European Journal of Neurology最新文献

{"title":"Speech-In-Noise Perception in Alzheimer's Disease and Primary Progressive Aphasia","authors":"Sophie A. Froud,&nbsp;Benjamin A. Levett,&nbsp;Jessica Jiang,&nbsp;Lucy B. Core,&nbsp;Nehzat Koohi,&nbsp;Madison Tutton,&nbsp;Tim Green,&nbsp;Doris-Eva Bamiou,&nbsp;Charles R. Marshall,&nbsp;Chris J. D. Hardy,&nbsp;Jason D. Warren","doi":"10.1111/ene.70701","DOIUrl":"10.1111/ene.70701","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Understanding speech despite background noise is essential for everyday communication, but makes heavy neural processing demands. It is therefore potentially vulnerable to neurodegenerative diseases, particularly those led by communication deficits (primary progressive aphasia). However, how speech-in-noise perception is affected in these diseases is poorly understood.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Here we addressed this in 59 patients representing typical Alzheimer's disease and canonical logopenic, nonfluent/agrammatic and semantic variant syndromes of primary progressive aphasia, compared with 24 cognitively-well, older controls. We administered a digit triplet test of speech-in-noise perception based on the task used in the UK Biobank study, alongside pure tone audiometry and a general neuropsychological assessment. Voxel-based morphometry of patients' brain MRI scans was used to identify structural neuroanatomical associations of speech-in-noise perception.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>After adjusting for age, peripheral hearing and general cognitive function, the Alzheimer's, logopenic and nonfluent primary progressive aphasia groups performed significantly worse on speech-in-noise perception than controls. The nonfluent primary progressive aphasia and Alzheimer groups additionally had significantly worse peripheral hearing function than controls. Speech-in-noise perceptual performance correlated with grey matter atrophy in the right supramarginal gyrus.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Profiles of central (brain) and peripheral hearing impairment stratify major dementias, with implications for diagnosis and development of interventions to improve real-world communication in people living with dementia.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70701","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148435691","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":"Comorbidities and Complications in Parkinson’s Disease in Primary Care","authors":"Olaitan Okunoye,&nbsp;Laura Horsfall,&nbsp;Louise Marston,&nbsp;Kate Walters,&nbsp;Anette Schrag","doi":"10.1111/ene.70697","DOIUrl":"10.1111/ene.70697","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Parkinson's disease (PD) is associated with an increased rate of mortality. It is unclear whether this is due to complications and comorbidities associated with the disease or the disease process of PD itself.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We undertook a cohort study in patients with PD in an electronic primary care record database. Mortality rate ratios and risk differences per 1000 person-years were calculated for each complication and comorbidity using multivariable Poisson regression analysis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>There were 10,104 patients with incident PD and 55,664 controls. The rate of falls, dementia, hallucinations, postural hypotension, stroke, depression, anxiety, sleep disorders, gastrointestinal and urinary disorders, headaches, epilepsy, and myocardial infarction was higher in individuals with PD compared to controls. Conversely, the rates of chronic obstructive pulmonary disease (COPD), congestive heart failure, and gout were lower. In the PD group, the comorbidities of dementia, falls, stroke, cancer, chronic heart failure, COPD, myocardial infarction, and gastrointestinal disorders were associated with higher mortality rates. However, for all comorbidities, the increase in mortality rate was similar to or lower in the PD group compared to people without PD, except for falls which tended to be associated with higher mortality in the PD group.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This study provides data on the increased rate of mortality associated with different complications and comorbidities in PD. The mortality associated with these alone, however, does not explain the increased mortality in PD. This underscores the need for treatments to slow the underlying disease process.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70697","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148435648","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":"Late Cognitive or Mood Alterations With ‘Status Cribrosum’ and Diffuse White Matter Lesions: A New Cerebral Small Vessel Disease Phenotype Associated With Rare COL4A1 Variants Located Within Exon 23","authors":"Hélène Morel,&nbsp;Sophie Dufourd-Delalande,&nbsp;William Bloch,&nbsp;Mathilde Graber,&nbsp;Sacha Weber,&nbsp;Carla Machado,&nbsp;Thibault Coste,&nbsp;Hugues Chabriat,&nbsp;Elisabeth Tournier-Lasserve,&nbsp;Stéphanie Guey","doi":"10.1111/ene.70692","DOIUrl":"10.1111/ene.70692","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To report an atypical phenotype associated with two rare <i>COL4A1</i> glycine missense variants located in exon 23.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Clinical, neuropsychological, and brain imaging data of four patients with such variants were reported.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Four unrelated patients presented with late-onset cognitive alterations starting between 55 and 65 years of age. Brain magnetic resonance imaging (MRI) showed extensive white-matter hyperintensities on T2 or Flair images in all subjects, associated with multiple dilated perivascular spaces in the basal ganglia with features of <i>status cribrosum</i> in the three oldest individuals. None of the patients had a history of haemorrhagic stroke. Three of these patients had previously experienced mood disturbances. All had a family history of depression and/or suicide. Three of these unrelated patients shared a rare missense variant p.(Gly474Arg) in <i>COL4A1</i>, whereas the fourth one carried a p.(Gly486Glu) variant; these two variants affect two closely linked glycine residues encoded by exon 23 and located in a major cell-binding site of the triple helix.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Missense variants affecting closely clustered glycine residues within the glycine–X–Y repeats encoded by exon 23 of <i>COL4A1</i> are associated with an atypical, late-onset form of cerebral small vessel disease, characterised by diffuse leukoencephalopathy with a <i>status cribrosum</i> pattern and predominantly cognitive and/or neuropsychiatric manifestations.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70692","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148430085","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":"Plasma Proteomic Changes in GRN and C9orf72 Frontotemporal Dementia","authors":"Joel Simrén,&nbsp;Andrea L. Benedet,&nbsp;Guglielmo Di Molfetta,&nbsp;Ilaria Pola,&nbsp;Kübra Tan,&nbsp;Valentina Cantoni,&nbsp;Ilenia Libri,&nbsp;Jasmine Rivolta,&nbsp;Roberta Ghidoni,&nbsp;Henrik Zetterberg,&nbsp;Barbara Borroni,&nbsp;Nicholas J. Ashton","doi":"10.1111/ene.70704","DOIUrl":"10.1111/ene.70704","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Biomarkers reflecting the complex pathophysiology of genetic frontotemporal dementia (FTD) will be increasingly important with the advent of therapeutic trials aiming to slow or prevent the disease. In this study, we aimed to identify blood biomarker candidates using a multiplex panel of CNS-related proteins.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We cross-sectionally evaluated 67 carriers (21 presymptomatic and 46 symptomatic) of pathogenic FTD-causing mutations in the <i>GRN</i> (<i>n</i> = 30 symptomatic) and <i>C9orf72</i> (<i>n</i> = 16 symptomatic) genes and 42 matched non-carriers. Clinical severity was estimated using the CDR Dementia Staging Instrument with National Alzheimer Coordinating Centre Frontotemporal Lobar Degeneration component (CDR plus NACC FTLD). A total of 124 CNS-related proteins were measured in plasma using the NUcleic acid Linked Immuno-Sandwich Assay (NULISA) CNS panel. Group-level changes were then investigated using linear and non-linear regression models.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In <i>GRN-</i> and <i>C9orf72</i>-FTD, neurofilament light (NfL) was the most clearly altered protein compared with non-carriers (<i>GRN:</i> β [95% CI] = 4.0 standard deviations [3.6–4.4], <i>C9orf72</i>: β = 2.8 [2.2–3.4]), followed by neurofilament heavy (NfH; <i>GRN</i>: β = 0.83 [0.39–1.3], <i>C9orf72</i>: β = 1.4 [0.8–2.0]). Proteins exclusively altered in <i>GRN</i>-FTD included glial fibrillary acidic protein (GFAp; β = 0.50 [0.20–0.81]) and vascular cell adhesion protein 1 (VCAM1; Standardized β = −0.90 [−1.4 to −0.38]), changing with increasing disease severity. Neuronal pentraxin receptor (NPTXR; β = −0.94 [−1.5 to −0.4]) was selectively reduced in <i>C9orf72-</i>FTD. Nominally changed proteins in <i>C9orf72</i>-FTD included several inflammatory mediators.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Using this multiplex panel, established markers recapitulated previously established trends, while less-studied biomarker candidates were also identified. If validated in independent cohorts, these candidates could broaden the repertoire of blood biomarkers reflecting genetic FTD pathophysiology.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70704","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148430047","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":"A Novel RORA Hinge-Region Variant in Adult IDDECA With Cerebellar Atrophy and Marked Response to Valproate","authors":"V. Busco,&nbsp;G. Di Lazzaro,&nbsp;A. T. Cimmino,&nbsp;F. Bove,&nbsp;L. Saccinto,&nbsp;G. Silvestri,&nbsp;A. R. Bentivoglio","doi":"10.1111/ene.70699","DOIUrl":"10.1111/ene.70699","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>To expand the <i>RORA</i> mutational and clinical spectrum by reporting a novel hinge-region variant associated with an adult progressive phenotype and a marked therapeutic response to valproate.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We describe a 61-year-old woman with lifelong intellectual disability who developed subacute severe motor deterioration in adulthood. Evaluation included brain MRI and whole-exome sequencing.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Whole-exome sequencing identified a novel heterozygous variant, c.551C&gt;G (p.Pro184Arg), in the hinge region (domain D) of <i>RORA</i>, a region not previously implicated in human disease. While <i>RORA</i>-related disorders are typically described as neurodevelopmental conditions, our patient showed progressive motor decline with loss of independent ambulation. Brain MRI demonstrated marked vermian atrophy, supporting a possible progressive component. Withdrawal of valproate was followed by severe clinical worsening, whereas reintroduction led to marked improvement in tremor and gait, restoring independent ambulation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>This case expands the mutational and phenotypic spectrum of <i>RORA</i>-related disorders and suggests that hinge-region variants may contribute to a more severe phenotype. It also supports a role for cortical hyperexcitability in the motor manifestations and highlights a potentially treatable component, with implications for clinical management.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70699","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148430070","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":"Neuropsychological Tests for Detecting Prodromal DLB: A Systematic Review","authors":"Francesco Mori,&nbsp;Fabrizia D'Antonio,&nbsp;Giulia Zazzaro,&nbsp;Desirée Conti,&nbsp;Micaela Mitolo,&nbsp;Annachiara Cagnin,&nbsp;Annalena Venneri","doi":"10.1111/ene.70681","DOIUrl":"10.1111/ene.70681","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The prodromal phase of Dementia with Lewy bodies (DLB) remains unexplored and poorly defined. This systematic review scrutinised studies on the earliest cognitive DLB deficits, focusing on the most useful neuropsychological tests.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>From an initial pool of 2307 articles, 37 articles met inclusion criteria including either a prodromal DLB population or DLB cohorts comprehensive of individuals in prodromal or Mild Cognitive Impairment (MCI) due to Lewy Bodies pathology. These studies included comparisons with healthy controls, MCI due to Alzheimer's Disease (MCI-AD), or other aetiologies, and patients with established dementia.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Mild impairments were found in attention, executive functions, visual perception, and working memory in prodromal DLB patients. Prodromal DLB exhibited worse performance in memory and language than healthy controls, while showing better memory but worse performance in visual perception, attention, and executive tasks than MCI-AD. Comparisons in some articles with MCI of other aetiologies confirmed the distinctiveness for prodromal DLB of this pattern of impairment. The most used tests to detect prodromal DLB included the Frontal Assessment Battery (FAB), Digit Symbol Substitution Test (DSST), Stroop test, AX continuous performance test (AX-CPT), digit span backward, Rey-Osterrieth Complex Figure copy, pareidolia test, and Visual Object and Space Perception battery (VOSP).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Prodromal DLB can be detected using test batteries focused on specific cognitive domains and facilitating early diagnosis. It is possible to standardise a targeted test battery centred on specific visuoperceptual and attention domains for early detection of subtle cognitive decline due to Lewy body pathology.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70681","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148419825","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":"Individual Variability of CD19+ B-Cell Repopulation in People With Multiple Sclerosis Treated With Extended Interval Dosing of Ocrelizumab","authors":"Laura Hogenboom,&nbsp;Lisa G. Schoof,&nbsp;Liza M. Y. Gelissen,&nbsp;Jop Mostert,&nbsp;Luuk van Rooij,&nbsp;Elske Hoitsma,&nbsp;Caspar E. P. van Munster,&nbsp;Jeroen van Eijk,&nbsp;Nienke F. Kalkers,&nbsp;Kirsten S. Adriani,&nbsp;Anke Vennegoor,&nbsp;Ilse M. P. Arts,&nbsp;Liselore Mensing,&nbsp;Marijke Eurelings,&nbsp;Koen de Gans,&nbsp;Oliver Gerlach,&nbsp;Erwin L. J. Hoogervorst,&nbsp;Mark E. Kloosterziel,&nbsp;Jolijn J. Kragt,&nbsp;Ruben P. Portier,&nbsp;Ide Smets,&nbsp;Björn M. van Geel,&nbsp;Jessica Nielsen,&nbsp;Christiaan M. Roosendaal,&nbsp;Jurgen R. Piet,&nbsp;Esther Zeinstra,&nbsp;Onno N. Groeneveld,&nbsp;Bob W. van Oosten,&nbsp;Brigit A. de Jong,&nbsp;Bernard M. J. Uitdehaag,&nbsp;Eva M. M. Strijbis,&nbsp;Birgit I. Lissenberg-Witte,&nbsp;Joep Killestein,&nbsp;Zoé L. E. van Kempen","doi":"10.1111/ene.70695","DOIUrl":"10.1111/ene.70695","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Objectives</h3>\u0000 \u0000 <p>B-cell repopulation patterns in ocrelizumab treated patients with multiple sclerosis are highly variable between individuals, but the course of B-cell reoccurrence after subsequent doses within an individual is not yet determined. Our aim was to determine the intraindividual variability of CD19+ B-cell repopulation after each ocrelizumab dose when using CD19+ B-cell guided interval dosing.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a prospective cohort study, as part of the ongoing BLOOMS trial, investigating participants randomised for B-cell guided interval dosing of ocrelizumab with ≥ 2 dosing intervals. Coefficients of variation were calculated for time from last ocrelizumab dose to first CD19+ B-cell measurement ≥ 0.01 × 10⁹ cells/L.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Seventy-five participants with a total of 209 B-cell guided intervals were included. Time from last dose to first appearance of CD19+ B-cell count ≥ 0.01 × 10⁹ cells/L showed wide variability between individuals (20.6–72.1 weeks), but the median variation was 5.6% (IQR: 3.1–8.4) within an individual. This translated to a variation of 2 weeks per dosing interval on average.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>Time to CD19+ B-cell repopulation after each ocrelizumab dose is individually stable. This finding could pave the way for easier and more accessible future personalised interval dosing of B-cell depleting therapies if this stability is confirmed in long-term treatment.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70695","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148396146","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":"Correction to ‘Pharmacological Interventions for Hereditary Transthyretin-Related Amyloidosis With Polyneuropathy: Systematic Review and Network Meta-Analysis’","authors":"","doi":"10.1111/ene.70696","DOIUrl":"10.1111/ene.70696","url":null,"abstract":"<p>G. Duarte, T. Machado, F. Rodrigues, I. Conceição, D. Pareyson, J. da Costa, and on behalf of the Task Force of the EAN/PNS Joint Guideline for the Management of Neurological Manifestations in Hereditary Transthyretin Amyloidosis, “Pharmacological Interventions for Hereditary Transthyretin-Related Amyloidosis With Polyneuropathy: Systematic Review and Network Meta-Analysis,” <i>European Journal of Neurology</i> 33, no. 6 (2026): e70627. https://doi.org/10.1111/ene.70627.</p><p>In this article, two errors were identified.</p><p>In the list of authors, the fourth name was published incorrectly as Isabel Conceção. It should have been Isabel Conceição.</p><p>These corrections refine the interpretation of the findings without affecting the conclusions; the underlying data, analyses, and results are unchanged.</p><p>The authors apologize for these errors.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70696","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148396128","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":"Diagnostic Value of Neurofilament Light Chain and Glial Fibrillary Acidic Protein in Differentiating Primary From Serious Secondary Headache","authors":"Sara-Jessica Camerin,&nbsp;Cihan Atila,&nbsp;Claudine A. Blum,&nbsp;Sofia Sandgren,&nbsp;Aleksandra Maleska,&nbsp;Athina Papadopoulou,&nbsp;Jens Kuhle,&nbsp;Mira Katan,&nbsp;Mirjam Christ-Crain","doi":"10.1111/ene.70691","DOIUrl":"10.1111/ene.70691","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Headache is one of the most frequent reasons for emergency department (ED) visits, and distinguishing primary from secondary headache remains challenging. Blood biomarkers may improve early risk stratification. This study evaluates serum neurofilament light chain (sNfL) and glial fibrillary acidic protein (sGFAP) as potential biomarkers in patients presenting with non-traumatic headache to the ED.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Secondary analysis of a prospective multicenter case–control study at University Hospital Basel and Aarau, Switzerland. Patients presenting with acute non-traumatic headache were included. Final headache diagnoses were adjudicated by two board-certified neurologists. Blood samples obtained at ED presentation were analyzed for sNfL and sGFAP, excluding patients with conditions known to affect biomarker levels. Primary headache samples were matched to serious secondary headache samples. Biomarker levels were expressed as age-, BMI-, and sex-adjusted Z-scores.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 82 patients were analyzed (42 serious secondary, 40 primary headache). Median[IQR] sNfL <i>Z</i>-scores were significantly higher in serious secondary compared with primary headache (0.8 [0.0–1.8] vs. 0.2 [−0.3–0.8]; <i>p</i> &lt; 0.01). sGFAP Z-scores showed an even greater difference (1.2 [−0.1–2.3] vs. −0.2 [−0.7–0.2]; <i>p</i> &lt; 0.001). Both biomarkers demonstrated a specificity of 85%, but sGFAP showed higher sensitivity (57% vs. 43%), NPV (65% vs. 59%), and PPV (80% vs. 75%). In multivariable analysis adjusted for clinical risk factors, sGFAP remained independently associated with serious secondary headache (OR 1.9, 95%-CI 1.2–3.4), whereas sNfL did not.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>sGFAP may represent a promising biomarker to support differentiation between primary and non-traumatic serious secondary headache in the ED, warranting validation in larger cohorts.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70691","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148390220","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":"Neuropsychiatric Adverse Events Associated With Foslevodopa/Foscarbidopa Continuous Subcutaneous Infusion in Clinical Practice: A Multicenter Study","authors":"David Campo-Caballero,&nbsp;Jon Rodriguez-Antiguedad,&nbsp;Arnau Puig-Davi,&nbsp;Javier Ruiz-Martinez,&nbsp;Ana Vinagre-Aragón,&nbsp;Elisabet Mondragón,&nbsp;Lara Pardina Vilella,&nbsp;Nuria López-Ariztegui,&nbsp;Nuria Caballol,&nbsp;Guillermo González-Ortega,&nbsp;Iria Cabo,&nbsp;Rocío García Ramos,&nbsp;Inés Legarda,&nbsp;Marta Blázquez-Estrada,&nbsp;Pilar Sánchez-Alonso,&nbsp;Pablo Mir,&nbsp;Juan Carlos Romero Fábrega,&nbsp;Silvia Martí Martinez,&nbsp;María Álvarez Sauco,&nbsp;Diego Santos-García,&nbsp;DATs-PD GETM Spanish Registry Group","doi":"10.1111/ene.70693","DOIUrl":"10.1111/ene.70693","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Foslevodopa/foscarbidopa continuous subcutaneous infusion (LDp/CDp CSI) has emerged as an effective and well-tolerated therapy for reducing OFF and increasing non-troublesome ON in advanced Parkinson's disease (PD). Neuropsychiatric adverse events (AEs) have been reported in both clinical trials and real-world studies, with some <i>real-world</i> cohorts suggesting higher rates among patients with prior hallucinations or cognitive impairment. The present study aimed to determine the incidence and risk factors of neuropsychiatric AEs in a large prospective <i>real-world</i> cohort.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We analyzed data from the DATs-PD GETM Spanish Registry, an observational, prospective, multicenter, open-label study.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>214 patients treated with LDp/CDp CSI were included. Median age was 69 years, and median disease duration was 12 years. At baseline, 35% had cognitive impairment, 25.7% hallucinations/psychosis, and 26.2% impulse control disorders (ICDs). During follow-up after initiation (median 163 days), 19.2% developed at least one neuropsychiatric AE, mostly mild–moderate, and only 2.3% required device removal. Most events occurred more than 1 month after treatment initiation. In adjusted Cox, none of the evaluated variables were associated with the development of hallucinations/psychosis/confusion. The presence of ICD at baseline was associated with an increased risk of ICD-related AEs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Neuropsychiatric AEs, mainly hallucinations/psychosis, occurred in a clinically relevant proportion of patients treated with LDp/CDp CSI. However, they were generally mild-to-moderate and rarely led to treatment discontinuation. Except for ICD, baseline cognitive and psychotic features were not associated with higher incidence. These findings support its use in appropriately selected patients while highlighting the importance of individualized careful clinical monitoring.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 7","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70693","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148390301","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}