Journal of Neurology, Neurosurgery, and Psychiatry最新文献

{"title":"Tract-specific white matter hyperintensities and neuropsychiatric syndromes: a multicentre memory clinic study.","authors":"Cheuk Ni Kan, Mirthe Coenen, Xin Xu, Saima Hilal, Frederik Barkhof, Thomas Benke, Peter Dal-Bianco, Charles DeCarli, Marco Duering, Christian Enzinger, Lieza G Exalto, Evan F Fletcher, Edith Hofer, Huiberdina L Koek, Hugo J Kuijf, Pauline M Maillard, Justine E F Moonen, Janne M Papma, Yolande A L Pijnenburg, Reinhold Schmidt, Rebecca M E Steketee, Esther van den Berg, Wiesje M van der Flier, Narayanaswamy Venketasubramanian, Meike W Vernooij, Frank J Wolters, Geert Jan Biessels, Christopher Li-Hsian Chen, J Matthijs Biesbroek, Chin Hong Tan","doi":"10.1136/jnnp-2024-334264","DOIUrl":"10.1136/jnnp-2024-334264","url":null,"abstract":"<p><strong>Background: </strong>White matter hyperintensities (WMH) have been implicated in the pathogenesis of neuropsychiatric symptoms of dementia but the functional significance of WMH in specific white matter (WM) tracts is unclear. We investigate whether WMH burden within major WM fibre classes and individual WM tracts are differentially associated with different neuropsychiatric syndromes in a large multicentre study.</p><p><strong>Method: </strong>Neuroimaging and neuropsychiatric data of seven memory clinic cohorts through the Meta VCI Map consortium were harmonised. Class-based analyses of major WM fibres (association, commissural and projection) and region-of-interest-based analyses on 11 individual WM tracts were used to evaluate associations of WMH volume with severity of hyperactivity, psychosis, affective and apathy syndromes.</p><p><strong>Results: </strong>Among 2935 patients (50.4% women; mean age=72.2 years; 19.8% subjective cognitive impairment, 39.8% mild cognitive impairment, and 40.4% dementia), larger WMH volume within projection fibres (B=0.24, SE=0.10, p=0.013) was associated with greater apathy. Larger WMH volume within association (B=0.31, SE=0.12, p=0.009), commissural (B=0.47, SE=0.17, p=0.006) and projection (B=0.39, SE=0.16, p=0.016) fibres was associated with greater hyperactivity, driven by the inferior fronto-occipital fasciculus (B=0.50, SE=0.18, p=0.006), forceps major (B=0.48, SE=0.18, p=0.009) and anterior thalamic radiation (B=0.49, SE=0.19, p=0.011), respectively. Larger WMH volume in the uncinate fasciculus (B=1.82, SE=0.67, p=0.005) and forceps minor (B=0.61, SE=0.19, p=0.001) were additionally associated with greater apathy. No associations with affective and psychosis were observed.</p><p><strong>Conclusions: </strong>Tract-syndrome specificity of WMH burden with apathy and hyperactivity suggests that disruption of strategic neuronal pathways may be a potential mechanism through which small vessel disease affects emotional and behavioural regulation in memory clinic patients.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":"96 7","pages":"697-704"},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144289473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Silent burden: recognising and managing invisible symptoms in neuromyelitis optica.","authors":"Lorena Lorefice, Antonio Carotenuto, Giuseppe Fenu","doi":"10.1136/jnnp-2025-336041","DOIUrl":"https://doi.org/10.1136/jnnp-2025-336041","url":null,"abstract":"<p><p>Aquaporin-4-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) is an autoimmune astrocytopathy primarily characterised by visual, motor, and sensory symptoms, depending on lesion location. However, patients frequently experience invisible symptoms such as fatigue, pain, neuropsychiatric symptoms, and bladder/bowel dysfunction, which are often overlooked despite their substantial impact on quality of life. A systematic literature review was conducted using PubMed, with keywords related to fatigue, pain, bladder/bowel dysfunction, and neuropsychiatric symptoms in the context of AQP4+NMOSD. The review explores the prevalence, assessment, and management of these frequently neglected symptoms. There is a critical need for improved detection and monitoring of invisible symptoms in AQP4+NMOSD. Enhanced assessment is crucial not only for developing targeted therapies but also for determining whether these symptoms reflect underlying disease activity, ultimately leading to optimised treatment strategies.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144285013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Environmental multiple sclerosis (MS) risk factors, genetic MS risk, and brain development in a general paediatric population.","authors":"Casper Louk de Mol, Sander Lamballais, Ryan Muetzel, Liesbeth Duijts, Joost Smolders, Tonya White, Rinze Frederik Neuteboom","doi":"10.1136/jnnp-2024-335053","DOIUrl":"10.1136/jnnp-2024-335053","url":null,"abstract":"<p><strong>Background: </strong>Neuroaxonal loss occurs in the early stages of multiple sclerosis (MS), but whether it results from early inflammatory brain damage or an ongoing neurodegenerative process remains unclear. We hypothesise that genetic and childhood environmental risk factors for MS may already have an impact on neurodevelopment before the typical age of onset for MS in the general population.</p><p><strong>Methods: </strong>We examined associations and interactions of genetic and environmental risk factors for MS with brain MRI outcomes, including volumetric (n=5350) and diffusion data (n=5649), at ages 9 and 13 years in a large, population-based childhood cohort without MS diagnoses. Polygenic risk scores (PRSs) were used to assess genetic burden, with rs10191329 as a marker of MS severity. Environmental factors at age 5 included Epstein-Barr virus (EBV) serology, vitamin D status, body mass index, duration of outdoor activities, and household parental smoking.</p><p><strong>Results: </strong>Genetic data were available for 2817 and 2970 participants with volumetric and diffusion data, respectively. The MS-PRS was positively associated with EBV viral capsid antigen titres among EBV-positive children (β=0.15, p=2.98×10^-6). A negative association was observed between the MS-PRS and subcortical grey matter volume (β=-0.03, p=0.014). Interaction between the MS-PRS and household parental smoking was negatively linked to total brain (β=-0.21, p=0.025) and thalamic volumes (β=-0.22, p=0.003), where a higher MS-PRS and household smoking were associated with lower volumes. No associations were observed for rs10191329 with brain outcomes.</p><p><strong>Conclusions: </strong>Genetic and environmental risk factors for MS interact to influence brain volumes in childhood, suggesting a potential window for preventing MS in genetically susceptible individuals by reducing exposure to household smoking.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"679-685"},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142807266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sex hormone-related factors and the risk of PIRA in women with multiple sclerosis.","authors":"Antonino Giordano, Arianna Giliberti, Ferdinando Clarelli, Kaalindi Misra, Elisabetta Mascia, Melissa Sorosina, Giulia Visentin, Monica Margoni, Lucia Moiola, Maria A Rocca, Massimo Filippi, Federica Esposito","doi":"10.1136/jnnp-2024-335547","DOIUrl":"10.1136/jnnp-2024-335547","url":null,"abstract":"<p><strong>Background: </strong>Sex-related differences affect multiple sclerosis (MS), but the impact of sex hormones on disease progression remains unclear. We investigated whether sex hormone-related factors influence progression independent of relapse activity (PIRA) in women with MS over a long-term follow-up.</p><p><strong>Methods: </strong>The study analysed 1210 female MS patients from the San Raffaele MS Center using data from an environmental survey (2019-2023). PIRA was defined as 12-week confirmed disability progression independent of recent relapses (<30 days). Cox proportional-hazard models (adjusted for confounding factors) were used to assess the effect of hormone-related factors on PIRA risk.</p><p><strong>Results: </strong>Patients who used oral contraceptives before MS diagnosis had a 26% lower risk of PIRA and a delayed median time to the first PIRA event (9.94 vs 7.5 years; HR=0.74; 95% CI 0.61 to 0.89; p=0.0018). Conversely, menopause at diagnosis (HR=1.82; 95% CI 1.24 to 2.67; p=0.0022) and pregnancy before diagnosis (HR=1.22; 95% CI 1.006 to 1.47; p=0.043) were associated with a shorter time to PIRA. No significant differences were found with abortion, menstrual irregularity or fertility therapy.</p><p><strong>Conclusions: </strong>This study suggests that early oral contraceptives may delay future disability progression, supporting the importance of sex hormones in MS and prompting further prospective investigations on oral contraceptives to slow disease progression.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"686-689"},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143624962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fewer relapses and worse outcomes of patients with late-onset myelin oligodendrocyte glycoprotein antibody-associated disease.","authors":"Yuxin Fan, Zhouzhou Wang, Yuhang Wu, Lei Zhou, Liang Wang, Wenjuan Huang, Hongmei Tan, Xuechun Chang, Jingzi ZhangBao, Chao Quan","doi":"10.1136/jnnp-2024-334613","DOIUrl":"10.1136/jnnp-2024-334613","url":null,"abstract":"<p><strong>Background: </strong>To delineate the clinical characteristics and outcomes of late-onset myelin oligodendrocyte glycoprotein antibody-associated disease (LO-MOGAD) and compare them with those of early-onset MOGAD (EO-MOGAD).</p><p><strong>Methods: </strong>This observational cohort study included 199 adult patients with MOGAD. We reviewed the patients' demographic and clinical data and performed comparative analyses between EO-MOGAD and LO-MOGAD (onset age 18-50 and ≥50 years, respectively).</p><p><strong>Results: </strong>Among the 199 patients, 42 had LO-MOGAD. Compared with patients with EO-MOGAD, those with LO-MOGAD patients exhibited a significantly higher incidence of optic neuritis both at the initial attack (66.67% vs 43.31%, p=0.007) and throughout all attacks (72.15% vs 52.51%, p=0.001). Over a similar disease duration, patients with LO-MOGAD exhibited significantly fewer relapsing courses (45.16% vs 70.97%), higher Expanded Disability Status Scale (EDSS) and visual functional system scores at the last visit (all p<0.05). Compared with patients with EO-MOGAD, those with LO-MOGAD had a significantly lower risk of relapse (HR 0.512, 95% CI 0.268 to 0.978, p=0.034), but higher risks of reaching EDSS ≥2 (HR 2.893, 95% CI 1.524 to 5.494, p<0.001) and visual acuity ≤0.6 (HR 3.097, 95% CI 1.073 to 8.937, p=0.022). Immunosuppressive therapies significantly reduced the annualised relapse rates of patients with LO-MOGAD, although adverse events leading to drug discontinuation and hospitalisation were observed.</p><p><strong>Conclusions: </strong>Compared with patients with EO-MOGAD, patients with LO-MOGAD exhibited fewer relapsing courses but worse disability outcomes and should be actively treated.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"655-664"},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142791837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Seizures in pregnancy and child outcomes.","authors":"Kimford J Meador","doi":"10.1136/jnnp-2025-335945","DOIUrl":"10.1136/jnnp-2025-335945","url":null,"abstract":"","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"619-620"},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143788444","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Blood biomarkers for predicting disability worsening in progressive multiple sclerosis: a multinational, individual participant-level analysis.","authors":"Ahmed Abdelhak, Franziska Bachhuber, Kiarra Ning, Pascal Benkert, W John Boscardin, Aleksandra Maleska Maceski, Sabine Schaedelin, Lutz Achtnichts, Sebastian Finkener, Patrice H Lalive, Marjolaine Uginet, Caroline Pot, Renaud Du Pasquier, Robert Hoepner, Andrew Chan, Claudio Gobbi, Chiara Zecca, Stefanie Müller, Patrick Roth, Cristina Granziera, Tanuja Chitnis, Evan Madill, Howard L Weiner, Ari J Green, Stephen L Hauser, Bruce Ac Cree, Tania Kümpfel, Joachim Havla, Thomas Skripuletz, Stefan Gingele, Makbule Senel, Ioannis Vardakas, Daniela Taranu, Ulf Ziemann, Markus C Kowarik, Ingo Kleiter, Muna-Miriam Hoshi, Uwe K Zettl, Axel Haarmann, Simon Thebault, Mark S Freedman, Hailey P Bergman, Ellen Iacobaeus, Mohsen Khademi, Diana Ferraro, Martina Cardi, Sara Mariotto, Manuel Comabella, Xavier Montalban, Andreu Vilaseca-Jolonch, Eva M Strijbis, Mark Hj Wessels, Joep Killestein, Bernhard Hemmer, Friederike Held, Finn Sellebjerg, Helene Højsgaard Chow, Roberto Alvarez-Lafuente, Maria Inmaculada Domínguez-Mozo, Harald Hegen, Klaus Berek, Florian Deisenhammer, Eric Thouvenot, Hanane Agherbi, Konrad Rejdak, Magda Gąsior, Dimitrios Tzanetakos, John S Tzartos, Maria Pia Sormani, Irena Dujmovic Basuroski, Georgina Arrambide, Michael Khalil, Fredrik Piehl, Charlotte E Teunissen, Jens Kuhle, Hayrettin Tumani","doi":"10.1136/jnnp-2025-335831","DOIUrl":"https://doi.org/10.1136/jnnp-2025-335831","url":null,"abstract":"<p><strong>Background and objectives: </strong>Biologically informative markers like glial fibrillary acidic protein (GFAP) and neurofilament light chain (NfL) may help predict confirmed disability worsening (CDW) in multiple sclerosis (MS). However, data on the prognostic value of their blood concentrations in progressive MS (PMS) are limited, and there are substantial discrepancies in the published literature. This international collaboration uses individual participant data to define the prognostic value of serum GFAP and NfL in people with PMS (pwPMS).</p><p><strong>Methods: </strong>Data were collected from BioMS-eu network centres and collaborating cohorts. pwPMS with primary progressive MS (PPMS) or secondary progressive MS (SPMS) with at least one GFAP value and at least three follow-up expanded disability status scale (EDSS) scores were included. The prognostic value of serum GFAP and NfL age- and sex-adjusted Z-scores for future CDW was evaluated using Cox regression models, accounting for sex, age, baseline disease duration and EDSS, and dominant treatment during follow-up.</p><p><strong>Results: </strong>1058 participants and 7530 encounters were included (median age 53 years (IQR: 44 to 59), 57% female, follow-up 4.6 years (2.9 to 8.4)) with median baseline GFAP of 0.74 (-0.10 to 1.55) and NfL of 0.64 (-0.36 to 1.51). 723 CDW events were recorded. Each GFAP Z-score increase was associated with ~10% higher CDW risk (adjusted HR (aHR) 1.107 (1.001 to 1.225), p=0.049). Results were mainly driven by SPMS participants (n=613, aHR 1.242 (1.073 to 1.438), p=0.004). Higher NfL Z-scores predicted CDW only in PPMS participants (1.236 (1.092 to 1.399), p=0.001).</p><p><strong>Conclusions: </strong>GFAP was a prognostic indicator for future CDW in pwPMS, especially in pwSPMS. On the other hand, NfL was predictive of CDW only in pwPPMS.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144285012","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Optical coherence tomography in secondary progressive multiple sclerosis: cross-sectional and longitudinal exploratory analysis from the MS-SMART randomised controlled trial.","authors":"Floriana De Angelis, James R Cameron, Arman Eshaghi, Richard Parker, Peter Connick, Jonathan Stutters, Domenico Plantone, Anisha Doshi, Nevin John, Thomas Williams, Alberto Calvi, David MacManus, Frederik Barkhof, Siddharthan Chandran, Christopher J Weir, Ahmed Toosy, Jeremy Chataway","doi":"10.1136/jnnp-2024-334801","DOIUrl":"10.1136/jnnp-2024-334801","url":null,"abstract":"<p><strong>Background: </strong>Optical coherence tomography (OCT) inner retinal metrics reflect neurodegeneration in multiple sclerosis (MS). We explored OCT measures as biomarkers of disease severity in secondary progressive MS (SPMS).</p><p><strong>Methods: </strong>We investigated people with SPMS from the Multiple Sclerosis-Secondary Progressive Multi-Arm Randomisation Trial OCT substudy, analysing brain MRIs, clinical assessments and OCT at baseline and 96 weeks. We measured peripapillary retinal nerve fibre layer (pRNFL) and macular ganglion cell-inner plexiform layer (GCIPL) thicknesses. Statistical analysis included correlations, multivariable linear regressions and mixed-effects models.</p><p><strong>Results: </strong>Of the 212 participants recruited at baseline, 192 attended at 96 weeks follow-up. Baseline pRNFL and GCIPL thickness correlated with Symbol Digit Modalities Test (SDMT) (respectively, r=0.33 (95% CI 0.20 to 0.47); r=0.39 (0.26 to 0.51)) and deep grey matter volume (respectively, r=0.21 (0.07 to 0.35); r=0.28 (0.14 to 0.41)).pRNFL was associated with Expanded Disability Status Scale (EDSS) score change (normalised beta (B)=-0.12 (-0.23 to -0.01)). Baseline pRNFL and GCIPL were associated with Timed 25-Foot Walk change (T25FW) (respectively, B=-0.14 (-0.25 to -0.03); B=-0.20 (-0.31 to -0.10)) and 96-week percentage brain volume change (respectively, B=0.14 (0.03 to 0.25); B=0.23 (0.12 to 0.34)). There were significant annualised thinning rates: pRNFL (-0.83 µm/year) and GCIPL (-0.37 µm/year).</p><p><strong>Conclusions: </strong>In our cohort of people with SPMS and long disease duration, OCT measures correlated with SDMT and deep grey matter volume at baseline; EDSS, T25FW and whole brain volume change at follow-up.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"647-654"},"PeriodicalIF":8.7,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Biopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients.","authors":"Milani Deb-Chatterji, Parnia Aghel, Hans Pinnschmidt, Christina Krüger, Juliane Herm, Julia Layer, Hebun Erdur, Felix J Bode, Christian H Nolte, Tim Magnus","doi":"10.1136/jnnp-2025-335764","DOIUrl":"https://doi.org/10.1136/jnnp-2025-335764","url":null,"abstract":"<p><strong>Background: </strong>Reports of primary angiitis of the central nervous system (PACNS) are mainly restricted to clinically suspected cases, but biopsy-proven ones are rare. Here, we present results from a large multicentre cohort of patients with biopsy-proven PACNS (BP-PACNS). In particular, we provide insights into characteristics and treatment responses of PACNS subtypes.</p><p><strong>Methods: </strong>BP-PACNS patients treated between 1999 and 2021 were analysed. The outcome was assessed by the modified Rankin Scale (mRS). Between-group comparisons were performed by Kruskal-Wallis, χ² or Fisher's exact tests.</p><p><strong>Results: </strong>In total, 57 patients were analysed (52% male). Of these, n=37 (65%) had a lymphocytic (L-PACNS), n=9 (16%) an amyloid-beta-related angiitis (ABRA), n=8 (14%) a granulomatous (G-PACNS) and n=3 (5%) a necrotising (N-PACNS) PACNS subtype. At the time of diagnosis, age differed significantly between groups (median age (years) L-PACNS 47, ABRA 64.5, G-PACNS 37, N-PACNS 65; p=0.008). The clinical course was mostly monophasic in L-PACNS and ABRA (65% and 75%, respectively), while relapsing-remitting in G-PACNS (63%). Median mRS at last follow up was 2 (IQR 1.25-4) in the study group. Worst outcome (median mRS 4) and highest mortality (25%) were seen in G-PACNS. Good induction treatment response was achieved in 77% of all BP-PACNS patients but was low in those with G-PACNS (29%).</p><p><strong>Conclusions: </strong>In this large, multicentre series of BP-PACNS patients, G-PACNS had a worse functional outcome, a predominant relapsing-remitting disease and a poorer response to the induction therapy. An optimal first-line treatment regimen for G-PACNS patients should be further examined and established in larger studies to improve the outcome of G-PACNS patients.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266352","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"PACNS: biopsy positive, negative or nought?","authors":"William Powers","doi":"10.1136/jnnp-2025-336655","DOIUrl":"https://doi.org/10.1136/jnnp-2025-336655","url":null,"abstract":"","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}