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    Journal of Neurology

    Journal of Neurology

    JournalISSN 0340-5354eISSN 1432-1459中科院 医学 2区

    年发文量

    研究主题

    论文(133300)

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    1NewDiscontinuation and Optimization of Device-Aided Therapies in Parkinson’s Disease: a Prospective Comparative Real-World Study
    Diego Santos García,Ángela Solleiro Vidal,Marta Blázquez Estrada,Pablo Mir,Déborah Alonso Modino,Inés Legarda,Nuria López Ariztegui,Rocío García Ramos,Nuria Caballol, Asunción Ávila Rivera,Iria Cabo,Jorge Hernández Vara,

    Comparative real-world evidence on device-aided therapy (DAT) persistence in Parkinson’s disease (PD) is limited. We assessed discontinuation, reasons, optimization time, and associated factors. We analyzed the prospective multicenter DATs-PD GETM Spanish Registry (January 2024–May 2026), including deep brain stimulation (DBS), continuous subcutaneous apomorphine infusion (CSAI), foslevodopa/foscarbidopa (fLD/fCD), levodopa–carbidopa intestinal gel (LCIG), and levodopa–entacapone–carbidopa intestinal gel (LECIG). Persistence and associated factors were examined using Kaplan–Meier and Cox regression analyses. Among 565 patients receiving 591 DATs, 170 (28.8

    2026
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    2NewCladribine in Data-Driven Secondary Progressive Multiple Sclerosis: an Italian Registry Analysis
    Tommaso Guerra,Massimiliano Copetti,Giuseppe Lucisano,Francesco Patti,Clara Grazia Chisari,Emilio Portaccio,Matteo Foschi,Diana Ferraro,Roberta Fantozzi,Giorgia Teresa Maniscalco,Giuseppe Salemi,Carla Tortorella,
    2026
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    3NewLong-term Outcomes and Predictors of Response to Rituximab in Anti-Mag Neuropathy: a Multicenter Real-World Study
    Emilien Delmont,Pascal Cintas,Céline Tard,José Boucraut,Laurent Magy,Simon Frachet,Céline Labeyrie,Andoni Echaniz-Laguna,Nicolas Noel,Juliette Svahn,Antoine Pegat,Françoise Bouhour,

    Rituximab (RTX) is the most widely used treatment for anti-myelin-associated glycoprotein (MAG) neuropathy, although its efficacy has not been demonstrated in controlled trials. Clarifying the optimal use of RTX in anti-MAG neuropathy remains clinically important. We conducted a multicenter retrospective study of 149 French patients treated with RTX monotherapy, focusing on long-term clinical outcomes and predictors of treatment response. Patients with anti-MAG antibody titers > 10,000 BTU were included. Clinical outcomes were assessed using the Overall Neuropathy Limitation Scale (ONLS) at baseline, 1 year, 2 years, and the last follow-up. Treatment success was defined as an improvement of ≥ 1 point in the ONLS score compared with baseline. The mean follow-up was 4 years (range 1–19 years). RTX treatment was associated with ONLS improvement in 60/146 patients (41

    2026
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    4NewUncovering Disability Progression in People with Multiple Sclerosis: a Practical Guide
    Bart Van Wijmeersch,Celia Oreja-Guevara,Melinda Magyari,Laura Airas,Andrew Chan,Florian Deisenhammer,Nikolaos Grigoriadis,Hans-Peter Hartung,Maura Pugliatti,Patrick Vermersch,Tjalf Ziemssen,Jelena Drulovic,

    People with multiple sclerosis (pwMS) often experience disability progression even when receiving disease-modifying therapies that effectively suppress relapses. This phenomenon is commonly referred to as progression independent of relapse activity (PIRA). Currently, it is challenging to characterize pwMS who are experiencing disability progression in clinical practice. Existing definitions of PIRA rely primarily on the Expanded Disability Status Scale (EDSS), which is biased towards ambulation and does not effectively capture the gradual accumulation of symptoms and signs across other clinical areas associated with disability progression. Here, we propose a step-by-step approach that neurologists can use in clinical practice to identify pwMS experiencing disability progression irrespective of age, EDSS, or disease duration. By using this approach, neurologists can assess the presence or absence of specific variables to help determine if disability is stable or progressing. This could enable appropriate treatments to be implemented in a timely manner, particularly as new disability-targeting therapies become available.

    2026
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    5NewSubtype and Stage Inference of Tract-Level Diffusion Abnormalities in Amyotrophic Lateral Sclerosis
    Yangyang Yin, Ye Tu, Baoyi Wang, Yu Chen,Yali Zhao,Jiahui Tang,Min Zhang, Jing Zhang

    Amyotrophic Lateral Sclerosis (ALS) shows substantial heterogeneity in clinical presentation and white matter involvement, but conventional diffusion tensor imaging analyses provide limited information on whether tract-level abnormalities follow distinct patient level patterns. This retrospective secondary analysis included 73 patients with ALS and 30 healthy controls. Fractional anisotropy (FA) and mean diffusivity (MD) from 11 predefined tracts were used as SuStaIn biomarkers, with healthy controls providing the normative reference for Z-score standardization. SuStaIn subtypes and stages were examined against Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), King’s stage, and peak width of skeletonized mean diffusivity (PSMD). The three-subtype model classified patients into cingulate callosal motor mixed subtype (n = 34), hippocampal cingulum posterior projection subtype (n = 32), and posterior callosal projection, MD predominant subtype (n = 7). Exploratory comparisons showed lower PSMD in subtype 0 than subtypes 1 and 2 (both p = 0.026), lower ALSFRS-R in subtype 2 than subtype 1 (p = 0.026), and higher SuStaIn stage in subtype 2 than subtype 0 (p = 0.025). Higher SuStaIn stage correlated with higher King’s stage (rho = 0.318, p = 0.006) and lower ALSFRS-R (rho = − 0.289, p = 0.013). After adjustment, higher SuStaIn stage remained associated with lower ALSFRS-R (β = − 0.333, p = 0.013) and more advanced King’s stage (odds ratio = 1.181, p < 0.001). Tract-level diffusion SuStaIn identified anatomically interpretable patterns of white matter involvement in ALS and provided patient-level stage estimates associated with clinical disease burden.

    2026
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    高被引作者

    作者引用发文
    Bradley F. Boeve40002374
    Jeffrey L. Cummings38990114
    Guy M. McKhann II3853335
    David A Drachman3753818
    Marshal Folstein372006
    David S. Knopman36725395
    Emanuel M Stadlan362232
    Robert Katzman362171
    Ludwig Kappos36105636
    Filippi Massimo35463709

    高产作者

    作者引用发文
    Comi Giancarlo28022924
    Filippi Massimo35463709
    Ludwig Kappos36105636
    Richard B. Lipton31822513
    Maria Assunta Rocca11346434
    David S. Knopman36725395
    Frederik Barkhof24698388
    Bradley F. Boeve40002374
    Bianca Weinstock-Guttman8291368
    Douglas Arnold8701368

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