Journal of Neurology, Neurosurgery, and Psychiatry最新文献

{"title":"Real-world multicentre cohort study on choices and effectiveness of immunotherapies in NMOSD and MOGAD.","authors":"Vivien Häußler, Corinna Trebst, Daniel Engels, Hanna Pellkofer, Joachim Havla, Ankelien Duchow, Patrick Schindler, Carolin Schwake, Thivya Pakeerathan, Katinka Fischer, Marius Ringelstein, Gero Lindenblatt, Martin W Hümmert, Daria Tkachenko, Franziska Bütow, Katrin Giglhuber, Martina Flaskamp, Insa Schiffmann, Mirjam Korporal-Kuhnke, Sven Jarius, Eva Dawin, Lisa Revie, Makbule Senel, Mariella Herfurth, Annette Walter, Mosche Pompsch, Ingo Kleiter, Klemens Angstwurm, Matthias Kaste, Matthias Grothe, Jonathan Wickel, Paulus Stefan Rommer, Jörn Peter Sieb, Markus Krämer, Florian Then Bergh, Hayrettin Tumani, Luisa Klotz, Brigitte Wildemann, Orhan Aktas, Ilya Ayzenberg, Judith Bellmann-Strobl, Friedemann Paul, Tania Kümpfel, Tim Friede, Achim Berthele, Jan-Patrick Stellmann","doi":"10.1136/jnnp-2024-334764","DOIUrl":"https://doi.org/10.1136/jnnp-2024-334764","url":null,"abstract":"<p><strong>Background: </strong>Recurrent attacks in neuromyelitis optica spectrum disorders (NMOSDs) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can lead to severe disability. We aimed to analyse the real-world use of immunotherapies in patients with NMOSD and MOGAD, focusing on changes in treatment strategies, effects on attack rates (ARR) and risk factors for attacks.</p><p><strong>Methods: </strong>This longitudinal registry-based cohort study included 493 patients (320 with aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive NMOSD (65%), 44 with AQP4-IgG seronegative NMOSD (9%) and 129 MOGAD (26%)) with 1247 treatments from 19 German and one Austrian centre from the registry of the neuromyelitis optica study group (NEMOS). We analysed unadjusted ARR and implemented survival analyses and Cox proportional hazard regression to assess efficiency and risk factors for subsequent attacks over time.</p><p><strong>Results: </strong>Rituximab and azathioprine are the most widely used immunotherapies in NMOSD as well as in MOGAD, with changes in distribution over the last decade. Immunotherapy demonstrated significant therapeutic effects in NMOSD but less pronounced effects in MOGAD. Risk factors for attacks included younger age and prior attacks under the same therapy. Efficacy varied among the different immunotherapies, with azathioprine, rituximab and eculizumab showing significant risk reductions in AQP4-IgG seropositive NMOSD.</p><p><strong>Conclusions: </strong>This study provides insights into the evolving treatment landscape and effectiveness of immunotherapies in NMOSD and MOGAD. Established off-label therapies continue to play an important role, especially for patients with stable disease, with emerging evidence supporting newly approved therapies. Future studies are needed to refine treatment algorithms and address the ongoing uncertainties in MOGAD management.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142895447","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":"Long-term exposure to multiple air pollutants and risk of Parkinson's disease: a population-based multipollutant model study.","authors":"Szu-Ju Chen, Shih-Chun Pan, Chih-Da Wu, Hsun Li, Yue Leon Guo, Chin-Hsien Lin","doi":"10.1136/jnnp-2024-334825","DOIUrl":"https://doi.org/10.1136/jnnp-2024-334825","url":null,"abstract":"<p><strong>Background: </strong>Recent evidence suggests brain-first Parkinson's disease (PD) may start from the olfactory system, indicating potential inhalational exposure to causal agents. We investigated the impact of long-term exposure to various air pollutants on PD incidence using both single- and multi-pollutant models to account for interactions between pollutants.</p><p><strong>Methods: </strong>This retrospective population study used data from Taiwan's National Health Insurance Research Database (2006 and 2018) and included individuals aged 40-65 without PD. Personal exposure levels to various air pollutants, including PM_2.5, PM₁₀, NO₂, O₃, SO₂ and CO, were calculated using the hybrid Kriging/land-use regression method. Cox regression models were used to analyse the association between pollutants and PD incidence, adjusting for covariates.</p><p><strong>Results: </strong>A total of 5 113 322 individuals without PD (mean age 50.1±6.9 years, 47.3% men) were followed for an average of 11.2±2.4 years, during which 20 694 incident cases of PD were identified. In the single-pollutant model, exposure to PM_2.5 (HR 2.65 (95% CI 2.59 to 2.72)), PM₁₀ (HR 3.13 (3.04 to 3.22)), NO₂ (HR 1.74 (1.68 to 1.80)) and SO₂ (HR 1.68 (1.65 to 1.71)) was associated with an increased risk of PD. These associations remained robust in the multipollutant model. A positive association between exposure to O₃ and an increased risk of PD (HR 1.29 (1.25-1.33)) was observed after adjusting for co-pollutants.</p><p><strong>Conclusions: </strong>This nationwide cohort study employing multiple-pollutant models for considering the interaction effects revealed an association between exposure to multiple air pollutants and the risk of PD, emphasising the need for early prevention strategies.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142895443","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":"Left atrial appendage occlusion in patients with atrial fibrillation and intracerebral haemorrhage associated with cerebral amyloid angiopathy: a multicentre observational study and pooled analysis of published studies.","authors":"Kitti Thiankhaw, Jonathan Best, Sonal Srivastava, Ishika Prachee, Smriti Agarwal, Serena Tan, Patrick A Calvert, Asim Chughtai, Richard Ang, Oliver R Segal, David J Werring","doi":"10.1136/jnnp-2024-334718","DOIUrl":"https://doi.org/10.1136/jnnp-2024-334718","url":null,"abstract":"<p><strong>Background: </strong>Cerebral amyloid angiopathy (CAA) is a common cause of intracerebral haemorrhage (ICH) with a high recurrence risk. Left atrial appendage occlusion (LAAO) is a method for ischaemic stroke prevention in patients with atrial fibrillation (AF), potentially reducing the risk of intracranial bleeding in CAA-associated ICH. We aimed to determine the outcomes of patients with AF with CAA-associated ICH undergoing LAAO.</p><p><strong>Methods: </strong>We conducted a multicentre study of patients with CAA-associated ICH who underwent LAAO for stroke prevention. We pooled our findings with data from a systematic review of relevant published studies of LAAO for AF in ICH survivors reporting CAA diagnosis.</p><p><strong>Results: </strong>We included data from two published studies (n=65) with CAA-specific data and our cohort study (n=37), providing a total of 102 participants (mean age 76.2±8.0 years, 74.6% male) with CAA-related symptomatic ICH and AF treated with LAAO. The median follow-up period was 9.4 months (IQR 4.2-20.6). Postprocedural antithrombotic regimens varied between single (73.0%) or dual antiplatelet therapy (16.2%), or direct oral anticoagulant (DOAC) (10.8%), with a median duration of 42 days (IQR 35-74). Postprocedural complications were uncommon, but included transient arrhythmias (2.1%) and non-life-threatening tamponade (2.1%). Pooled incidence rates of ischaemic stroke and ICH during follow-up were 5.16 (95% CI 1.36 to 17.48) and 2.73 (95% CI 0.41 to 13.94) per 100 patient years, respectively.</p><p><strong>Conclusions: </strong>LAAO followed by short-term antithrombotic therapy might be a safe and effective ischaemic stroke preventive strategy in people with CAA-associated ICH and AF. However, randomised controlled trials are needed to determine how LAAO compares with long-term DOAC in this population.</p><p><strong>Prospero registration number: </strong>CRD42023415354.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854476","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":"How well do plasma Alzheimer's disease biomarkers reflect the CSF amyloid status?","authors":"Jemma Hazan, Emily Abel, Miguel Rosa Grilo, Deborah Alawode, Ines Laranjinha, Amanda J Heslegrave, Kathy Y Liu, Jonathan M Schott, Robert Howard, Henrik Zetterberg, Nick C Fox","doi":"10.1136/jnnp-2024-334122","DOIUrl":"https://doi.org/10.1136/jnnp-2024-334122","url":null,"abstract":"<p><strong>Background: </strong>Can plasma biomarkers as well as cerebrospinal fluid (CSF) perform in the separation of amyloid-beta-positive (Aβ+) vs amyloid-beta-negative (Aβ-) groups across an age range seen in an NHS cognitive disorder clinic?</p><p><strong>Methods: </strong>As part of the routine diagnostic investigation of 111 clinic patients who had contemporaneous blood and CSF samples taken, patients were categorised into Aβ+ and Aβ- groups based on their CSF in an Aβ42/40 ratio. We then evaluated four single molecule array (Simoa) Quanterix assays, quantifying single plasma analytes and ratios (p-tau217, p-tau217/Aβ42 ratio, p-tau181, p-tau181/Aβ42 ratio and Aβ42/40 ratio) in their ability to distinguish between these groups and the effect of age.</p><p><strong>Results: </strong>The median (range) age of participants was 66 (55-79) years with 48 females (43.2%). The areas under the curve (AUC), not accounting for age, for the ability to discriminate Aβ+ from Aβ- groups were plasma p-tau217 AUC=0.94, Aβ42/40 AUC=0.78 and p-tau181 AUC=0.77. Combining p-tau217/Aβ42 increased the AUC to 0.97. The difference between the groups was influenced by age with less separation in older individuals: a significant negative interaction term between age and group for plasma p-tau217 concentrations (-0.037, p=0.013) and p-tau217/Aβ42 ratio (-0.007, p=0.008).</p><p><strong>Conclusions: </strong>There was variable performance of plasma biomarkers to recapitulate the CSF assay. Both p-tau217 and p-tau217/Aβ42 showed excellent promise as surrogates of CSF amyloid status, although with slightly reduced performance in older individuals. There was poorer discriminatory ability for p-tau181 and Aβ42/40. Further research is needed to address potential age-related confounds.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854456","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":"https://doi.org/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":""},"PeriodicalIF":8.7,"publicationDate":"2024-12-18","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":"The relationship between corticospinal excitability and structural integrity in stroke patients.","authors":"Lina Daghsen, Thomas Checkouri, Aymric Wittwer, Romain Valabregue, Damien Galanaud, François-Xavier Lejeune, Mohammed Doulazmi, Jean-Charles Lamy, Pierre Pouget, Emmanuel Roze, Charlotte Rosso","doi":"10.1136/jnnp-2023-331996","DOIUrl":"10.1136/jnnp-2023-331996","url":null,"abstract":"<p><strong>Background: </strong>Evaluation of the structural integrity and functional excitability of the corticospinal tract (CST) is likely to be important in predicting motor recovery after stroke. Previous reports are inconsistent regarding a possible link between CST structure and CST function in this setting. This study aims to investigate the structure‒function relationship of the CST at the acute phase of stroke (<7 days).</p><p><strong>Methods: </strong>We enrolled 70 patients who had an acute ischaemic stroke with unilateral upper extremity (UE) weakness. They underwent a multimodal assessment including clinical severity (UE Fugl Meyer at day 7 and 3 months), MRI to evaluate the CST lesion load and transcranial magnetic stimulation to measure the maximum amplitude of motor evoked potential (MEP).</p><p><strong>Results: </strong>A cross-sectional lesion load above 87% predicted the absence of MEPs with an accuracy of 80.4%. In MEP-positive patients, the CST structure/function relationship was bimodal with a switch from a linear relationship (rho=-0.600, 95% CI -0.873; -0.039, p<0.03) for small MEP amplitudes (<0.703 mV) to a non-linear relationship for higher MEP amplitudes (p=0.72). In MEP-positive patients, recovery correlated with initial severity. In patients with a positive MEP <0.703 mV but not in patients with an MEP ≥0.703 mV, MEP amplitude was an additional independent predictor of recovery. In MEP-negative patients, we failed to identify any factor predicting recovery.</p><p><strong>Conclusion: </strong>This large multimodal study on the structure/function of the CST and stroke recovery proposes a paradigm change for the MEP-positive patients phenotypes and refines the nature of the link between structural integrity and neurophysiological function, with implications for study design and prognostic information.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"85-94"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142145809","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":"Phenotypic spectrum of myelin protein zero-related neuropathies: a large cohort study from five mutation clusters across Italy.","authors":"Alessandro Bertini, Luca Gentile, Tiziana Cavallaro, Stefano Tozza, Paola Saveri, Massimo Russo, Sara Massucco, Yuri Matteo Falzone, Emilia Bellone, Federica Taioli, Alessandro Geroldi, Giuseppe Occhipinti, Moreno Ferrarini, Eleonora Cavalca, Luca Crivellari, Paola Mandich, Francesca Balistreri, Stefania Magri, Franco Taroni, Stefano Carlo Previtali, Angelo Schenone, Marina Grandis, Fiore Manganelli, Gian Maria Fabrizi, Anna Mazzeo, Davide Pareyson, Chiara Pisciotta","doi":"10.1136/jnnp-2024-333842","DOIUrl":"10.1136/jnnp-2024-333842","url":null,"abstract":"<p><strong>Background: </strong>We aimed to investigate the clinical features of a large cohort of patients with myelin protein zero (<i>MPZ</i>)-related neuropathy, focusing on the five main mutation clusters across Italy.</p><p><strong>Methods: </strong>We retrospectively gathered a minimal data set of clinical information in a series of patients with these frequent mutations recruited among Italian Charcot-Marie-Tooth (CMT) registry centres, including disease onset/severity (CMTES-CMT Examination Score), motor/sensory symptoms and use of orthotics/aids.</p><p><strong>Results: </strong>We collected data from 186 patients: 60 had the p.Ser78Leu variant ('classical' CMT1B; from Eastern Sicily), 42 the p.Pro70Ser (CMT2I; mainly from Lombardy), 38 the p.Thr124Met (CMT2J; from Veneto), 25 the p.Ser44Phe (CMT2I; from Sardinia) and 21 the p.Asp104ThrfsX13 (mild CMT1B; from Apulia) mutation. Disease severity (CMTES) was higher (p<0.001) in late-onset axonal forms (p.Thr124Met=9.2±6.6; p.Ser44Phe=7.8±5.7; p.Pro70Ser=7.6±4.8) compared with p.Ser78Leu (6.1±3.5) patients. Disease progression (ΔCMTES/year) was faster in the p.Pro70Ser cohort (0.8±1.0), followed by p.Ser44Phe (0.7±0.4), p.Thr124Met (0.4±0.5) and p.Ser78Leu (0.2±0.4) patients. Disease severity (CMTES=1.2±1.5), progression (ΔCMTES/year=0.1±0.4) and motor involvement were almost negligible in p.Asp104ThrfsX13 patients, who, however, frequently (78%, p<0.001) complained of neuropathic pain. In the other four clusters, walking difficulties were reported by 69-85% of patients, while orthotic and walking aids use ranged between 40-62% and 16-28%, respectively.</p><p><strong>Conclusions: </strong>This is the largest <i>MPZ</i> (and late-onset CMT2) cohort ever collected, reporting clinical features and disease progression of 186 patients from five different clusters across Italy. Our findings corroborate the importance of differentiating between 'classical' childhood-onset demyelinating, late-onset axonal and mild <i>MPZ</i>-related neuropathy, characterised by different pathomechanisms, in view of different therapeutic targets.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"47-53"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11672051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141262098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictors of a relapsing course in myelin oligodendrocyte glycoprotein antibody-associated disease.","authors":"Akash Virupakshaiah, Vinicius A Schoeps, Jonathan Race, Michael Waltz, Siefaddeen Sharayah, Zahra Nasr, Carson E Moseley, Scott S Zamvil, Cristina Gaudioso, Allison Schuette, Theron Charles Casper, John Rose, Eoin P Flanagan, Moses Rodriguez, Jan-Mendelt Tillema, Tanuja Chitnis, Mark P Gorman, Jennifer S Graves, Leslie A Benson, Mary Rensel, Aaron Abrams, Lauren Krupp, Timothy E Lotze, Gregory Aaen, Yolanda Wheeler, Teri Schreiner, Amy Waldman, Janet Chong, Soe Mar, Emmanuelle Waubant","doi":"10.1136/jnnp-2024-333464","DOIUrl":"10.1136/jnnp-2024-333464","url":null,"abstract":"<p><strong>Background: </strong>Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described demyelinating disorder, and children represent about 50% of all cases. Almost half of the patients experience relapses, but very few studies have evaluated predictors of relapse risk, challenging clinical management. The study aimed to identify predictors at MOGAD onset that are associated with a relapsing course.</p><p><strong>Methods: </strong>Prospectively collected data from paediatric patients with MOGAD seen by the US Network of Paediatric MS Centres were leveraged. Univariable and adjusted multivariable models were used to predict recurrent disease.</p><p><strong>Results: </strong>We identified 326 MOGAD cases (mean age at first event 8.9 years [SD 4.3], 57% female, 77% white and 74% non-Hispanic) and 46% relapsed during a mean follow-up of 3.9 years (SD 4.1). In the adjusted multivariable model, female sex (HR 1.66, 95% CI 1.17 to 2.36, p=0.004) and Hispanic/Latino ethnicity (HR 1.77, 95% CI 1.19 to 2.64, p=0.005) were associated with a higher risk of relapsing MOGAD. Maintenance treatment initiated before a second event with rituximab (HR 0.25, 95% CI 0.07 to 0.92, p=0.037) or intravenous immunoglobulin (IVIG) (HR 0.35, 95% CI 0.14 to 0.88, p=0.026) was associated with lower risk of a second event in multivariable analyses. Conversely, maintenance steroids were associated with a higher estimated relapse risk (HR 1.76, 95% CI 0.90 to 3.45, p=0.097).</p><p><strong>Conclusion: </strong>Sex and ethnicity are associated with relapsing MOGAD. Use of rituximab or IVIG therapy shortly after onset is associated with a lower risk of the second event. Preventive treatment after a first event could be considered for those with a higher relapse risk.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"68-75"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11652255/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141534648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Visuospatial dysfunction predicts dementia-first phenoconversion in isolated REM sleep behaviour disorder.","authors":"Jing Wang, Bei Huang, Li Zhou, Shi Tang, Hongliang Feng, Joey W Y Chan, Steven W H Chau, Jihui Zhang, Shirley X Li, Vincent Mok, Yun Kwok Wing, Yaping Liu","doi":"10.1136/jnnp-2024-333865","DOIUrl":"10.1136/jnnp-2024-333865","url":null,"abstract":"<p><strong>Objective: </strong>While isolated rapid eye movement sleep behaviour disorder (iRBD) is known as a prodrome of α-synucleinopathies, the prediction for its future phenoconversion to parkinsonism-first or dementia-first subtype remains a challenge. This study aimed to investigate whether visuospatial dysfunction predicts dementia-first phenoconversion in iRBD.</p><p><strong>Methods: </strong>Patients with iRBD and control subjects were enrolled in this prospective cohort study. Baseline neuropsychological assessment included the Unified Parkinson's Disease Rating Scale part III, Montreal Cognitive Assessment (MoCA), Rey-Osterrieth complex figure (ROCF), Colour Trails test (CTT), Farnsworth-Munsell 100-hue test and Digit Span test. The anterior and posterior subscores of MoCA as well as their modified versions were explored. A composite score derived from ROCF and CTT was also explored. Regular follow-up was conducted to determine the phenoconversion status of iRBD patients.</p><p><strong>Results: </strong>The study included 175 iRBD patients and 98 controls. During a mean follow-up of 5.1 years, 25.7% of patients experienced phenoconversion. Most of the neuropsychological tests could differentiate dementia-first but not parkinsonism-first convertors from non-convertors. The modified posterior subscore of MoCA, by integrating the Alternating Trail Making and Clock Drawing components into original the posterior subscore, which mainly reflects visuospatial function, was the strongest predictor for dementia-first phenoconversion (adjusted HR 5.48, 95% CI 1.67 to 17.98).</p><p><strong>Conclusion: </strong>Visuospatial dysfunction, as reflected mainly by the modified posterior subscore of MoCA, is a predictive factor for dementia-first phenoconversion in iRBD, suggesting its potential for being a biomarker for clinical prognostic prediction and potential neuroprotective trials aiming to delay or prevent dementia.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"76-84"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141457468","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":"Novel therapies in CIDP.","authors":"Devan Mair, Heba Madi, Filip Eftimov, Michael P Lunn, Stephen Keddie","doi":"10.1136/jnnp-2024-334165","DOIUrl":"10.1136/jnnp-2024-334165","url":null,"abstract":"<p><p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous but clinically well-described disease within circumscribed parameters. It is immunologically mediated through several poorly understood mechanisms. First-line therapies with steroids, intravenous immunoglobulin (IVIG) or plasma exchange are each effective in about two-thirds of patients. These treatments are seldom associated with complete resolution or cure, and often pose considerable practical, financial and medical implications.Our understanding of many of the key pathological processes in autoimmune diseases is expanding, and novel targeted therapeutics are being developed with promise in several autoimmune neurological disorders.This narrative review looks first at detailing key pathogenic mechanisms of disease in CIDP, followed by an in-depth description of potential novel therapies and the current evidence of their application in clinical practice.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"38-46"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365473","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}