European Journal of Neurology最新文献

{"title":"Non-Invasive Intracranial Pressure Waveform Assessment as a Screening Test for Abnormal Lumbar Puncture Results","authors":"Gabriel Taricani Kubota,&nbsp;Alessandra Santos da Fonseca,&nbsp;Cintya Yukie Hayashi,&nbsp;Rebecca Gabriela de Oliveira Pereira,&nbsp;Thauan Gonçalves,&nbsp;Helio Rodrigues Gomes,&nbsp;Valquíria Aparecida da Silva,&nbsp;Ida Fortini,&nbsp;Eberval Gadelha Figueiredo,&nbsp;Lin Tchia Yeng,&nbsp;Manoel Jacobsen Teixeira,&nbsp;Daniel Ciampi de Andrade","doi":"10.1111/ene.70776","DOIUrl":"10.1111/ene.70776","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Lumbar puncture (LP) is essential for detecting intracranial hypertension (IH) but is often non-diagnostic and not without complications. A skull extensometer-based non-invasive intracranial pressure waveform assessment (NIWA) has shown promise in detecting IH in neurocritical settings. This study investigated its feasibility and accuracy among outpatient neurological individuals.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This cross-sectional study enrolled adults referred to routine outpatient LP at a tertiary-care hospital. Subjects underwent baseline supine NIWA, then were sequentially divided (2:1:1 ratio) to: LP then follow-up supine NIWA; follow-up NIWA while sitting then LP; or follow-up NIWA during Valsalva maneuver then LP. Several waveform parameters and their variation between baseline and follow-up assessments were compared with LP results. IH was defined as LP opening pressure &gt; 25cmH₂O.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The study included 130 subjects, and 28 (21.5%) presented with IH. Dicrotic peak slope &lt; 0.143 predicted IH with 89.5% sensitivity and 29.7% specificity (AUROC = 0.680, 95% CI 0.541–0.819). Equations derived from multivariate logistic regression models including mostly latency-related parameters of the entire waveform and its percussion and dicrotic peaks predicted cerebrospinal fluid (CSF) chemocytology changes with AUROC = 0.661 (95% CI 0.542–0.780) and overall abnormal LP results (IH and/or altered CSF chemocytology) with 96.2% sensitivity and 24.1% specificity (AUROC = 0.696, 95% CI 0.572–0.820). Parameter changes after LP, during Valsalva maneuver, and sitting position did not improve diagnostic accuracy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>NIWA was feasible and detected IH and CSF changes with high sensitivity among real-world neurological outpatients. While LP remains pivotal to many cases, pre-LP screening with NIWA may enhance diagnostic yield and mitigate complications and healthcare-related costs.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13664666/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180243","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Altered Saccades, Blink and Pupil Responses in Functional Motor Disorder: New Insight Into Neurobiological Mechanisms","authors":"Katarína Šútorová,&nbsp;Heidi C. Riek,&nbsp;Isabell C. Pitigoi,&nbsp;Donald C. Brien,&nbsp;Brian C. Coe,&nbsp;Barbora Křupková,&nbsp;Lucia Nováková,&nbsp;Tomáš Sieger,&nbsp;Douglas P. Munoz,&nbsp;Tereza Serranová","doi":"10.1111/ene.70774","DOIUrl":"10.1111/ene.70774","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Functional motor disorder (FMD) is a common and disabling condition with incompletely understood pathophysiology. Eye-tracking offers a method to objectively examine cognitive and motor control processes and their underlying neural pathways. We aimed to quantify saccade, blink, and pupil responses in FMD and healthy controls performing an interleaved pro-/anti-saccade task, and to investigate the relationships between oculomotor measures and motor and non-motor symptom severity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted video-based eye-tracking in 104 patients with clinically definite FMD and 115 age- and sex-matched healthy controls performing the saccade task. Patients completed questionnaires on depressive, pain-related, dissociative, and non-motor somatic symptoms. Clinician-rated motor severity and centrally acting medication were recorded in FMD patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Compared to controls, FMD patients showed increased anti-saccade error rates (<i>p</i> &lt; 0.001), anticipatory saccades (<i>p</i> ≤ 0.003), altered blink distribution (<i>p</i> &lt; 0.001), and reduced pupil dilation velocity (<i>p</i> &lt; 0.001). However, reduced pupil dilation velocity was not significant in the subsample of unmedicated patients. Higher anti-saccade error rates were significantly associated with depressive symptoms, pain severity, dissociative symptoms, non-motor somatic symptom burden, and motor severity (all <i>p</i> &lt; 0.05).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>We hypothesize that the altered saccade and blink responses result from altered processing in the frontal cortex and basal ganglia, which provide critical input to brainstem oculomotor control areas in FMD. These results support neurobiological models proposing altered predictive and attentional processing underlying FMD. Association between oculomotor measures and symptom severity suggests that specific cognitive abnormalities may play a role in the pathophysiology of these symptoms in FMD.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70774","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Migraine in Older Adults: Age-Related Clinical Features From the Italian RICe Registry","authors":"Giulia Paparella,&nbsp;Luigi Francesco Iannone,&nbsp;Giulia Paparella,&nbsp;Grazia Sances,&nbsp;Roberto De Icco,&nbsp;Gloria Vaghi,&nbsp;Cristina Tassorelli,&nbsp;Andrea Burgalassi,&nbsp;Francesco De Cesaris,&nbsp;Alberto Chiarugi,&nbsp;Francesca Gabriele,&nbsp;Raffaele Ornello,&nbsp;Simona Sacco,&nbsp;Maria Pia Prudenzano,&nbsp;Martino Gentile,&nbsp;Damiano Paolicelli,&nbsp;Giorgia Dalla Volta,&nbsp;Marco Bolchini,&nbsp;Innocenzo Rainero,&nbsp;Elisa Rubino,&nbsp;Maria Albanese,&nbsp;Fabrizio Vernieri,&nbsp;Gianluca Coppola,&nbsp;Gianluca Avino,&nbsp;Edoardo Mampreso,&nbsp;Delfina Ferrandi,&nbsp;Marta Altieri,&nbsp;Stefania Battistini,&nbsp;Antonio Granato,&nbsp;Simona Guerzoni,&nbsp;Antonio Russo,&nbsp;Marina de Tommaso,&nbsp;the RICe Study Group","doi":"10.1111/ene.70761","DOIUrl":"10.1111/ene.70761","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Primary headaches, particularly migraine, are prevalent disorders insufficiently characterized in older adults. This multicentre study evaluated headache diagnoses, focusing on migraine, across age groups in headache centers participating in the Italian Registry for Headache Disorders (RICe).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Using a post hoc design, we evaluated 3477 patients enrolled at 30 headache centers (2019–2025). Patients were stratified into young adults (18–30 years), adults (31–45 years), middle-aged (46–59 years), and elderly individuals (≥ 60 years). Demographic and clinical migraine characteristics were collected. Group differences were assessed using <i>χ</i>² tests and Kruskal–Wallis ANOVAs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 3477 patients, 644 (18.5%) were elderly, 1285 (36.9%) middle-aged, 990 (28.5%) adults, and 558 (16.1%) young adults. Among migraine patients (<i>n</i> = 3013, 86.7%), diagnoses differed across age groups (<i>χ</i>²(6) = 71.5, <i>p</i> &lt; 0.001). Elderly individuals more frequently had chronic migraine (CM) (<i>z</i> = 4.18), whereas migraine with aura was more common in young and adult patients (<i>z</i> = 3.62 and 4.97, respectively). Medication-overuse headache was more frequent in the elderly (<i>z</i> = 3.56), who reported more monthly migraine days but lower pain intensity and fewer severe attacks (all <i>p</i> &lt; 0.02). Stress and physical activity were common triggers. Among patients with available treatment data, some differences in prior preventive treatment patterns were observed, with more frequent use of beta-blockers and onabotulinumtoxinA in the elderly, whereas anti-calcitonin gene-related peptide (CGRP) monoclonal antibody prescriptions did not differ across groups.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Older adults attending headache centers more frequently presented CM, with higher attack frequency, lower pain severity, greater medication overuse, and distinct triggers, supporting tailored diagnostic and therapeutic strategies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13664672/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Blood Glial Fibrillary Acidic Protein in MRI-Negative Attack-Like Episodes of Neuromyelitis Optica Spectrum Disorder","authors":"Su-Hyun Kim,&nbsp;Jieun Chung,&nbsp;Rosah May Palermo Payumo,&nbsp;Jae-Won Hyun,&nbsp;Ho Jin Kim","doi":"10.1111/ene.70784","DOIUrl":"10.1111/ene.70784","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Magnetic resonance imaging (MRI) is widely used to support relapse diagnosis in neuromyelitis optica spectrum disorder (NMOSD), yet some patients present with attack-like symptoms without corresponding lesions. We assessed serum glial fibrillary acidic protein (sGFAP) levels in MRI-negative attack-like episodes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively analyzed aquaporin-4-IgG–positive NMOSD patients presenting with relapse-like symptoms but without corresponding new MRI lesions. sGFAP was measured from stored serum collected at presentation and converted to demographic-adjusted <i>z</i>-scores. A predefined attack-associated threshold (<i>z</i>-score ≥ 3.0) was based on prior work.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We identified 39 MRI-negative attack-like episodes from 34 patients. Most episodes were clinically mild or subjective, with objective worsening observed in 23%. Eight episodes (21%) showed elevated sGFAP levels (median <i>z</i>-score 4.56 [1748 pg/mL]). Compared with episodes without elevated sGFAP, these episodes occurred closer to prior attacks (8 vs. 33 months) and more frequently met objective worsening criteria (50% vs. 16%). In all episodes with elevated sGFAP, remission samples showed normalization below the threshold.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>Marked sGFAP elevation in a subset of MRI-negative episodes suggests that some events may represent relapse not captured by conventional imaging. sGFAP may serve as a complementary biomarker to support attack identification when clinical and MRI findings are inconclusive.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660715/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180220","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Science Is a Lot of Fun: How Professor Jes Olesen Made Discoveries in Migraine","authors":"Elena R. Lebedeva","doi":"10.1111/ene.70775","DOIUrl":"10.1111/ene.70775","url":null,"abstract":"<p>The human provocation model, cortical spreading depression during the aura and the identification of CGRP all came from one investigator following the same method for 60 years. Marking the 85th birthday of Professor Jes Olesen, Honorary Member of the EAN and founder of the European Journal of Neurology, this interview describes how each discovery was actually obtained. Migraine, once a disorder in which nothing could be measured, now has an objectively documented aura, an established molecular physiology, criteria used worldwide and drugs designed against a target these experiments identified.\u0000 <figure>\u0000 <div><picture>\u0000 <source></source></picture><p></p>\u0000 </div>\u0000 </figure></p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660710/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progressive Amyloid-Related Imaging Abnormalities After Discontinuation of Lecanemab in Early Alzheimer Disease in an APOE ε2/ε4 Carrier","authors":"Elisabeth Stögmann,&nbsp;Lukas Haider,&nbsp;Joel Leinkauf,&nbsp;Thomas Nakuz,&nbsp;Helena Untersteiner,&nbsp;Clemens Farr,&nbsp;Sara Silvaieh,&nbsp;Tandis Parvizi,&nbsp;Raphael Wurm,&nbsp;Gregor Kasprian,&nbsp;Thomas Berger,&nbsp;Ellen Gelpi,&nbsp;Evelyn Berger Sieczkowski","doi":"10.1111/ene.70779","DOIUrl":"10.1111/ene.70779","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Amyloid-related imaging abnormalities (ARIA) are the most relevant adverse events associated with anti-amyloid therapies in Alzheimer's disease.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We report a case of progressive ARIA after discontinuation of lecanemab in a patient with early-onset Alzheimer's disease carrying the rare APOE ε2/ε4 genotype.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>After two infusions, mild ARIA-edema (ARIA-E) and ARIA-hemorrhage (ARIA-H) were detected on routine MRI, prompting treatment interruption. Despite partial radiologic regression, follow-up MRI 8 weeks after discontinuation demonstrated marked progression with multifocal ARIA-H displaying as superficial siderosis, while the patient remained clinically asymptomatic. Corticosteroid treatment was initiated and lecanemab permanently discontinued.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This case highlights that severe ARIA may progress beyond active treatment exposure and emphasizes the importance of continued MRI surveillance even after therapy cessation. Furthermore, the APOE ε2/ε4 genotype may represent a distinct risk factor for cerebral amyloid angiopathy-related vascular fragility and hemorrhagic ARIA.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13656894/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149164540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurological Manifestations in Whipple's Disease: A Scoping Review of Clinical Features, Laboratory Findings, and Neuroimaging Characteristics of 111 Cases","authors":"Lucia Moro,&nbsp;Domenicantonio Tropepi,&nbsp;Dora Buonfrate,&nbsp;Cristina Mazzi,&nbsp;Giovanni Foti,&nbsp;Andrea Fittipaldo,&nbsp;Andrea Angheben,&nbsp;Anna Beltrame","doi":"10.1111/ene.70772","DOIUrl":"10.1111/ene.70772","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Whipple disease (WD) is a rare, potentially fatal infection caused by <i>Tropheryma whipplei</i> (TW). Central Nervous System involvement (CNS-WD) is a severe complication of WD with a challenging diagnosis, due to limited data integrating neurological features, imaging, cerebrospinal fluid analysis, PCR, and intestinal histopathology. Prompt recognition and treatment are essential to improve patient outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A scoping review of the literature from 2000 to 2025 was conducted, including all CNS-WD case reports and series, to outline its clinical spectrum and diagnostic workup.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Included studies were 89, accounting for 111 CNS-WD cases. Most patients were male (79.8%), with a mean age of 53.6 years. Notably, 13.2% presented with CNS involvement in the absence of systemic WD. Pathognomonic signs were rare (13/111, 11.7%). Most patients (82.9%, 92/111) presented with two or more neurological symptoms, the most frequently reported being altered mentation, followed by ataxia, eye movement abnormalities, and other movement disorders. CSF was abnormal in 74.7% (56/75) of patients and TW PCR was positive in 75% (48/64); 64% (55/86) of cases showed intestinal histopathology consistent with WD. TW PCR on gastrointestinal biopsies was positive in 67.9% (19/28) cases. CNS imaging revealed abnormalities in 85.4% (82/96) of patients, with no distinctive pattern.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>CNS-WD diagnosis remains challenging due to nonspecific symptoms, variable imaging, and occasional discordance among CSF, TW PCR or biopsy findings. Our review suggests that CNS-WD should be considered in the differential diagnosis of unexplained neurological syndromes, even in patients with atypical clinical or radiological profiles, supporting broader indications for TW testing.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13655652/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149167763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transcutaneous Carbon Dioxide Monitoring in Amyotrophic Lateral Sclerosis: A Scoping Review","authors":"Cláudia Santos Silva,&nbsp;Mamede de Carvalho","doi":"10.1111/ene.70782","DOIUrl":"10.1111/ene.70782","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Respiratory failure is the leading cause of death in amyotrophic lateral sclerosis (ALS). Conventional pulmonary function tests are effort-dependent and may fail to detect early nocturnal hypoventilation. Transcutaneous carbon dioxide (PtcCO₂) measurement is a non-invasive method for assessing ventilation, but its role in ALS remains unclear. We performed a scoping review to map the current evidence.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>PubMed, Web of Science, Scopus, and Google Scholar were searched from inception to April 2026. Original studies evaluating PtcCO₂ monitoring in ALS were included. Due to heterogeneity in study designs, populations, and outcomes, a narrative synthesis was performed, categorized by study characteristics, measurement protocols, and clinical endpoints.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-one studies were included. Most were observational and conducted in tertiary centres. Twelve studies evaluated PtcCO₂ as a surrogate marker for nocturnal hypoventilation, mainly to guide initiation or titration of non-invasive ventilation. Definitions of hypoventilation and reported PtcCO₂ metrics varied widely. Only three studies evaluated PtcCO₂ in relation to respiratory impairment or hypercapnia, but none reported formal measures of diagnostic accuracy. Evidence regarding the prognostic value was limited and inconsistent. Three studies showed that nocturnal hypercapnia preceded abnormalities in spirometry or daytime hypercapnia.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>PtcCO₂ monitoring shows promise as a non-invasive method for assessing nocturnal ventilatory abnormalities in ALS, including nocturnal hypoventilation. However, due to methodological heterogeneity, its diagnostic and prognostic roles remain uncertain. Current evidence is insufficient to support PtcCO₂ as a standalone criterion for NIV initiation. Large-scale prospective studies with standardized protocols are essential to delineate its clinical utility.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13656895/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149165302","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparator Event Rates, Baseline Activity, and Operational Selection Pressure in Relapsing Multiple Sclerosis Trials: A Meta-Epidemiological Analysis","authors":"Steffen Pfeuffer,&nbsp;Lars Masanneck,&nbsp;Marc Pawlitzki,&nbsp;Hans-Peter Hartung","doi":"10.1111/ene.70781","DOIUrl":"https://doi.org/10.1111/ene.70781","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Reference-arm annualized relapse rates (ARRs) in relapsing multiple sclerosis trials have declined. We examined whether baseline disease activity and the treatment environment during recruitment changed alongside this trend.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We analyzed 15 treated-reference arms from nine Phase 3 programs. ARR was related to recruitment era, prior-year relapse activity, MRI markers, and an Operational Selection Pressure Index (OSPI) combining recruitment geography with time since ocrelizumab approval. Associations were descriptive; disability outcomes were audited separately.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Reference-arm ARR declined from 0.370 in TEMSO and 0.320 in TOWER to 0.109–0.140 in BTK inhibitor-era programs (<i>ρ</i> = −0.906; 95% CI, −0.985 to −0.279; <i>n</i> = 15). Prior-year relapse activity correlated with ARR (<i>ρ</i> = 0.739; 95% CI, 0.158 to 0.928; <i>n</i> = 13) and declined over time (<i>ρ</i> = −0.725; 95% CI, −0.965 to −0.157; <i>n</i> = 13). MRI findings were inconsistent. OSPI clustered at 0–7.3 in six programs and 65.2–80.5 in BTK inhibitor-era programs and correlated inversely with ARR (<i>ρ</i> = −0.892; 95% CI, −1.000 to −0.569; <i>n</i> = 9). The association persisted across sensitivities but was negligible after adjustment for recruitment midpoint (partial <i>ρ</i> = −0.020). Disability trends depended on reporting metric and effective follow-up.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Lower baseline relapse activity accompanied declining event yield. OSPI made a treatment-ecosystem transition measurable but could not be separated from calendar era or establish mechanism. Trials require conservative event assumptions, harmonized reporting, and validation using screening and country- or period-specific outcomes.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70781","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149146568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Co-Occurrence of Facioscapulohumeral Muscular Dystrophy and Myasthenia Gravis: A Systematic Review and Meta-Analysis of Individual Patient Data","authors":"Pricilla Yani Gunawan,&nbsp;Jeremiah Hilkiah Wijaya","doi":"10.1111/ene.70752","DOIUrl":"10.1111/ene.70752","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Co-occurrence of facioscapulohumeral muscular dystrophy (FSHD) and myasthenia gravis (MG) is rare but clinically critical, as treatable MG symptoms may be misattributed to FSHD progression. This systematic review and meta-analysis aimed to comprehensively characterize patients with both conditions and to quantitatively synthesize the available individual patient data.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Following PRISMA 2020 guidelines, we searched four databases until 3 April 2026 for original reports providing individual-patient data on confirmed FSHD and MG co-occurrence. We performed descriptive and quantitative syntheses of clinical, genetic, and therapeutic variables.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Nine studies were systematically reviewed, with 29 patients contributing to on. FSHD onset significantly preceded MG onset, with a pooled mean interval of 24.21 years. The pooled mean age at MG onset was 62.62 years. All tested patients were acetylcholine receptor antibody positive. MG frequently presented with ocular and bulbar manifestations. Crucially, the pooled estimate for clinical improvement following standard MG-directed therapies was 89.7%.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>MG in FSHD patients typically emerges late in adulthood as an antibody-positive autoimmune synaptopathy. Recognizing new ocular or bulbar weakness as superimposed MG is essential, providing a highly treatable avenue to reverse functional decline in patients with established myopathy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70752","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149135489","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}