Journal of the Peripheral Nervous System最新文献_第8页

A Case Series of Unilateral Peripheral Neuropathy 单侧周围神经病变1例

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-26 DOI: 10.1111/jns.70033

Caroline Kramarz, Marion Masingue, Françoise Bouhour, Christophe Vial, Philippe Latour, Christophe Vandendries, Thierry Maisonobe, Jan Coebergh, Julian Blake, Mary M. Reilly, Tanya Stojkovic, Alexander M. Rossor

Background and Aims

Peripheral neuropathy may present with a variety of phenotypes depending on the pattern of weakness and sensory loss, the neurophysiological characteristics (axonal or demyelinating) and additional features such as involvement of the autonomic nervous system or the cranial nerves. The most common phenotype is a symmetrical length-dependent sensory and motor neuropathy. Other phenotypes include non-length-dependent forms such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) or a sensory neuronopathy or ganglionopathy. Asymmetric forms of neuropathy are mostly represented by mononeuritis multiplex and Lewis-Sumner syndrome or focal CIDP. Unilateral weakness or sensory loss respecting the midline is mainly due to pathology in the central nervous system and is unusual in peripheral neuropathy.

Methods

We evaluated the clinical and genetic features of three unrelated individuals with a peripheral neuropathy affecting one side of the body.

Results

We describe three unrelated patients (two female and one male) with a slowly progressive peripheral neuropathy restricted to one side of the body. Each case is marked by onset in early childhood with the absence of a family history or a structural lesion of the central nervous system. Neurophysiology demonstrated an axonal type of neuropathy in two cases and conduction slowing supportive of a demyelinating neuropathy type in one. Genetic testing was performed in the three cases, specifically looking for variants in genes associated with Charcot-Marie-Tooth disease (CMT) but none were identified in DNA extracted from blood.

Interpretation

A unilateral, slowly progressive peripheral neuropathy is a rare phenomenon, and we propose the cause of this unusual phenotype to be due to a mosaic or chimeric form of Charcot-Marie-Tooth disease (CMT).

背景和目的周围神经病变可能表现为多种表型，这取决于虚弱和感觉丧失的模式、神经生理特征（轴突或脱髓鞘）和其他特征，如自主神经系统或颅神经的受累。最常见的表型是对称长度依赖的感觉和运动神经病变。其他表型包括非长度依赖性形式，如慢性炎症性脱髓鞘性多根神经病变（CIDP）或感觉神经病变或神经节病变。不对称形式的神经病变主要以多发性单神经炎和Lewis-Sumner综合征或局灶性CIDP为代表。关于中线的单侧无力或感觉丧失主要是由于中枢神经系统的病理，在周围神经病变中并不常见。方法我们评估了三个不相关的影响身体一侧的周围神经病变个体的临床和遗传特征。结果我们描述了三名不相关的患者（两名女性和一名男性）缓慢进展的周围神经病变局限于身体的一侧。每个病例的特点是在儿童早期发病，没有家族史或中枢神经系统的结构性病变。神经生理学证实两例为轴突型神经病，一例为脱髓鞘性神经病。对三个病例进行了基因检测，专门寻找与腓骨肌萎缩症（CMT）相关的基因变异，但从血液中提取的DNA中没有发现任何变异。单侧，缓慢进展的周围神经病变是一种罕见的现象，我们提出这种不寻常的表型的原因是由于马赛克或嵌合形式的沙科-玛丽-图斯病（CMT）。

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引用次数: 0

Validity and Responsiveness of Balance Measurements Using Posturography in Patients With Immune-Mediated Neuropathies 在免疫介导的神经病变患者中使用姿势测量平衡的有效性和反应性

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-26 DOI: 10.1111/jns.70031

Milou R. Michael, Robin van Veen, Luuk Wieske, Ingemar S. J. Merkies, Ivo N. van Schaik, Filip Eftimov

Background and Aims

Validated objective measures for balance in immune mediated neuropathies are lacking. In this study, we investigated the clinimetric properties of posturography using a force platform, a quantitative assessment of postural control.

Methods

We assessed patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and IgM-related polyneuropathy (IgM-PNP) using sway parameters (path, area and amplitude) measured at multiple time points. Validity was investigated by assessing differences in sway path between patients with and without reported balance symptoms and by assessing correlations of sway path with (established) impairment measures related to balance, disability and quality of life (QoL). Responsiveness was assessed by means of an anchor-based approach, using a patient anchor and two disability scales.

Results

We included 52 CIDP and 13 IgM-PNP patients. In CIDP, sway path was 25% longer in patients reporting balance symptoms relative to patients without balance symptoms (p = 0.03). There was excellent reliability between consecutive measurements in both CIDP and IgM-PNP. Moderate to good correlations were observed between sway path and an ataxia scale (CIDP: Spearman's ρ = 0.46, 95% CI: 0.2–0.69; IgM-PNP: Spearman's ρ = 0.72, 95% CI: 0.28–0.96) while correlations with related disability measures and QoL were poor. Changes in sway parameters over time were not consistently associated with changes in other outcome measures.

Interpretation

Posturography measurements showed poor validity and responsiveness. Therefore, despite excellent reliability, using a force platform in clinical practice or trials for immune-mediated neuropathies cannot be recommended.

背景和目的免疫介导的神经病变缺乏有效的客观测量方法。在这项研究中，我们使用一个力平台来研究姿势测量的临床特性，这是一种对姿势控制的定量评估。方法对慢性炎症性脱髓鞘性多神经病变（CIDP）和igm相关性多神经病变（IgM-PNP）患者采用多个时间点的摇摆参数（路径、面积和振幅）进行评估。通过评估有和没有报告平衡症状的患者之间摇摆路径的差异，以及评估摇摆路径与（已建立的）与平衡、残疾和生活质量（QoL）相关的损害措施的相关性，来调查有效性。反应性通过基于锚点的方法进行评估，使用患者锚点和两个残疾量表。结果纳入52例CIDP和13例IgM-PNP患者。在CIDP中，有平衡症状的患者的摇摆路径比没有平衡症状的患者长25% （p = 0.03）。在CIDP和IgM-PNP连续测量之间有极好的可靠性。在摇摆路径和共济失调量表之间观察到中度至良好的相关性(CIDP: Spearman's ρ = 0.46, 95% CI: 0.2-0.69；IgM-PNP: Spearman's ρ = 0.72, 95% CI: 0.28-0.96)，而相关残疾措施和生活质量的相关性较差。随着时间的推移，摇摆参数的变化与其他结果测量的变化并不一致。解释姿势测量显示较差的有效性和反应性。因此，尽管具有良好的可靠性，在临床实践或免疫介导的神经病变试验中使用力平台是不推荐的。

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引用次数: 0

SLC5A6 Mutations in Axonal Sensorimotor Polyneuropathy Patients Concurrent With Sodium Dependent Multivitamin Transporter Deficiency and Improved Effects by Multivitamin Therapy SLC5A6突变并发钠依赖性多种维生素转运体缺乏的轴突感觉运动多发性神经病患者及多种维生素治疗改善效果

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-21 DOI: 10.1111/jns.70030

Byung Kwon Pi, Ah. Jin Lee, Soo Hyun Nam, Ki Wha Chung, Byung-Ok Choi

Background and Aims

The SLC5A6 gene encodes a transmembrane protein responsible for transporting biotin, pantothenic acid, and lipoic acid. Mutations in SLC5A6 have shown a wide spectrum of clinical phenotypes, such as sodium-dependent multivitamin transporter deficiency (SMVTD), childhood-onset biotin-responsive peripheral motor neuropathy (COMNB), and mixed axonal and demyelinating sensory motor neuropathy. The purpose of this study was to identify pathogenic SLC5A6 mutations in the Korean CMT cohort.

Methods

This study performed whole exome sequencing to identify the genetic cause for two independent patients with early onset axonal sensorimotor polyneuropathy and SMVTD. We also examined the therapeutic effects of multivitamin replenishment on a patient with SLC5A6 mutations.

Results

We identified compound heterozygous variants of SLC5A6 in two patients (p.Arg94X and p.Phe522Ser in patient 1; p.Cys443Tyr and p.Phe513_Lys515delinsLeu in patient 2). In patient 2, an oral regimen comprising biotin, lipoic acid, and pantothenic acid demonstrated significant therapeutic effects, including cessation of cyclic vomiting, resolution of skin lesions on the fingers, and improvements in muscle weakness affecting both the upper and lower extremities.

Interpretation

This study represents the first report of novel heterozygous SLC5A6 mutations in patients with axonal CMT and SMVTD, expanding the phenotypic spectrum associated with SLC5A6 mutations. Notably, we observed significant therapeutic effects from multivitamin treatment in a patient.

背景和目的SLC5A6基因编码一种跨膜蛋白，负责运输生物素、泛酸和硫辛酸。SLC5A6突变已显示出广泛的临床表型，如钠依赖性多维生素转运体缺乏症（SMVTD）、儿童期发病的生物素反应性周围运动神经病变（COMNB）以及混合性轴突和脱髓鞘感觉运动神经病变。本研究的目的是鉴定韩国CMT队列中的致病SLC5A6突变。方法对2例独立的早发性轴突感觉运动多神经病变和SMVTD患者进行全外显子组测序，确定遗传原因。我们还研究了补充多种维生素对SLC5A6突变患者的治疗效果。结果我们在2例患者中发现了SLC5A6的复合杂合变异体（p.Arg94X和p.Phe522Ser）；p. cyp443tyr和p.Phe513_Lys515delinsLeu（患者2）。在患者2中，由生物素、硫辛酸和泛酸组成的口服方案显示出显著的治疗效果，包括停止周期性呕吐，缓解手指皮肤病变，改善影响上肢和下肢的肌肉无力。本研究首次报道了轴突性CMT和SMVTD患者中新的杂合SLC5A6突变，扩大了与SLC5A6突变相关的表型谱。值得注意的是，我们观察到多种维生素治疗对患者的显著治疗效果。

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引用次数: 0

Soleal Sling Syndrome: A Narrative Review Soleal Sling综合征：叙述性回顾

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-20 DOI: 10.1111/jns.70032

Laura Hilbig-Vlatten, Jennifer N. Grauberger, Toni F. Engmann, Lilly F. Stadelmeier, Raymond M. Dunn, Ross Mandeville, Jason H. Ko, Pierre D'Hemecourt, Sammy Dowlatshahi

Soleal sling syndrome is a rare cause of lower extremity neuropathy due to compression of the proximal tibial nerve under the fibromuscular arch of the soleus muscle. It presents with plantar numbness/paresthesias, calf pain, and tenderness over the proximal calf. Chronic compression can lead to toe flexor weakness. This study reviews the clinical presentation, diagnostic workup, and treatment options for soleal sling syndrome. A query of the PubMed database up until August 30, 2022, was conducted to gather relevant clinical, anatomic, and radiographic findings. The literature review identified key features of soleal sling syndrome, highlighting the importance of considering it in patients with calf pain/tenderness and plantar foot neurosensory changes. Diagnosis typically relies on history and physical examination, often with a positive Tinel's sign, though imaging modalities show inconsistent utility. Soleal sling syndrome is underrecognized and overlaps with other syndromes. Radiological imaging modalities can rule out secondary causes of proximal tibial nerve compression but lack consistency for diagnosing idiopathic cases. Surgical decompression of the nerve, via a medial or posterior approach, is the definitive treatment for all causes of tibial nerve compression.

摘要比目鱼肌悬吊症候群是一种罕见的下肢神经病变，其原因是比目鱼肌纤维肌弓下的胫近端神经受到压迫。表现为足底麻木/感觉异常，小腿疼痛，小腿近端压痛。慢性压迫可导致脚趾屈肌无力。本研究回顾了soleal sling综合征的临床表现、诊断检查和治疗方案。对PubMed数据库进行查询，直到2022年8月30日，收集相关的临床、解剖和放射学结果。文献综述确定了脚底悬吊综合征的主要特征，强调了在小腿疼痛/压痛和足底神经感觉改变的患者中考虑它的重要性。诊断通常依赖于病史和体格检查，通常有阳性的蒂内尔征象，尽管影像学表现不一致。Soleal sling综合征未被充分认识，并与其他综合征重叠。影像学检查可以排除胫骨近端神经压迫的继发原因，但对特发性病例的诊断缺乏一致性。手术减压神经，通过内侧或后方入路，是确定的治疗胫骨神经压迫的所有原因。

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引用次数: 0

Feasibility, Validity, and Reliability of the Virtual CMT Infant Toddler Scale (vCMTInfS): A Remote Evaluation of Infants/Toddlers With CMT 虚拟CMT婴幼儿量表（vCMTInfS）的可行性、效度和信度：对CMT婴幼儿的远程评估

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-20 DOI: 10.1111/jns.70029

Rosemary Shy, Amanda Dragon, Shawna M. E. Feely, Gabrielle Donlevy, Kayla Cornett, Melissa Mandarakas, Tim Estilow, Joshua Burns, Michael E. Shy

Background and Aims

The CMT Infant Scale (CMTInfS) enables evaluation of infants/toddlers in clinic. Our aim was to evaluate the feasibility, reliability, and validity of a virtual version of the CMTInfS (vCMTInfS).

Methods

Children aged 55 months or less were evaluated either in clinic using CMTInfS or remotely via telemedicine using the vCMTInfS. A trained clinical evaluator remotely directed activities with assistance from the parent/caregiver. vCMTInfS scores were calculated using the CMTInfS calculator available at www.ClinicalOutcomeMeasures.org. Clinical evaluators also used the Brazelton Neonatal Behavior assessment scale to give insight into the behavior of the child during the exam.

Results

Twenty children (10 males and 10 females) aged 6–55 months with confirmed or at risk for CMT were evaluated. The mean in person (IP) CMT Infant and Toddler Scale (CMTInfS) raw score (4.11, SD = 2.76) was not significantly different from the mean initial virtual (V1) CMTInfS raw score (3.78, SD = 2.59) using a two-tailed test (t = 1.000, p = 0.347). Differences between the first and second (V2) visits as well as between the IP and V2 visits were also nonsignificant.

Interpretation

Our data demonstrate that children aged 55 months or less can be effectively evaluated remotely using the vCMTInfS, which will expand the number of very young children who can be evaluated with rare forms of CMT.

背景与目的CMT婴儿量表（CMTInfS）用于临床对婴幼儿进行评估。我们的目的是评估虚拟版本的CMTInfS （vCMTInfS）的可行性、可靠性和有效性。方法对55月龄及以下的儿童进行临床和远程评估，分别采用CMTInfS和vCMTInfS。训练有素的临床评估员在父母/照顾者的协助下远程指导活动。vCMTInfS分数是使用可在www.ClinicalOutcomeMeasures.org上获得的CMTInfS计算器计算的。临床评估人员还使用Brazelton新生儿行为评估量表来深入了解孩子在考试期间的行为。结果对20例6-55月龄确诊或有CMT风险的儿童（男10名，女10名）进行了评估。使用双尾检验（t = 1.000, p = 0.347），面对面CMT婴幼儿量表（CMTInfS）原始得分均值（4.11,SD = 2.76）与初始虚拟（V1） CMTInfS原始得分均值（3.78,SD = 2.59）无显著差异。第一次和第二次（V2）访问之间以及IP和V2访问之间的差异也不显著。我们的数据表明，55个月以下的儿童可以使用vCMTInfS进行有效的远程评估，这将扩大可以用罕见形式的CMT评估非常年幼的儿童的数量。

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引用次数: 0

Correction to “Results From a Phase 1 Study Evaluating the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics, and Efficacy of ANX005, a C1q Inhibitor, in Patients With Guillain–Barré Syndrome” 对“评估C1q抑制剂ANX005在格林-巴罗综合征患者中的安全性、耐受性、药代动力学、药效学和疗效的1期研究结果”的更正

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-15 DOI: 10.1111/jns.70024

Q. D. Mohammad, Z. Islam, N., Papri, et al. “Results From a Phase 1 Study Evaluating the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics, and Efficacy of ANX005, a C1q Inhibitor, in Patients With Guillain–Barré Syndrome,” Journal of the Peripheral Nervous System 30 (2025): e70009. https://doi.org/10.1111/jns.70009

FIGURE 7 | Change in MRC from baseline for ANX005 and placebo (N = 26). MRC, Medical Research Council.

We apologize for this error.

Q. D. Mohammad， Z. Islam， N., Papri，等。“C1q抑制剂ANX005在格林-巴勒综合征患者中的安全性、耐受性、药代动力学、药效学和疗效的1期研究结果”，《外周神经系统杂志》30 (2025):e70009。https://doi.org/10.1111/jns.70009FIGURE 7 |与基线相比，ANX005和安慰剂的MRC变化（N = 26）。医学研究委员会我们为这个错误道歉。

引用次数: 0

Patient-Reported Outcome Measures for Assessing Health-Related Quality of Life in Patients With Polyneuropathies, Focusing on Guillain-Barré Syndrome and Chronic Inflammatory Demyelinating Polyneuropathy: A Systematic Review of Measurement Properties 评估多神经病变患者健康相关生活质量的患者报告结果测量，重点是格林-巴勒综合征和慢性炎症性脱髓鞘多神经病变：测量特性的系统回顾

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-14 DOI: 10.1111/jns.70022

Farah Pelouto, Adája E. Baars, Nowshin Papri, Juanita A. Haagsma, Bart C. Jacobs, Caroline B. Terwee

Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are immune-mediated peripheral neuropathies. Despite treatment, patients may report residual deficits, pain, and fatigue with considerable impact on quality of life. A systematic review was conducted of the methodological quality of current patient-reported outcome measures (PROMs) for measuring health-related quality of life (HRQoL) in patients with GBS and CIDP. A literature search was conducted in EMBASE, MEDLINE, Web of Science, and Google Scholar. PROMs developed to measure (aspects of) HRQoL in patients with polyneuropathy were classified using the Wilson and Cleary model. Measurement properties were evaluated in accordance with Consensus-based Standards for selection of health Measurement Instruments (COSMIN) guideline. A total of 57 articles identified 31 unique PROMs that are used for measuring HRQoL in patients with polyneuropathies. Of these, 22 measured symptom status, 19 functional status, and 4 general health perception. Eight PROMs were developed or validated in patients with GBS/CIDP. None of the PROMs demonstrated sufficient content validity for recommendation in this population. Only the Rasch-built Fatigue Severity Scale (R-FSS) performed sufficiently across all other measurement properties. The Inflammatory Rasch-built Overall Disability Scale (I-RODS) and IN-QoL are not recommended for use because of insufficient construct validity. GBS Patient Experience Questionnaire, Chronic Acquired Polyneuropathy Patient-Reported Index (CAP-PRI), Fatigue Severity Scale (FSS), R-FSS, Rotterdam Handicap Scale (RHS) and the 36-Item Short Form Health Survey (SF-36) need further validation. PROMs of good quality assessing all relevant aspects of HRQoL are required for better insight in HRQoL in patients with GBS and CIDP.

格林-巴利综合征（GBS）和慢性炎症性脱髓鞘多神经病变（CIDP）是免疫介导的周围神经病变。尽管进行了治疗，但患者可能会报告对生活质量有相当大影响的残余缺陷、疼痛和疲劳。对目前用于测量GBS和CIDP患者健康相关生活质量（HRQoL）的患者报告结果测量（PROMs）的方法学质量进行了系统评价。在EMBASE、MEDLINE、Web of Science和b谷歌Scholar中进行文献检索。采用Wilson和Cleary模型对多神经病变患者的HRQoL（各方面）进行分类。测量特性按照基于共识的卫生测量仪器选择标准（COSMIN）指南进行评估。共有57篇文章确定了31种独特的PROMs，用于测量多发性神经病患者的HRQoL。其中22项测量症状状态，19项测量功能状态，4项测量总体健康感知。在GBS/CIDP患者中开发或验证了8种PROMs。在这一人群中，没有一个prom显示出足够的内容效度。只有rasch构建的疲劳严重程度量表（R-FSS）在所有其他测量属性中表现充分。由于结构效度不足，不建议使用炎性rasch构建的整体残疾量表（I-RODS）和IN-QoL。GBS患者体验问卷、慢性获得性多神经病变患者报告指数（CAP-PRI）、疲劳严重程度量表（FSS）、R-FSS、鹿特丹残疾量表（RHS）和36项简短健康调查（SF-36）有待进一步验证。为了更好地了解GBS和CIDP患者的HRQoL，需要评估HRQoL所有相关方面的高质量prom。

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引用次数: 0

Advanced Hybrid Closed-Loop Insulin Delivery Is Associated With Improved Glycemic Indicators and Normalization of Small Nerve Fibre Structure in Adults With Type 1 Diabetes 先进的混合型闭环胰岛素递送与成人1型糖尿病患者血糖指标改善和小神经纤维结构正常化相关

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-14 DOI: 10.1111/jns.70026

Hoda Gad, Einas Elgassim, Kawsar Mohamed Ayon Mohamud, Najlaa Sultan Al-Naimi, Hamad Ali Al-Sharshani, Ibrahim Mohammed, Mariam A. Al-Malaheem, Dorothy J. Quadros, Alhanoof AlJalahma, Neila Lamine, Ahmad Yaser Alhaddad, Hussein Ahmed Hussein Zaky Aly, John-John Cabibihan, Abdulaziz Al-Ali, Kishor Kumar Sadasivuni, Ioannis N. Petropoulos, Georgios Ponirakis, Hamda A. Ali, Dabia AlMohanadi, Khaled Baagar, Rayaz A. Malik

Background

Hyperglycemia is a major driver of diabetic peripheral neuropathy (DPN) in type 1 diabetes mellitus (T1DM). Advanced hybrid closed-loop (AHCL) technologies improve glycemic control and reduce glycemic variability and may improve DPN.

Methods

Patients with T1DM treated for 9.8 ± 0.32 months with the 780G SmartGuard system (n = 14) were compared with patients with T1DM on MDI (n = 20) and healthy controls (n = 15). Time in range (TIR), time above range (TAR), time below range (TBR), glycemic variability, and HbA_1c were evaluated, and corneal confocal microscopy (CCM) was undertaken to quantify corneal nerve fiber density (CNFD), branch density (CNBD), fiber length (CNFL), and inferior whorl length (IWL).

Results

Participants using the 780G system had a significantly higher TIR (p < 0.001), lower glycemic variability (p = 0.02), and less time in level 2 hyperglycemia (p = 0.01), level 1 hyperglycemia (p = 0.04), and level 2 hypoglycemia (p = 0.008) compared with the MDI group. CNFD (p = 0.02), CNBD (p = 0.04), CNFL (p = 0.04), and IWL (p < 0.001) were significantly higher in the 780G group compared with the MDI group and comparable to healthy controls.

Conclusion

The MiniMed 780G with SmartGuard improves glycemic control and variability with an improvement in small nerve fiber morphology in patients with DPN.

背景：高血糖是1型糖尿病（T1DM）患者糖尿病周围神经病变（DPN）的主要驱动因素。先进的混合闭环（AHCL）技术改善血糖控制，降低血糖变异性，并可能改善DPN。方法将使用780G SmartGuard系统治疗9.8±0.32个月的T1DM患者（n = 14）与MDI治疗的T1DM患者（n = 20）和健康对照组（n = 15）进行比较。评估范围内时间（TIR）、范围上时间（TAR）、范围下时间（TBR）、血糖变异性和糖化血红蛋白（HbA1c），并采用角膜共聚焦显微镜（CCM）量化角膜神经纤维密度（CNFD）、分支密度（CNBD）、纤维长度（CNFL）和下轮长度（IWL）。结果与MDI组相比，使用780G系统的参与者有更高的TIR (p < 0.001)，更低的血糖变异性（p = 0.02），出现2级高血糖（p = 0.01）， 1级高血糖（p = 0.04）和2级低血糖（p = 0.008）的时间更短。780G组的CNFD （p = 0.02）、CNBD （p = 0.04）、CNFL （p = 0.04）和IWL （p < 0.001）显著高于MDI组，与健康对照组相当。结论使用SmartGuard的MiniMed 780G可改善DPN患者的血糖控制和变异性，改善小神经纤维形态。

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引用次数: 0

Clinical and Electrophysiological Characterization of Diabetic Neuropathy in a Sub-Saharan African Cohort 撒哈拉以南非洲人群糖尿病神经病变的临床和电生理特征

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-09 DOI: 10.1111/jns.70021

Samuel Eric Chokote, Gaelle Lemdjo, Juan Francisco Idiaquez Rios, Aurelien Tejiozem Anakeu, Leonard Ngarka, Leonard N. Nfor, Michel K. Mengnjo, Wepnyu Y. Njamnshi, Herman Nestor Tsague Kengni, Ruth Joelle Ngongang, Gilles Simeni, Lylian Piameu, Alain Balla Nkonda, Faustin Yepnjio, Godwin Y. Tatah, Umapathi N. Thirugnanam, Alfred Kongnyu Njamnshi

Background

Diabetic neuropathy (DN) is the most frequent complication of diabetes mellitus, contributing to increased morbidity and mortality. Previous clinical studies on DN in sub-Saharan Africa (sSA) have used purely clinical approaches, potentially underestimating the true magnitude of this disease. This study was designed to determine the prevalence of definite diabetic neuropathy and describe the different subtypes using objective small and large fiber function measures.

Methods

This was a hospital-based cross-sectional study that included diabetes and prediabetes patients, followed up at Jordan Medical Services, Yaoundé, Cameroon, between March 2022 and February 2023. The “Toronto Clinical Neuropathy Score” and “Douleur Neuropathique en 4” questionnaires were used for clinical evaluation. Autonomic symptoms were equally recorded. Nerve conduction studies and Sudoscan were used for electrophysiological assessments of large and small fibre functions.

Results

Eighty-four participants were included; 91.7% had type 2 DM, 2.4% had type 1 DM, and 6% had glucose intolerance. DN was found in 73/84 (86.9%). Diabetic sensorimotor polyneuropathy (DSP) was the most frequent subtype (63.8%), followed by diabetic autonomic neuropathy (40.5%), mononeuropathy (36.9%), asymmetric axonal sensory neuropathy (4.8%) and treatment-induced neuropathy of diabetes (TIND) in 1.2% of patients. The prevalence of large and small fibre neuropathies was 38.1% and 25.0%, respectively.

Conclusion

The prevalence of DN and specifically DSP in our study was higher than previously described in African literature. We identified subtypes never before reported in sSA, mainly small fibre neuropathy and TIND. This may have management and policy implications.

背景：糖尿病性神经病变（DN）是糖尿病最常见的并发症，导致发病率和死亡率增高。以前在撒哈拉以南非洲（sSA）对DN的临床研究使用了纯粹的临床方法，可能低估了这种疾病的真正严重程度。本研究旨在确定明确的糖尿病神经病变的患病率，并通过客观的小纤维和大纤维功能测量来描述不同的亚型。方法：这是一项以医院为基础的横断面研究，包括糖尿病和前驱糖尿病患者，于2022年3月至2023年2月在喀麦隆雅温得约旦医疗服务中心进行随访。采用“多伦多临床神经病评分”和“Douleur神经症en 4”问卷进行临床评价。自主神经症状同样被记录。神经传导研究和Sudoscan用于大纤维和小纤维功能的电生理评估。结果共纳入84例受试者；91.7%患有2型糖尿病，2.4%患有1型糖尿病，6%患有葡萄糖耐受不良。73/84例（86.9%）发现DN。糖尿病感觉运动多神经病变（DSP）是最常见的亚型（63.8%），其次是糖尿病自主神经病变（40.5%）、单神经病变（36.9%）、不对称轴突感觉神经病变（4.8%）和治疗性糖尿病神经病变（TIND），占1.2%。大纤维神经病和小纤维神经病的患病率分别为38.1%和25.0%。结论本研究中DN特别是DSP的患病率高于非洲文献先前的报道。我们确定了sSA中从未报道过的亚型，主要是小纤维神经病和TIND。这可能会对管理和政策产生影响。

{"title":"Clinical and Electrophysiological Characterization of Diabetic Neuropathy in a Sub-Saharan African Cohort","authors":"Samuel Eric Chokote, Gaelle Lemdjo, Juan Francisco Idiaquez Rios, Aurelien Tejiozem Anakeu, Leonard Ngarka, Leonard N. Nfor, Michel K. Mengnjo, Wepnyu Y. Njamnshi, Herman Nestor Tsague Kengni, Ruth Joelle Ngongang, Gilles Simeni, Lylian Piameu, Alain Balla Nkonda, Faustin Yepnjio, Godwin Y. Tatah, Umapathi N. Thirugnanam, Alfred Kongnyu Njamnshi","doi":"10.1111/jns.70021","DOIUrl":"https://doi.org/10.1111/jns.70021","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Diabetic neuropathy (DN) is the most frequent complication of diabetes mellitus, contributing to increased morbidity and mortality. Previous clinical studies on DN in sub-Saharan Africa (sSA) have used purely clinical approaches, potentially underestimating the true magnitude of this disease. This study was designed to determine the prevalence of definite diabetic neuropathy and describe the different subtypes using objective small and large fiber function measures.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a hospital-based cross-sectional study that included diabetes and prediabetes patients, followed up at Jordan Medical Services, Yaoundé, Cameroon, between March 2022 and February 2023. The “Toronto Clinical Neuropathy Score” and “Douleur Neuropathique en 4” questionnaires were used for clinical evaluation. Autonomic symptoms were equally recorded. Nerve conduction studies and Sudoscan were used for electrophysiological assessments of large and small fibre functions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Eighty-four participants were included; 91.7% had type 2 DM, 2.4% had type 1 DM, and 6% had glucose intolerance. DN was found in 73/84 (86.9%). Diabetic sensorimotor polyneuropathy (DSP) was the most frequent subtype (63.8%), followed by diabetic autonomic neuropathy (40.5%), mononeuropathy (36.9%), asymmetric axonal sensory neuropathy (4.8%) and treatment-induced neuropathy of diabetes (TIND) in 1.2% of patients. The prevalence of large and small fibre neuropathies was 38.1% and 25.0%, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The prevalence of DN and specifically DSP in our study was higher than previously described in African literature. We identified subtypes never before reported in sSA, mainly small fibre neuropathy and TIND. This may have management and policy implications.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 2","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70021","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143925751","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

Differential Effects of Visceral and Subcutaneous Adiposity on Peripheral Neuropathy 内脏和皮下脂肪对周围神经病变的不同影响

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System

Pub Date : 2025-05-09 DOI: 10.1111/jns.70025

Georgios Ponirakis, Leeza Peerzada, Ioannis N. Petropoulos, Hoda Gad, Sidra Abdulshakoor, Jenneth M. Concepcion, Sara H. Khalfalla, Iynas S. A. Elamin, Abeer T. H. AlZawqari, Einas Elgassim, Areej Baraka, Ziyad R. Mahfoud, Marwa A. El Deeb, Nahla Afifi, Rayaz A. Malik

Objective

Obesity increases the risk of diabetic neuropathy. This study investigates the impact of visceral (VAT) and subcutaneous adipose tissue (SAT) volume on peripheral neuropathy.

Methods

A total of 302 adults from the Qatar Biobank (QBB) underwent iDXA to measure VAT and SAT volumes, intima media thickness (IMT), and peripheral neuropathy assessments using corneal confocal microscopy (CCM), vibration perception threshold (VPT), and the DN4 questionnaire.

Results

The QBB cohort was aged 43.9 ± 12.9 years, of whom 43.7% were women, 42.1% had obesity, 17.4% had type 2 diabetes (T2D) and 10.9% had hypertension. VAT was associated with T2D, hypertension, higher HbA1c, diastolic blood pressure, triglycerides, and inflammatory markers, and lower HDL (p < 0.0001). There were no significant associations between SAT and these cardiovascular risk factors. VAT volume was associated with lower corneal nerve inferior whorl length (IWL) (p < 0.05) and higher VPT (p = 0.01), partially mediated by elevated HbA1c (p < 0.05, p = 0.001) and IMT (p < 0.0001), while its association with neuropathic symptoms was fully mediated by systolic blood pressure (p < 0.05), T2D (p < 0.01), and triglycerides (p = 0.05). SAT showed no associations with measures of neuropathy.

Conclusions

VAT but not SAT is associated with peripheral neuropathy. This study underscores the need to target VAT to improve neuropathy.

肥胖增加糖尿病性神经病变的风险。本研究探讨了内脏（VAT）和皮下脂肪组织（SAT）体积对周围神经病变的影响。方法来自卡塔尔生物银行（QBB）的302名成年人接受iDXA测量VAT和SAT体积、内膜中膜厚度（IMT），并使用角膜共聚焦显微镜（CCM）、振动感知阈值（VPT）和DN4问卷进行周围神经病变评估。结果QBB队列患者年龄43.9±12.9岁，其中女性43.7%，肥胖42.1%，2型糖尿病（T2D） 17.4%，高血压10.9%。VAT与T2D、高血压、较高的HbA1c、舒张压、甘油三酯和炎症标志物以及较低的HDL相关（p < 0.0001）。SAT和这些心血管危险因素之间没有明显的关联。VAT容积与角膜下神经环长（IWL）（p < 0.05）和VPT升高（p = 0.01）相关，部分由HbA1c升高（p < 0.05, p = 0.001）和IMT升高（p < 0.0001）介导，而与神经病变症状的相关性由收缩压（p < 0.05）、T2D （p < 0.01）和甘油三酯（p = 0.05）完全介导。SAT与神经病变的测量没有关联。结论VAT与周围神经病变相关，而SAT与周围神经病变无关。这项研究强调了靶向VAT以改善神经病变的必要性。

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引用次数: 0