Journal of Clinical Neuromuscular Disease最新文献_第10页

Rituximab Responsive Relapsing–Remitting IgG4 Anticontactin 1 Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Membranous Nephropathy: A Case Description and Brief Review 慢性炎症性脱髓鞘性多根神经病变伴膜性肾病:一例描述和简要回顾

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000395

G. Remiche, Marta Lamartine S Monteiro, C. Catalano, J. Hougardy, E. Delmont, J. Boucraut, N. Mavroudakis

引用次数: 1

Academic and Employment Status in Patients With Generalized Myasthenia Gravis Treated With Eculizumab: A Case Series. Eculizumab治疗全身性重症肌无力患者的学业和就业状况:一个病例系列

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000391

Seung Ah Kang, Megan Sweeney, Raghav Govindarajan

{"title":"Academic and Employment Status in Patients With Generalized Myasthenia Gravis Treated With Eculizumab: A Case Series.","authors":"Seung Ah Kang, Megan Sweeney, Raghav Govindarajan","doi":"10.1097/CND.0000000000000391","DOIUrl":"https://doi.org/10.1097/CND.0000000000000391","url":null,"abstract":"Objectives: To evaluate the impact of treatment with eculizumab, a terminal complement inhibitor, on academic and employment status in patients with refractory generalized myasthenia gravis (MG).Methods: Case review of 7 US patients.Results: Six patients were aged ≤65 years; one was a full-time student and the remainder were in employment before MG diagnosis. After diagnosis, all patients gave up work (n = 3) or reduced their study/working hours (n = 4). In the 12 months after eculizumab initiation, patients who had stopped work resumed working in some capacity, whereas those who had changed their work/study hours returned to their original work/study pattern. Patients also experienced a reduction in the number of MG exacerbations, and a clinically significant improvement in MG-Activities of Daily Living scores, and were able to reduce other MG medications.Conclusions: These results suggest that treatment with eculizumab may help maintain education/employment activity in patients with refractory generalized MG.","PeriodicalId":39645,"journal":{"name":"Journal of Clinical Neuromuscular Disease","volume":"23 4","pages":"210-218"},"PeriodicalIF":0.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d9/e3/jcnd-23-210.PMC9126260.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10131317","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Clinical Utility of Repetitive Nerve Stimulation Test in Differentiating Multifocal Motor Neuropathy From Progressive Muscular Atrophy. 重复神经刺激试验鉴别多灶性运动神经病与进行性肌萎缩的临床应用。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000401

Shunsuke Watanabe, Kenji Sekiguchi, Yoshikatsu Noda, Riki Matsumoto

{"title":"Clinical Utility of Repetitive Nerve Stimulation Test in Differentiating Multifocal Motor Neuropathy From Progressive Muscular Atrophy.","authors":"Shunsuke Watanabe, Kenji Sekiguchi, Yoshikatsu Noda, Riki Matsumoto","doi":"10.1097/CND.0000000000000401","DOIUrl":"https://doi.org/10.1097/CND.0000000000000401","url":null,"abstract":"Objectives: To evaluate the utility of repetitive nerve stimulation test (RNS) for differentiating multifocal motor neuropathy (MMN) and progressive muscular atrophy (PMA).Methods: We retrospectively enrolled 20 patients with MMN or PMA. We extracted the results of the initial 3-Hz RNS in the ulnar and accessory nerves and compared the percentage and frequency of abnormal decremental responses between both groups.Results: RNS was performed in 8 ulnar and 9 accessory nerves in patients with MMN, and in 8 ulnar and 10 accessory nerves in patients with PMA. Patients with MMN had a significantly lower decrement percentage (0.6 ± 4.0% in MMN vs. 10.3 ± 6.5% in PMA, P < 0.01) and frequency of abnormal decremental response (0 of 9 in MMN vs. 6 of 10 in PMA, P = 0.01) than patients with PMA in the accessory nerve.Conclusions: The RNS has clinical utility for differentiating MMN from PMA.","PeriodicalId":39645,"journal":{"name":"Journal of Clinical Neuromuscular Disease","volume":"23 4","pages":"175-182"},"PeriodicalIF":0.0,"publicationDate":"2022-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fd/7a/jcnd-23-175.PMC9126258.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10131319","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Parsonage Turner Syndrome Following Vaccination With mRNA-1273 SARS-CoV-2 Vaccine. 接种 mRNA-1273 SARS-CoV-2 疫苗后出现特纳综合征。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000411

James H Bernheimer, Gregory Gasbarro

引用次数: 0

Parsonage-Turner Syndrome: Fascicular Involvement and Focal Constriction. 帕森纳-特纳综合征:肌束受累和局灶性收缩。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000407

W. Waheed, D. Sneag

引用次数: 1

Electrodiagnostic Findings in Riboflavin Transporter Deficiency Type 2. 核黄素转运蛋白缺乏2型的电诊断表现。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000390

J. A. Sánchez, Rebecca E. Traub, S. Trau, J. Howard

引用次数: 1

Pfizer mRNA COVID-19 Vaccination and Acute Inflammatory Demyelinating Polyneuropathy. 辉瑞mRNA COVID-19疫苗与急性炎性脱髓鞘性多神经病变。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000397

Katrina Bernardo, A. Misra

引用次数: 1

Use of Neuromuscular Ultrasound in the Diagnosis of Disulfiram Polyneuropathy. 神经肌肉超声在二硫仑多发性神经病诊断中的应用。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000380

Sabrina Isabel Coronel, J. Dorman

引用次数: 0

What is in the Neuromuscular Junction Literature? 什么是神经肌肉接点文献?

Journal of Clinical Neuromuscular Disease Pub Date : 2022-06-01 DOI: 10.1097/CND.0000000000000403

Tawfiq Al-Lahham, David Lacomis

引用次数: 0

A Case of Probable Multifocal Motor Neuropathy With Clinical Stability for Ten Years After a Single Treatment of Rituximab. 一例可能的多灶性运动神经病变，单次利妥昔单抗治疗后临床稳定10年。

Journal of Clinical Neuromuscular Disease Pub Date : 2022-03-01 DOI: 10.1097/CND.0000000000000358

Natalia L Gonzalez, Vern C Juel, Saša A Živković

{"title":"A Case of Probable Multifocal Motor Neuropathy With Clinical Stability for Ten Years After a Single Treatment of Rituximab.","authors":"Natalia L Gonzalez, Vern C Juel, Saša A Živković","doi":"10.1097/CND.0000000000000358","DOIUrl":"https://doi.org/10.1097/CND.0000000000000358","url":null,"abstract":"Abstract: Multifocal motor neuropathy is a rare, immune-mediated motor neuropathy with asymmetric, often debilitating progressive weakness. The efficacy of intravenous immunoglobulin in this disease is well established; however, the response typically wanes over time. No other agent has shown similar therapeutic efficacy. We describe a case of anti-ganglioside GM1 IgM-positive multifocal motor neuropathy with typical incomplete and diminishing response to intravenous immunoglobulin over time. Sixteen years after symptom onset, rituximab was administered at 2 g/m2 over 2 weeks. No significant progression of disease has occurred over the following 10 years despite no additional treatments, including intravenous immunoglobulin, being given. Only case reports and small, mostly uncontrolled studies have reported the use of rituximab in multifocal motor neuropathy with mixed results. However, given its potential benefits and lack of an established second-line agent, treatment with rituximab may be considered in select patients with refractory multifocal motor neuropathy.","PeriodicalId":39645,"journal":{"name":"Journal of Clinical Neuromuscular Disease","volume":"23 3","pages":"136-142"},"PeriodicalIF":0.0,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10121939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0