Revue neurologique最新文献

{"title":"Amyloid PET imaging in France: One-year experience and perspectives","authors":"A. Verger , P. Payoux , S. Heyer , M.O. Habert , A. Flaus , M. Ribeiro , N. De Leiris , J. Pariente , D. Wallon , M. Ceccaldi , S. Bombois , E. Guedj , Groupe de travail « neurologie » de la Société française de médecine nucléaire (GT neurologie SFMN)","doi":"10.1016/j.neurol.2025.08.001","DOIUrl":"10.1016/j.neurol.2025.08.001","url":null,"abstract":"","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 699-702"},"PeriodicalIF":2.3,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144856180","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":"Training in movement disorders during neurology residency in France: A national survey on educational gaps and future perspectives","authors":"M. Aubignat ,&nbsp;S. Potel ,&nbsp;G. Carey ,&nbsp;V. Schneider ,&nbsp;V. Mira ,&nbsp;T. Ollivier ,&nbsp;S. Grimaldi ,&nbsp;T. Wirth ,&nbsp;E. Roze ,&nbsp;C. Desjardins ,&nbsp;behalf of the MAJE study group","doi":"10.1016/j.neurol.2025.07.014","DOIUrl":"10.1016/j.neurol.2025.07.014","url":null,"abstract":"<div><div>Movement disorders (MDs) subspecialty is a key discipline in neurology, requiring dedicated expertise and training for accurate diagnosis and management. However, structured data on neurology residency training in this field in France are lacking. This study aims to evaluate the accessibility, quality, and consistency of MDs education among French neurology residents and to identify areas for improvement. A national online survey was conducted from August to November 2024 among neurology residents across France. The questionnaire assessed theoretical and practical exposure to MDs, procedural training, self-reported confidence in managing MDs, and residents’ expectations regarding training. Responses were analyzed descriptively, with comparisons across residency years and hospital types. Data were obtained from 151 neurology residents at 24 university hospitals. MDs training was highly heterogeneous: 47.3% reported receiving ≤<!--> <!-->5<!--> <!-->h of theoretical MDs education, while only 2.7% exceeded 20<!--> <!-->h. Practical exposure was limited, with 25.2% of residents having attended MD consultations under supervision, and only 4% having performed botulinum toxin injections under supervision. Confidence in managing MDs was generally low, particularly for dystonia (60.9% felt unprepared) and Tourette syndrome/tics (79.5% felt unprepared). Residents identified major gaps in structured education, procedural training, and access to mentorship. They advocated for a standardized MDs curriculum, increased supervised practical training, and the development of innovative educational tools. In conclusion, MD training during neurology residency in France is inconsistent, with significant gaps in both theoretical and practical exposure. Standardizing MD education, expanding supervised procedural training, and fostering mentorship programs are essential steps to improve resident skills.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 808-816"},"PeriodicalIF":2.3,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144856183","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":"Quality of life in patients with spontaneous intracranial hypotension: A systematic review and meta-analysis","authors":"J. Ognard ,&nbsp;G. El Hajj ,&nbsp;S. Ghozy ,&nbsp;J.K. Cutsforth-Gregory ,&nbsp;A.A. Madhavan ,&nbsp;R. Kadirvel ,&nbsp;D.F. Kallmes ,&nbsp;W. Brinjikji","doi":"10.1016/j.neurol.2025.08.002","DOIUrl":"10.1016/j.neurol.2025.08.002","url":null,"abstract":"<div><h3>Background</h3><div>Not only are diagnosis and management of spontaneous intracranial hypotension (SIH) challenging due to heterogeneous symptoms and limited treatment effectiveness, but SIH's impact on health-related quality of life (HRQoL) is under-documented.</div></div><div><h3>Objectives</h3><div>In this systematic review, we aim to evaluate the assessment of QoL in SIH patients, identify impacted QoL domains, and explore treatment-related changes in QoL with a meta-analysis.</div></div><div><h3>Methods</h3><div>Following PRISMA recommendations, we conducted a systematic literature search using a comprehensive set of keywords related to QoL and SIH. Databases were searched from the inception to July 2025. Studies were included if they provided reports on the quality of life for SIH patients. A meta-analysis using mean difference (MD) of baseline and after-treatment QoL scores was conducted. The risk of bias was assessed using the Newcastle-Ottawa scale.</div></div><div><h3>Results</h3><div>Of 1435 initial publications, 20 studies met the inclusion criteria, representing a total of 1106 patients with SIH. EQ-5D-5L and HIT-6 were the most frequently used tools, with pooled results showing significant improvement post-treatment in perceived health (Visual analog scale score improved from 38.9 to 72.2; MD of 42.4 [95% CI 26.2–58.7]) and headache impact (HIT-6 scores improved from 66.1 to 49.3; MD of 20.1 [95% CI: 14.7–25.6]). Despite treatment, studies reported moderate to severe physical, mental, and social limitations.</div></div><div><h3>Discussion</h3><div>The reporting of QoL is inconsistent and the tools used to assess QoL in SIH patients are heterogenous. While treatment provides help, some symptoms persist and highlight the need for specific QoL assessment, with tools tailored to SIH.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 725-741"},"PeriodicalIF":2.3,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144856182","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":"Utility of tissue-based assays to elucidate an autoantibody-negative paraneoplastic syndrome: Lessons from a case report","authors":"G. Cogan ,&nbsp;D. Reguigne ,&nbsp;L.D. Do ,&nbsp;G. Picard ,&nbsp;M. Mongin ,&nbsp;B. Degos","doi":"10.1016/j.neurol.2025.07.013","DOIUrl":"10.1016/j.neurol.2025.07.013","url":null,"abstract":"","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 817-818"},"PeriodicalIF":2.3,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144856184","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":"Climate-related challenges to brain health: A European perspective review","authors":"J. Reis ,&nbsp;A. Buguet ,&nbsp;Z. Tulek ,&nbsp;A.-M. Landtblom ,&nbsp;M.W. Radomski ,&nbsp;Ş. Öztürk ,&nbsp;M. Pugliatti ,&nbsp;U. Kallweit ,&nbsp;P.S. Spencer","doi":"10.1016/j.neurol.2025.07.010","DOIUrl":"10.1016/j.neurol.2025.07.010","url":null,"abstract":"<div><div>In this paper, we provide a brief overview on the assessment of the numerous effects of climate change on human health, with particular emphasis on brain health. After reviewing the epidemiological data concerning neuro-psychiatric mortality and morbidity, we will examine the known effects of global warming in a European context. We will consider respectively, neurological diseases (epilepsy, migraine, stroke, sleep, neurodegenerative and neuroinfectious disorders), neurological involvement of climate-change induced health conditions, neuro-psychological aspects (post-traumatic stress and sleep disorders, eco-anxiety) and emerging risks for the brain (toxins and infections). We suggest some underlying mechanisms impacting the brain health. Given the paucity of available data, we call for increased research attention to the adverse effects of climate change. This perspective is intended to support a higher degree of preparedness, the development of improved preventive measures, and the promotion of targeted education for health professionals.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 713-724"},"PeriodicalIF":2.3,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144856181","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 predicting seizure control and delivery outcomes in women with epilepsy with planned and unplanned pregnancy","authors":"S. Melikova ,&nbsp;A. Mammadbayli ,&nbsp;A. Guekht","doi":"10.1016/j.neurol.2025.07.006","DOIUrl":"10.1016/j.neurol.2025.07.006","url":null,"abstract":"<div><h3>Objective</h3><div>To determine and ascertain factors influencing seizure control and maternal and neonatal outcomes in women with epilepsy (WWE) with planned versus unplanned pregnancies.</div></div><div><h3>Methods</h3><div>One hundred twelve pregnant WWE were prospectively evaluated for over an eight-year period. Patients were subsequently evaluated at 3, 6, and 12 months after delivery and then were followed up by a neurologist at least three years after delivery.</div></div><div><h3>Results</h3><div>The proportion of the patients with increased seizure frequency was higher in focal epilepsy versus generalized epilepsy (<em>P</em> <!-->&lt;<!--> <!-->0.05). The occurrence of seizures during pregnancy was associated with poor seizure control one year prior to the pregnancy and nonadherence to treatment (<em>P</em> <!-->&lt;<!--> <!-->0.0001). Structural brain abnormalities were associated with a higher risk of seizures during pregnancy (<em>P</em> <!-->=<!--> <!-->0.03). Women with seizures during pregnancy (<em>P</em> <!-->=<!--> <!-->0.0069) and with non-adherence to antiseizure medication (ASM) treatment had a higher rate of cesarean section (<em>P</em> <!-->=<!--> <!-->0.0069 and <em>P</em> <!-->&lt;<!--> <!-->0.05, respectively). The rates of adverse delivery outcomes were not higher in women with planned pregnancies compared to women with unplanned pregnancies. However, offspring of mothers with unplanned pregnancies and nonadherence to treatment were at higher risk of an Apgar score at 5<!--> <!-->minutes<!--> <!-->≤<!--> <!-->7 compared with infants of planned pregnancies (<em>P</em> <!-->=<!--> <!-->0.02 and <em>P</em> <!-->=<!--> <!-->0.0001, respectively).</div></div><div><h3>Conclusion</h3><div>Seizure control prior to pregnancy, epilepsy type, adherence to ASM therapy, and pregnancy planning are potential factors influencing both seizure control and delivery outcomes in WWE. Our findings imply that systematic preconception counseling is crucial to preventing seizure deterioration in pregnancy and reducing the maternal and fetal complications.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 790-800"},"PeriodicalIF":2.3,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144812283","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":"Presymptomatic multiple sclerosis: Insights from the Radiologically Isolated Syndrome.","authors":"C Lebrun-Frenay, M Cohen, D T Okuda","doi":"10.1016/j.neurol.2025.06.013","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.06.013","url":null,"abstract":"<p><p>Radiologically isolated syndrome (RIS) represents the initial phase of multiple sclerosis (MS) and is identified incidentally in asymptomatic individuals who display typical brain or spinal cord lesions indicative of autoimmune inflammatory demyelination. The 2023 RIS criteria enhance diagnostic precision against imaging mimics by requiring one T2-weighted hyperintense lesion in two of four specified locations (periventricular, juxtacortical/cortical, infratentorial, spinal cord) alongside at least two of the following: a spinal cord lesion, CSF-restricted oligoclonal bands (OCB), or a new T2/gadolinium-enhancing lesion on MRI at any point, defining dissemination in time (DIT). After confirming the diagnosis, established risk factors for transition to clinical MS need to be assessed. Key factors include a younger age, male sex, the presence of infratentorial, spinal cord, or gadolinium-enhancing lesions, and CSF-restricted OCBs or increased kappa-free light chains. Two randomized trials showed the efficacy of two oral disease-modifying therapies in delaying the first clinical event in RIS. However, as some individuals remain asymptomatic, it's crucial to identify suitable candidates to balance treatment benefits with potential risks. Reviewing each RIS case with an MS expert team is advisable for better care and monitoring. The updated 2024 McDonald criteria classify RIS patients with additional features, such as positive CSF and susceptibility MRI biomarkers, as having preclinical MS.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144804573","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":"Hyperoxemia and its impact on in-hospital mortality in intracerebral hemorrhage patients: A retrospective cohort study","authors":"C. Tian ,&nbsp;H. Zhou ,&nbsp;M. Yuan","doi":"10.1016/j.neurol.2025.07.003","DOIUrl":"10.1016/j.neurol.2025.07.003","url":null,"abstract":"<div><h3>Introduction</h3><div>This study aims to explore the relationship between arterial partial pressure of oxygen (PaO₂) and in-hospital mortality in patients admitted for the first time with intracerebral hemorrhage, with emphasis on the effect of hyperoxia on mortality.</div></div><div><h3>Methods</h3><div>We screened the MIMIC IV database for 1985 patients with cerebral hemorrhage admitted for the first time between 2008 and 2019. Exclusions included cases with duplicate records, patients with tumors, aneurysms, traumatic cerebral hemorrhage, cerebral hemorrhage due to blood disorders, and cases missing PaO₂ values — 828 patients for final analysis. Based on PaO₂ levels within 24<!--> <!-->hours of admission, patients were divided into three groups: high (&gt;<!--> <!-->150<!--> <!-->mmHg), medium (100–150<!--> <!-->mmHg), and low (&lt;<!--> <!-->100<!--> <!-->mmHg). A logistic mixed-effects regression model was used to analyze the relationship between PaO₂ and hospital mortality.</div></div><div><h3>Results</h3><div>The overall mortality rate during hospitalization was 37.1%. After adjusting for confounding factors, the mortality risk ratio (OR) of the low PaO₂ group and high PaO₂ group were 1.78 (95%CI: 1.04–3.03, <em>P</em> <!-->=<!--> <!-->0.034) and 2.09 (95%CI: 1.28–3.42, <em>P</em> <!-->=<!--> <!-->0.003), respectively. However, no significant associations were found between PaO₂levels and 28-, 60-, or 90-day mortality. Subgroup analysis showed an interaction between in-hospital mortality and PaO₂ level in patients with chronic lung disease (<em>P</em> <!-->=<!--> <!-->0.002). Sensitivity analysis showed that the relationship still existed after excluding extreme PaO₂ values.</div></div><div><h3>Conclusions</h3><div>Elevated arterial oxygen tension within the first 24<!--> <!-->hours of admission was independently associated with increased in-hospital mortality in patients with intracerebral hemorrhage.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 748-758"},"PeriodicalIF":2.3,"publicationDate":"2025-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144800099","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":"Influence of age and sex on presymptomatic phases of neurodegenerative diseases: Focus on multiple sclerosis and Alzheimer's disease.","authors":"B Zeydan, K Kantarci","doi":"10.1016/j.neurol.2025.06.015","DOIUrl":"10.1016/j.neurol.2025.06.015","url":null,"abstract":"<p><p>Neurodegenerative diseases such as multiple sclerosis (MS) and Alzheimer's dementia (AD) demonstrate an ever-evolving disease continuum. The presymptomatic phase of neurodegenerative diseases provides a window of opportunity to detect disease-specific changes and abnormalities early on and potentially intervene right away, before clinical symptoms occur. Age and sex are key modifiers of the presymptomatic phase of neurodegenerative diseases. In presymptomatic MS, younger age and male sex are main risk factors for transition to symptomatic MS, whereas older age and male sex are important predictors of the direct transition from presmyptomatic MS to primary progressive MS. In cognitively unimpaired adults, age is the strongest risk factor for AD and the lifetime AD risk after>65 years is higher in women versus men. The prevalence and future disease severity of AD is further modified by factors such as apolipoprotein Eɛ4, ovarian hormones, and menopause in women. Biomarkers are instrumental in detecting and monitoring abnormalities and underlying disease mechanisms in vivo, that are already present in the presymptomatic phase. Evaluating the influence of age and sex on presymptomatic phase of neurodegenerative diseases, particularly through biomarkers, contributes to the enhanced patient selection for clinical trials, optimization and individualization of patient management and development of new therapeutics.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12416211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144800100","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":"Homozygous DNAJB4 deletion revealing myopathy with acute respiratory failure","authors":"D.M. Chitimus ,&nbsp;C. Adam ,&nbsp;C. Cauquil ,&nbsp;B. Keren ,&nbsp;N. Heming ,&nbsp;S. Amthor ,&nbsp;D. Annane ,&nbsp;G. Nicolas ,&nbsp;P. Laforêt ,&nbsp;C. Métay ,&nbsp;C. Lefeuvre","doi":"10.1016/j.neurol.2025.07.004","DOIUrl":"10.1016/j.neurol.2025.07.004","url":null,"abstract":"<div><div>Homozygous mutations in the <em>DNAJB4</em> (NM_007034) gene impair HSP40 function, leading to early respiratory failure due to diaphragm involvement and rigid-spine-like characteristics. We describe the case of a 23-year-old male patient who was admitted for acute respiratory failure and motor deficit of the distal upper limbs. Creatine kinase values were elevated (10x upper normal limit), while the pulmonary function tests showed restrictive respiratory syndrome (forced vital capacity at 20% of theoretical values). The deltoid muscle biopsy findings were consistent with myofibrillar myopathy. Genetic analysis by NGS panel sequencing identified a homozygous deletion c.(?_1)_(1014_?)del, p.? (HGVS nomenclature) of the entire <em>DNAJB4</em> gene, confirmed by qPCR. Both healthy parents exhibited the variant at the heterozygous state. Our results demonstrate that homozygous c.(?1)(1014_?)del, p.? deletion in <em>DNAJB4</em> leads to a hereditary myopathy, further underscoring the gene's crucial role in muscle maintenance and function.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 759-764"},"PeriodicalIF":2.3,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144795263","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}