Journal of the Peripheral Nervous System最新文献

{"title":"Behavioral Thermoceptive Responses and Morphologic Correlates in Mouse Models of CMT1A, HNPP, and Aging","authors":"Vaibhav Oberoi,&nbsp;James O. Campbell,&nbsp;Nelson Akpabli-Tsigbe,&nbsp;Fatima Imran,&nbsp;Silvano Bond,&nbsp;Sara Ngo Tenlep,&nbsp;Charles D. Brennan,&nbsp;Meifang Wang,&nbsp;Smita Saxena,&nbsp;Kathryn R. Moss,&nbsp;De-Pei Li,&nbsp;W. David Arnold,&nbsp;Ryan Castoro","doi":"10.1111/jns.70113","DOIUrl":"10.1111/jns.70113","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Thermoceptive dysfunction is a frequent but understudied feature of peripheral neuropathies and aging. Patients often report abnormal heat perception, yet the underlying sensory mechanisms remain unclear. This study evaluated thermoceptive behavior and corresponding structural changes in mouse models of inherited dysmyelinating neuropathy and natural aging to identify shared and divergent mechanisms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Thermal preference was assessed using a user-independent gradient apparatus spanning physiological to noxious temperatures, with automated quantification of time in zone, distance traveled, and velocity. Nocifensive responses were evaluated by hot plate latency. Intraepidermal nerve fiber density (IENFD) was measured in paw pads, and TRPV1-positive dorsal root ganglion (DRG) neurons were analyzed by immunofluorescence and confocal imaging.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Thermal gradient testing revealed preserved temperature preference in CMT1A and HNPP mice but significantly altered behavior in aged animals, which spent less time in warmer zones. Hot plate testing showed prolonged times to nocifensive behavior in aged and CMT1A mice, whereas HNPP mice exhibited variable responses. IENFD was markedly reduced in aged mice but preserved in CMT1A and HNPP. DRG analysis revealed smaller soma diameters and reduced proportions of TRPV1-positive Aδ neurons in aged mice, while CMT1A animals maintained normal morphology.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Aging produces thermoceptive deficits through axonal degeneration and selective Aδ-fiber vulnerability, whereas CMT1A mice display conduction-related impairment due to dysmyelination. Both models reproduce key human sensory phenotypes and provide translational platforms for studying small-fiber dysfunction and therapeutic interventions in peripheral neuropathies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147377969","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":"Cranial Nerve Involvement With Diplopia as Presenting Feature of CMT1H Caused by Recurring FBLN5 Variant.","authors":"Georgios Koutsis, Zoi Kontogeorgiou, Charalampos Tzempetzis, Nikolaos Ragazos, Evgenia Efthymiou, Chrisoula Kartanou, Chrysoula Koniari, Nikolaos Giagkou, Klio Chatzistefanou, Georgios Velonakis, Georgia Karadima, Vasiliki Zouvelou","doi":"10.1111/jns.70115","DOIUrl":"10.1111/jns.70115","url":null,"abstract":"<p><strong>Background and aims: </strong>CMT1H is a rare, autosomal dominant, demyelinating subtype of CMT caused by variants in FBLN5. Symptomatic cranial nerve involvement has never been reported in patients with CMT1H.</p><p><strong>Case report: </strong>We report a 45-year-old woman with a history of long-standing diplopia. On examination, she had bilateral limitation of eye abduction, with double vision in all directions of gaze, particularly horizontally. She was unable to heel-walk, had mild lower limb distal weakness, decreased tendon reflexes with absent Achilles reflexes, reduced distal vibration sense, pes cavus, and hammertoes. A dominant family history was noted. Brain MRI revealed bilateral contrast enhancement and thickening of cranial nerves III through XII. Genetic testing with whole exome sequencing revealed a known, recurring, heterozygous, likely pathogenic missense variant in FBLN5 [c.1117C>T; p. (Arg373Cys)], consistent with a diagnosis of CMT1H.</p><p><strong>Interpretation: </strong>This is the first reported case of FBLN5-related CMT1H with symptomatic cranial nerve involvement, expanding the known phenotypic spectrum of the disease.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":"e70115"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12960836/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147355472","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":"A New Simple SCreening Tool for Painful Diabetic Neuropathy (ACT) in Ecuador.","authors":"Hoda Gad, María Cristina Moreno, Angel Pluas, María Belen Tello, Maribel Burgos, Karla Diaz, Estefanía Icaza, Mabel Sanchez, Mariella Vecchionacce, Rayaz A Malik, Carlos Solis","doi":"10.1111/jns.70118","DOIUrl":"10.1111/jns.70118","url":null,"abstract":"<p><strong>Background: </strong>Diabetic painful neuropathy is a cause of significant disability and sleep disturbance but may be undiagnosed in up to 80% of patients with diabetes. Various neuropathic pain (NP) questionnaires have good diagnostic utility but are impractical in resource-constrained and busy clinical settings. A simple sCreening Tool (ACT) was developed as a concise, patient-led tool to rapidly detect NP. This study evaluates the screening validity and psychometric properties of ACT compared with the DN4 questionnaire (reference standard).</p><p><strong>Methods: </strong>We conducted an observational study employing clinic-based recruitment of patients with diabetes at a tertiary outpatient clinic in Guayaquil, Ecuador. All enrolled participants completed the ACT, DN4, and MNSI questionnaires during the study visit. MNSI was administered as part of the study to confirm the diagnosis of DPN using predefined cut-offs (MNSI-q ≥ 4 and MNSI-e ≥ 2.5). Time to completion, inter-item consistency, sensitivity, specificity, Youden's index, and the area under the receiver operating characteristic (ROC) curve were calculated for ACT using DN4 ≥ 4 as the criterion for NP.</p><p><strong>Results: </strong>Of the 300 participants (median age 63 years, diabetes duration 10 years), 65 (21.7%) had NP per DN4. Total ACTq+e (questionnaire+examination) yielded a cut-off score of ≥ 6 items as optimal, with area under the ROC curve 0.836, sensitivity 80.0% and specificity 75.7% for painful DPN. ACT demonstrated moderate internal consistency (Cronbach's alpha = 0.65) and was well received by patients and clinicians on face validity. The completion time for ACT was comparable to that of DN4.</p><p><strong>Conclusion: </strong>The ACT tool is a rapid screening tool for identifying neuropathic pain in the busy clinic, with acceptable sensitivity and specificity compared to DN4. The brevity and ease of patient self-administration of ACT make it promising for integration into busy outpatient workflows to improve the diagnosis of painful DPN.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":"e70118"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12999269/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147480906","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":"SOX5 Overexpression in Schwann Cell Combined With Chitosan-Based Conduit Promotes Peripheral Nerve Regeneration.","authors":"Bo Ma, Fengshi Zhang, Junyu Su, Baixin Gu, Lingxin Duan, Wenjing Zhang, Yuhui Kou","doi":"10.1111/jns.70120","DOIUrl":"10.1111/jns.70120","url":null,"abstract":"<p><strong>Background and aims: </strong>Severe peripheral nerve injury (PNI) remains a major clinical challenge, and functional recovery after conventional neurorrhaphy is often unsatisfactory due to fascicular mismatch, suture tension, and limited Schwann cell viability. To address these limitations, we previously developed a small-gap chitosan-based conduit that provides a controlled microenvironment for regenerative interventions. This study aimed to investigate whether SOX5 overexpression enhances Schwann cell regenerative potential and, when combined with this conduit, synergistically promotes peripheral nerve regeneration.</p><p><strong>Methods: </strong>Schwann cells were transduced with SOX5 lentivirus and assessed for proliferation, migration, and neurotrophic factor secretion in vitro. In a rat sciatic nerve transection model (2-mm gap), animals received a chitosan conduit with intraluminal injection of SOX5 lentivirus. Histological, electrophysiological, and behavioral assessments were conducted at 12 weeks post-surgery.</p><p><strong>Results: </strong>SOX5 overexpression significantly enhanced Schwann cell proliferation, migration, and secretion of BDNF, NGF, CNTF, and VEGF, while maintaining the dedifferentiated repair phenotype. In vivo, the combination of SOX5 lentivirus and chitosan conduit improved axonal regeneration, reduced muscle atrophy, and increased conduction velocity and locomotor recovery relative to the empty conduit group.</p><p><strong>Interpretation: </strong>Lentivirus-mediated SOX5 overexpression drives Schwann cells toward a repair phenotype and, when integrated with a small-gap chitosan-based conduit, effectively promotes structural and functional nerve regeneration.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":"e70120"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147504118","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":"Motor Neuronopathy With Widespread Fasciculations in MCM3AP-Related Disorder: Clinical and Muscle MRI Insights.","authors":"Ana Flávia Andrade Lemos, Rodrigo Siqueira Soares Frezatti, Antônio Carlos Dos Santos, Pedro José Tomaselli, Wilson Marques","doi":"10.1111/jns.70112","DOIUrl":"10.1111/jns.70112","url":null,"abstract":"<p><strong>Background: </strong>Biallelic pathogenic variants in MCM3AP, encoding the germinal center-associated nuclear protein (GANP), have been linked to autosomal recessive peripheral neuropathies variably accompanied by cognitive impairment and multisystem involvement. To date, anterior horn cell involvement has not been documented in association with MCM3AP-related disorders.</p><p><strong>Objective: </strong>To describe a patient with biallelic MCM3AP variants presenting with a motor neuronopathy phenotype and to provide the first whole-body muscle MRI characterization associated with this gene.</p><p><strong>Methods and results: </strong>A 53-year-old woman born to non-consanguineous parents presented with early-onset motor neuronopathy and lifelong learning difficulties. Neurological examination revealed generalized areflexia and widespread fasciculations without sensory abnormalities. Electroneuromyography demonstrated diffuse mixed acute-on-chronic denervation process. Whole-body muscle MRI showed a selective non-length-dependent pattern of fatty infiltration. Whole-exome sequencing identified two likely pathogenic heterozygous variants in the MCM3AP gene.</p><p><strong>Standard protocol approvals, registrations, and patient consents: </strong>According to the policies of our institution, single-patient case reports do not require review or approval by the institutional ethics committee. Written informed consent for participation and for publication of clinical information, photographs, electrophysiological data, and muscle MRI images was obtained from the patient. No clinical trial registration was applicable.</p><p><strong>Conclusion: </strong>This case extends the phenotypic spectrum of MCM3AP-related disorders to include a slowly progressive, non-syndromic motor neuronopathy with electrophysiological evidence of active denervation and distinctive MRI findings. These observations highlight the hidden boundaries between hereditary motor neuropathies and anterior horn cell diseases, emphasizing the need for integrated clinical, neurophysiological, and genetic evaluation.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":"e70112"},"PeriodicalIF":3.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12981946/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147444379","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":"Trajectory of Recovery and Burden of Residual Symptoms Following Guillain–Barré Syndrome: A Single-Center Prospective Cohort Study From Sri Lanka","authors":"Bimsara Senanayake,&nbsp;Arun Rajaratnam,&nbsp;Thilani Thennakoon,&nbsp;Nimasha Ekanayake,&nbsp;Pramith Ruwanpathirana","doi":"10.1111/jns.70105","DOIUrl":"10.1111/jns.70105","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Guillain–Barré syndrome (GBS) is classically a monophasic illness. In resource-limited settings, patients with GBS are not routinely followed up. However, these patients may suffer from residual symptoms and disability. In this study, we assessed the trajectory of recovery and burden of residual symptoms in patients who developed GBS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This prospective cohort study was conducted at the National Hospital of Sri Lanka from January 1, 2020 to April 1, 2025. We assessed the patients during the illness and then at 1, 3, and 6 months after discharge. The primary outcome was the GBS Disability Score (0 [<i>no disability</i>]–6 [<i>death</i>]), and the secondary outcomes were the individual residual symptoms. All patients with GBS were included. The serial improvement of the GBS Disability Score was assessed using the generalized equation estimate. The burden of residual symptoms was given as percentages.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We included 144 patients with GBS (mean age 46.2 ± 17.3 years; male:female 0.92), and 67 completed the follow-up. There was no in-hospital mortality; one patient (1.5%) died within 6 months. The disability improved significantly, with only 5.9% unable to walk independently at 6 months. Increasing age and axonal variants (AMAN/AMSAN, 37%) were independent negative predictors of recovery. The residual symptoms at 6 months included positive sensory symptoms (42.6%), fatigue (30.9%), and subjective limb weakness (29.4%). Forty-eight percent had to make some change in their occupation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Although many patients achieve motor recovery, there is a high burden of residual symptoms at 6 months in patients with GBS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147276398","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":"Climatic and Geographic Variations of Antecedent Infections in Guillain–Barré Syndrome: A Long-Term Longitudinal Analysis From Bangladesh","authors":"Mantaka Rahman,&nbsp;Nowshin Papri,&nbsp;Asif Mohammed,&nbsp;Imran Hasan,&nbsp;Sharmin Sultana,&nbsp;Israt Jahan,&nbsp;Shoma Hayat,&nbsp;Zhahirul Islam","doi":"10.1111/jns.70111","DOIUrl":"10.1111/jns.70111","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Guillain–Barré syndrome (GBS) is an acute post-infectious autoimmune neuropathy, frequently triggered by antecedent infections. While climate influences the seasonality and distribution of infections, its role in triggering GBS remains underexplored. We aimed to evaluate the impact of climatic and geographical variations on infections preceding GBS in Bangladesh.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Five hundred and sixty-six GBS patients were enrolled from four prospective cohorts (2010–2024). Antecedent events were recorded within 4 weeks of weakness onset. Meteorological data were retrieved from the World Bank's Climate Change Knowledge Portal (2010–2020) and Bangladesh Meteorological Department (2021–2024). Patients were categorized by regions (northern, central, southwestern, and eastern), seasons (summer, autumn, winter, spring). Analysis were performed in R using chi-square, <i>t</i>-tests, Kaplan–Meier, and multivariable logistic regression.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 566 patients (69% male, median age: 31 years). Antecedent events were reported in 70%, predominantly GE (46%) and URTI (19%). The axonal subtype predominated (64%), with 31% receiving intravenous immunoglobulin or plasma exchange. GE was associated with higher disability (GBS-DS = 4, 61% vs. 46%, <i>p = 0.014</i>) and cranial nerve involvement (57% vs. 43%, <i>p</i> = 0.017) compared to URTI. GE peaked during spring (31%) and URTI in winter (35%, <i>p</i> = 0.009), with no subtype-season association after adjustment (<i>p</i> = 0.180). GE occurred more at higher mean temperatures (26.06°C vs. 25.05°C, <i>p</i> = 0.048) and at higher precipitation (162.65 mm vs. 105.04 mm, <i>p</i> = 0.0001). GE cases correlated positively, while URTI inversely, with temperature (<i>r</i>_<i>s</i> = 0.185) and precipitation (<i>r</i>_<i>s</i> = 0.053).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretations</h3>\u0000 \u0000 <p>Warmer temperature and higher precipitation, particularly spring and summer, favor GE-related GBS. Further studies are warranted to explore climate-sensitive infectious triggers of GBS in Bangladesh.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147276442","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":"Austin J. Sumner: In Memoriam","authors":"Richard A. Lewis,&nbsp;David R. Cornblath","doi":"10.1111/jns.70110","DOIUrl":"https://doi.org/10.1111/jns.70110","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147320889","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":"Pathological Features of Vasculitic Neuropathy and the Role of Nerve Biopsy","authors":"Nan Hu,&nbsp;Jia Li,&nbsp;Hongzhi Guan,&nbsp;Yanhuan Zhao,&nbsp;Haitao Ren,&nbsp;Yuzhou Guan,&nbsp;Mingsheng Liu,&nbsp;Min Qian,&nbsp;Lin Chen","doi":"10.1111/jns.70109","DOIUrl":"10.1111/jns.70109","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To compare the pathological features of patients with different forms of vasculitic neuropathy (VN).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients with clinically probable VN were enrolled. Clinical characteristics and ancillary examinations were collected and evaluated. Nerve biopsies were performed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 48 patients with VN were involved, including 20 primary systemic VN (PSVN), 17 secondary systemic VN (SSVN), and 11 non-systemic VN (NSVN). Patients that fulfilled the pathologically definite, probable, and possible VN were 20 (41.67%), 8 (16.67%), and 12 (25.00%), respectively. The frequencies of acute vascular damage showed no significant difference across three subgroups (PSVN 80.00%, SSVN 82.35%, NSVN 90.91%). Chronic vascular damage was more frequently observed in PSVN (90.00%) and SSVN (76.47%) than in NSVN (54.55%) with no significance. Perivascular inflammatory cell infiltration in the endoneurium was more common in NSVN (45.45%) than PSVN (15.00%, <i>p</i> = 0.004) and none with SSVN (0.00%, <i>p</i> = 0.002).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The overall rate of pathologically definite and probable VN in nerve biopsy was 58%. Acute vascular lesion is commonly seen in all forms of VN, while chronic vascular damage is more frequently observed in SVN. Perivascular inflammatory cell infiltration in the epineurium is primarily found in NSVN.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>The study further elucidated the clinical significance of nerve biopsy in VN.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146202100","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":"Elucidating Causal Associations Between Immune Cells, Circulating Inflammatory Proteins, and Chronic Inflammatory Demyelinating Polyneuropathy: A Two-Sample Two-Step Mendelian Randomization Study","authors":"Ann-Ching Wang,&nbsp;Meng-Chang Lee,&nbsp;Hueng-Chuen Fan,&nbsp;Ming-Hua Wang,&nbsp;Chung-Hsing Chou,&nbsp;Pei-Yi Wu","doi":"10.1111/jns.70108","DOIUrl":"10.1111/jns.70108","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Immune cells and circulating inflammatory proteins play crucial roles in chronic inflammatory demyelinating polyneuropathy (CIDP) pathogenesis. However, the causal associations between these factors and CIDP remain unclear. Herein, we aimed to explore these associations using a two-sample, two-step Mendelian randomization (MR) approach.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Two-sample MR analysis was conducted using genome-wide association studies data to assess links between immune cells, inflammatory proteins, and CIDP. Data on 731 immune cell traits were obtained from a cohort of 3757 Sardinians, 91 inflammatory proteins from 14 824 Europeans across 11 cohorts, and CIDP data from 456 348 Europeans in the UK Biobank. Fixed-effect inverse variance weighted and Bayesian-weighted MR methods were used, along with a two-step MR to assess mediation effects. Sensitivity analyses were performed to check for horizontal pleiotropy and heterogeneity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-five immune cell traits were found to be significantly associated with CIDP. Of these, 17 immunophenotypes increased CIDP risk, whereas 8 were protective. Among inflammatory proteins, cystatin D (CST5) and interleukin-18 (IL-18) were linked to CIDP risk. Although not statistically significant, CST5 appeared to partially mediate the association between CD8+ NKT %T cells and CIDP (OR, 1.006; 95% CI, 1.00–1.02), accounting for approximately 3% of the mediation effect.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our findings highlight several novel immune cell and inflammatory protein interactions implicated in CIDP. These results present new immune targets for future CIDP therapies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146157647","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}