Journal of the Peripheral Nervous System最新文献

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Hypoxia-Induced Lactylation of NSUN3 Promotes m5C Methylation of SETD2 to Regulate Peripheral Nerve Injury Repair. 缺氧诱导的NSUN3乳酸化促进SETD2的m5C甲基化,调节周围神经损伤修复。
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-09-01 DOI: 10.1111/jns.70166
Shuai An, Jingfei Shi, Jiang Huang, Zheng Li, Jingbo Cheng, Mingli Feng
{"title":"Hypoxia-Induced Lactylation of NSUN3 Promotes m5C Methylation of SETD2 to Regulate Peripheral Nerve Injury Repair.","authors":"Shuai An, Jingfei Shi, Jiang Huang, Zheng Li, Jingbo Cheng, Mingli Feng","doi":"10.1111/jns.70166","DOIUrl":"10.1111/jns.70166","url":null,"abstract":"<p><strong>Background: </strong>Peripheral nerve injury (PNI) repair remains challenging, with metabolic regulation and neuronal senescence playing critical roles. This study explored whether intermittent hypoxia modulates injured dorsal root ganglion (DRG) neuron regeneration via lactate-mediated mechanisms.</p><p><strong>Methods: </strong>DRG neurons were subjected to impairment via acrylamide (ACR) to develop an in vitro injury model, followed by the induction of intermittent hypoxia and lactate production inhibitors including 2-deoxy-d-glucose (2-DG) and oxamate. In vivo experiments employed a rat PNI model exposed to intermittent hypoxia. Cellular evaluations comprised the CCK-8 assay to determine cell viability, β-galactosidase staining to identify cellular senescence, and immunofluorescence for the detection of p21 and Lamin B1. The m5C-RIP-qPCR assay was used to assess m5C modification of SETD2 mRNA. The immunoprecipitation was applied to examine the lactylation of NSUN3 protein.</p><p><strong>Results: </strong>Intermittent hypoxia increased lactate production, improved ACR-impaired DRG viability, reduced ROS and MDA levels, and promoted damaged neuron regeneration, effects reversed by 2-DG or oxamate. Hypoxia inhibited ACR-induced neuron senescence, which was abrogated by lactate inhibitors. Mechanistically, hypoxia-induced lactate promoted NSUN3 lactylation, further enhanced m5C modification and stability of SETD2 mRNA. NSUN3 knockdown exacerbated senescence and suppressed mitochondrial biogenesis, while SETD2 overexpression reversed these impacts.</p><p><strong>Conclusion: </strong>Intermittent hypoxia facilitated the regeneration of injured DRG neurons through lactate-mediated NSUN3 lactylation. This process was correlated with the stability of SETD2 mRNA via m5C modification, thereby mitigating cellular senescence and promoting mitochondrial function. These findings present novel targets for strategies aimed at nerve repair.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":"e70166"},"PeriodicalIF":3.0,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13527880/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865383","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}
引用次数: 0
Correction to "Epidemiology, Presentation, Management and Outcomes in Chronic Inflammatory Demyelinating Polyneuropathy in Birmingham, UK: The Impact of Ethnicity". 更正“英国伯明翰慢性炎症性脱髓鞘性多神经病变的流行病学、表现、管理和结果:种族的影响”。
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-09-01 DOI: 10.1111/jns.70169
{"title":"Correction to \"Epidemiology, Presentation, Management and Outcomes in Chronic Inflammatory Demyelinating Polyneuropathy in Birmingham, UK: The Impact of Ethnicity\".","authors":"","doi":"10.1111/jns.70169","DOIUrl":"https://doi.org/10.1111/jns.70169","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":"e70169"},"PeriodicalIF":3.0,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149016399","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}
引用次数: 0
Impact of Baseline Polyneuropathy Severity on Eplontersen Efficacy in the NEURO-TTRansform Clinical Trial. 在neurot - transform临床试验中,基线多发性神经病严重程度对Eplontersen疗效的影响。
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-09-01 DOI: 10.1111/jns.70167
Thomas H Brannagan, Markus Weiler, Márcia Waddington Cruz, Sami Khella, John L Berk, Morie Gertz, Ahmad Masri, Mathew S Maurer, Jonas Wixner, Jonatan Nåtman, T Jesse Kwoh, Maksym Pola, Laura Obici
{"title":"Impact of Baseline Polyneuropathy Severity on Eplontersen Efficacy in the NEURO-TTRansform Clinical Trial.","authors":"Thomas H Brannagan, Markus Weiler, Márcia Waddington Cruz, Sami Khella, John L Berk, Morie Gertz, Ahmad Masri, Mathew S Maurer, Jonas Wixner, Jonatan Nåtman, T Jesse Kwoh, Maksym Pola, Laura Obici","doi":"10.1111/jns.70167","DOIUrl":"10.1111/jns.70167","url":null,"abstract":"<p><strong>Background and aims: </strong>In the NEURO-TTRansform clinical trial (NCT04136184), eplontersen improved neuropathy impairment and quality of life (QoL) through Week 66 versus the NEURO-TTR historical placebo in patients with hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN). This analysis assessed the impact of baseline ATTRv-PN severity on eplontersen response in patients from NEURO-TTRansform.</p><p><strong>Methods: </strong>This post hoc analysis grouped patients into tertiles by baseline Neuropathy Impairment Score (NIS): T1 (least baseline impairment: 3.5 to < 27.5; n = 67), T2 (27.5 to < 55.0; n = 67), and T3 (55.0 to < 127.8; n = 66). Outcomes assessed were neuropathy impairment (modified NIS+7 [mNIS+7] and NIS, Neuropathy Symptom and Change, Polyneuropathy Disability), QoL (Norfolk QoL-Diabetic Neuropathy), physical functioning (36-Item Short-Form Health Survey Physical Component Summary), nutritional status (modified body mass index), and serum transthyretin levels; these were compared with NEURO-TTR historical placebo. Autonomic dysfunction (Composite Autonomic Symptom Score-31), disability (Rasch-built Overall Disability Scale), and walking speed (10-Meter Walk Test) were also assessed. Final assessments were carried out following 65/66 or 81/85 weeks of treatment.</p><p><strong>Results: </strong>Mean mNIS+7 composite scores were maintained over 85 weeks with eplontersen (changes from baseline of -4.5 [T1], -1.3 [T2], and -2.6 [T3] points). Other disease parameter scores were similarly maintained or improved with eplontersen. Patients receiving placebo experienced disease worsening across outcomes. T1 mean disease scores were typically better than in T2 and T3.</p><p><strong>Interpretation: </strong>Consistent, sustained benefits of eplontersen were observed regardless of baseline ATTRv-PN severity. These findings strengthen the importance of early treatment initiation for patients with ATTRv-PN across the disease spectrum.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":"e70167"},"PeriodicalIF":3.0,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13545155/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891971","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}
引用次数: 0
Reply to the Letter to the Editor "From Discrimination to Clinical Utility: Next Steps for the Erasmus Polyneuropathy Symptom Score". 回复编辑“从歧视到临床应用:伊拉斯谟多发性神经病变症状评分的下一步”。
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-09-01 DOI: 10.1111/jns.70170
Noor E Taams, Merel Huijg, Pieter A van Doorn
{"title":"Reply to the Letter to the Editor \"From Discrimination to Clinical Utility: Next Steps for the Erasmus Polyneuropathy Symptom Score\".","authors":"Noor E Taams, Merel Huijg, Pieter A van Doorn","doi":"10.1111/jns.70170","DOIUrl":"https://doi.org/10.1111/jns.70170","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":"e70170"},"PeriodicalIF":3.0,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012562","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}
引用次数: 0
Neuropathy in Val122Ile Hereditary Transthyretin (ATTR) Amyloidosis: A Multicenter Retrospective Cohort Study Val122Ile遗传性甲状腺素转移(ATTR)淀粉样变性的神经病变:一项多中心回顾性队列研究
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-08-28 DOI: 10.1111/jns.70160
Anna Paula Paranhos, Marcela Câmara Machado Costa, Carolina Lavigne Moreira, Diogo Fernandes dos Santos, Marcondes Cavalcante França, Renan Flavio de França Nunes, Osvaldo José Moreira do Nascimento, Jemima Araujo da Silva Batista, Eduardo Boiteux Uchôa Cavalcanti, Cleonisio Leite Rodrigues, Ândrea Virgínia Ferreira Chaves, Marcelo Soares Kerstenetzky, Lara Albuquerque de Brito, Carlo Domênico Marrone, Marcus Vinicius Magno Gonçalves, Pedro José Tomaselli, Wilson Marques
{"title":"Neuropathy in Val122Ile Hereditary Transthyretin (ATTR) Amyloidosis: A Multicenter Retrospective Cohort Study","authors":"Anna Paula Paranhos,&nbsp;Marcela Câmara Machado Costa,&nbsp;Carolina Lavigne Moreira,&nbsp;Diogo Fernandes dos Santos,&nbsp;Marcondes Cavalcante França,&nbsp;Renan Flavio de França Nunes,&nbsp;Osvaldo José Moreira do Nascimento,&nbsp;Jemima Araujo da Silva Batista,&nbsp;Eduardo Boiteux Uchôa Cavalcanti,&nbsp;Cleonisio Leite Rodrigues,&nbsp;Ândrea Virgínia Ferreira Chaves,&nbsp;Marcelo Soares Kerstenetzky,&nbsp;Lara Albuquerque de Brito,&nbsp;Carlo Domênico Marrone,&nbsp;Marcus Vinicius Magno Gonçalves,&nbsp;Pedro José Tomaselli,&nbsp;Wilson Marques","doi":"10.1111/jns.70160","DOIUrl":"10.1111/jns.70160","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>The Val122Ile ATTR Amyloidosis has traditionally been linked to cardiac manifestations. Recent studies suggest that neuropathy may be relevant. In this study, we characterized its peripheral nerve manifestations in depth.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a national, multicenter, observational, retrospective study. Patients underwent careful clinical and neurophysiological evaluation. We excluded those with alternative etiologies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We identified 246 Val122Ile carriers, including 240 heterozygous, 4 homozygous, and 2 compound heterozygous. Gender distribution was similar. Age of onset: 19–89 years, mean 53 ± 17. Self-reported ethnicity: White (26.7%), Black (17.3%), and Mixed (Pardo) (56.0%). Birthplaces spanned all Brazilian regions, mainly the Northeast. Among heterozygotes, 52 of 122 (42.6%) were symptomatic. Carpal tunnel syndrome (CTS) preceded polyneuropathy and/or cardiomyopathy in 69.2%. The most frequent onset manifestations, excluding CTS, were cardiomyopathy (52.9%), neuropathy (37.3%), and mixed phenotype (9.8%). Age of onset: 29–86 years (mean: 64.9 years). During follow-up, 14/27 cardiac patients developed neuropathy, and 8/19 neurologic patients developed cardiomyopathy, significantly increasing the mixed phenotype from 9.8% to 52.9%. Most patients with neuropathy presented with sensory or sensory and motor disease (62.2%), followed by small fiber neuropathy (32.4%), and isolated dysautonomia (5.4%).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Neuropathy is underrecognized in Val122Ile ATTR Amyloidosis, is frequent at disease onset, may be the sole manifestation, and may occur in White persons. CTS frequently preceded neuropathy, usually an axonal polyneuropathy, although atypical patterns were observed. This variant clusters in the Northeast region of Brazil.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70160","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840742","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}
引用次数: 0
Comparing the Diagnostic Characteristics of Neuropathy Questionnaires in Adults With Obesity 成人肥胖患者神经病变问卷的诊断特点比较
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-08-21 DOI: 10.1111/jns.70163
Nicholas Heaton, Evan L. Reynolds, Fallon Koenig, Lavanya Muthukumar, Merel Huijg, Noor E. Taams, Pieter A. van Doorn, Brian C. Callaghan
{"title":"Comparing the Diagnostic Characteristics of Neuropathy Questionnaires in Adults With Obesity","authors":"Nicholas Heaton,&nbsp;Evan L. Reynolds,&nbsp;Fallon Koenig,&nbsp;Lavanya Muthukumar,&nbsp;Merel Huijg,&nbsp;Noor E. Taams,&nbsp;Pieter A. van Doorn,&nbsp;Brian C. Callaghan","doi":"10.1111/jns.70163","DOIUrl":"https://doi.org/10.1111/jns.70163","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>To compare the diagnostic characteristics of three peripheral neuropathy (PN) patient-completed questionnaires.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We recruited participants with obesity from a previously completed randomized controlled trial. Patient-completed PN questionnaires included the Michigan Neuropathy Screening Instrument questionnaire (MNSIq), the Diabetic Neuropathy Symptom (DNS) score, and the Erasmus Polyneuropathy Symptom Score (E-PSS). PN was defined using the Toronto consensus definition for confirmed neuropathy. Secondary PN definitions included probable neuropathy and definitions based on skin biopsy, nerve conduction studies, and clinical scales. Diagnostic accuracy was determined using the area under the receiver operating characteristic curve (AUC), and significant differences in AUC were assessed with DeLong's test. Potential diagnostic thresholds and corresponding predictive values and likelihood ratios were calculated.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We enrolled 59 participants (mean [SD] age: 53 [7] years, 76% female, 81% White), and 20.3% had neuropathy. E-PSS had the largest AUC (95% confidence interval) for confirmed neuropathy (0.84, 0.71–0.97), followed by MNSIq (0.76, 0.63–0.90) and the DNS (0.72, 0.54–0.90), but these differences were not significant (<i>p</i> &gt; 0.05). No significant differences were seen for secondary PN definitions (all <i>p</i> &gt; 0.05). All three questionnaires were highly correlated with one another (<i>r</i> range: 0.70–0.72, all <i>p</i> &lt; 0.01), and diagnostic thresholds were estimated.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>All three patient-completed questionnaires displayed good diagnostic accuracy for PN and were highly correlated. Our findings suggest that questionnaires can be used to determine PN in large epidemiological studies and might be useful as screening instruments in clinical care.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70163","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148784566","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}
引用次数: 0
Professor Vera Bril (1948–2026): Scientist, Mentor, and Builder of Modern Peripheral Nerve Medicine 维拉·布里尔教授(1948-2026):现代周围神经医学的科学家、导师和缔造者
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-08-19 DOI: 10.1111/jns.70157
Bruce A. Perkins, Carolina Barnett-Tapia, Juan Francisco Idiaquez, Catherine Zahn, Hans Katzberg
{"title":"Professor Vera Bril (1948–2026): Scientist, Mentor, and Builder of Modern Peripheral Nerve Medicine","authors":"Bruce A. Perkins,&nbsp;Carolina Barnett-Tapia,&nbsp;Juan Francisco Idiaquez,&nbsp;Catherine Zahn,&nbsp;Hans Katzberg","doi":"10.1111/jns.70157","DOIUrl":"https://doi.org/10.1111/jns.70157","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148943335","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}
引用次数: 0
RFC1 Repeat Expansions in Chronic Idiopathic Axonal Polyneuropathy: Prevalence, Phenotype, and Diagnostic Implications 慢性特发性轴突多发性神经病的RFC1重复扩增:患病率、表型和诊断意义
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-08-12 DOI: 10.1111/jns.70155
Vicente Gajate-García, María Fenollar-Cortés, Lucía Galán, Raluca Oancea-Ionescu, Antonio Guerrero-Solá, Alejandro Horga
{"title":"RFC1 Repeat Expansions in Chronic Idiopathic Axonal Polyneuropathy: Prevalence, Phenotype, and Diagnostic Implications","authors":"Vicente Gajate-García,&nbsp;María Fenollar-Cortés,&nbsp;Lucía Galán,&nbsp;Raluca Oancea-Ionescu,&nbsp;Antonio Guerrero-Solá,&nbsp;Alejandro Horga","doi":"10.1111/jns.70155","DOIUrl":"10.1111/jns.70155","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Chronic idiopathic axonal polyneuropathy (CIAP) accounts for approximately 20%–30% of adult-onset axonal polyneuropathies. Pathogenic <i>RFC1</i> repeat expansions have emerged as a frequent cause of idiopathic sensory neuropathy, but their recognition in routine clinical practice may be challenging, particularly in the presence of potentially confounding comorbidities. We aimed to determine the prevalence of pathogenic <i>RFC1</i> repeat expansions in a well-defined CIAP cohort, characterize the associated clinical and electrophysiological phenotype, and evaluate whether coexisting well-controlled diabetes mellitus (DM) or monoclonal gammopathy of undetermined significance (MGUS) may hinder recognition of <i>RFC1</i>-related neuropathy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We performed a retrospective observational study of adult patients with CIAP followed at a tertiary neuromuscular unit. All patients underwent <i>RFC1</i> genetic testing. Clinical and electrophysiological features were compared between <i>RFC1+</i> and <i>RFC1</i>− patients in the full cohort and after exclusion of patients with DM or MGUS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Ninety patients met CIAP criteria and were analyzed. Twenty-four (27%) carried biallelic pathogenic AAGGG repeat expansions in <i>RFC1</i>, of whom 6 (25%) had coexisting DM or MGUS. Compared with <i>RFC1</i>− patients, <i>RFC1+</i> individuals more frequently exhibited dysautonomic symptoms, unsteadiness, history of falls, need for walking support, chronic cough, impaired vibration sense in the upper limbs and up to the knees in the lower limbs, brisk upper-limb reflexes, mild cerebellar signs, an abnormal head-impulse test, and a positive Romberg's test. Most of these differences persisted after exclusion of DM or MGUS. Electrophysiological studies in <i>RFC1</i>+ patients showed widespread sensory nerve involvement, including the upper limbs, with relative motor sparing, whereas <i>RFC1</i>− patients exhibited a more typical length-dependent pattern.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Biallelic AAGGG repeat expansions in <i>RFC1</i> were identified in 27% of patients with CIAP. Specific clinical and electrophysiological features may help distinguish <i>RFC1</i>-related disease from other forms of CIAP and identify candidates for genetic testing, even in the presence of potentially confounding comorbidities such as well-controlled DM or MGUS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148713052","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}
引用次数: 0
Switching From Intravenous to Subcutaneous Immunoglobulin Maintenance Treatment in Chronic Inflammatory Demyelinating Polyradiculoneuropathy 从静脉注射到皮下免疫球蛋白维持治疗在慢性炎性脱髓鞘性多根神经病变中的转换。
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-08-08 DOI: 10.1111/jns.70159
Yelda Su, Pieter A. van Doorn, Bart C. Jacobs, Krista Kuitwaard
{"title":"Switching From Intravenous to Subcutaneous Immunoglobulin Maintenance Treatment in Chronic Inflammatory Demyelinating Polyradiculoneuropathy","authors":"Yelda Su,&nbsp;Pieter A. van Doorn,&nbsp;Bart C. Jacobs,&nbsp;Krista Kuitwaard","doi":"10.1111/jns.70159","DOIUrl":"10.1111/jns.70159","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Subcutaneous immunoglobulin (SCIg) is an alternative maintenance therapy to intravenous immunoglobulin (IVIg) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The CIDP guideline considers switching from IVIg to SCIg at an equivalent dosage reasonable; however, the optimal dosing strategy is unknown. This case series examines whether a 1:1 IVIg-to-SCIg switch maintains clinical stability.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>IVIg-dependent, clinically stable CIDP patients who switched from IVIg to SCIg in the Erasmus Medical Center were retrospectively evaluated. Clinical deterioration was defined as a decline in grip strength or muscle strength, or an increase in disability based on minimal clinically important difference criteria within 4 months after the switch.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 10 consecutive CIDP patients on stable IVIg regimens switched to an equivalent (1:1) SCIg dose. One patient remained clinically stable, while nine patients deteriorated. Three of these became clinically stable after increasing the SCIg dose to 1:1.3 or 1:1.5, whereas three patients did not regain stability despite a dose increase to 1:1.3. Clinical stability was maintained in the patient receiving a low dose (0.4 g/kg every 3 weeks), whereas patients receiving higher doses (≥ 1 g/kg every 3 weeks) deteriorated. Five patients switched back to IVIg, while five remained on SCIg throughout follow-up.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>A 1:1 IVIg-to-SCIg switch was insufficient to maintain clinical stability in most CIDP patients, including those on higher IVIg maintenance doses. Some but not all patients regained stability after increasing the SCIg dose. Optimal dose adjustment strategies, including whether temporary higher dosing is required, remain to be determined in future studies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 3","pages":""},"PeriodicalIF":3.0,"publicationDate":"2026-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13451955/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148689451","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}
引用次数: 0
Burden and Treatment Preference of Probable Neuropathic Pain in 13 592 people: A Cross-Sectional and Discrete Choice Experiment in the General Population in Malaysia 13,592人可能神经性疼痛的负担和治疗偏好:马来西亚普通人群的横断面和离散选择实验。
IF 3 3区 医学
Journal of the Peripheral Nervous System Pub Date : 2026-08-04 DOI: 10.1111/jns.70154
Wee-Kiat Tan, Maznah Dahlui, Azhari Rosman, Hiang Leng Tan, Noraidah Guntalib, Siew Pheng Chan, Shalini Sree Dharan, Pei Chiek Teh, Daniel Yi Jie Wong, Kenny Tan, Solomon Tesfaye, Lee-Ling Lim
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