Journal of the Peripheral Nervous System最新文献

{"title":"Rituximab With or Without Chemotherapy for Anti-MAG Neuropathy: A Retrospective Multicenter Real-World Study","authors":"Delmont Emilien,&nbsp;Cintas Pascal,&nbsp;Tard Céline,&nbsp;Boucraut José,&nbsp;Magy Laurent,&nbsp;Frachet Simon,&nbsp;Labeyrie Céline,&nbsp;Echaniz-Laguna Andoni,&nbsp;Noel Nicolas,&nbsp;Svahn Juliette,&nbsp;Pegat Antoine,&nbsp;Bouhour Françoise,&nbsp;Cluse Florent,&nbsp;Puma Angela,&nbsp;Magot Armelle,&nbsp;Farnault Laure,&nbsp;Aurran Schleinitz Thérèse,&nbsp;Gendre Thierry,&nbsp;Planté-Bordeneuve Violaine,&nbsp;Dormeuil Alice,&nbsp;Cassereau Julien,&nbsp;Chanson Jean-Baptiste,&nbsp;Mallaret Martial,&nbsp;Stojkovic Tanya,&nbsp;Noury Jean-Baptiste,&nbsp;Taieb Guillaume,&nbsp;Camdessanché Jean-Philippe,&nbsp;Poncet-Megemont Louis,&nbsp;Grapperon Aude-Marie,&nbsp;Fortanier Etienne,&nbsp;Attarian Shahram","doi":"10.1111/jns.70175","DOIUrl":"10.1111/jns.70175","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>In anti-myelin-associated glycoprotein (MAG) neuropathy, the efficacy of rituximab (RTX) is not demonstrated in randomized controlled trials. Combinations of RTX with conventional chemotherapy (immunochemotherapy, ICT) may provide potential benefit, but systematic evaluations are lacking. We aimed to compare the efficacy and safety of RTX and ICT in a multicenter retrospective real-world cohort.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients with anti-MAG antibody titers &gt; 10 000 Buhlmann titer units (BTUs) were included. Treatment response was defined as an improvement of ≥ 1 point in the Overall Neuropathy Limitation Scale (ONLS) compared to baseline. Propensity score matching was used to account for differences in baseline characteristics between treatment groups.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 230 patients were included: 150 received RTX and 80 received ICT. Mean follow-up was 4 years (range, 1–20 years). One year after treatment, clinical improvement occurred in 61/147 (41%) patients treated with RTX and 39/78 (50%) treated with ICT (OR = 1.4 [0.8–2.5] <i>p</i> = 0.3). At last follow-up, clinical improvement occurred in 55/150 (37%) patients treated with RTX and 31/80 (39%) treated with ICT (OR = 1.1 [0.6–1.9] <i>p</i> = 0.8). Propensity score matching yielded similar results. Among ICT regimens, dexamethasone–RTX–cyclophosphamide was associated with the highest response rate. RTX induction, 1 g days 1 and 15, seemed superior to four-weekly infusions. Adverse events were more frequent with ICT than with RTX (28% vs. 15%; OR = 2.20 [1.07–4.54], <i>p</i> = 0.02).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>In this cohort, adding chemotherapy to RTX was not associated with greater long-term improvement, despite a higher rate of adverse events. Prospective controlled studies are needed.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 4","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149081014","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response to “Guillain-Barré Syndrome in Chile: The Impact of Case Definitions on Incidence Estimates”","authors":"Gonzalo Rivera-Lillo,&nbsp;Rayén Galiá-Llaña,&nbsp;Matías Otto-Yáñez,&nbsp;Rodrigo Torres-Castro,&nbsp;Roberto Vera-Uribe","doi":"10.1111/jns.70171","DOIUrl":"10.1111/jns.70171","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 4","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149075130","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Guillain–Barré Syndrome in Chile: The Impact of Case Definitions on Incidence Estimates","authors":"Juan Francisco Idiáquez Rios,&nbsp;Rodrigo Salinas,&nbsp;Gabriel Cea","doi":"10.1111/jns.70161","DOIUrl":"10.1111/jns.70161","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 4","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149075153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiovascular Autonomic Neuropathy in Charcot–Marie–Tooth Disease: A Genotype-Stratified Study of PMP22 Duplication and GJB1 Mutation Carriers","authors":"Kryštof Prášil,&nbsp;Veronika Potočková,&nbsp;Tomáš Nedělka,&nbsp;Alžběta Prášilová,&nbsp;Radim Mazanec","doi":"10.1111/jns.70173","DOIUrl":"10.1111/jns.70173","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Data on cardiovascular autonomic neuropathy (CAN) are sparse in Charcot–Marie–Tooth disease. We aimed to determine the prevalence of CAN in CMT1A and CMTX1 and its associations with neuropathy severity and heart rate variability (HRV), compared with age- and sex-matched controls.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Thirty-six CMT1A, 24 CMTX1 patients, and 62 controls underwent cardiovascular autonomic testing including the modified Ewing battery (Expiration: Inspiration ratio, 30:15 ratio, Valsalva ratio; complete in 41/62 controls), orthostatic blood pressure measurement, and HRV analysis from 10-min supine–standing recordings. Abnormality was defined using age-stratified 2.3rd-percentile cutoffs. CMT Neuropathy Score version 2 (CMTNSv2) and Survey of Autonomic Symptoms were recorded. Comparisons used Fisher's exact test and the Kruskal–Wallis test with Holm correction; HRV was age- and sex-adjusted.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>CAN was present in 30.6% of CMT1A and 33.3% of CMTX1, versus 10.0% of controls (<i>p</i> = 0.011). Definite CAN occurred only in CMT1A (11.1%), although the CMT1A–CMTX1 contrast was not significant (<i>p</i> = 0.14). The Valsalva ratio was lower in CMT1A than in CMTX1 (median 1.54 vs. 1.89, <i>p</i> = 0.001), with abnormal values confined to CMT1A (14.7%). After age and sex adjustment, vagal HRV was reduced in CMT1A but not CMTX1.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>CAN affects approximately one-third of patients with CMT1A and CMTX1, roughly three times the control prevalence. Overall prevalence was similar in the two genotypes, but Valsalva impairment was confined to CMT1A, indicating that similar prevalence does not imply an identical pattern of involvement. Cardiovascular autonomic assessment warrants consideration in these patients, particularly perioperatively.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 4","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13616590/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149069729","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fampridine for Symptomatic Treatment in Chronic Inflammatory Demyelinating Polyneuropathy: A Randomized, Double-Blinded, Placebo-Controlled Crossover Study","authors":"Peter Nørregaard Hansen,&nbsp;Lars Markvardsen,&nbsp;Hatice Tankisi,&nbsp;Henning Andersen,&nbsp;Thomas Krøigård,&nbsp;Søren Sindrup","doi":"10.1111/jns.70172","DOIUrl":"https://doi.org/10.1111/jns.70172","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated neuropathy that may cause persistent disability despite immunoglobulin treatment. Ion channel dysfunction has been demonstrated in CIDP, but no therapies specifically target this mechanism. We investigated whether fampridine improves clinical function and electrophysiological measures in CIDP patients with residual symptoms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this two-center, randomized, double-blind, placebo-controlled crossover trial, patients with CIDP receiving stable subcutaneous or intravenous immunoglobulin were assigned to 4 weeks of fampridine (10 mg twice daily) or placebo, separated by a washout period. Co-primary outcomes were changes in Six-Spot-Step Test (SSST) and Nine-Hole Peg Test (9-HPT). Secondary outcomes included clinical scores, patient-reported outcomes, nerve conduction studies, and nerve excitability testing.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-eight patients were included in the study. No significant differences were observed between fampridine and placebo for SSST (<i>p</i> = 0.154) or 9-HPT (<i>p</i> = 0.857). Grip strength and R-ODS worsened during fampridine compared with placebo (Bonferroni-adjusted <i>p</i> = 0.007 for both), while other clinical outcomes showed no differences. Median nerve CMAP amplitude with proximal stimulation increased significantly during fampridine treatment (adjusted <i>p</i> = 0.040). No significant changes were observed in other electrophysiological variables, including nerve excitability testing. No serious adverse events occurred and adverse effects were predominantly mild.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our study did not provide evidence that fampridine improves clinical function in CIDP patients with residual disability during stable immunoglobulin therapy. Targeting ion channel dysfunction in CIDP remains of interest, but alternative strategies may be required.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 4","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70172","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149029213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Newly Developing IgM Anti-GD1a/GD1b Ganglioside IgM Monoclonal Gammopathy in a Patient With Chronic Inflammatory Demyelinating Polyradiculoneuropathy","authors":"Ruby F. Ross,&nbsp;Jennifer A. Langsdorf,&nbsp;Norman Latov","doi":"10.1111/jns.70174","DOIUrl":"https://doi.org/10.1111/jns.70174","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Chronic inflammatory demyelinating neuropathy (CIDP) is a rare, immune-mediated neuropathy that is distinct from other immune-mediated, paraneoplastic, and paraproteinemic neuropathies that may have overlapping clinical features. Identification of the specific diagnosis in each patient is important as each of these disorders has distinct pathophysiology and treatment response.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Report</h3>\u0000 \u0000 <p>Here, we present an atypical case of CIDP in which the patient developed an IgM monoclonal gammopathy with positive anti-ganglioside antibodies over 20 years into his disease course.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Patients with CIDP may be predisposed to develop IgM autoantibodies or monoclonal gammopathies. If a patient with CIDP experiences clinical worsening while on maintenance therapy, it may be helpful to test for anti-ganglioside antibodies and monoclonal gammopathies and reassess if alternative treatment would be useful.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 4","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149029211","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Abstract","authors":"","doi":"10.1111/jns.70152","DOIUrl":"https://doi.org/10.1111/jns.70152","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 S2","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148974951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complement-Regulated Nodal Vulnerability in Guillain–Barré Syndrome and CIDP","authors":"Nobuhiro Yuki","doi":"10.1111/jns.70165","DOIUrl":"10.1111/jns.70165","url":null,"abstract":"<div>\u0000 \u0000 <p>The immunobiology of Guillain–Barré syndrome (GBS) has long been organized around a dichotomy: acute motor axonal neuropathy (AMAN) is an antibody-mediated nodal disorder, whereas acute inflammatory demyelinating polyneuropathy (AIDP) has been interpreted mainly through T-cell–mediated models of compact-myelin injury. Four successive findings challenge this separation. Intraneural injection of GBS sera produced demyelination without transfer of immune cells; pathological studies in AIDP localized complement activation to the Schwann-cell surface before macrophage-associated myelin stripping. Serum IgG from a substantial subset of patients with AIDP bound nodal or paranodal surface domains; and identification of gelsolin-3 defined an AIDP subset in which patient IgG, together with active complement, produced nodal disruption before internodal demyelination. Peripheral-myelin-reactive T cells further indicate that T-cell–mediated and antibody-mediated immunity may coexist. Within this evidence hierarchy, AMAN and a subset of AIDP have distinct initiating targets but converge on nodal dysfunction. CD55 and CD59 are membrane-bound complement regulators: CD55 limits complement amplification, whereas CD59 prevents assembly of the membrane attack complex. Both are detectable in compact myelin but not at human nodes of Ranvier, revealing a localized discontinuity in complement control. This local absence of complement regulators may influence whether antibody binding progresses to conduction failure, axonal degeneration, or internodal demyelination, but should be regarded as a permissive substrate for injury rather than as evidence of lesion localization. Observations in chronic inflammatory demyelinating polyneuropathy (CIDP) suggest that antibody-mediated functional injury may precede structural demyelination. This Review proposes complement-regulated nodal vulnerability to integrate evidence across AMAN and subsets of AIDP and CIDP while preserving differences in targets, tempo, and mechanistic strength.</p>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148880796","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"High Resolution 3-T Magnetic Resonance Tractography Cannot Localise Conduction Block in Multifocal Motor Neuropathy With Conduction Block.","authors":"Ryan Y S Keh, Laura Zambreanu, Stephen Wastling, Carolynne M Doherty, Hamza A Salhab, Chandrashekar Hoskote, Sachit Shah, Julian Blake, Aisling S Carr, John Thornton, Jasper M Morrow, Michael P Lunn","doi":"10.1111/jns.70164","DOIUrl":"10.1111/jns.70164","url":null,"abstract":"<p><strong>Background and aims: </strong>Definite diagnosis of multifocal motor neuropathy with conduction block (MMNCB) requires identification of electrophysiological motor conduction block (CB), which is sometimes challenging to demonstrate. Magnetic resonance (MR) neurography is recommended as a supportive diagnostic criterion. Brachial plexus short tau inversion recovery (STIR) hyperintensity is described in MMNCB but the utility of tractography (DTI) is less well understood. We sought evidence for the utility of DTI and STIR parameters of MMNCB forearm nerve segments with clear NCS-demonstrated CB, which could be applied to less accessible nerves.</p><p><strong>Methods: </strong>Ten MMNCB individuals with definite CB and 10 healthy controls underwent dedicated forearm 3-T MRI with DTI and STIR. Axial, radial and mean diffusivity (AD, RD and MD) and fractional anisotropy (FA) were calculated. DTI profiles were visually inspected for possible abnormalities, and pooled values of individual diffusion parameters were analysed for group differences. STIR sequences were reviewed for areas of hyperintensity. MRI and defined NCS abnormalities were correlated.</p><p><strong>Results: </strong>Composite nerve segment profile inspection showed occasional patterns (elevated AD/MD/RD, reduced FA) in regions of CB, but indistinguishable changes were also seen in MMNCB nerves without CB and controls (sensitivity 28.6%-42.9%, positive predictive value 44.4%-54.5%). Pooled diffusion parameter data identified no statistically significant group differences. Some MMNCB nerves demonstrated focal STIR hyperintensity poorly correlating with CB.</p><p><strong>Interpretation: </strong>Existing DTI and STIR techniques cannot identify useful characteristics of MMNCB pathology in forearm nerves. Whilst statistically significant group differences can be identified, inspection of individual images cannot identify consistently found abnormalities, and the differences are too small to be clinically useful.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":"e70164"},"PeriodicalIF":3.0,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13572773/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"From Discrimination to Clinical Utility: Next Steps for the Erasmus Polyneuropathy Symptom Score.","authors":"Yasin Taşkın","doi":"10.1111/jns.70168","DOIUrl":"https://doi.org/10.1111/jns.70168","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":"e70168"},"PeriodicalIF":3.0,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012631","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}