Journal of the Peripheral Nervous System最新文献

{"title":"Safety and Cost Analysis of Immunoglobulin Cessation Trials in Chronic Inflammatory Demyelinating Polyradiculoneuropathy","authors":"Shiwen Koay,&nbsp;Yi-Chun Chen,&nbsp;George Ransley,&nbsp;Laura Compton,&nbsp;Michael P. Lunn,&nbsp;Aisling S. Carr","doi":"10.1111/jns.70007","DOIUrl":"https://doi.org/10.1111/jns.70007","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic autoimmune neuropathy worldwide. A significant proportion of CIDP patients enter spontaneous or medication-related remission, remaining stable without immunotherapy. Overtreatment of CIDP has clinical and financial implications. We examined performance of IVIg cessation trials in our CIDP cohort and report safety and cost analysis outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In individuals with CIDP on maintenance IVIg treatment, a cessation trial was proposed in clinically stable patients with a static IVIg regimen over a 12-month period. We explored the proportion who were stable off treatment for 12 or more months and the time to recovery in those who declined and were re-treated. We examined cost implications of this approach.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>45/125 individuals met criteria for clinical stability, with median age 58 years, I-RODS 37/48, MRC-SS 69/70 and annual treatment costs £107 000/person. Nine individuals had cessation trials resulting in decline within 2 years prior and were not re-challenged, leaving 36 eligible individuals. 12 of 36 (33.3%) consented to cessation trial and eight of those (66.7%) remained stable off treatment for ≥ 12 months. The successful cessation trials resulted in a cost saving of £855 000/year, with a potential further saving of £1.7 million/year if all the eligible individuals had consented. All patients who deteriorated were rescued to previous baseline on retreatment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Individuals with CIDP should be counselled about the natural history of the disease and future scheduled, targeted cessation trials. A dedicated clinical infrastructure is vital to safely perform cessation trials.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70007","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143438946","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":"Unique Nerve Tissue-Restricted T-Cell Clones in Chronic Inflammatory Demyelinating Polyneuropathy","authors":"G. G. A. van Lieverloo,&nbsp;D. C. Anang,&nbsp;M. E. Adrichem,&nbsp;B. A. Coert,&nbsp;A. E. Aronica,&nbsp;L. Wieske,&nbsp;I. N. van Schaik,&nbsp;N. de Vries,&nbsp;F. Eftimov","doi":"10.1111/jns.70006","DOIUrl":"https://doi.org/10.1111/jns.70006","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 disorder characterized by peripheral nerve damage. Although T lymphocytes (T-cells) are implicated in the pathogenesis of CIDP, we previously observed that the frequency of highly expanded T-cell clones (HECs) in peripheral blood of CIDP patients was not different from healthy controls. To investigate if local T-cells might be pathogenic, we employed next-generation sequencing to compare the TCRβ repertoire between peripheral blood and nerve tissue of CIDP patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Adaptive immune receptor repertoire sequencing (AIRR-Seq) of the TCRβ chain was conducted on peripheral blood and nerve tissue obtained from three newly diagnosed CIDP patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>All patients showed high numbers of highly expanded TCRβ clones in nerve tissue that were not detected or detected only in very low frequencies in blood, whereas in blood other HECs were found. Clustering analysis based on CDR3-similarity showed that these nerve tissue-restricted TCRβ clones were distinct from blood clones, as evidenced by the absence of prominent clusters.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Unique nerve tissue-restricted TCRβ clones may indicate a highly localized immune response with localized expansion and/or retention of T-cells that could contribute to the pathomechanism of CIDP.</p>\u0000 \u0000 <p>Further characterization of the phenotype, antigen target and functionality of these T-cells is essential to determine their pathogenic role.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143438945","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":"Differential Impact of Metabolic Factors and Comorbidities on Peripheral Neuropathy","authors":"Georgios Ponirakis,&nbsp;Ioannis N. Petropoulos,&nbsp;Hoda Gad,&nbsp;Sidra Abdulshakoor,&nbsp;Jenneth M. Concepcion,&nbsp;Sara H. Khalfalla,&nbsp;Iynas S. A. Elamin,&nbsp;Abeer T. H. AlZawqari,&nbsp;Einas Elgassim,&nbsp;Areej Baraka,&nbsp;Aljazi M. Al-Khalifa,&nbsp;Ziyad R. Mahfoud,&nbsp;Marwa A. El Deeb,&nbsp;Nahla Afifi,&nbsp;Rayaz A. Malik","doi":"10.1111/jns.70004","DOIUrl":"https://doi.org/10.1111/jns.70004","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>This study aimed to assess the impact of metabolic factors and comorbidities on peripheral neuropathy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Qatari nationals and long-term residents from the Qatar Biobank underwent clinical/metabolic assessments, including iDXA to measure visceral adipose tissue (VAT) and subcutaneous (SAT) volumes, inflammation, thyroid function, carotid intima media thickness (CIMT), corneal confocal microscopy (CCM), vibration perception threshold (VPT), and DN4 questionnaire.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In 332 adults aged 43.4 ± 12.7 years, the prevalence of neuropathy was 3.9%. The prevalence of T2D was 16.6%, and the prevalence of neuropathy was significantly higher in T2D (14.5% vs. 1.8%, <i>p</i> &lt; 0.0001). A higher HbA1c (<i>p</i> = 0.05) and lower eGFR (<i>p</i> &lt; 0.01) were associated with reduced inferior whorl length (IWL) and lower FT3 was associated with reduced corneal nerve fiber length (CNFL) (<i>p</i> &lt; 0.01). Triglycerides were associated with increased neuropathic symptoms (<i>p</i> = 0.05). All the risk factors in this study contributed to 39% of neuropathy in T2D but had a minimal impact in those without T2D.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This study highlights the importance of additional risk factors beyond traditional risk factors associated with peripheral neuropathy in T2D.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143423839","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":"Hip Dysplasia in Charcot–Marie–Tooth Disease: Insights From a Large Cohort of Children and Adolescents","authors":"Khian Aun Tan,&nbsp;Monique M. Ryan,&nbsp;Rachel A. Kennedy,&nbsp;Kate Carroll,&nbsp;Katy de Valle,&nbsp;Carrie M. Kollias,&nbsp;Eppie M. Yiu","doi":"10.1111/jns.70002","DOIUrl":"10.1111/jns.70002","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Despite the known association of hip dysplasia and Charcot Marie Tooth disease (CMT), evidence is limited regarding its exact prevalence. Available studies pre-date genetic confirmation of CMT subtypes and current hip reconstruction surgical options. This study examined the prevalence of hip dysplasia in CMT in a tertiary neuromuscular center.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a retrospective study of children with CMT who had at least one pelvic radiograph between 2000 and 2020. Reimer's migration percentage, acetabular index and lateral center edge angle were used to identify hip dysplasia.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 178 children were included with a median age of 6.4 (IQR 3.4–11.3) years at CMT diagnosis. First pelvic radiographs were performed at a median age of 8.0 (IQR 4.6–12.2) years and 64 (35.8%) had hip dysplasia, of which 20 normalized over time. Repeat radiographs were done in 96/178 children (53.9%), and six children with originally normal radiographs developed later radiographic hip dysplasia. At the time of last follow up, 50/178 children (28.1%) had hip dysplasia and 17/178 children (9.6%) required surgical intervention. The frequency of hip dysplasia in specific CMT subtypes was: 28/100 in CMT1A, 5/7 in Dejerine-Sottas disease, 3/10 in CMT2A, and 4/4 in <i>TRPV4</i>-related CMT.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>The prevalence of hip dysplasia in children with CMT in this cohort was estimated to be between 9.6% and 28.1%. Serial imaging is important to monitor outcomes into adulthood. Specific CMT subtypes were more likely to be associated with hip dysplasia.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11783581/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143066424","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":"Association Between MScanFit Motor Unit Number Estimation and Clinical Function and Response to Immunoglobulin Therapy in Chronic Inflammatory Demyelinating Polyneuropathy","authors":"Peter N. Hansen,&nbsp;Abdullahi A. Mohammed,&nbsp;Lars K. Markvardsen,&nbsp;Henning Andersen,&nbsp;Hatice Tankisi,&nbsp;Søren H. Sindrup,&nbsp;Thomas Krøigård","doi":"10.1111/jns.70001","DOIUrl":"10.1111/jns.70001","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Loss of motor units in chronic inflammatory demyelinating polyneuropathy is difficult to assess by conventional nerve conduction due to collateral innervation. We aimed to assess the association between a motor unit number estimate (MUNE) derived from the compound muscle action potential (CMAP) scan using MScanFit and hand function and the clinical response to intravenous immunoglobulin (IVIG).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Forty-nine CIDP patients and 52 control subjects were included. CMAP scan recordings were obtained from the right abductor pollicis brevis muscle. The primary outcome was the correlation between MUNE and the duration of the nine-hole-peg test (9-HPT) at baseline and the change in the duration of the 9-HPT following treatment with IVIG. Secondary outcomes were grip strength, 10-m-walk test, six-spot-step test, medical research council sum score, inflammatory neuropathy cause and treatment sensory sum score, overall neuropathy limitations scale, and the Rasch-built overall disability scale (R-ODS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>MScanFit analysis suggested both loss of motor units (reduced MUNE (<i>p</i> = 0.022) and N50 (<i>p</i> &lt; 0.0001)) and collateral reinnervation (increased median amplitude (<i>p</i> &lt; 0.0001) and size of the largest unit (<i>p</i> &lt; 0.0001)) in CIDP patients compared to controls. In CIDP patients, there was a statistically significant correlation between MUNE and the duration of the 9-HPT (Spearman's <i>r</i> = −0.342; <i>p</i> = 0.016). Further, patients with a low MUNE had the largest reduction in the duration of the 9-HPT following IVIG treatment (<i>r</i> = −0.577; <i>p</i> = 0.043). MUNE also correlated significantly with R-ODS (<i>r</i> = −0.722; <i>p</i> = 0.007).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>MScanFit MUNE could be a useful method for assessing motor axonal loss in CIDP, which correlates with the clinical function and treatment response.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11783579/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143066416","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":"Inter-Laboratory Validation of Nodal/Paranodal Antibody Testing","authors":"Cinta Lleixà,&nbsp;Maarten Titulaer,&nbsp;Sophia Rohrbacher,&nbsp;Victor Mgbachi,&nbsp;Susan Halstead,&nbsp;Janev Fehmi,&nbsp;Elba Pascual-Goñi,&nbsp;Louisa Zhu,&nbsp;Luise Appeltshauser,&nbsp;Suzanne Franken,&nbsp;Manuela Paunovic,&nbsp;Patrick Waters,&nbsp;Hugh Willison,&nbsp;Claudia Sommer,&nbsp;Luis Querol,&nbsp;Ruth Huizinga,&nbsp;Kathrin Doppler,&nbsp;Simon Rinaldi","doi":"10.1111/jns.70000","DOIUrl":"10.1111/jns.70000","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Reliable detection of antibodies against nodal targets is vital for the diagnosis of autoimmune nodopathies. The performance characteristics of recently developed in-house assays are unknown. We compared testing at four centres.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Each submitted 29–40 serum samples to a coordinating centre from one of three groups: (1) autoimmune nodopathy patients, with positive nodal/paranodal antibodies; (2) seronegative patients with other inflammatory neuropathies, and (3) healthy individuals or those with other neurological diseases. The coordinating centre recoded all samples and returned 160 identical aliquots to each testing centre for blinded testing. Once data from all centres had been received by the coordinating centre, unblinded results were returned for analysis. Sensitivity was defined by the proportion of group 1 samples returned as positive. Accuracy was defined as 0.075(sensitivity) + 0.925(specificity).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Centres performed various combinations of ELISA, cell-based (CBAs) and teased-nerve fibre assays. All labs produced highly accurate results (96%–100%) and concordance for the overall result across at least 3 or all 4 test centres was observed for 98% and 89% of the samples respectively. However, 10/30 individual assays (6/14 CBAs and 4/16 ELISAs) were less than 90% sensitive. Only 3 assays had more than 1 false positive result (2 ELISAs and 1 CBA). Combining different assay modalities to produce an overall result did not improve accuracy. Inter-laboratory consistency in the determination of antibody subclasses was poor.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Although most samples were correctly categorised in all 4 centres, the use of a specific test modality or multiple tests did not guarantee accuracy. Early and repeated interlaboratory testing with sharing of samples is important to understand test performance and reproducibility, identify areas for improvement and maintain consistency. To aid this, we provide detailed methods for the best performing tests. Further standardisation of antibody subclass determination is required.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11780190/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143066426","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":"Broadening Research Priorities in Peripheral Neuropathy: A Response to “A Call to Action for Peripheral Neuropathy Research Funding—Time to Consolidate Funding Under One NIH Initiative?”","authors":"Lien-Chung Wei,&nbsp;Hsien-Jane Chiu","doi":"10.1111/jns.70003","DOIUrl":"10.1111/jns.70003","url":null,"abstract":"","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143066422","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":"Neurological Performance and Clinical Outcomes Related to Patients With Oropouche-Associated Guillain–Barré Syndrome","authors":"Frank D. Martos-Benítez,&nbsp;Iliovanys Betancourt-Plaza,&nbsp;Isleidys Osorio-Carmenates,&nbsp;Nadieska J. González-Martínez,&nbsp;Ileana Moráles-Suárez,&nbsp;Carilda E. Peña-García,&nbsp;Yudeily L. Pérez-Matos,&nbsp;Zurina Lestayo-O'Farrill,&nbsp;José R. de Armas-Fernández,&nbsp;Raysa C. Cárdenas-González,&nbsp;Judet Izquierdo-Castañeda,&nbsp;Ernesto Sánchez-de la Rosa,&nbsp;Versis Orama-Requejo","doi":"10.1111/jns.12683","DOIUrl":"10.1111/jns.12683","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>A recent study reported that Oropouche virus (OROV) infection may play a role in the etiology of Guillain–Barré syndrome. We aimed to identify the neurological performance, disease-modifying therapies, and clinical outcomes related to patients with Oropouche-associated Guillain–Barré syndrome admitted to the critical care unit.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was an analysis of 210 patients diagnosed with Guillain–Barré syndrome and suspicion of Oropouche viral infection admitted to the critical care units from June 2024 to September 2024 using the national administrative healthcare data. OROV was identified by reverse-transcriptase–polymerase-chain-reaction. Patients with Guillain–Barré syndrome and Oropouche infection were compared with those without Oropouche infection in terms of demography features, neurological performance, disease-modifying therapies, and clinical outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Most patients had a severe disease. Mechanical ventilation was required in 28.6%. Overall mortality rate was 14.3%. The median time from onset of weakness to intensive care unit discharge, and the median time from hospital admission to intensive care unit discharge was 18 days (IQR: 13–24.3 days) and 13 days (IQR: 9–19 days), respectively. Oropouche viral infection was detected in 43 (20.5%) patients. There were no differences among patients with and without Oropouche viral infection regarding general characteristics, neurological performance, disease-modifying therapies, and outcomes. After adjusting for confounders in multivariate logistic regression analysis, Oropouche viral infection (OR: 1.94; 95% CI: 0.72–5.20; <i>p</i> = 0.189) was not related to increased mortality.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Oropouche viral infection does not modify the clinical course, disease severity, and outcomes of patients with Guillain–Barré syndrome.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143033390","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":"A call to action for peripheral neuropathy research funding—Time to consolidate funding under one NIH initiative?","authors":"Stéphanie A. Eid,&nbsp;Kristy L. Townsend,&nbsp;Vincenza Spallone,&nbsp;Daniela M. Menichella,&nbsp;Emily J. Koubek,&nbsp;Eva L. Feldman","doi":"10.1111/jns.12681","DOIUrl":"10.1111/jns.12681","url":null,"abstract":"&lt;p&gt;Peripheral neuropathies (PNs) pose a significant clinical challenge in the field of neurological disorders, with a prevalence of 2.4% in the general population that rises with age to over 8% in patients aged 55 years and older.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; Symmetrical, distal-to-proximal axonal loss is the most common form of PN and accounts for most cases.&lt;span&gt;&lt;sup&gt;2, 3&lt;/sup&gt;&lt;/span&gt; It is characterized by damage to the peripheral nerves that typically, especially for diabetes (the leading cause of PN), impacts small-diameter axons beginning in the feet and progresses proximally in a length-dependent manner. PN results in a range of debilitating symptoms such as numbness, tingling, weakness, as well as burning or shooting pain.&lt;span&gt;&lt;sup&gt;4&lt;/sup&gt;&lt;/span&gt; Along with these painful symptoms, patients may experience depression, anxiety, and sleep disturbances.&lt;span&gt;&lt;sup&gt;5&lt;/sup&gt;&lt;/span&gt; As PN progresses, individuals may present with diminished sensation to mechanical and thermal stimuli, making it challenging to perceive or effectively heal injuries or trauma, which increases the risk of non-healing ulcers. In severe cases, the cumulative effects of sensation loss and non-healing ulcers can necessitate lower limb amputations.&lt;span&gt;&lt;sup&gt;6&lt;/sup&gt;&lt;/span&gt; In fact, patients with PN are almost four times at greater risk of undergoing lower-limb amputation than those without.&lt;span&gt;&lt;sup&gt;7&lt;/sup&gt;&lt;/span&gt; Additionally, PN also leads to an increased risk of falls due to compromised balance and proprioception, further exacerbating the potential for injury and disability in affected individuals. PN likely impacts far more tissues and organs than previously appreciated.&lt;span&gt;&lt;sup&gt;8&lt;/sup&gt;&lt;/span&gt; Data now indicate diabetic PN exists in the muscle, liver, adipose tissue, pancreas, gastrointestinal tract, and heart.&lt;span&gt;&lt;sup&gt;9-17&lt;/sup&gt;&lt;/span&gt; PN profoundly impacts the lives of patients, with limited therapeutic options to mitigate pain symptoms, prevent progression, or regenerate lost axons.&lt;/p&gt;&lt;p&gt;While clinical presentations may appear similar, PN can result from a range of causes, including both inherited and acquired conditions. Hereditary neuropathies (HN), such as Charcot–Marie-Tooth (CMT) disease and hereditary sensory and autonomic neuropathies, comprise a diverse group of inherited PN disorders, with an overall prevalence of 1:2500.&lt;span&gt;&lt;sup&gt;18&lt;/sup&gt;&lt;/span&gt; These differ in their inheritance patterns (autosomal dominant, recessive, or X-linked), electrophysiological characteristics (demyelinating, axonal, or intermediate), and clinical features. While not as common as the other PN types, HN highlight the importance of genetic factors in neuropathic disorders.&lt;/p&gt;&lt;p&gt;Of the acquired neuropathies, diabetic peripheral neuropathy (DPN) is the most prevalent, accounting for 32%–53% of total cases.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; As cases of diabetes are expected to rise from 537 to 783 million by 2045, DPN, which affects 50% or more of patients and increases in frequency with di","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11725771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142971584","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":"Distinguishing Chronic Inflammatory Demyelinating Polyneuropathy From Mimic Disorders: The Role of Statistical Modeling","authors":"Grace Swart,&nbsp;Michael P. Skolka,&nbsp;Shahar Shelly,&nbsp;Richard A. Lewis,&nbsp;Jeffrey A. Allen,&nbsp;Divyanshu Dubey,&nbsp;Zhiyv Niu,&nbsp;Judith Spies,&nbsp;Ruple S. Laughlin,&nbsp;Smathorn Thakolwiboon,&nbsp;Ashley R. Santilli,&nbsp;Hebatallah Rashed,&nbsp;Igal Mirman,&nbsp;Alexander Swart,&nbsp;Sarah E. Berini,&nbsp;Kamal Shouman,&nbsp;Marcus V. Pinto,&nbsp;Michelle L. Mauermann,&nbsp;John R. Mills,&nbsp;P. James B. Dyck,&nbsp;William S. Harmsen,&nbsp;Jay Mandrekar,&nbsp;Christopher J. Klein","doi":"10.1111/jns.12682","DOIUrl":"10.1111/jns.12682","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is difficult to distinguish from mimicking disorders, with misdiagnosis resulting in IVIG overutilization. We evaluate a clinical-electrophysiological model to facilitate CIDP versus mimic neuropathy prediction.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Using the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 CIDP guidelines we derived 26 clinical and 144 nerve conduction variables. The model was generated and validated utilizing total CIDP (<i>n</i> = 129) and mimics (<i>n</i> = 309); including (1) IgG4-nodopathies; (2) POEMS (polyneuropathy–organomegaly–endocrinopathy–monoclonal protein-skin changes); (3) anti-myelin-associated-glycoprotein; (4) paraneoplastic; (5) Waldenström B-cell lymphoma; (6) diabetic neuropathies; (7) amyloidosis; (8) Charcot–Marie–Tooth; (9) motor neuropathies/neuronopathies; and (10) idiopathic-inflammatory-myopathies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We analyzed 9282 clinical and 51 408 electrophysiological data points. Univariate analysis identified 11 of 26 clinical variables with significant odds ratios. A multivariate regression model using four clinical and two electrophysiologic variables achieved 93% area-under-curve (95% CI 91–95): progression over 8 weeks (OR 40.66, 95% CI 5.31–311.36), absent autonomic involvement (OR 17.82, 95% CI 2.93–108.24), absent muscle atrophy (OR 16.65, 95% CI 3.27–84.73), proximal weakness (OR 3.63, 95% CI 1.58–8.33), ulnar motor conduction velocity slowing &lt; 35.7 m/s (OR 5.21, 95% CI 2.13–12.76), and ulnar motor conduction block (OR 13.37, 95% CI 2.47–72.40). A web-based probability calculator (https://news.mayocliniclabs.com/cidp-calculator/) was developed, with 100% sensitivity and 68% specificity at a 92% probability threshold. Specificity improved to 93% when considering “red flags,” electrophysiologic criteria, and laboratory testing.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>A probability calculator using clinical electrophysiological variables assists CIDP differentiation from mimics, with scores below 92% unlikely to have CIDP. The highest specificity is achieved by considering clinical “red flags,” electrophysiologic demyelination, and laboratory testing.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 1","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142971585","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}