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Prospective trial of natalizumab personalised extended interval dosing by therapeutic drug monitoring in relapsing-remitting multiple sclerosis (NEXT-MS) J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Marcus Koch
In JNNP , Toorop and coworkers present the results of the NEXT-MS trial, a clinical trial on individualised extended interval dosing (EID) of natalizumab (NTZ) in people with relapsing-remitting multiple sclerosis (RRMS).1 NTZ was one of the first high-efficacy treatments in RRMS, and has remained popular since its introduction in the late 2000s. NTZ is typically administered at a dose of 300 mg every
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Prospective trial of natalizumab personalised extended interval dosing by therapeutic drug monitoring in relapsing-remitting multiple sclerosis (NEXT-MS) J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Alyssa A Toorop, Zoë YGJ van Lierop, Liza MY Gelissen, Elske Hoitsma, Esther MPE Zeinstra, Luuk C van Rooij, Caspar EP van Munster, Anke Vennegoor, Jop P Mostert, Beatrijs HA Wokke, Nynke F Kalkers, Erwin LJ Hoogervorst, Jeroen JJ van Eijk, Christiaan M Roosendaal, Jolijn J Kragt, Marijke Eurelings, Jessie van Genugten, Jessica Nielsen, LGF Sinnige, Mark E Kloosterziel, Edo PJ Arnoldus, Gert W van
Background Extended interval dosing (EID) of natalizumab is a promising strategy to optimise treatment in multiple sclerosis (MS). Personalised EID by therapeutic drug monitoring can enable further extension of treatment intervals. Methods The NEXT-MS trial is an investigator-initiated prospective phase IV non-randomised study. Adults with a diagnosis of relapsing-remitting MS who received ≥6 natalizumab
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Exploring the cost-effectiveness of EBV vaccination to prevent multiple sclerosis in an Australian setting J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Andrew J Palmer, Ting Zhao, Bruce V Taylor, Ingrid van der Mei, Julie A Campbell
Background Increasing evidence suggests the potential of Epstein-Barr virus (EBV) vaccination in preventing multiple sclerosis (MS). We aimed to explore the cost-effectiveness of a hypothetical EBV vaccination to prevent MS in an Australian setting. Methods A five-state Markov model was developed to simulate the incidence and subsequent progression of MS in a general Australian population. The model
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Serum biomarker levels predict disability progression in patients with primary progressive multiple sclerosis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Nicolás Fissolo, Pascal Benkert, Jaume Sastre-Garriga, Neus Mongay-Ochoa, Andreu Vilaseca-Jolonch, Sara Llufriu, Yolanda Blanco, Harald Hegen, Klaus Berek, Francisco Perez-Miralles, Konrad Rejdak, Luisa M Villar, Enric Monreal, Roberto Alvarez-Lafuente, Onder K Soylu, Ahmed Abdelhak, Franziska Bachhuber, Hayrettin Tumani, Sergio Martínez-Yélamos, Antonio J Sánchez-López, Antonio García-Merino, Lucía
Background We aimed to investigate the potential of serum biomarker levels to predict disability progression in a multicentric real-world cohort of patients with primary progressive multiple sclerosis (PPMS). Methods A total of 141 patients with PPMS from 18 European MS centres were included. Disability progression was investigated using change in Expanded Disability Status Scale (EDSS) score over
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Predictive value of retinal atrophy for cognitive decline across disease duration in multiple sclerosis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Salut Alba-Arbalat, Elisabeth Solana, Elisabet Lopez-Soley, Anna Camos-Carreras, Eloy Martinez-Heras, Francesc Vivó, Irene Pulido-Valdeolivas, Magi Andorra, Maria Sepulveda, Jose María Cabrera, Elianet Fonseca, Alberto Calvi, Rafel Alcubierre, Marina Dotti-Boada, Albert Saiz, Elena H Martinez-Lapiscina, Pablo Villoslada, Yolanda Blanco, Bernardo Sanchez-Dalmau, Sara Llufriu
Background We investigated the association between changes in retinal thickness and cognition in people with MS (PwMS), exploring the predictive value of optical coherence tomography (OCT) markers of neuroaxonal damage for global cognitive decline at different periods of disease. Method We quantified the peripapillary retinal nerve fibre (pRFNL) and ganglion cell-inner plexiform (GCIPL) layers thicknesses
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Evolution of brain MRI lesions in paediatric myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and its relevance to disease course J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Omar Abdel-mannan, Dimitrios Champsas, Carmen Tur, Vanessa Lee, Sharmila Manivannan, Haroon Usman, Alison Skippen, Ishita Desai, Manali Chitre, Rob Forsyth, Rachel Kneen, Dipak Ram, Sithara Ramdas, Thomas Rossor, Siobhan West, Sukhvir Wright, Jacqueline Palace, Evangeline Wassmer, Cheryl Hemingway, Ming J Lim, Kshitij Mankad, Olga Ciccarelli, Yael Hacohen
Background Lesion resolution is often observed in children with myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and asymptomatic lesions are less commonly reported in MOGAD than in multiple sclerosis (MS). Objective We aimed to evaluate brain MRI changes over time in paediatric MOGAD. Methods Retrospective study in eight UK paediatric neuroscience centres. Acute brain MRI and
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Use, tolerability, benefits and side effects of orthotic devices in Charcot-Marie-Tooth disease J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Alessandro Bertini, Fiore Manganelli, Gian Maria Fabrizi, Angelo Schenone, Lucio Santoro, Tiziana Cavallaro, Matteo Tagliapietra, Marina Grandis, Stefano Carlo Previtali, Yuri Matteo Falzone, Isabella Allegri, Luca Padua, Costanza Pazzaglia, Irene Tramacere, Eleonora Cavalca, Paola Saveri, Andrea Quattrone, Paola Valentino, Stefano Tozza, Luca Gentile, Massimo Russo, Anna Mazzeo, Giuseppe Vita, Valeria
Background Shoe inserts, orthopaedic shoes, ankle-foot orthoses (AFOs) are important devices in Charcot-Marie-Tooth disease (CMT) management, but data about use, benefits and tolerance are scanty. Methods We administered to Italian CMT Registry patients an online ad hoc questionnaire investigating use, complications and perceived benefit/tolerability/emotional distress of shoe inserts, orthopaedic
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Effectiveness of conservative non-pharmacological interventions in people with muscular dystrophies: a systematic review and meta-analysis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Enza Leone, Anand Pandyan, Alison Rogers, Richa Kulshrestha, Jonathan Hill, Fraser Philp
Introduction Management of muscular dystrophies (MD) relies on conservative non-pharmacological treatments, but evidence of their effectiveness is limited and inconclusive. Objective To investigate the effectiveness of conservative non-pharmacological interventions for MD physical management. Methods This systematic review and meta-analysis followed Preferred Reporting Items for Systematic Reviews
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Methodological considerations for observational studies of treatment effectiveness in neurology: a clinician’s guide J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Tomas Kalincik, Izanne Roos, Sifat Sharmin, Charles B Malpas
Data from cohorts, registries, randomised trials, electronic medical records and administrative claims databases have increasingly been used to inform the use of therapies for neurological diseases. While novel sophisticated methods are enabling us to use existing data to guide treatment decisions, the complexity of statistical methodology is making appraisal of clinical evidence increasingly demanding
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Unravelling the influence of affective stimulation on functional neurological symptoms: a pilot experiment examining potential mechanisms J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Susannah Pick, LS Merritt Millman, Emily Ward, Eleanor Short, Biba Stanton, AAT Simone Reinders, Joel S Winston, Timothy R Nicholson, Mark J Edwards, Laura H Goldstein, Anthony S David, Trudie Chalder, Matthew Hotopf, Mitul A Mehta
Background Differences in affective processing have previously been shown in functional neurological disorder (FND); however, the mechanistic relevance is uncertain. We tested the hypotheses that highly arousing affective stimulation would result in elevated subjective functional neurological symptoms (FNS), and this would be associated with elevated autonomic reactivity. The possible influence of
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Pragmatic computerised perfusion diagnostics for non-convulsive status epilepticus: a prospective observational study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Elena Merli, Michele Romoli, Simone Galluzzo, Lorenzo Bevacqua, Emanuele Saverio Cece, Gabriele Ricci, Stefania Testoni, Anna Zaniboni, Maria Maddalena Viola, Luigi Simonetti, Francesca Bisulli, Sara Contardi, Paolo Tinuper, Andrea Zini
Background Non-convulsive status epilepticus (NCSE) is a time-dependent neurological disorder often misdiagnosed in the emergency setting. Electroencephalography (EEG) is often not available on a 24/7 basis, and Salzburg criteria may at times miss the diagnosis. Here, we tested the accuracy of hyperperfusion on CT perfusion imaging (CTP) in the identification of NCSE against Salzburg criteria, to define
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Binary reversals: a diagnostic sign in primary progressive aphasia J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Eoin Mulroy, Lucy B Core, Anthipa Chokesuwattanaskul, Jeremy CS Johnson, Phillip D Fletcher, Charles R Marshall, Anna Volkmer, Jonathan D Rohrer, Chris JD Hardy, Martin N Rossor, Jason D Warren
Background Binary reversals (exemplified by ‘yes’/‘no’ confusions) have been described in patients with primary progressive aphasia (PPA) but their diagnostic value and phenotypic correlates have not been defined. Methods We conducted a retrospective cohort study analysing demographic, clinical, neuropsychological, linguistic and behavioural data from patients representing all major PPA syndromes
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Clinical biomarker-based biological ageing and future risk of neurological disorders in the UK Biobank J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Jonathan K L Mak, Christopher E McMurran, Sara Hägg
Background Many common neurological disorders are associated with advancing chronological age, but their association with biological age (BA) remains poorly understood. Methods We studied 325 870 participants in the UK Biobank without a diagnosed neurological condition at baseline and generated three previously-described measures of BA based on 18 routinely measured clinical biomarkers (PhenoAge, Klemera-Doubal
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Haemorrhagic myelitis as a manifestation of MOG antibody-associated disease J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-05-01 Jonathan Ciron, Damien Biotti, Chloé Bost, Fabrice Bonneville, Romain Marignier
We read with interest the recent article ‘MOG antibody-associated encephalitis in adult: clinical phenotypes and outcomes’ by Lee et al 1 and the letter to the editor entitled ‘Acute haemorrhagic leucoencephalitis as clinical manifestation of MOG antibody-associated disease’ by Skarsta et al .2 In the first article, the authors illustrate three different phenotypes of MOG (myelin oligodendrocyte glycoprotein)
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Effects of high-intensity interval training and moderate-intensity continuous training on non-motor symptoms in patients with Parkinson’s disease: a randomised pilot trial J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-05 Ryul Kim, Seohee Choi, Nyeonju Kang, Kiwon Park, Heehyun Shin, Hanall Lee, Hyungwoo Lee, Jin-Sun Jun, Beomseok Jeon, Kyeongho Byun
The benefits of engaging in regular exercise for individuals with Parkinson’s disease (PD) have been widely recognised.1 With respect to non-motor symptoms, previous studies have demonstrated the efficacy of physical exercises on cognitive function and depressive and sleep-related symptoms.2–4 However, it is largely unknown whether exercise improves other non-motor symptoms and how such effects differ
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Routine CSF parameters as predictors of disease course in multiple sclerosis: an MSBase cohort study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-03 Cathérine Dekeyser, Matthias Hautekeete, Melissa Cambron, Vincent Van Pesch, Francesco Patti, Jens Kuhle, Samia Khoury, Jeanette Lechner Scott, Oliver Gerlach, Alessandra Lugaresi, Davide Maimone, Andrea Surcinelli, Pierre Grammond, Tomas Kalincik, Mario Habek, Barbara Willekens, Richard Macdonell, Patrice Lalive, Tunde Csepany, Helmut Butzkueven, Cavit Boz, Valentina Tomassini, Matteo Foschi, José
Background It remains unclear whether routine cerebrospinal fluid (CSF) parameters can serve as predictors of multiple sclerosis (MS) disease course. Methods This large-scale cohort study included persons with MS with CSF data documented in the MSBase registry. CSF parameters to predict time to reach confirmed Expanded Disability Status Scale (EDSS) scores 4, 6 and 7 and annualised relapse rate in
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Trends in the prevalence and pharmacological management of migraine during pregnancy in the UK, 2000–2018 J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-03 Katherine Phillips, Krishnarajah Nirantharakumar, Benjamin R Wakerley, Francesca L Crowe
Background Migraine is common in women of reproductive age. This study aimed to (1) describe the prevalence of migraine in pregnant women in the UK, (2) identify drugs commonly prescribed for migraine during pregnancy and (3) identify characteristics associated with being prescribed medication for migraine during pregnancy. Methods The Clinical Practice Research Datalink pregnancy register, a database
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Peripheral hearing loss at age 70 predicts brain atrophy and associated cognitive change J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-03 Thomas D Parker, Chris Hardy, Sarah Keuss, William Coath, David M Cash, Kirsty Lu, Jennifer M Nicholas, Sarah-Naomi James, Carole Sudre, Sebastian Crutch, Doris-Eva Bamiou, Jason D Warren, Nick C Fox, Marcus Richards, Jonathan M Schott
Background Hearing loss has been proposed as a modifiable risk factor for dementia. However, the relationship between hearing, neurodegeneration, and cognitive change, and the extent to which pathological processes such as Alzheimer’s disease and cerebrovascular disease influence these relationships, is unclear. Methods Data from 287 adults born in the same week of 1946 who underwent baseline pure
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Prognostic factors for disease activity in newly diagnosed teriflunomide-treated patients with multiple sclerosis: a nationwide Danish study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-03 Mie Reith Mahler, Melinda Magyari, Luigi Pontieri, Frederik Elberling, Rolf Pringler Holm, Arkadiusz Weglewski, Mai Bang Poulsen, Lars Kristian Storr, Plamen Anzhelov Bekyarov, Zsolt Illes, Matthias Kant, Tobias Sejbaek, Morten Leif Stilund, Peter V Rasmussen, Maria Brask, Inga Urbonaviciute, Finn Sellebjerg
Background Clinicians frequently rely on relapse counts, T2 MRI lesion load (T2L) and Expanded Disability Status Scale (EDSS) scores to guide treatment decisions for individuals diagnosed with multiple sclerosis (MS). This study evaluates how these factors, along with age and sex, influence prognosis during treatment with teriflunomide (TFL). Methods We conducted a nationwide cohort study using data
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Neuroanatomical and prognostic associations of depression in Parkinson’s disease J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-03 James B Badenoch, Alvar Paris, Benjamin Meir Jacobs, Alastair J Noyce, Charles R Marshall, Sheena Waters
Background Depression is reported as a risk factor, prodromal feature and late consequence of Parkinson’s disease (PD). We aimed to evaluate the timing, neuroanatomy and prognostic implications of depression in PD. Methods We used data from 434 023 participants from UK Biobank with 14.1 years of follow-up. Multivariable regression models established associations of depression with incident PD and regional
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Validation of the international MOGAD panel proposed criteria: a single-centre US study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-03 Angeliki G Filippatou, Yana Said, Haiwen Chen, Eleni S Vasileiou, Gelareh Ahmadi, Elias S Sotirchos
Background Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a demyelinating disorder of the central nervous system. We aimed to evaluate the diagnostic performance of recently proposed MOGAD diagnostic criteria in a real-world patient cohort at a tertiary referral centre. Methods We identified all patients who were evaluated at Johns Hopkins and were MOG-IgG seropositive
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T cell activation markers CD38 and HLA-DR indicative of non-seroconversion in anti-CD20-treated patients with multiple sclerosis following SARS-CoV-2 mRNA vaccination J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-28 Niels J M Verstegen, Ruth R Hagen, Christine Kreher, Lisan H Kuijper, Jet van den Dijssel, Thomas Ashhurst, Laura Y L Kummer, Virginia Palomares Cabeza, Maurice Steenhuis, Mariël C Duurland, Rivka de Jongh, C Ellen van der Schoot, Veronique A L Konijn, Erik Mul, Katherine Kedzierska, Koos P J van Dam, Eileen W Stalman, Laura Boekel, Gertjan Wolbink, Sander W Tas, Joep Killestein, Theo Rispens, Luuk
Background Messenger RNA (mRNA) vaccines provide robust protection against SARS-CoV-2 in healthy individuals. However, immunity after vaccination of patients with multiple sclerosis (MS) treated with ocrelizumab (OCR), a B cell-depleting anti-CD20 monoclonal antibody, is not yet fully understood. Methods In this study, deep immune profiling techniques were employed to investigate the immune response
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Venous thromboembolism risk in amyotrophic lateral sclerosis: a hospital record-linkage study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-28 Raph Goldacre, Michael Trubshaw, Eva J A Morris, Kevin Talbot, Michael J Goldacre, Alexander Guy Thompson, Martin R Turner
Background Venous thromboembolism (VTE) can occur in amyotrophic lateral sclerosis (ALS) and pulmonary embolism causes death in a minority of cases. The benefits of preventing VTE must be weighed against the risks. An accurate estimate of the incidence of VTE in ALS is crucial to assessing this balance. Methods This retrospective record-linkage cohort study derived data from the Hospital Episode Statistics
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Effectiveness of autologous haematopoietic stem cell transplantation versus natalizumab in progressive multiple sclerosis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-27 Tomas Kalincik, Sifat Sharmin, Izanne Roos, Jennifer Massey, Ian Sutton, Barbara Withers, Mark S Freedman, Harold Atkins, Eva Krasulova, Eva Kubala Havrdova, Marek Trneny, Tomas Kozak, Joachim Burman, Richard Macdonell, Øivind Torkildsen, Lars Bø, Anne Kristine Lehmann, Basil Sharrack, John Snowden
Background Natalizumab was not shown to modify disability in progressive multiple sclerosis (MS). This matched observational study compared the effectiveness of autologous haematopoietic stem cell transplantation (AHSCT) with natalizumab in progressive MS. Methods Patients with primary/secondary progressive MS from seven AHSCT MS centres and the MSBase registry, treated with AHSCT or natalizumab, were
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Epidemiology of myasthenia gravis in Denmark, Finland and Sweden: a population-based observational study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-27 John Vissing, Sari Atula, Mari Savolainen, Juha Mehtälä, Laila Mehkri, Tina Bech Olesen, Tero Ylisaukko-oja, Ingrid Lindberg-Schager, Fredrik Berggren, Fredrik Piehl
Background Incidence and prevalence rates of myasthenia gravis (MG) vary considerably across studies, and mortality risk is rarely addressed. We examined the prevalence and incidence rates, mortality and factors associated with mortality with MG. Method This was a registry linkage study based on nationwide health and administrative registries of Denmark, Finland and Sweden (populations of 5.9, 5.6
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Prevalence of autoimmune diseases in functional neurological disorder: influence of psychiatric comorbidities and biological sex J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-21 Anna Joseph, Gaston Baslet, Mary A O’Neal, Ginger Polich, Irene Gonsalvez, Andrea N Christoforou, Barbara A Dworetzky, Primavera A Spagnolo
Background Functional neurological disorder (FND) is a common and disabling neuropsychiatric condition, which disproportionally affects women compared with men. While the etiopathogenesis of this disorder remains elusive, immune dysregulation is emerging as one potential mechanism. To begin to understand the role of immune dysfunctions in FND, we assessed the prevalence of several common autoimmune
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Clinicoradiological and neuropathological evaluation of primary progressive aphasia J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-21 Dror Shir, Nick Corriveau-Lecavalier, Camilo Bermudez Noguera, Leland Barnard, Nha Trang Thu Pham, Hugo Botha, Joseph R Duffy, Heather M Clark, Rene L Utianski, David S Knopman, Ronald C Petersen, Bradley F Boeve, Melissa E Murray, Aivi T Nguyen, R Ross Reichard, Dennis W Dickson, Gregory S Day, Walter K Kremers, Neill R Graff-Radford, David T Jones, Mary M Machulda, Julie A Fields, Jennifer L Whitwell
Background Primary progressive aphasia (PPA) defines a group of neurodegenerative disorders characterised by language decline. Three PPA variants correlate with distinct underlying pathologies: semantic variant PPA (svPPA) with transactive response DNA-binding protein of 43 kD (TDP-43) proteinopathy, agrammatic variant PPA (agPPA) with tau deposition and logopenic variant PPA (lvPPA) with Alzheimer’s
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Circuit-based neuromodulation enhances delayed recall in amnestic mild cognitive impairment J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-19 Jie Ma, Jia-Jia Wu, Xiang-Xin Xing, Xin Xue, Yun-Ting Xiang, Xiao-Min Zhen, Jian-Hua Li, Juan-Juan Lu, Jun-Peng Zhang, Mou-Xiong Zheng, Xu-Yun Hua, Jian-Guang Xu
Background This study aimed to investigate the efficacy of circuits-based paired associative stimulation (PAS) in adults with amnestic mild cognitive impairment (aMCI). Methods We conducted a parallel-group, randomised, controlled clinical trial. Initially, a cohort of healthy subjects was recruited to establish the cortical-hippocampal circuits by tracking white matter fibre connections using diffusion
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Dissociative seizures in the emergency department: how to solve the problem of diagnostic confusion leading to iatrogenic harm? J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Mahinda Yogarajah
Over the last 20 years, there has been a renaissance in the study of functional neurological disorders (FNDs). Alongside an improved understanding of their involuntary nature,1 and mechanistic and aetiological complexities,2 there is an increasing recognition of the iatrogenic harm and socioeconomic costs associated with these disorders. Dissociative seizures are the most common form of FND. They look
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Dissociative seizures in the emergency room: room for improvement J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Ozan Cengiz, Johannes Jungilligens, Rosa Michaelis, Jörg Wellmer, Stoyan Popkirov
Background Dissociative seizures, also known as functional or psychogenic non-epileptic seizures, account for 11%–27% of all emergency seizure presentations. Misdiagnosis as epileptic seizures is common and leads to ineffective and potentially harmful treatment escalations. We assess the potential for diagnostic improvement at different stages of emergency workup and estimate the utility of benzodiazepines
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GPi-DBS-induced brain metabolic activation in cervical dystonia J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Emma A Honkanen, Jaana Rönkä, Eero Pekkonen, Juho Aaltonen, Maija Koivu, Olli Eskola, Hazem Eldebakey, Jens Volkmann, Valtteri Kaasinen, Martin M Reich, Juho Joutsa
Background Deep brain stimulation (DBS) of the globus pallidus interna (GPi) is a highly efficacious treatment for cervical dystonia, but its mechanism of action is not fully understood. Here, we investigate the brain metabolic effects of GPi-DBS in cervical dystonia. Methods Eleven patients with GPi-DBS underwent brain 18F-fluorodeoxyglucose positron emission tomography imaging during stimulation
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Are patients with GBA–Parkinson disease good candidates for deep brain stimulation? A longitudinal multicentric study on a large Italian cohort J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Micol Avenali, Roberta Zangaglia, Giada Cuconato, Ilaria Palmieri, Alberto Albanese, Carlo Alberto Artusi, Marco Bozzali, Giovanna Calandra-Buonaura, Francesco Cavallieri, Roberto Cilia, Antoniangela Cocco, Filippo Cogiamanian, Fabiana Colucci, Pietro Cortelli, Alessio Di Fonzo, Roberto Eleopra, Giulia Giannini, Alberto Imarisio, Gabriele Imbalzano, Claudia Ledda, Leonardo Lopiano, Maria Chiara Malaguti
Background GBA variants increase the risk of developing Parkinson disease (PD) and influence its outcome. Deep brain stimulation (DBS) is a recognised therapeutic option for advanced PD. Data on DBS long-term outcome in GBA carriers are scarce. Objective To elucidate the impact of GBA variants on long-term DBS outcome in a large Italian cohort. Methods We retrospectively recruited a multicentric Italian
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Temporal course of cognitive and behavioural changes in motor neuron diseases J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Caroline A McHutchison, Joanne Wuu, Corey T McMillan, Rosa Rademakers, Jeffrey Statland, Gang Wu, Evadnie Rampersaud, Jason Myers, Jessica P Hernandez, Sharon Abrahams, Michael Benatar
Background Cognitive and behavioural dysfunction may occur in people with motor neuron disease (MND), with some studies suggesting an association with the C9ORF72 repeat expansion. Their onset and progression, however, is poorly understood. We explored how cognition and behaviour change over time, and whether demographic, clinical and genetic factors impact these changes. Methods Participants with
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Seroreactivity against lytic, latent and possible cross-reactive EBV antigens appears on average 10 years before MS induced preclinical neuroaxonal damage J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Daniel Jons, Viktor Grut, Tomas Bergström, Henrik Zetterberg, Martin Biström, Martin Gunnarsson, Magnus Vrethem, Nicole Brenner, Julia Butt, Kaj Blennow, Staffan Nilsson, Ingrid Kockum, Tomas Olsson, Tim Waterboer, Peter Sundström, Oluf Andersen
Background Multiple sclerosis (MS) and presymptomatic axonal injury appear to develop only after an Epstein-Barr virus (EBV) infection. This association remains to be confirmed across a broad preclinical time range, for lytic and latent EBV seroreactivity, and for potential cross-reacting antigens. Methods We performed a case–control study with 669 individual serum samples obtained before clinical
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Best practices in phase III clinical trials on DMTs for multiple sclerosis: a systematic analysis and appraisal of published trials J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Marta Mascarenas-Garcia, Alejandro Rivero-de-Aguilar, Mónica Pérez-Ríos, Alberto Ruano-Raviña, Miguel Angel Llaneza-Gonzalez, Cristina Candal-Pedreira, Julia Rey-Brandariz, Leonor Varela-Lema
Background Great advances have been made in the field of multiple sclerosis (MS) therapy due to the publication of numerous randomised clinical trials (RCTs). In this study, we carried out a critical appraisal of phase III RCTs of disease-modifying therapies (DMTs) for MS published after 2010, intending to identify critical areas of improvement. Methods We performed a systematic search of published
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COVID-19 has no impact on disease activity, progression and cognitive performance in people with multiple sclerosis: a 2-year study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Federico Montini, Agostino Nozzolillo, Nicolò Tedone, Damiano Mistri, Paola MV Rancoita, Chiara Zanetta, Alessandra Mandelli, Roberto Furlan, Lucia Moiola, Vittorio Martinelli, Maria A Rocca, Massimo Filippi
Background Sequelae of COVID-19 in people with multiple sclerosis (PwMS) have not been characterised. We explored whether COVID-19 is associated with an increased risk of disease activity, disability worsening, neuropsychological distress and cognitive dysfunction during the 18–24 months following SARS-COV-2 infection. Methods We enrolled 174 PwMS with history of COVID-19 (MS-COVID) between March 2020
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Do lower limb motor-evoked potentials predict walking outcomes post-stroke? J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Marie-Claire Smith, Benjamin J Scrivener, Cathy M Stinear
Background This observational study examined whether lower limb (LL) motor-evoked potentials (MEPs) 1 week post-stroke predict recovery of independent walking, use of ankle-foot orthosis (AFO) or walking aid, at 3 and 6 months post-stroke. Methods Non-ambulatory participants were recruited 5 days post-stroke. Transcranial magnetic stimulation was used to determine tibialis anterior MEP status and clinical
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Alzheimer’s disease marker phospho-tau181 is not elevated in the first year after moderate-to-severe TBI J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Neil Graham, Karl Zimmerman, Amanda J Heslegrave, Ashvini Keshavan, Federico Moro, Samia Abed-Maillard, Adriano Bernini, Vincent Dunet, Elena Garbero, Giovanni Nattino, Arturo Chieregato, Enrico Fainardi, Camelia Baciu, Primoz Gradisek, Sandra Magnoni, Mauro Oddo, Guido Bertolini, Jonathan M Schott, Henrik Zetterberg, David Sharp
Background Traumatic brain injury (TBI) is associated with the tauopathies Alzheimer’s disease and chronic traumatic encephalopathy. Advanced immunoassays show significant elevations in plasma total tau (t-tau) early post-TBI, but concentrations subsequently normalise rapidly. Tau phosphorylated at serine-181 (p-tau181) is a well-validated Alzheimer’s disease marker that could potentially seed progressive
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Premorbid brain structure influences risk of amyotrophic lateral sclerosis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Alexander G Thompson, Bernd Taschler, Stephen M Smith, Martin R Turner
Background Amyotrophic lateral sclerosis (ALS) is a disease of the motor network associated with brain structure and functional connectivity alterations that are implicated in disease progression. Whether such changes have a causal role in ALS, fitting with a postulated influence of premorbid cerebral architecture on the phenotypes associated with neurodegenerative disorders is not known. Methods This
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The T1-weighted/T2-weighted ratio as a biomarker of anti-NMDA receptor encephalitis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Tim Julian Hartung, Graham Cooper, Valentin Jünger, Darko Komnenić, Lara Ryan, Josephine Heine, Claudia Chien, Friedemann Paul, Harald Prüss, Carsten Finke
Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis rarely causes visible lesions in conventional MRI, yet advanced imaging detects extensive white matter damage. To improve prognostic capabilities, we evaluate the T1-weighted/T2-weighted (T1w/T2w) ratio, a measure of white matter integrity computable from clinical MRI sequences, in NMDAR encephalitis and examine its associations with
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Understanding the pathophysiology of idiopathic intracranial hypertension (IIH): a review of recent developments J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Blake D Colman, Frederique Boonstra, Minh NL Nguyen, Subahari Raviskanthan, Priya Sumithran, Owen White, Elspeth J Hutton, Joanne Fielding, Anneke van der Walt
Idiopathic intracranial hypertension (IIH) is a condition of significant morbidity and rising prevalence. It typically affects young people living with obesity, mostly women of reproductive age, and can present with headaches, visual abnormalities, tinnitus and cognitive dysfunction. Raised intracranial pressure without a secondary identified cause remains a key diagnostic feature of this condition
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Dementia prevention: the Mendelian randomisation perspective J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 Emma Louise Anderson, Neil M Davies, Roxanna Korologou-Linden, Mika Kivimäki
Understanding the causes of Alzheimer’s disease and related dementias remains a challenge. Observational studies investigating dementia risk factors are limited by the pervasive issues of confounding, reverse causation and selection biases. Conducting randomised controlled trials for dementia prevention is often impractical due to the long prodromal phase and the inability to randomise many potential
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Correction: ICARUS study: prevalence and clinical features of impulse control disorders in Parkinson’s disease J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-04-01 BMJ Publishing Group Ltd
Antonini A, Barone P, Bonuccelli U, et al . ICARUS study: …
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Revisiting the jumping to conclusions bias in functional movement disorders J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-07 Raquel Sainz-Amo, Jessenia Morillo-González, Jorge Gómez-Corral, Cristina Moreno, Araceli Alonso Cánovas, Juan Carlos Martinez Castrillo, Mark J Edwards, Daniel Hernández-Huerta, Isabel Pareés
Functional movement disorders (FMD) are part of the spectrum of functional neurological disorders (FND), which are common and disabling. Nowadays, it is widely accepted that key aspects of the underlying pathophysiology include the tendency to form abnormal beliefs about symptoms, disturbance of attentional control and disruption of mechanisms regulating sense of agency.1 ‘Jumping to conclusions’ bias
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Central vein sign and trigeminal lesions of multiple sclerosis visualised by 7T MRI J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-07 Jing Jing, Zhe Zhang, Lei Su, Chenyang Gao, Ai Guo, Xinyao Liu, Huabing Wang, Xinghu Zhang, Yaou Liu, Giancarlo Comi, Emmanuelle Waubant, Fu-Dong Shi, De-Cai Tian
Background Although trigeminal nerve involvement is a characteristic of multiple sclerosis (MS), its prevalence across studies varies greatly due to MRI resolution and cohort selection bias. The mechanism behind the site specificity of trigeminal nerve injury is still unclear. We aim to determine the prevalence of trigeminal nerve involvement in patients with MS in a consecutive 7T brain MRI cohort
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Patient-perceived progression in multiple system atrophy: natural history of quality of life J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-07 Tiphaine Saulnier, Margherita Fabbri, Mélanie Le Goff, Catherine Helmer, Anne Pavy-Le Traon, Wassilios G. Meissner, Olivier Rascol, Cecile Proust-Lima, Alexandra Foubert-Samier
Background Health-related quality of life (Hr-QoL) scales provide crucial information on neurodegenerative disease progression, help improve patient care and constitute a meaningful endpoint for therapeutic research. However, Hr-QoL progression is usually poorly documented, as for multiple system atrophy (MSA), a rare and rapidly progressing alpha-synucleinopathy. This work aimed to describe Hr-QoL
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Comparing ocrelizumab to interferon/glatiramer acetate in people with multiple sclerosis over age 60 J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-07 Yi Chao Foong, Daniel Merlo, Melissa Gresle, Katherine Buzzard, Michael Zhong, Wei Zhen Yeh, Vilija Jokubaitis, Mastura Monif, Olga Skibina, Serkan Ozakbas,, Francesco Patti, Pierre Grammond, Maria Pia Amato, Tomas Kalincik, Dana Horakova, Eva Kubala Havrdova, Bianca Weinstock-Guttman, Jeanette Lechner Scott, Cavit Boz, Maria Jose Sa, Helmut Butzkueven, Anneke van der Walt, Chao Zhu
Background Ongoing controversy exists regarding optimal management of disease modifying therapy (DMT) in older people with multiple sclerosis (pwMS). There is concern that the lower relapse rate, combined with a higher risk of DMT-related infections and side effects, may alter the risk-benefit balance in older pwMS. Given the lack of pwMS above age 60 in randomised controlled trials, the comparative
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Risk of stroke with different levels of leisure-time physical activity: a systematic review and meta-analysis of prospective cohort studies J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-05 Federico De Santis, Michele Romoli, Matteo Foschi, Francesco Domenico Sciancalepore, Lucio D'Anna, Lorenzo Barba, Samir Abu-Rumeileh, Simona Sacco, Raffaele Ornello
Background Leisure-time physical activity (LTPA) protects against vascular diseases. Whether and to what extent different levels of LTPA, including lower ones, benefit stroke prevention is still unclear. Methods We searched prospective cohort studies, indexed on PubMed and Scopus, published in English up to 22 April 2023, that investigated, in a general healthy population, the relationship between
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Resective epilepsy surgery and its impact on depression in adults: a systematic review, meta-analysis, and implications for future research J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-05 Natalia Hernandez Poblete, Florian Gay, Francesco Salvo, Jean-Arthur Micoulaud-Franchi, Thomas Bienvenu, Julien Coelho, Jerome Aupy
Background How epilepsy surgery influences the bidirectional relationship of epilepsy and depression remains poorly defined. Method For a better understanding of this question, we conducted a systematic review and meta-analysis of risk ratio on depression prevalence before and after epilepsy surgery, using Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines. Three databases
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Does sex influence the natural history of idiopathic adult-onset dystonia? J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-01 Vittorio Velucci, Sarah Idrissi, Roberta Pellicciari, Marcello Esposito, Assunta Trinchillo, Daniele Belvisi, Giovanni Fabbrini, Gina Ferrazzano, Carmen Terranova, Paolo Girlanda, Giovanni Majorana, Vincenzo Rizzo, Francesco Bono, Giovanni Idone, Vincenzo Laterza, Laura Avanzino, Francesca Di Biasio, Roberta Marchese, Anna Castagna, Marina Ramella, Christian Lettieri, Sara Rinaldo, Maria Concetta Altavista
Background Several earlier studies showed a female predominance in idiopathic adult-onset dystonia (IAOD) affecting the craniocervical area and a male preponderance in limb dystonia. However, sex-related differences may result from bias inherent to study design. Moreover, information is lacking on whether sex-related differences exist in expressing other dystonia-associated features and dystonia spread
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Prognostic factors of first-onset optic neuritis based on diagnostic criteria and antibody status: a multicentre analysis of 427 eyes J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-28 Young Gi Min, Yeji Moon, Young Nam Kwon, Byung Joo Lee, Kyung-Ah Park, Jae Yong Han, Jinu Han, Haeng-Jin Lee, Seol-Hee Baek, Byung-Jo Kim, Jun-Soon Kim, Kyung Seok Park, Nam-Hee Kim, Martha Kim, Tai-Seung Nam, Seong-Il Oh, Jae Ho Jung, Jung-Joon Sung, Myoung-Jin Jang, Seong-Joon Kim, Sung-Min Kim
Background Optic neuritis (ON) prognosis is influenced by various factors including attack severity, underlying aetiologies, treatments and consequences of previous episodes. This study, conducted on a large cohort of first ON episodes, aimed to identify unique prognostic factors for each ON subtype, while excluding any potential influence from pre-existing sequelae. Methods Patients experiencing their
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Exercise as medicine in Parkinson’s disease J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-28 Martin Langeskov-Christensen, Erika Franzén, Lars Grøndahl Hvid, Ulrik Dalgas
Parkinson’s disease (PD) is an incurable and progressive neurological disorder leading to deleterious motor and non-motor consequences. Presently, no pharmacological agents can prevent PD evolution or progression, while pharmacological symptomatic treatments have limited effects in certain domains and cause side effects. Identification of interventions that prevent, slow, halt or mitigate the disease
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NICE guideline on ME/CFS: robust advice based on a thorough review of the evidence J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-28 Peter Walter Barry, Kate Kelley, Toni Tan, Ilora Finlay
In 2021, the National Institute for Health and Care Excellence produced an evidence-based guideline on the diagnosis and management of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), a disabling long-term condition of unknown cause. The guideline provides clear support for people living with ME/CFS, their families and carers, and for clinicians. A recent opinion piece published in the
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Effects of motor cortical and peripheral axonal hyperexcitability on survival in amyotrophic lateral sclerosis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-28 Ryo Otani, Kazumoto Shibuya, Yo-Ichi Suzuki, Tomoki Suichi, Marie Morooka, Yuya Aotsuka, Moeko Ogushi, Satoshi Kuwabara
Background Increased ‘cortical’ and ‘peripheral’ excitability are reportedly associated with shorter survival in amyotrophic lateral sclerosis (ALS) patients, suggesting that hyperexcitability contributes to motor neuron death. However, whether upper or lower motor function has a greater impact on survival is unclear. We aimed to investigate the component that strongly impacts the prognosis of ALS
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Primary lateral sclerosis: application and validation of the 2020 consensus diagnostic criteria in an expert opinion-based PLS cohort J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-22 Simon Witzel, Veronika Micca, Hans P Müller, André Huss, Franziska Bachhuber, Johannes Dorst, Dorothée E Lulé, Hayrettin Tumani, Jan Kassubek, Albert C Ludolph
Background Validation of the 2020 consensus criteria for primary lateral sclerosis (PLS) is essential for their use in clinical practice and future trials. Methods In a large cohort of patients diagnosed with PLS by expert opinion prior to the new criteria with detailed clinical baseline evaluation (n=107) and longitudinal follow-up (n=63), we applied the new diagnostic criteria and analysed the clinical
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Incidence and determinants of seizures in multiple sclerosis: a meta-analysis of randomised clinical trials J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-21 Valeria Pozzilli, Shalom Haggiag, Massimiliano Di Filippo, Fioravante Capone, Vincenzo Di Lazzaro, Carla Tortorella, Claudio Gasperini, Luca Prosperini
Background Seizures are reported to be more prevalent in individuals with multiple sclerosis (MS) compared with the general population. Existing data predominantly originate from population-based studies, which introduce variability in methodologies and are vulnerable to selection and reporting biases. Methods This meta-analysis aims to assess the incidence of seizures in patients participating in
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Pain and functional neurological disorder: a systematic review and meta-analysis J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-21 Moritz Steinruecke, Isabel Mason, Mairi Keen, Laura McWhirter, Alan J Carson, Jon Stone, Ingrid Hoeritzauer
Background Functional neurological disorder (FND) is characterised by neurological symptoms, such as seizures and abnormal movements. Despite its significance to patients, the clinical features of chronic pain in people with FND, and of FND in people with chronic pain, have not been comprehensively studied. Methods We systematically reviewed PubMed, Embase and PsycINFO for studies of chronic pain in
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Therapeutic interventions increasing seizure risk in multiple sclerosis: resolving discordant meta-analyses J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-21 John P Ioannidis
In their JNNP meta-analysis Pozzilli et al concludes that sphingosine-1-phosphate receptor (S1PR) modulators substantially increase seizure risk in multiple sclerosis.1 Conversely, another meta-analysis by Dang et al 2 recently concluded that ‘no evidence of association was found between disease-modifying therapy and seizure risk’. Both meta-analyses focused on randomised controlled trials, but used
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Genotype-specific spinal cord damage in spinocerebellar ataxias: an ENIGMA-Ataxia study J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-02-21 Thiago Junqueira Ribeiro Rezende, Isaac Adanyaguh, Orlando G P Barsottini, Benjamin Bender, Fernando Cendes, Leo Coutinho, Andreas Deistung, Imis Dogan, Alexandra Durr, Juan Fernandez-Ruiz, Sophia L Göricke, Marina Grisoli, Carlos R Hernandez-Castillo, Christophe Lenglet, Caterina Mariotti, Alberto R M Martinez, Breno K Massuyama, Fanny Mochel, Lorenzo Nanetti, Anna Nigri, Sergio E Ono, Gülin Öz, José
Background Spinal cord damage is a feature of many spinocerebellar ataxias (SCAs), but well-powered in vivo studies are lacking and links with disease severity and progression remain unclear. Here we characterise cervical spinal cord morphometric abnormalities in SCA1, SCA2, SCA3 and SCA6 using a large multisite MRI dataset. Methods Upper spinal cord (vertebrae C1–C4) cross-sectional area (CSA) and
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Genetics, cell biology and a novel mechanism for ALS J. Neurol. Neurosurg. Psychiatry (IF 11.0) Pub Date : 2024-03-01 Michael E Shy
Syeda and colleagues used an elegant combination of genetics, meticulous clinical evaluation and cell biology to demonstrate that disrupting serine palmitoyl transferase (SPT) can cause amyotrophic lateral sclerosis (ALS) in very young children.1 SPT is the initial rate-limiting step in sphingolipid biosynthesis, joining serine and palmitoyl CoA in a decarboxylating condensation reaction that ultimately