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":"97 1","pages":"0"},"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}
{"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":"11 1","pages":"0"},"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}
T. Fullam, J. Jacobsohn, Swathy Chandrashekhar, Omar Jawdat, M. Dimachkie
{"title":"A rare potential cause of mononeuropathy multiplex: LYG Mononeuropathy multiplex","authors":"T. Fullam, J. Jacobsohn, Swathy Chandrashekhar, Omar Jawdat, M. Dimachkie","doi":"10.17161/rrnmf.v2i5.15711","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15711","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"51 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132783329","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}
Gloria Ortiz Guerrero, A. Heim, M. Pasnoor, L. Herbelin, Omar Jawdat, Melanie D. Glenn, J. Statland, D. Jabari, C. Farmakidis, R. Barohn
{"title":"Timing of Decremental Response During Repetitive Nerve Stimulation in Myasthenia Gravis","authors":"Gloria Ortiz Guerrero, A. Heim, M. Pasnoor, L. Herbelin, Omar Jawdat, Melanie D. Glenn, J. Statland, D. Jabari, C. Farmakidis, R. Barohn","doi":"10.17161/rrnmf.v3i1.15216","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.15216","url":null,"abstract":"Background: A decrement >10% detected during repetitive nerve stimulation (RNS) is supportive of considering a diagnosis of myasthenia gravis (MG). Several studies have found that most of this decrement is seen between 4 to 6 min post-exercise. However, there are not available studies analyzing if shorter timing would be sufficient. \u0000Objective: The objective of this study was to evaluate if RNS up to 2 min post-exercise is sufficient to detect a decrement response >10%. \u0000Methods: We performed a retrospective chart review study of patients referred to our neuromuscular clinic at The University of Kansas Medical Center with symptoms suggestive of MG from 2013 to 2017. \u0000Results: A total of 76 patients with MG and 100 controls were identified. A significant decrement was detected in 95% of MG patients with abnormal RNS within 2 minutes post-exercise. \u0000Conclusion: RNS up to 2 min post-exercise might be sufficient to detect a significant decrement in MG patients. ","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128961404","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}
Swathi Beladakere Ramaswamy, J. Stanford, S. Iyadurai, R. Govindarajan, R. Barohn
{"title":"Effect of 3,4-diaminopyridine phosphate in symptomatic SOD1-G93A mice","authors":"Swathi Beladakere Ramaswamy, J. Stanford, S. Iyadurai, R. Govindarajan, R. Barohn","doi":"10.17161/rrnmf.v2i5.15641","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15641","url":null,"abstract":"Objective: To study the effect of 3,4-diaminopyridine phosphate (3,4-DAPP) on body weight, grip strength, neurological score and survival in symptomatic SOD1-G93A mice. \u0000 \u0000Method: We administered 3,4-diaminopyridine phosphate (3,4-DAPP) at 0, 8, and 16 mg/kg to SOD1-G93A mice 5 days/week beginning at 90 days of age. We measured body weight, grip strength, neurological score and survival in this model of ALS. \u0000 \u0000Results: 3,4-DAPP had no influence on body weight, grip strength, neurological score or survival in this transgenic mouse model. \u0000 \u0000Conclusion: Our study showed that 3,4-DAPP administration had no effects on survival, body weight, grip strength and neurological score of mice with SOD1 mutation. Since the results of this study are of limited significance, larger animal studies are required to investigate the utility of 3,4-DAPP.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"47 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132479376","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}
{"title":"Black History Month: Remembering a Pioneer in Medicine","authors":"R. Barohn","doi":"10.17161/rrnmf.v3i1.16443","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.16443","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"320 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115342887","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}
{"title":"Steps in Inclusion Body Myositis","authors":"T. Trevor, H. Kushlaf","doi":"10.17161/rrnmf.v2i5.15839","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15839","url":null,"abstract":"Not applicable. This is a poem. ","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"15 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131866653","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}
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":"12 1","pages":"0"},"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}
{"title":"Methotrexate Use in Generalized Autoimmune Myasthenia Gravis: A Case series","authors":"Preston Eibling, Yuebing Li, R. Marquardt","doi":"10.17161/rrnmf.v2i5.15795","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15795","url":null,"abstract":"Methotrexate (MTX) is an inexpensive and well-tolerated immunosuppressive medication that is used anecdotally in autoimmune myasthenia gravis (MG). However, the efficacy in MG is unclear at this time. This retrospective analysis describes six patients with acetylcholine receptor antibody positive MG who were treated with MTX and corticosteroids. The efficacy of MTX was measured by steroid-sparing effect and the Myasthenia Gravis Foundation of America (MGFA) classification. MTX initiation was associated with a reduction in prednisone dosage in all patients. Minimal manifestation status was reached at an average duration of 10 months in 5 patients. No patients were hospitalized for myasthenia gravis exacerbations. There were no major side effects experienced with MTX use. This retrospective analysis suggests that MTX is safe and probably efficacious as a corticosteroid-sparing agent in the management of MG.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"55 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116947790","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}
{"title":"FDA approves Alzheimer's drug against the recommendation of its scientific panel. Be very concerned.","authors":"Joshua Freeman, MD","doi":"10.17161/rrnmf.v2i5.15979","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15979","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"72 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123586618","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}