Journal of the Peripheral Nervous System最新文献_第10页

Distinguishing Chronic Inflammatory Demyelinating Polyneuropathy From Mimic Disorders: The Role of Statistical Modeling 区分慢性炎症性脱髓鞘性多神经病变与模拟疾病：统计模型的作用。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-01-12 DOI: 10.1111/jns.12682

Grace Swart, Michael P. Skolka, Shahar Shelly, Richard A. Lewis, Jeffrey A. Allen, Divyanshu Dubey, Zhiyv Niu, Judith Spies, Ruple S. Laughlin, Smathorn Thakolwiboon, Ashley R. Santilli, Hebatallah Rashed, Igal Mirman, Alexander Swart, Sarah E. Berini, Kamal Shouman, Marcus V. Pinto, Michelle L. Mauermann, John R. Mills, P. James B. Dyck, William S. Harmsen, Jay Mandrekar, Christopher J. Klein

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.

背景与目的：慢性炎症性脱髓鞘性多根神经病变（CIDP）与模拟性疾病难以区分，常因误诊而导致IVIG的过度使用。我们评估了一种临床电生理模型，以促进CIDP与模拟神经病变的预测。方法：根据欧洲神经病学学会/周围神经学会（EAN/PNS） 2021年CIDP指南，我们导出了26个临床和144个神经传导变量。利用总CIDP （n = 129）和mimics （n = 309）生成模型并进行验证；包括(1)IgG4-nodopathies；(2) POEMS（多发性神经病-器官肿大-内分泌病-单克隆蛋白-皮肤变化）；(3) anti-myelin-associated-glycoprotein;(4)多种;(5) Waldenström b细胞淋巴瘤；(6)糖尿病性神经病；(7)淀粉样变;(8);腓骨肌萎缩(9)运动神经病/神经病变；（10）特发性炎症性肌病。结果：我们分析了9282个临床和51 408个电生理数据点。单因素分析发现26个临床变量中有11个具有显著优势比。使用4个临床和2个电生理变量的多变量回归模型达到了93%的曲线下面积（95% CI 91-95）： 8周内的进展（OR 40.66, 95% CI 5.31-311.36），无自主神经受累（OR 17.82, 95% CI 2.93-108.24），无肌肉萎缩（OR 16.65, 95% CI 3.27-84.73），近端无力（OR 3.63, 95% CI 1.58-8.33），尺侧运动传导速度减慢使用临床电生理变量的概率计算器有助于将CIDP与模拟患者区分开来，得分低于92%的患者不太可能患有CIDP。最高的特异性是通过考虑临床“危险信号”、电生理脱髓鞘和实验室检测来实现的。

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引用次数: 0

Minimal invasive biopsies are highly sensitive for amyloid detection in hereditary transthyretin amyloidosis with polyneuropathy 微创活检是高度敏感的淀粉样蛋白检测遗传性转甲状腺蛋白淀粉样变性合并多发性神经病变。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-01-12 DOI: 10.1111/jns.12680

Luca Leonardi, Clovis Adam, Guillemette Beaudonnet, Diane Beauvais, Cécile Cauquil, Adeline Not, Olivier Morassi, Olivier Trassard, Andoni Echaniz-Laguna, David Adams, Céline Labeyrie

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.

目的：评价单用唇小涎腺活检（LSGB）或联合穿孔皮肤活检（SB）检测遗传性甲状腺转蛋白淀粉样变性伴多神经病变（ATTRv-PN）患者淀粉样蛋白沉积的有效性。方法：在这项单中心回顾性研究中，对2012年至2023年连续评估的ATTRv-PN患者进行微创LSGB （4 mm）和SB （3 mm）刚刚红染色。结果：171例ATTRv-PN的组织病理学资料，其中早发型p.Val50Met 49例，晚发型p.Val50Met 58例，非p.Val50Met 64例。Val50Met，进行了审查。LSGB和SB分别在123/171（72%）和131/171（77%）患者中发现淀粉样蛋白沉积（p = 0.2）。结合LSGB和SB可使淀粉样蛋白检出率提高到150/171(88%)，尤其是在迟发性p.Val50Met（48/58[83%]）和非p。Val50Met患者（55/64[86%]）。LSGB和SB在早发性p.Val50Met患者中淀粉样蛋白沉积的检出率相似（94%）。此外，LSGB/SB联合检测在89%（55/62）的早期ATTRv-PN患者中发现了淀粉样变性。结论：在我们的研究中，结合LSGB和SB可以在88%的ATTRv-PN患者中检测到淀粉样蛋白沉积。LSGB/SB分析可能是确认ATTRv-PN在非常早期进入疾病的主要兴趣，对疾病改善治疗的开始具有意义。

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引用次数: 0

Ofatumumab for treating autoimmune nodopathy Ofatumumab用于治疗自身免疫性肿瘤病。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-01-12 DOI: 10.1111/jns.12679

Jianian Hu, Yongsheng Zheng, Chong Sun, Jian Sun, Jianying Xi, Sushan Luo, Kai Qiao, Chongbo Zhao, Jie Lin

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.

背景和目的研究自身免疫性结节病（AN）患者使用伊妥单抗的治疗方法：在AN患者中开展了一项开放标签、前瞻性、观察性研究。治疗方案为在第0、7、14、28天皮下注射20毫克的ofatumumab，随后每4周一次，共24周。研究的主要终点是证实临床症状得到改善的患者比例：8名患者（100%）均在第24周时病情有所好转。病情好转的中位时间为 8 周（IQR：7-10 周）。之前接受过利妥昔单抗治疗的四名患者和不规则注射 OFatumumab (OFA) 的两名患者病情均有改善。第24周时，调整后的INCAT评分、MRC总分、cI-RODS和非支配手握力与基线相比均有显著改善。在神经传导研究中，有数据可查的六名患者（100%）的病情均有所改善。第 8 周时，sNfL 中位数较基线明显降低。7名患者的抗NF155抗体在第20周时比基线降低。8名患者中有7名患者的CD19+ B细胞在第4周时明显减少。无严重不良事件报告：治疗方案为第0、7、14、28天皮下注射20毫克ofatumumab，从第4周开始每4周一次，共24周。ofatumumab疗法可为AN患者提供更方便、更安全的治疗，同时也可作为利妥昔单抗无效患者的有效替代疗法。

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引用次数: 0

Abstracts of the 35th Annual Meeting of the Japanese Peripheral Nerve Society (JPNS) 日本外周神经学会第35届年会摘要。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-01-09 DOI: 10.1111/jns.12678

September 6–7, 2024

Kagoshima, Japan

President of JPNS: Ken-ichi Kaida

Congress Chair: Hiroshi Takashima

Scientific Committee (Editors of JPNS): Kazunori Sango, Yoshiki Sekijima, Shigeru Kurimoto, Ayato Hayashi, Ryosuke Ikeguchi, Norimasa Iwasaki, Haruki Koike, Norito Kokubun, Hiroki Mizukami, Yasumasa Nishiura, Akinori Sakai, Kazuma Sugie, Hiroshi Takashima

Organizing Committee: Hiroshi Takashima, Akihiro Hashiguchi, Yujiro Higuchi, Masahiro Ando

JPNS Editorial Staff: Kana Shimada

JPNS Secretariat: Munehisa Izuno, Haruna Tanaka

Organizing Secretariat: www.congre.co.jp/jpns2024/

2024年9月6日至7日，日本鹿儿岛JPNS会长：kaikenichi大会主席：高岛浩科学委员会（JPNS编辑）：sangunori, Sekijima，栗本茂，hayato Hayashi，池口良介，岩崎守正，小池春树，国本典，水上博树，西村康正，堺明典，Sugie Kazuma，高岛浩JPNS编辑人员：岛名ajpns秘书处：出野宗久，田中春，组织秘书处：www.congre.co.jp/jpns2024/

引用次数: 0

Validation of the Korean version of inflammatory Rasch-built Overall Disability Scale in patients with inflammatory neuropathy 韩式炎性rasch量表在炎性神经病变患者中的应用验证。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2024-12-10 DOI: 10.1111/jns.12676

Woohee Ju, Young Gi Min, Jong Su Kim, Jiwon Choi, Jiwon Lee, Seok-Jin Choi, Sung-Min Kim, Yoon-Ho Hong, Jung-Joon Sung

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.

背景和目的：炎症性拉希德构建的整体残疾量表（I-RODS）是一种用于炎性周围神经病变的有效活动测量方法。本研究的目的是验证韩国版I-RODS在慢性炎症性脱髓鞘多神经病变（CIDP）、格林-巴勒综合征（GBS）、抗髓鞘相关糖蛋白（MAG）神经病变和自身免疫性淋巴结病患者中的应用。方法：对120例患者进行临床评估，包括i - rod、炎症性神经病变病因与治疗（INCAT）评估和Jamar握力（kg）测量。对83例患者在常规门诊就诊期间进行随访评估。为了估计I-RODS的重测可靠性，在初始测试后2-7天内将量表重新应用于16名患者的子集。总体而言，评估了i - rod的信度、效度和反应性。结果：内部一致性好，人分离指数为0.966。原始和标准化Cronbach’s alpha值均为0.974。用类内相关系数（ICC）分析的重测信度也很高（ICC = 0.972）。I-RODS与INCAT评分有很强的相关性（ρ = -0.81, p）解释：韩国版I-RODS是测量炎症性神经病变患者残疾的可靠有效工具。I-RODS对临床实践和研究应用都很有用。

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引用次数: 0

Digital nerve reconstruction with a new composite silk fibroin nerve conduit 用新型复合丝纤维神经导管重建数字神经。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2024-11-26 DOI: 10.1111/jns.12675

Olga Politikou, Florian S. Frueh, Martina Greminger, Inga S. Besmens, Giuliano Freddi, Antonio Alessandrino, Maurizio Calcagni

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.

背景和目的：在无法进行直接修复的情况下，周围神经损伤往往需要桥接。神经自体移植是金标准，但可能导致供体部位发病。基于蚕丝纤维素的神经导管（如新型 SILKBridge）是一种很有前景的替代方法。这项试验性研究评估了首次使用 SILKBridge 进行人体数字神经重建的中期效果，重点关注感觉恢复、并发症发生率、患者报告结果和生物整合：这项研究包括四名使用 SILKBridge 重建数字神经缺损的患者。临床评估包括两点辨别、Semmes-Weinstein 单丝测试以及使用数字评分量表进行疼痛评估。此外，还进行了声学评估，以评估导管的生物整合性和潜在并发症：结果：在平均 32 个月的随访中，所有患者都表现出令人满意的感觉恢复，并报告称疼痛极轻甚至没有疼痛。声学评估证实生物整合效果良好，没有炎症或疤痕迹象：对首次使用 SILKBridge 进行人体数字神经重建的中期评估显示，该技术具有安全性、高效性和良好的生物相容性。为了验证这些研究结果并将其与其他神经修复方法进行比较，还需要进行更大规模的研究。

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引用次数: 0

Dorsal root ganglia CSF1+ neuronal subtypes have different impact on macrophages and microglia after spared nerve injury 神经损伤后，背根神经节 CSF1+ 神经元亚型对巨噬细胞和小胶质细胞的影响不同。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2024-11-24 DOI: 10.1111/jns.12674

Andreea Violeta Grosu, Roxana-Olimpia Gheorghe, Alexandru Filippi, Alexandru Florian Deftu, Manon Isler, Marc Suter, Violeta Ristoiu

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.

背景和目的：集落刺激因子1（CSF1）是一种由背根神经节（DRG）神经元分泌的生长因子，对DRG巨噬细胞和脊髓（SC）小胶质细胞损伤诱导的增殖和活化非常重要，特别是在神经损伤（SNI）后释放。在这项研究中，我们研究了SNI诱导的CSF1表达和小鼠DRG神经元周围的巨噬细胞神经元环是否在L3-L5 DRG之间和随神经元类型而变化，以及SC背角的CSF1+神经元突起是否与相应神经层的小胶质细胞数量增加有关：方法：手术七天后，收集、冷冻和切割 L3-L5 DRG 及其 SC 水平的相应节段。用 CSF1 和 NF200、CGRP 或 IB4 抗体对 DRG 切片进行双重免疫染色，用荧光 Nissl 染色法对 SC 切片进行免疫染色，并用内部 ImageJ 插件分析 CX3CR1-GFP 小胶质细胞的数量和分布：结果：我们的研究结果表明，SNI诱导的CSF1表达在小鼠DRG神经元的所有亚型中都很常见，以DRG依赖的方式吸引更多的巨噬细胞驻留在神经元周围，其中L4神经元的反应更强烈，CSF1+/NF200+神经元的发生率最高。尽管SC同侧背角的小胶质细胞总数在SNI后有所增加，但其特定板层投射部位的增加并不反映CSF1+神经元中DRG神经元亚型的发生率：综上所述，这些结果有助于更全面地了解SNI后CSF1与巨噬细胞/小胶质细胞反应之间的联系，并强调了在研究小鼠SNI-神经性疼痛发病机制时单独考虑L3-L5 DRG的重要性。

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引用次数: 0

Prediction of respiratory failure and prolonged mechanical ventilation in Guillain-Barré syndrome: A prospective cohort study in Bangladesh 吉兰-巴雷综合征呼吸衰竭和长期机械通气的预测：孟加拉国前瞻性队列研究。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2024-11-24 DOI: 10.1111/jns.12673

Nowshin Papri, Alex Y. Doets, Linda Luijten, Quazi D. Mohammad, Hubert P. Endtz, Hester F. Lingsma, Bart C. Jacobs, Zhahirul Islam

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.

背景和目的：本研究旨在对伊拉斯谟 GBS 呼吸功能不全评分（EGRIS）进行验证和地区性调整，并确定孟加拉吉兰-巴雷综合征（GBS）患者长期机械通气（PMV）的潜在预测因素：我们从孟加拉国进行的四项前瞻性观察队列研究中招募了吉兰-巴雷综合征患者。EGRIS 预测 14 天内 MV 需求的准确性。通过 Cox 回归评估了 PMV 的潜在预测因素：共招募了 594 名年龄≥6 岁的 GBS 患者；其中 541 名患者在 MV 前拥有完整的 EGRIS 数据，并被纳入验证分析。EGRIS 能正确区分 81% 的患者是否需要 MV（AUC = 0.81）。与观察到的概率相比，EGRIS 高估了 MV 的概率（41% 对 20%），通过更新模型截距解决了这一问题。在 MV 开始的第 7 天，髋关节不能屈曲是 PMV 的最强预测因子，预测概率为 82%：EGRIS准确预测了孟加拉国GBS患者对中风的需求。这项研究开发了针对特定地区的 EGRIS 版本，并确定了 PMV 的预测因素。这些发现有助于临床医生识别呼吸衰竭高风险患者和需要进行 MV 的患者，以确保在资源匮乏的环境中为患者及时插管和进行气管造口术。

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引用次数: 0

Assessing corneal dendritic cells in glucose dysregulation small-fibre neuropathy 评估葡萄糖失调小纤维神经病变中的角膜树突状细胞。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2024-11-12 DOI: 10.1111/jns.12671

Juan Francisco Idiaquez, Carolina Barnett-Tapia, Bruce A. Perkins, Vera Bril

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.

背景和目的：小纤维神经病变（SFN）与葡萄糖失调有关，包括糖耐量受损（IGT）和 2 型糖尿病（T2D）。角膜共聚焦显微镜（CCM）是评估角膜神经损伤和树突状细胞密度（DCD）的非侵入性工具。在这项研究中，我们调查了 SFN 和血糖失调（定义为 IGT 或 T2D）患者的角膜 DCD：我们招募了 38 名 SFN + 血糖失调患者、51 名 SFN + 非血糖失调患者和 20 名健康对照者。所有参与者均接受了神经系统检查、神经电生理学检查和CCM检查：结果：与健康对照组相比，SFN 和血糖失调患者的 DCD 更高（P = .01），IGT SFN 患者的成熟 DCD 比 T2D 患者更高：与对照组和已确诊的 T2D 患者相比，IGT 患者的 DCD 较高，这可能表明 DCD 是早期神经病变的生物标志物。

{"title":"Assessing corneal dendritic cells in glucose dysregulation small-fibre neuropathy","authors":"Juan Francisco Idiaquez, Carolina Barnett-Tapia, Bruce A. Perkins, Vera Bril","doi":"10.1111/jns.12671","DOIUrl":"10.1111/jns.12671","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>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.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Individuals with SFN and glucose dysregulation had higher DCD compared with healthy controls (<i>p</i> = .01), and mature DCD was higher in IGT SFN patients than in T2D patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Higher DCD in IGT compared with controls and patients with established T2D may suggest that DCD is a biomarker of early neuropathy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"29 4","pages":"400-405"},"PeriodicalIF":3.9,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11625976/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142622992","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

Long-term safety and tolerability of hyaluronidase-facilitated subcutaneous immunoglobulin 10% as maintenance therapy for chronic inflammatory demyelinating polyradiculoneuropathy: Results from the ADVANCE-CIDP 3 trial 透明质酸酶促进的 10%皮下免疫球蛋白作为慢性炎症性脱髓鞘多发性神经病维持疗法的长期安全性和耐受性：ADVANCE-CIDP 3试验结果。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2024-11-11 DOI: 10.1111/jns.12672

Robert D. M. Hadden, Henning Andersen, Vera Bril, Ivana Basta, Konrad Rejdak, Kim Duff, Erin Greco, Shabbir Hasan, Colin Anderson-Smits, Hakan Ay

Background and Aims

Hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) consists of subcutaneous human immunoglobulin G (IgG) 10% with recombinant human hyaluronidase (rHuPH20) and can be administered at the same dose and interval as intravenous IgG (IVIG). fSCIG recently received US approval as maintenance therapy for adults with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and European approval for adults and children with CIDP after stabilization with IVIG.

Methods

ADVANCE-CIDP 3 (NCT02955355) was an open-label long-term extension of the Phase 3 double-blind randomized placebo-controlled ADVANCE-CIDP 1 study (NCT02549170) that examined fSCIG safety and efficacy as maintenance CIDP therapy. Primary outcomes were safety, tolerability, and immunogenicity. Efficacy was an exploratory outcome.

Results

The study provided 220 patient-years of follow-up data from 85 patients. Median (range) exposure was 33 (0–77) months. Patients received fSCIG every 4 weeks (88.2%) or every 3 weeks (11.8%). Median (range) 4-weekly IgG dose equivalent was 64.0 (28.0–200.0) g. Mean (standard deviation) infusion duration was 135.5 (62.8) minutes. Most adverse events (AEs) were mild or moderate and self-limiting. Of the 1406 AEs, only 48 were severe and 30 were serious. fSCIG-related AEs (n = 798) included infusion site reactions such as pain, redness, and pruritus. Three infusions (0.1%) were reduced in rate, interrupted, or stopped due to intolerability. Relapse occurred in 10 of 77 patients (13.0%); annual relapse rate was 4.5%. An anti-rHuPH20 antibody titer ≥1:160 was detected in 14 of 84 patients (16.7%); patients who tested positive (≥1:160) had similar relapse rates versus those who tested negative (16.7% vs. 12.3%, respectively).

Interpretation

ADVANCE-CIDP 3 demonstrated favorable fSCIG long-term safety and tolerability consistent with its established safety profile, and a low relapse rate, supporting use as maintenance CIDP treatment.

背景和目的：透明质酸酶促进皮下免疫球蛋白（fSCIG）由10%的重组人透明质酸酶（rHuPH20）和皮下注射人免疫球蛋白G（IgG）组成，其给药剂量和间隔与静脉注射IgG（IVIG）相同。fSCIG 最近获得美国批准，可作为慢性炎症性脱髓鞘多发性神经病（CIDP）成人患者的维持疗法，并获得欧洲批准，可用于 IVIG 稳定后的 CIDP 成人和儿童患者：ADVANCE-CIDP 3（NCT02955355）是3期双盲随机安慰剂对照ADVANCE-CIDP 1研究（NCT02549170）的一项开放标签长期扩展研究，该研究考察了fSCIG作为CIDP维持疗法的安全性和有效性。主要结果是安全性、耐受性和免疫原性。疗效是一项探索性结果：该研究提供了 85 名患者 220 年的随访数据。中位（范围）暴露时间为 33（0-77）个月。患者每 4 周（88.2%）或每 3 周（11.8%）接受一次 fSCIG 治疗。平均（标准差）输注时间为 135.5（62.8）分钟。大多数不良反应（AEs）为轻度或中度，且具有自限性。与 fSCIG 相关的不良反应（n = 798）包括输液部位反应，如疼痛、发红和瘙痒。有 3 例输液（0.1%）因不能耐受而降低输液速度、中断或停止。77 例患者中有 10 例（13.0%）复发；年复发率为 4.5%。84例患者中有14例（16.7%）检测到抗rHuPH20抗体滴度≥1:160；检测结果为阳性（≥1:160）的患者与检测结果为阴性的患者复发率相似（分别为16.7%和12.3%）：ADVANCE-CIDP 3证明了fSCIG良好的长期安全性和耐受性，符合其已建立的安全性特征，而且复发率低，支持将其用作CIDP的维持治疗。

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引用次数: 0