RRNMF Neuromuscular Journal最新文献_第10页

Characteristics of Triple Seronegative Myasthenia Gravis: A Single Center Experience 三重血清阴性重症肌无力的特征:单中心经验

RRNMF Neuromuscular Journal Pub Date : 2022-03-11 DOI: 10.17161/rrnmf.v3i1.14989

J. Morena, B. Jiang, M. Freimer, J. Hoyle, Bakri H. Elsheikh, D. Arnold, S. Lorusso

{"title":"Characteristics of Triple Seronegative Myasthenia Gravis: A Single Center Experience","authors":"J. Morena, B. Jiang, M. Freimer, J. Hoyle, Bakri H. Elsheikh, D. Arnold, S. Lorusso","doi":"10.17161/rrnmf.v3i1.14989","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.14989","url":null,"abstract":"Background: There is variability in the literature regarding the characteristics of triple seronegative myasthenia gravis (SNMG) patients. Most studies were performed before LRP4 antibodies were discovered, and characterizations of triple seronegative patients are lacking in the literature. \u0000Methods: We retrospectively investigated patients diagnosed with myasthenia gravis (MG) at Ohio State University from 2009 to 2019. Triple SNMG was defined by a history and examination that was consistent with MG and positive SFEMG, RNS or edrophonium testing, but negative serology for AChR, MUSK, and LRP4 antibodies. \u0000Results: A total of 210 AChR+, 9 MuSK+, 6 LRP4+, 9 double SNMG, and 21 triple SNMG patients were reviewed. Triple SNMG patients required significantly fewer immunosuppressive agents compared with AChR+ patients (p=0.0001) and a trend towards a less frequent history of hospitalizations, myasthenic crises and intubations compared to all antibody positive groups. Triple SNMG patients had a significantly higher frequency of ocular disease (33%) compared to AChR+ patients (13%) (p=0.0250). One triple and one double SNMG patient had thymic hyperplasia and improved after thymectomy. 11 triple SNMG patients had negative genetic testing for CMS. \u0000Conclusion: Our results further elucidate the clinical characteristics of triple SNMG, which include the predominance for ocular disease and a less severe disease course. Although likely rare, investigation for thymic pathology should be a consideration even in SNMG, and thymectomy should be considered when there is thymic pathology. We did not find alternate diagnoses in SNMG patients and thus ancillary testing should be considered in carefully selected patients for cost-effective care.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114817736","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

Asymmetrical Onset of Leg Amyotrophic Diplegia (LAD): A Case Report 腿肌萎缩性双瘫(LAD)发病不对称1例

RRNMF Neuromuscular Journal Pub Date : 2022-03-11 DOI: 10.17161/rrnmf.v3i1.15640

R. Khodabandehlou

{"title":"Asymmetrical Onset of Leg Amyotrophic Diplegia (LAD): A Case Report","authors":"R. Khodabandehlou","doi":"10.17161/rrnmf.v3i1.15640","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.15640","url":null,"abstract":"Progressive muscular atrophy (PMA) comprises approximately 10% of patients with motor neuron disease (MND). Some of the patients presenting initially as PMA will develop when followed over time upper motor neuron findings leading to the diagnosis of ALS. True PMA cases represent a pure lower motor neuron presentation of sporadic motor neuron disease and are in a spectrum with ALS and PLS. While PMA typically affects both the arms and legs, some patients have predominantly upper extremity involvement and others may have selective leg weakness referred to respectively as brachial amyotrophic diplegia (BAD) 1,2 and leg amyotrophic diplegia (LAD).3 These cases of progressive muscle atrophy remain restricted to a body region for extended periods. These are considered slow regional variants of motor neuron disease. LAD is a leg onset variant of progressive muscular atrophy (PMA). LAD weakness is confined to the legs for at least 2 years, and there are no upper motor neuron signs.4A LMN syndrome confined to legs LAD was first described by Pierre Marie and his student Patrikios in 1918 and was known as the pseudopolyneuritic variant of ALS, the Marie-Patrikios form, or the peroneal form of ALS.Here is another case of LAD for the neuromuscular literature.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115665210","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

A rare potential cause of mononeuropathy multiplex: LYG Mononeuropathy multiplex 多发性单神经病变罕见的潜在病因:LYG多发性单神经病变

RRNMF Neuromuscular Journal Pub Date : 2022-03-11 DOI: 10.17161/rrnmf.v2i5.15711

T. Fullam, J. Jacobsohn, Swathy Chandrashekhar, Omar Jawdat, M. Dimachkie

引用次数: 0

Timing of Decremental Response During Repetitive Nerve Stimulation in Myasthenia Gravis 重症肌无力患者重复神经刺激过程中衰减反应的时间

RRNMF Neuromuscular Journal Pub Date : 2022-03-11 DOI: 10.17161/rrnmf.v3i1.15216

Gloria Ortiz Guerrero, A. Heim, M. Pasnoor, L. Herbelin, Omar Jawdat, Melanie D. Glenn, J. Statland, D. Jabari, C. Farmakidis, R. Barohn

引用次数: 0

Effect of 3,4-diaminopyridine phosphate in symptomatic SOD1-G93A mice 3,4-二氨基吡啶磷酸对SOD1-G93A症状小鼠的影响

RRNMF Neuromuscular Journal Pub Date : 2022-03-11 DOI: 10.17161/rrnmf.v2i5.15641

Swathi Beladakere Ramaswamy, J. Stanford, S. Iyadurai, R. Govindarajan, R. Barohn

引用次数: 0

Black History Month: Remembering a Pioneer in Medicine 黑人历史月:纪念一位医学先驱

RRNMF Neuromuscular Journal Pub Date : 2022-03-11 DOI: 10.17161/rrnmf.v3i1.16443

R. Barohn

引用次数: 0

Steps in Inclusion Body Myositis 包涵体肌炎的步骤

RRNMF Neuromuscular Journal Pub Date : 2021-12-20 DOI: 10.17161/rrnmf.v2i5.15839

T. Trevor, H. Kushlaf

引用次数: 0

Practice patterns in the management of myasthenia gravis: a cross-sectional survey of neurologists in the United States 重症肌无力管理的实践模式:美国神经科医生的横断面调查

RRNMF Neuromuscular Journal Pub Date : 2021-12-20 DOI: 10.17161/rrnmf.v2i5.15806

V. Bril, Jacqueline A Palace, T. Mozaffar, D. Gelinas, E. Brauer, Paul Nisbet, G. Wolfe

{"title":"Practice patterns in the management of myasthenia gravis: a cross-sectional survey of neurologists in the United States","authors":"V. Bril, Jacqueline A Palace, T. Mozaffar, D. Gelinas, E. Brauer, Paul Nisbet, G. Wolfe","doi":"10.17161/rrnmf.v2i5.15806","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15806","url":null,"abstract":"Background: Management of myasthenia gravis (MG), a rare immunoglobulin G autoantibody–mediated neuromuscular junction disorder, is driven by physician experience. To gain insight into current practices and physician needs, neurologists’ use of guidelines and disease activity evaluations to manage MG was assessed. Methods: In November and December of 2020, a quantitative, cross-sectional, 51-item, online survey–based study was used to collect data from 100 community neurologists, from 31 US states, who treat MG. Differences across ratio variables were analyzed via Chi-square and t tests, at a significance level of P<0.05. Results: Of respondents, 76% reported using clinical judgment rather than guidelines to inform treatment decisions, and only 29% reported awareness of the updated 2020 International Consensus Guidance for Management of Myasthenia Gravis. Treatment patterns reported include use of prednisone-equivalent corticosteroid doses ≤10 mg/day for ≥6 months (76% of respondents). When corticosteroids are contraindicated or after failure of an initial nonsteroidal immunosuppressant therapy (NSIST), immunoglobulin therapy is the respondents’ preferred initial treatment in patients with acetylcholine receptor antibody–positive generalized MG (vs a second NSIST). Respondents expressed interest in more guidance on crisis management, initiating/titrating maintenance medications, and managing patients with comorbidities. Conclusions: Respondents to this survey reported varied approaches to MG management and, in some clinical settings, heavier reliance on clinical judgment than on available consensus-based guidance. Also observed was potential underutilization of NSISTs in patients for whom corticosteroids are contraindicated, with reliance, instead, on immunoglobulin.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117211230","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: 1

Methotrexate Use in Generalized Autoimmune Myasthenia Gravis: A Case series 甲氨蝶呤在广泛性自身免疫性重症肌无力中的应用:一个病例系列

RRNMF Neuromuscular Journal Pub Date : 2021-12-20 DOI: 10.17161/rrnmf.v2i5.15795

Preston Eibling, Yuebing Li, R. Marquardt

引用次数: 0

FDA approves Alzheimer's drug against the recommendation of its scientific panel. Be very concerned. FDA不顾科学小组的建议批准了阿尔茨海默症药物。要非常关注。

RRNMF Neuromuscular Journal Pub Date : 2021-12-20 DOI: 10.17161/rrnmf.v2i5.15979

Joshua Freeman, MD

引用次数: 0