Mohammad QD, Islam Z, Papri N
… +9 more, Hayat S, Jahan I, Azad KAK, Artis DR, Hoehn B, Humphriss E, Lin P, Yednock T, Kroon HA
J Peripher Nerv Syst
· 2025 Mar · PMID 40000167
·
Full text
BACKGROUND AND AIMS: Guillain-Barré syndrome (GBS) is an acute autoimmune peripheral neuropathy driven by autoantibodies and classical complement pathway activation. Despite treatments with intravenous immunoglobulin or...BACKGROUND AND AIMS: Guillain-Barré syndrome (GBS) is an acute autoimmune peripheral neuropathy driven by autoantibodies and classical complement pathway activation. Despite treatments with intravenous immunoglobulin or plasma exchange, GBS remains characterized by variability in recovery and high incidence of residual disabilities. This randomized, double-blind, placebo-controlled Phase 1 trial evaluated the safety, tolerability, and pharmacokinetics of ANX005, a novel therapeutic targeting the classical complement cascade. METHODS: Patients with recent-onset GBS, who had no access to intravenous immunoglobulin or plasma exchange, received escalating doses of ANX005 or placebo as a single IV infusion. Primary objectives included assessments of safety. Secondary objectives included determination of pharmacokinetic and pharmacodynamic profiles and clinical outcomes through Week 8. Exploratory objectives included an evaluation of serum and cerebrospinal fluid (CSF) complement and tissue damage biomarkers. RESULTS: Fifty patients were randomized to receive either ANX005 (n = 38) or placebo (n = 12). ANX005 was well-tolerated across all doses with no dose-limiting toxicities, suggesting an acceptable safety profile. Pharmacodynamic data showed effective C1q inhibition and a reduction in neurofilament light chain levels, suggesting nerve damage mitigation. Exploratory endpoints evaluating clinical outcomes included improvements in Medical Research Council sum scores, GBS-Disability Score, and Overall Neuropathy Limitations Scale with ANX005 compared to placebo, particularly in patients receiving doses that inhibited serum C1q for ≥ 1 week and provided C1q blockade in the CSF. INTERPRETATION: These results support ANX005 as a targeted therapy for GBS that modulates the classical complement pathway. Further investigation in a larger Phase 3 trial is warranted to confirm these results and assess the long-term benefits of complement inhibition in patients with GBS.
Koay S, Chen YC, Ransley G
… +3 more, Compton L, Lunn MP, Carr AS
J Peripher Nerv Syst
· 2025 Mar · PMID 39967344
·
Full text
BACKGROUND AND AIMS: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic autoimmune neuropathy worldwide. A significant proportion of CIDP patients enter spontaneous or medication-...BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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. INTERPRETATION: 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.
van Lieverloo GGA, Anang DC, Adrichem ME
… +6 more, Coert BA, Aronica AE, Wieske L, van Schaik IN, de Vries N, Eftimov F
J Peripher Nerv Syst
· 2025 Mar · PMID 39967321
·
Full text
BACKGROUND AND AIMS: 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 o...BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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. INTERPRETATION: 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. Further characterization of the phenotype, antigen target and functionality of these T-cells is essential to determine their pathogenic role.
Ponirakis G, Petropoulos IN, Gad H
… +12 more, Abdulshakoor S, Concepcion JM, Khalfalla SH, Elamin ISA, AlZawqari ATH, Elgassim E, Baraka A, Al-Khalifa AM, Mahfoud ZR, El Deeb MA, Afifi N, Malik RA
J Peripher Nerv Syst
· 2025 Mar · PMID 39953748
·
Full text
OBJECTIVE: This study aimed to assess the impact of metabolic factors and comorbidities on peripheral neuropathy. METHODS: Qatari nationals and long-term residents from the Qatar Biobank underwent clinical/metabolic asse...OBJECTIVE: This study aimed to assess the impact of metabolic factors and comorbidities on peripheral neuropathy. METHODS: 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. RESULTS: 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%, p < 0.0001). A higher HbA1c (p = 0.05) and lower eGFR (p < 0.01) were associated with reduced inferior whorl length (IWL) and lower FT3 was associated with reduced corneal nerve fiber length (CNFL) (p < 0.01). Triglycerides were associated with increased neuropathic symptoms (p = 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. CONCLUSIONS: This study highlights the importance of additional risk factors beyond traditional risk factors associated with peripheral neuropathy in T2D.
Hansen PN, Mohammed AA, Markvardsen LK
… +4 more, Andersen H, Tankisi H, Sindrup SH, Krøigård T
J Peripher Nerv Syst
· 2025 Mar · PMID 39887824
·
Full text
BACKGROUND AND AIMS: 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 betw...BACKGROUND AND AIMS: 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). METHODS: 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). RESULTS: MScanFit analysis suggested both loss of motor units (reduced MUNE (p = 0.022) and N50 (p < 0.0001)) and collateral reinnervation (increased median amplitude (p < 0.0001) and size of the largest unit (p < 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 r = -0.342; p = 0.016). Further, patients with a low MUNE had the largest reduction in the duration of the 9-HPT following IVIG treatment (r = -0.577; p = 0.043). MUNE also correlated significantly with R-ODS (r = -0.722; p = 0.007). INTERPRETATION: MScanFit MUNE could be a useful method for assessing motor axonal loss in CIDP, which correlates with the clinical function and treatment response.
Lleixà C, Titulaer M, Rohrbacher S
… +15 more, Mgbachi V, Halstead S, Fehmi J, Pascual-Goñi E, Zhu L, Appeltshauser L, Franken S, Paunovic M, Waters P, Willison H, Sommer C, Querol L, Huizinga R, Doppler K, Rinaldi S
J Peripher Nerv Syst
· 2025 Mar · PMID 39887819
·
Full text
BACKGROUND AND AIMS: 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 comp...BACKGROUND AND AIMS: 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. METHODS: 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). RESULTS: 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. INTERPRETATION: 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.
Tan KA, Ryan MM, Kennedy RA
… +4 more, Carroll K, de Valle K, Kollias CM, Yiu EM
J Peripher Nerv Syst
· 2025 Mar · PMID 39887493
·
Full text
BACKGROUND AND AIMS: 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 subtyp...BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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 TRPV4-related CMT. INTERPRETATION: 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.
Martos-Benítez FD, Betancourt-Plaza I, Osorio-Carmenates I
… +10 more, González-Martínez NJ, Moráles-Suárez I, Peña-García CE, Pérez-Matos YL, Lestayo-O'Farrill Z, de Armas-Fernández JR, Cárdenas-González RC, Izquierdo-Castañeda J, la Rosa ES, Orama-Requejo V
J Peripher Nerv Syst
· 2025 Mar · PMID 39853692
·
Publisher ↗
BACKGROUND AND AIMS: 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 therapie...BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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; p = 0.189) was not related to increased mortality. INTERPRETATION: Oropouche viral infection does not modify the clinical course, disease severity, and outcomes of patients with Guillain-Barré syndrome.
Swart G, Skolka MP, Shelly S
… +20 more, Lewis RA, Allen JA, Dubey D, Niu Z, Spies J, Laughlin RS, Thakolwiboon S, Santilli AR, Rashed H, Mirman I, Swart A, Berini SE, Shouman K, Pinto MV, Mauermann ML, Mills JR, Dyck PJB, Harmsen WS, Mandrekar J, Klein CJ
J Peripher Nerv Syst
· 2025 Mar · PMID 39801067
·
Publisher ↗
BACKGROUND AND AIMS: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is difficult to distinguish from mimicking disorders, with misdiagnosis resulting in IVIG overutilization. We evaluate a clinical-elec...BACKGROUND AND AIMS: 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. METHODS: 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 (n = 129) and mimics (n = 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. RESULTS: 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 < 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. INTERPRETATION: 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.
Leonardi L, Adam C, Beaudonnet G
… +8 more, Beauvais D, Cauquil C, Not A, Morassi O, Trassard O, Echaniz-Laguna A, Adams D, Labeyrie C
J Peripher Nerv Syst
· 2025 Mar · PMID 39800979
·
Full text
OBJECTIVE: To assess the effectiveness of labial minor salivary gland biopsy (LSGB) alone or in combination with punch skin biopsy (SB) for the detection of amyloid deposits in hereditary transthyretin amyloidosis with p...OBJECTIVE: To assess the effectiveness of labial minor salivary gland biopsy (LSGB) alone or in combination with punch skin biopsy (SB) for the detection of amyloid deposits in hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN). METHODS: In this single-center retrospective study, Congo red staining of minimal invasive LSGB (4 mm) and SB (3 mm) was assessed in ATTRv-PN patients consecutively evaluated between 2012 and 2023. RESULTS: Histopathological data of 171 ATTRv-PN, including 49 early-onset p.Val50Met, 58 late-onset p.Val50Met, and 64 non-p.Val50Met, were reviewed. LSGB and SB identified amyloid deposits in 123/171 (72%) and 131/171 (77%) patients respectively (p = 0.2). Combining LSGB and SB increased the amyloid detection rate to 150/171 (88%), especially in late-onset p.Val50Met (48/58 [83%]) and non-p.Val50Met patients (55/64 [86%]). LSGB and SB have a similar rate of detection of amyloid depositions in early onset p.Val50Met patients (94%). Also, the LSGB/SB combination identified amyloidosis in 89% (55/62) of early-stage ATTRv-PN patients. CONCLUSIONS: In our study, combining LSGB and SB allowed the detection of amyloid deposits in 88% of ATTRv-PN patients. LSGB/SB analysis may be of major interest to confirm entry in the disease at very early-stage ATTRv-PN, with implications in disease-modifying treatment initiation.
Hu J, Zheng Y, Sun C
… +6 more, Sun J, Xi J, Luo S, Qiao K, Zhao C, Lin J
J Peripher Nerv Syst
· 2025 Mar · PMID 39800963
·
Publisher ↗
BACKGROUND AND AIMS: To investigate the treatment of ofatumumab in autoimmune nodopathy (AN). METHODS: An open-label, prospective, observational study was conducted in patients with AN. The regimen was 20 mg ofatumumab s...BACKGROUND AND AIMS: To investigate the treatment of ofatumumab in autoimmune nodopathy (AN). METHODS: An open-label, prospective, observational study was conducted in patients with AN. The regimen was 20 mg ofatumumab subcutaneously on day 0, 7, 14, 28, and subsequently every 4 weeks in a total of 24 weeks. The primary endpoint of the study was the proportion of patients with confirmed clinical improvement. RESULTS: All of the eight patients (100%) improved at Week 24. The median time to improvement was 8 (IQR: 7-10) weeks. The four patients previously treated with rituximab and two with irregular injections of ofatumumab (OFA) improved. At Week 24, the adjusted INCAT score, MRC sum score, cI-RODS, and grip strength in nondominant hand significantly improved from baseline. In nerve conduction studies, all of the six patients with available data (100%) improved. The median sNfL significantly reduced from baseline at Week 8. Anti-paranodal antibody in seven patients with anti-NF155 antibodies reduced from baseline at Week 20. In seven of the eight patients, CD19+ B cells were significantly reduced at Week 4. No serious adverse events were reported. INTERPRETATION: The regimen was 20 mg ofatumumab subcutaneously on day 0, 7, 14, 28, and every 4 weeks from Week 4, in a total of 24 weeks. The ofatumumab therapy may provide a more convenient and safer treatment for patients with AN, while serving as an effective alternative for those who did not respond to rituximab.
Ju W, Min YG, Kim JS
… +6 more, Choi J, Lee J, Choi SJ, Kim SM, Hong YH, Sung JJ
J Peripher Nerv Syst
· 2025 Mar · PMID 39655711
·
Publisher ↗
BACKGROUND AND AIMS: The Inflammatory Rasch-built Overall Disability Scale (I-RODS) is an effective activity measure for use in inflammatory peripheral neuropathy. The aim of this study was to validate the Korean version...BACKGROUND AND AIMS: The Inflammatory Rasch-built Overall Disability Scale (I-RODS) is an effective activity measure for use in inflammatory peripheral neuropathy. The aim of this study was to validate the Korean version of the I-RODS in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barré syndrome (GBS), anti-myelin-associated glycoprotein (MAG) neuropathy, and autoimmune nodopathy. METHODS: A total of 120 patients underwent clinical evaluations, which included the I-RODS, Inflammatory Neuropathy Cause and Treatment (INCAT) assessment, and Jamar grip strength (kg) measurement. Follow-up assessments were performed for 83 patients during their regular clinic visits. To estimate the test-retest reliability of the I-RODS, the scale was reapplied to a subset of 16 patients within 2-7 days of the initial test. Overall, reliability, validity, and responsiveness of the I-RODS were evaluated. RESULTS: Internal consistency was good, as indicated by a person separation index of 0.966. The raw and standardized Cronbach's alpha values were both 0.974. The test-retest reliability analyzed using the intraclass correlation coefficient (ICC) was also high (ICC = 0.972). The I-RODS showed a strong correlation with INCAT scores (ρ = -0.81, p < .001) and a moderate correlation with grip strength (ρ = 0.61, p < .001). Furthermore, the sensitivity for detecting clinically meaningful improvement was highest for grip strength (60.4%) followed by I-RODS (52.1%), while for capturing deterioration, it was highest for I-RODS (80.0%). INTERPRETATION: The Korean version of the I-RODS is a reliable and valid tool for measuring disability in patients with inflammatory neuropathy. The I-RODS is useful for both clinical practice and research applications.
Politikou O, Frueh FS, Greminger M
… +4 more, Besmens IS, Freddi G, Alessandrino A, Calcagni M
J Peripher Nerv Syst
· 2025 Mar · PMID 39592390
·
Publisher ↗
BACKGROUND AND AIMS: Peripheral nerve injuries often require bridging when direct repair is not feasible. Nerve autografts are the gold standard, but they can lead to donor site morbidity. Silk fibroin-based nerve condui...BACKGROUND AND AIMS: Peripheral nerve injuries often require bridging when direct repair is not feasible. Nerve autografts are the gold standard, but they can lead to donor site morbidity. Silk fibroin-based nerve conduits, like the novel SILKBridge, offer a promising alternative. This pilot study evaluates the mid-term outcomes of the first in-human digital nerve reconstruction using the SILKBridge, focusing on sensory recovery, complication rates, patient-reported outcomes, and biological integration. METHODS: This study included four patients with digital nerve defects reconstructed using the SILKBridge. Clinical assessments included two-point discrimination, Semmes-Weinstein monofilament testing, and pain evaluation using the Numeric Rating Scale. Sonographic assessments were also performed to evaluate the conduit's biointegration and potential complications. RESULTS: At a mean follow-up of 32 months, all patients demonstrated satisfactory sensory recovery and reported minimal to no pain. Sonographic assessments confirmed effective biointegration with no signs of inflammation or scarring. INTERPRETATION: The mid-term evaluation of the first in-human digital nerve reconstruction with the SILKBridge revealed safety, efficiency, and favorable biocompatibility properties. Further studies with larger cohorts are needed to validate these findings and compare them with other nerve repair methods.
Papri N, Doets AY, Luijten L
… +5 more, Mohammad QD, Endtz HP, Lingsma HF, Jacobs BC, Islam Z
J Peripher Nerv Syst
· 2024 Dec · PMID 39581760
·
Publisher ↗
BACKGROUND AND AIMS: The aim of this study is to validate and perform a region-specific adjustment of the Erasmus GBS Respiratory Insufficiency Score (EGRIS) and identify potential predictors of prolonged mechanical vent...BACKGROUND AND AIMS: The aim of this study is to validate and perform a region-specific adjustment of the Erasmus GBS Respiratory Insufficiency Score (EGRIS) and identify potential predictors of prolonged mechanical ventilation (PMV) among Guillain-Barré syndrome (GBS) patients from Bangladesh. METHODS: We enrolled GBS patients from four prospective observational cohort studies conducted in Bangladesh. Accuracy of EGRIS to predict the requirement of MV in <7 days of study entry was evaluated. Model performance was assessed by discrimination (ability of the model to differentiate between patients who needed MV or not) and calibration (accuracy of absolute risk estimates). PMV was defined as duration of MV >14 days. Potential predictors for PMV were evaluated by Cox regression. RESULTS: A total of 594 GBS patients aged ≥6 years old were enrolled; of whom 541 patients had complete EGRIS data prior to MV and were included in validation analysis. EGRIS correctly distinguished between patients requiring MV or not in 81% pairs (AUC = 0.81). EGRIS overestimated the probability of MV than the observed probability (41% vs. 20%) which was resolved by updating of the model intercept. Inability to flex hip at day 7 of start of MV was the strongest predictor for PMV with predicted probabilities of 82%. INTERPRETATION: EGRIS accurately predicts the need for MV in GBS patients from Bangladesh. This study developed a region-specific version of EGRIS and identified predictors of PMV. These findings can assist clinicians to identify patients at high risk of developing respiratory failure and requiring PMV to ensure timely intubation and tracheostomy of the patients in low resource settings.
Grosu AV, Gheorghe RO, Filippi A
… +4 more, Deftu AF, Isler M, Suter M, Ristoiu V
J Peripher Nerv Syst
· 2024 Dec · PMID 39581686
·
Full text
BACKGROUND AND AIMS: Colony-stimulating factor 1 (CSF1) is a growth factor secreted by dorsal root ganglia (DRG) neurons important for DRG macrophages and spinal cord (SC) microglia injury-induced proliferation and activ...BACKGROUND AND AIMS: Colony-stimulating factor 1 (CSF1) is a growth factor secreted by dorsal root ganglia (DRG) neurons important for DRG macrophages and spinal cord (SC) microglia injury-induced proliferation and activation, specifically released after spared nerve injury (SNI). In this study, we investigated if SNI-induced CSF1 expression and perineuronal rings of macrophages around mouse DRG neurons vary between L3-L5 DRG and with the neuronal type, and if the CSF1 neuronal projections at the SC dorsal horns were associated with an increased microglial number in the corresponding laminae. METHODS: Seven days after surgery, L3-L5 DRG as well as their corresponding segments at the SC level were collected, frozen, and cut. DRG sections were double-immunostained using antibodies against CSF1 and NF200, CGRP or IB4, while SC sections were immunostained using a fluorescent Nissl Stain and analyzed for CX3CR1-GFP microglia number and distribution by an in-house ImageJ Plug-in. RESULTS: Our results showed that SNI-induced CSF1 expression was common for all subtypes of mouse DRG neurons, being responsible for attracting more resident macrophages around them in a DRG-dependent manner, with L4 showing the stronger response and CSF1/NF200 neurons showing the highest incidence. Even though the total number of microglia in the SC ipsilateral dorsal horns increased after SNI, the increase at their specific laminar projection sites did not mirror the incidence of DRG neuronal subtypes among CSF1 neurons. INTERPRETATION: Taken together, these results contribute to a more comprehensive understanding of the connection between CSF1 and macrophage/microglia response after SNI and emphasize the importance of considering L3-L5 DRG individually when investigating SNI-neuropathic pain pathogenesis in mice.
Idiaquez JF, Barnett-Tapia C, Perkins BA
… +1 more, Bril V
J Peripher Nerv Syst
· 2024 Dec · PMID 39532698
·
Full text
BACKGROUND AND AIMS: Small-fibre neuropathy (SFN) is associated with glucose dysregulation, including impaired glucose tolerance (IGT) and type 2 diabetes (T2D). Corneal confocal microscopy (CCM) offers a non-invasive to...BACKGROUND AND AIMS: Small-fibre neuropathy (SFN) is associated with glucose dysregulation, including impaired glucose tolerance (IGT) and type 2 diabetes (T2D). Corneal confocal microscopy (CCM) offers a non-invasive tool to assess corneal nerve damage and dendritic cell density (DCD). In this study, we investigated corneal DCD in patients with SFN and glucose dysregulation, defined as IGT or T2D. METHODS: We enrolled 38 patients with SFN + glucose dysregulation, 51 with SFN + non-glucose dysregulation and 20 healthy controls. All participants underwent neurological examination, neurophysiology and CCM. RESULTS: Individuals with SFN and glucose dysregulation had higher DCD compared with healthy controls (p = .01), and mature DCD was higher in IGT SFN patients than in T2D patients. INTERPRETATION: Higher DCD in IGT compared with controls and patients with established T2D may suggest that DCD is a biomarker of early neuropathy.