Journal of Neurology, Neurosurgery, and Psychiatry最新文献

{"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}

{"title":"CSF sphingolipids are correlated with neuroinflammatory cytokines and differentiate neuromyelitis optica spectrum disorder from multiple sclerosis.","authors":"Lisa Shi, Laura Ghezzi, Chiara Fenoglio, Anna Margherita Pietroboni, Daniela Galimberti, Francesca Pace, Todd A Hardy, Laura Piccio, Anthony S Don","doi":"10.1136/jnnp-2024-333774","DOIUrl":"10.1136/jnnp-2024-333774","url":null,"abstract":"<p><strong>Background: </strong>There is a need for biomarkers of disease progression and therapeutic response in multiple sclerosis (MS). This study aimed to identify cerebrospinal fluid (CSF) lipids that differentiate MS from other neuroinflammatory conditions and correlate with Expanded Disability Status Scale (EDSS) scores, gadolinium-enhancing lesions or inflammatory mediators.</p><p><strong>Methods: </strong>Lipids and inflammatory cytokines/chemokines were quantified with liquid chromatography-tandem mass spectrometry and multiplex ELISA, respectively, in CSF from people with untreated MS, neuromyelitis optica spectrum disorder (NMOSD), other inflammatory neurological diseases and non-inflammatory neurological diseases (NIND). Analytes were compared between groups using analysis of variance, and correlations were assessed with Pearson's analysis.</p><p><strong>Results: </strong>Twenty-five sphingolipids and four lysophosphatidylcholines were significantly higher in NMOSD compared with MS and NIND cases, whereas no lipids differed significantly between MS and NIND. A combination of three sphingolipids differentiated NMOSD from MS with the area under the curve of 0.92 in random forest models. Ninety-four lipids, including those that differentiated NMOSD from MS, were positively correlated with macrophage migration inhibitory factor (MIF) and 37 lipids were positively correlated with CSF protein in two independent MS cohorts. EDSS was inversely correlated with cholesterol ester CE(16:0) in both MS cohorts. In contrast, MIF and soluble triggering receptor expressed on myeloid cells 2 were positively associated with EDSS.</p><p><strong>Conclusions: </strong>CSF sphingolipids are positively correlated with markers of neuroinflammation and differentiate NMOSD from MS. The inverse correlation between EDSS and CE(16:0) levels may reflect poor clearance of cholesterol released during myelin break-down and warrants further investigation as a biomarker of therapeutic response.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"54-67"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11672031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141283940","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":"Sodium valproate is associated with cortical thinning of disease-specific areas in juvenile myoclonic epilepsy.","authors":"Bernardo Crespo Pimentel, Giorgi Kuchukhidze, Fenglai Xiao, Lorenzo Caciagli, Julia Höfler, Lucas Rainer, Martin Kronbichler, Christian Vollmar, John S Duncan, Eugen Trinka, Matthias Koepp, Britta Wandschneider","doi":"10.1136/jnnp-2024-333703","DOIUrl":"10.1136/jnnp-2024-333703","url":null,"abstract":"<p><strong>Background: </strong>Juvenile myoclonic epilepsy (JME) is associated with cortical thinning of the motor areas. The relative contribution of antiseizure medication to cortical thickness is unknown. We aimed to investigate how valproate influences the cortical morphology of JME.</p><p><strong>Methods: </strong>In this cross-sectional study, individuals with JME with and without valproate, with temporal lobe epilepsy (TLE) with valproate and controls were selected through propensity score matching. Participants underwent T1-weighted brain imaging and vertex-wise calculation of cortical thickness.</p><p><strong>Results: </strong>We matched 36 individuals with JME on valproate with 36 individuals with JME without valproate, 36 controls and 19 individuals with TLE on valproate. JME on valproate showed thinning of the precentral gyri (left and right, p<0.001) compared with controls and thinning of the left precentral gyrus when compared with JME not on valproate (p<0.01) or to TLE on valproate (p<0.001). Valproate dose correlated negatively with the thickness of the precentral gyri, postcentral gyri and superior frontal gyrus in JME (left and right p<0.0001), but not in TLE.</p><p><strong>Conclusions: </strong>Valproate was associated with JME-specific and dose-dependent thinning of the cortical motor regions. This suggests that valproate is a key modulator of cortical morphology in JME, an effect that may underlie its high efficacy in this syndrome.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"11-14"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141751939","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":"Prognosis prediction and immunotherapy optimisation for cryptogenic new-onset refractory status epilepticus.","authors":"Yoonhyuk Jang, Soo Hyun Ahn, Kyung-Il Park, Bum-Sup Jang, Han Sang Lee, Jae-Han Bae, Yoonkyung Lee, Jun-Sang Sunwoo, Jin-Sun Jun, Keun Tae Kim, Su Yee Mon, Ji Hye You, Tae-Joon Kim, Hyunsuk Shin, Dohyun Han, Yong Won Cho, Divyanshu Dubey, Kon Chu, Sang Kun Lee, Soon-Tae Lee","doi":"10.1136/jnnp-2024-334285","DOIUrl":"10.1136/jnnp-2024-334285","url":null,"abstract":"<p><strong>Background: </strong>Cryptogenic new-onset refractory status epilepticus (cNORSE) currently lacks comprehensive knowledge regarding its clinical dynamics, prognostic factors and treatment guidance. Here we present the longitudinal clinical profiles, predictive factors for outcomes and the optimal duration of immunotherapy in patients with cNORSE.</p><p><strong>Methods: </strong>This retrospective secondary endpoint analysis investigated patients with cNORSE identified from a prospective autoimmune encephalitis cohort at a national referral centre in Korea. The main outcomes included longitudinal functional scales, seizure frequency and the number of antiseizure medications. Measures encompassed NORSE-related clinical parameters such as the duration of unconsciousness, immunotherapy profiles, cytokine/chemokine analysis, and serial MRI scans.</p><p><strong>Results: </strong>A total of 74 patients with cNORSE were finally analysed (mean age: 38.0±18.2; 36 (48.6%) male). All patients received first-line immunotherapy, and 91.9% (68/74) received second-line immunotherapy. A total of 83.8% (62/74) regained consciousness within a median duration of 30 days (14-56), and 50% (31/62) achieved good outcome (mRS ≤2) at 2 years. Poor 1-year outcomes (mRS ≥3) were predicted by the presence of mesial temporal lobe (mTL) and extra-mTL lesions at 3-month MRI, and prolonged unconsciousness (≥60 days). Those with mTL atrophy exhibited a higher seizure burden post-NORSE. The optimal duration of immunotherapy appeared to be between 18 weeks and 1-year post-NORSE onset.</p><p><strong>Conclusions: </strong>This study elucidates longitudinal clinical dynamics, functional outcomes, prognostic factors and immunotherapy response in patients with cNORSE. These findings might contribute to a more standardised understanding and clinical decision-making for cNORSE.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"26-37"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140350","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":"First-ever seizure and eligibility for commercial motor vehicle driving.","authors":"Nicholas Lawn, Judy Lee, John Dunne","doi":"10.1136/jnnp-2024-333684","DOIUrl":"10.1136/jnnp-2024-333684","url":null,"abstract":"<p><strong>Background: </strong>After a first-ever seizure, 6 months of seizure freedom is usually required before returning to driving a private motor vehicle, after which the annual risk of seizure recurrence has fallen to ≤20%. Stricter criteria apply for commercial driver's licence (CDL) holders, and a longer period of seizure freedom sufficient for the annual risk of recurrence to be <2% is recommended. However, CDL guidelines are based on little data with few studies having long-term follow-up.</p><p><strong>Methods: </strong>1714 patients with first-ever seizures were prospectively studied. Seizure recurrence was evaluated using survival analysis. The annual conditional risk of seizure recurrence was calculated for patients with first-ever unprovoked and acute symptomatic seizures, and according to the presence or absence of clinical, electroencephalogram (EEG) and neuroimaging risk factors for recurrence.</p><p><strong>Results: </strong>The annual risk of recurrence for unprovoked first seizures did not fall below 2% until after 9 years of seizure freedom. The annual risk after 5 years of seizure freedom was still 3.9% (95% CI 1.8% to 6.1%) including for those without epileptiform abnormalities on EEG and with normal imaging. For acute symptomatic first seizures, the annual recurrence risk was 4.5% (95% CI 2.3% to 6.7%) after 1 year and fell below 2% only after 4 years of seizure freedom.</p><p><strong>Conclusions: </strong>For unprovoked and acute symptomatic first-ever seizure and CDL, a higher-than-expected annual seizure risk persists beyond the currently recommended seizure-free periods, even in those without risk factors for recurrence. Our data can inform decisions regarding a return to driving for CDL holders after first-ever seizure.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"4-10"},"PeriodicalIF":8.7,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141457438","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}