tritan deficiencies being the most common pure type (39.9%). Fellow eyes demonstrated higher odds of composite CVD compared to the no-ON group (OR = 1.97, p = 0.016).</p><p><strong>Conclusions: </strong>CVD is highly prevalent in MS even without clinical ON, indicating diffuse, subclinical optic pathway damage. </p>.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"13-21"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12779286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145631467","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Disorders of Arousal and Sleep-Related Hypermotor Epilepsy in Adults: A Challenging but Necessary and Critical Distinctive Diagnosis.","authors":"Daniela Cimatti, Greta Mainieri, Luca Baldelli, Federica Provini","doi":"10.1159/000551918","DOIUrl":"10.1159/000551918","url":null,"abstract":"<p><strong>Background: </strong>Sleep-related hypermotor epilepsy (SHE) and disorders of arousal (DoA) are two conditions that, despite originating from distinct etiological mechanisms, share the manifestation of complex motor behaviours emerging from sleep. Their overlapping clinical features have long posed challenges for differential diagnosis, particularly in adults.</p><p><strong>Summary: </strong>Both SHE and DoA exhibit a close relationship with sleep physiology; consequently, increasing attention has recently been devoted to the neurophysiological mechanisms underlying these motor phenomena, with a specific focus on sleep microstructure and arousal system dynamics. K-complexes may play a key role in the onset of SHE seizures, as evidenced by the tendency of seizures to occur in quasi-periodic clusters at frequencies consistent with K-complexes and other physiological oscillations during light sleep. Analyses of the cyclic alternating pattern (CAP), an EEG marker of sleep instability, have shown that epileptic activity is not uniformly distributed across non-rapid eye movement sleep but is instead enhanced during specific CAP subtypes. A similar pattern has been observed in DoA, with episodes frequently arising during CAP phase A. The sleep instability observed in both SHE and DoA suggests that transient cortical activations may act as triggers for motor events, facilitating the expression of innate motor patterns through subcortical circuit activation. Although distinct in origin, physiological in DoA, and epileptic in SHE, both disorders reflect altered arousal regulation and disrupted cortical-subcortical interactions.</p><p><strong>Key messages: </strong>This review aimed to provide an integrated neurophysiological perspective on SHE and DoA, emphasizing their distinctive mechanisms and the broader implications for understanding sleep-related motor behaviours.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"138-149"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147688948","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":"Factors Affecting Prehospital Delay in Patients with Acute Ischemic Stroke in the Republic of Georgia: A Countrywide, Prospective Study.","authors":"Tamar Janelidze, Teona Janiashvili, Tamar Akhvlediani, Irine Pkhakadze, Nana Gelenidze, Natia Bitsadze, Ketevan Tsikhiseli, Mariam Ioseliani, Nino Sharia, Khatia Danelia, Ani Sergeenko, Vlasi Kakabadze, Nino Mdivnishvili, Nino Jalagonia, Giorgi Argvliani, Giorgi Abuladze, Giorgi Mamardashvili, Dali Mebonia, Ketevan Dzagoevi, Elene Koiava, Ketevan Paposhvili, Temur Margania, Nana Skhulukhia, Nino Fifia, Marine Todua, Zaza Katsarava","doi":"10.1159/000550217","DOIUrl":"10.1159/000550217","url":null,"abstract":"<p><strong>Introduction: </strong>Recently, a nationwide stroke care consisting of stroke care units (SCUs) and stroke centers (SCs) was established in the Republic of Georgia.</p><p><strong>Patients and methods: </strong>A hospital-based prospective registry to evaluate the quality of stroke care and an additional nationwide survey on factors leading to prehospital delay and subsequent low rate of intravenous thrombolysis (IVT) and endovascular thrombectomy (EVT).</p><p><strong>Results: </strong>A total of 5,385 consecutive patients with acute stroke (3,636 ischemic, 1,302 TIA, and 447 hemorrhagic) were included in the stroke registry, of whom 2,459 (46%) were female, with a mean age of 70.3±11.3 years. A total of 1,538 (28.6%) patients presented <6 h (1,160 [21.5%] <4.5 h). Of 3,636 patients with ischemic stroke, 121 (3.3%) patients received IVT, 72 (2.0%) received EVT, and 17 (0.5%) received both. An additional 1,067 patients with acute ischemic stroke (898) and TIA (169) were interviewed to understand the factors of prehospital delay. Of these, 472 (44.2%) were female, with a mean age of 70.5 ± 11.4 years, and 339 (31.8%) patients arrived <6 h (219 [20.5%] <4.5 h). Forty-two patients (4.7%) received IVT, 37 (4.1%) received EVT, and 6 (0.7%) received both. Pre-ictal education level, high socio-economic status, stroke awareness, calling the ambulance, and a knowledgeable ambulance team transferring patients to the stroke-ready hospital predicted timely arrival at the SC.</p><p><strong>Conclusions: </strong>The study reveals major problems in the prehospital management of acute stroke.</p><p><strong>Discussion: </strong>There is a great need for a nationwide geographic transfer plan for acute stroke service, connecting ambulances, SCUs, and SCs and for the educational campaigns to increase stroke awareness among the population.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"58-65"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145862554","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":"The Role of Stroke Severity in the Association between Hypoperfusion Intensity Ratio and Futile Reperfusion after Endovascular Treatment.","authors":"Liyuan Wang, Ziqi Xu, Hongjian Shen, Hao Wang, Hongchao Shi, Xiangliang Chen, Wenhua Liu, Wei Li, Kaixuan Yang, Guangshuo Li, Yahui Hao, Zhixin Cao, Manjun Hao, Na Wu, Pengfei Yang, Yunyun Xiong","doi":"10.1159/000552523","DOIUrl":"10.1159/000552523","url":null,"abstract":"<p><strong>Introduction: </strong>The hypoperfusion intensity ratio (HIR) was significantly correlated with the futile reperfusion (FR) and National Institutes of Health Stroke Scale (NIHSS) score. We aimed to quantify the direct and indirect effect of HIR on FR.</p><p><strong>Methods: </strong>We analyzed acute ischemic stroke patients with large vessel occlusion who underwent endovascular treatment at seven comprehensive stroke centers between 2017 and 2022 in China. HIR was automatically calculated on baseline perfusion imaging as the ratio of brain volume with time-to-max (Tmax) delay >10 s over volume with Tmax >6 s. The established threshold HIR >0.4 was regarded as poor tissue-level collaterals (TLCs). FR was defined as the modified Rankin Scale score of 4-6 at 90 days despite successful recanalization. Mediation analysis using the \"mediation\" package in R 4.2.2 was performed to examine the potential causal chain.</p><p><strong>Results: </strong>Among the 891 included patients, FR was observed in 320 (35.9%) patients. Inadequate TLC was significantly associated with a higher NIHSS score (adjusted common odds ratio [OR], 1.47; 95% confidence interval [CI], 1.12-1.93; p = 0.006) and higher rates of FR (aOR, 1.87; 95% CI, 1.31-2.68; p = 0.001) within 90 days. The baseline NIHSS score was a predictor of FR (aOR, 1.13; 95% CI, 1.10-1.16; p < 0.001). Causal mediation analyses revealed that 20.4% (95% CI, 5.5%-40.9%) of the relationship between HIR and FR was mediated by the baseline NIHSS score.</p><p><strong>Conclusion: </strong>Higher NIHSS score partially mediates the association between poorer HIR and FR at 90 days among patients after EVT. Our study provided primarily mechanistic and prognostic findings about the effect of HIR on FR.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"165-174"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147981351","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":"Plasma Brain-Derived Neurotrophic Factor Levels, Brain-Derived Neurotrophic Factor Val66Met Polymorphism, and Their Association with Phenoconversion in Isolated REM Sleep Behavior Disorder.","authors":"Yajie Zang, Hui Zhang, Zheng Ruan, Ting Wang, Yuan Li, Yuan Yuan, Qian Yu, Yanning Cai, Wei Mao","doi":"10.1159/000550711","DOIUrl":"10.1159/000550711","url":null,"abstract":"<p><p><p>Introduction: Brain-derived neurotrophic factor (BDNF) plays an important role in the survival of dopaminergic neurons. Clinical studies have suggested that serum BDNF levels are reduced in patients with Parkinson's disease (PD). However, no study has investigated peripheral BDNF levels and BDNF Val66Met polymorphism in the prodromal stage of PD and their relationship with disease conversion.</p><p><strong>Methods: </strong>In total, 120 patients with video-polysomnography confirmed isolated REM sleep behavior disorder (iRBD) and 120 healthy controls (HCs) were enrolled. Genetic analyses were performed, and plasma levels of BDNF were measured. All patients with iRBD underwent comprehensive clinical testing, and 107 iRBD patients were prospectively followed up.</p><p><strong>Results: </strong>Plasma BDNF levels were significantly lower in the iRBD group than in HCs (18,878.85 pg/mL vs. 24,649.85 pg/mL, p = 0.002), but no differences were observed in BDNF Val66Met carrier rates between the two groups. Plasma BDNF levels did not differ significantly between BDNF Val66Met carriers and noncarriers. Notably, higher plasma BDNF levels were associated with an increased risk of short-term disease conversion (hazard ratio = 3.418, 95% CI: 1.520-7.684, p = 0.003), whereas BDNF Val66Met carrier rates showed no such association.</p><p><strong>Conclusion: </strong>Our findings suggest that plasma BDNF is significantly associated with iRBD and may likely serve as a prognostic biomarker for the development of neurodegenerative disease. However, the BDNF Val66Met polymorphism may not be involved in the pathogenesis of iRBD as well as phenoconversion in the studied population. </p>.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"66-74"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146092527","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":"Association between Internal Carotid Artery Tortuosity and Hemorrhagic Transformation in Patients with Acute Ischemic Stroke.","authors":"Jae-Chan Ryu, Sang-Hun Lee, Eun-Tae Jeon, Ha-Na Song, Hangseok Choi, Hwan-Ho Cho, Hyunjin Park, In Young Baek, Kyungseo Kim, Seongvin Ju, Jong-Un Choi, Seong-Hoon Lee, Yun-Hwa Ha, Ji-Eun Lee, Woo-Keun Seo","doi":"10.1159/000549765","DOIUrl":"10.1159/000549765","url":null,"abstract":"<p><p><p>Introduction: Hemorrhagic transformation is one of the most common complications of acute ischemic stroke. However, the association between hemorrhagic transformation and internal carotid artery (ICA) tortuosity has rarely been investigated. This study investigated the effect of ICA tortuosity on hemorrhagic transformation after acute ischemic stroke.</p><p><strong>Methods: </strong>We conducted a retrospective analysis on stroke patients' data collected from prospective hospital-based stroke registry. The baseline demographics, clinical and radiological variables, including the Fazekas scale, advanced Fazekas scale (grade 0-9), white matter hyperintensity volume, presence of cerebral microbleeds, and hemorrhagic transformation, were compared based on the severity of ICA tortuosity and divided into quartiles (Q1: <25%, Q2+Q3: 25%-75%, and Q4: >75%). ICA tortuosity was examined using in-house vessel analysis software. The primary outcome was hemorrhagic transformation.</p><p><strong>Results: </strong>A total of 146 patients were analyzed, including 167 ICAs, which were vascular territories with acute cerebral infarction. Among the various risk factors considered, intravenous thrombolysis, endovascular thrombectomy, stroke severity, and ICA tortuosity index were associated with the occurrence of hemorrhagic transformation. In multivariate analysis, ICA tortuosity was also significantly associated with the occurrence of hemorrhagic transformation, while the severity of ICA tortuosity was negatively correlated (Q1: reference, Q2+Q3: 0.27 [0.09-0.84], Q4: 0.14 [0.03-0.70]; p = 0.023 for Q2+Q3, p = 0.016 for Q4).</p><p><strong>Conclusions: </strong>We identified a paradoxical relationship between ICA tortuosity and hemorrhagic transformation after acute ischemic stroke. Patients with more severe tortuosity experienced fewer hemorrhagic transformations, and vice versa. These findings highlight the potential of ICA tortuosity as a marker for identifying high-risk patients. </p>.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"47-57"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12807496/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145695922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Global Perspectives on the Link between REM Sleep Behavior Disorder and Neurodegenerative Disorders.","authors":"Haixia Fan, Lu Zhai, Shudan Deng, Limantian Wang, Yan Li, Huiyan Niu, Bomeng Zhao, Jie Gao, Xiaoling Gao","doi":"10.1159/000550504","DOIUrl":"10.1159/000550504","url":null,"abstract":"<p><strong>Introduction: </strong>Rapid eye movement sleep behavior disorder (RBD) represents a parasomnia marked by impaired muscle inhibition during REM sleep, commonly manifesting as vivid dream enactment with potentially violent movements. Its strong association with neurodegenerative diseases, particularly α-synucleinopathies such as Parkinson's disease (PD), has drawn increasing attention. This study presents a bibliometric overview of global RBD research from 2010 to 2025.</p><p><strong>Methods: </strong>We analyzed 4,204 publications from the Web of Science Core Collection and Scopus databases. Tools including CiteSpace, VOSviewer, Python, R (bibliometrix), Excel, and Charticulator were used to examine publication trends, geographic and institutional distribution, author influence, journal preferences, co-citation networks, and keyword evolution.</p><p><strong>Results: </strong>RBD-related publications increased steadily, with a marked rise after 2015. The USA, China, and Canada were leading contributors, and institutions such as the University of Toronto, Mayo Clinic, and Peking University showed strong productivity and collaboration. Key researchers included Schenck CH, Boeve BF, and Postuma RB. Top journals were Sleep, Neurology, and Movement Disorders. Research has shifted from clinical characterization to biomarker identification and early intervention. Recent themes include α-synuclein pathology, prodromal PD, and melatonin therapy.</p><p><strong>Conclusion: </strong>RBD research has progressed toward translational approaches focused on early detection and neurodegenerative risk prediction, supported by global collaboration and growing interest in mechanistic insights.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"103-121"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147347813","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":"Jean-Martin Charcot and the Clinical Foundations of Amyotrophic Lateral Sclerosis: A Historical Case Revisited.","authors":"Emmanuel Drouin, Patrick Hautecoeur, Arnaud Kwiatkowski","doi":"10.1159/000548249","DOIUrl":"10.1159/000548249","url":null,"abstract":"<p><p>This article revisits an original case of bulbar-onset amyotrophic lateral sclerosis (ALS) recorded by Jean-Martin Charcot in 1877-78, highlighting his clinical methodology and its enduring significance. The case of Eugénie Florentine P., a 62-year-old patient at the Salpêtrière, illustrates Charcot's rigorous approach: precise observation of clinical signs, longitudinal documentation of disease progression, and anatomoclinical correlation confirmed by autopsy findings. Charcot identified the combined role of anterior horn and lateral column lesions, establishing ALS as a distinct nosological entity. While predecessors such as Aran, Cruveilhier, and Duchenne contributed key observations, Charcot synthesized clinical and pathological data to define the disease later known as \"Charcot's disease.\" This historical perspective underscores the lasting value of bedside examination in an era dominated by neuroimaging and biomarkers. By comparing 19th-century clinical reasoning with current diagnostic and therapeutic challenges, the article demonstrates how Charcot's work continues to inform the modern multidisciplinary management of ALS.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"97-101"},"PeriodicalIF":2.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145058499","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}