Pub Date : 2022-06-22DOI: 10.17161/rrnmf.v3i2.18133
R. Barohn, J. Allen, D. Avila, A. Barboi, S. Biliciler, C. Abrams, Thomas Brannagan III, Michael Cartwright, N. Chahin, J. Chawla, Miguel Chuquilin, Michael Collins, A. Deboo, J. England, J. Fernandes, M. Friemer, S. Geisler, S. Herskovitz, N. Holland, Carlayne Jackson, V. Juel, D. Lacomis, Maxwell H Levy, Rabia N. Malik, D. Menichella, S. Nations, A. Peltier, M. Pulley, K. Rezania, J. Robinson-Papp, Katherine Ruzhansky, K. Sharma, A. Stino, Xiaowei W Su, A. Swenson, P. Thaisetthawatkul, S. Thawani, Rebecca E. Traub
{"title":"Determining Best or Inferior Drug(s) Using an Adaptive Platform for Cryptogenic Sensory Polyneuropathy","authors":"R. Barohn, J. Allen, D. Avila, A. Barboi, S. Biliciler, C. Abrams, Thomas Brannagan III, Michael Cartwright, N. Chahin, J. Chawla, Miguel Chuquilin, Michael Collins, A. Deboo, J. England, J. Fernandes, M. Friemer, S. Geisler, S. Herskovitz, N. Holland, Carlayne Jackson, V. Juel, D. Lacomis, Maxwell H Levy, Rabia N. Malik, D. Menichella, S. Nations, A. Peltier, M. Pulley, K. Rezania, J. Robinson-Papp, Katherine Ruzhansky, K. Sharma, A. Stino, Xiaowei W Su, A. Swenson, P. Thaisetthawatkul, S. Thawani, Rebecca E. Traub","doi":"10.17161/rrnmf.v3i2.18133","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i2.18133","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"128 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121477822","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}
Pub Date : 2022-03-11DOI: 10.17161/rrnmf.v2i5.15966
Michael Stanley
"Boots" is a lyrical, narrative poem from the perspective of a Maine Coon Cat rescued one winter by a boy, Johnny, who grows up to be a young man enlisting in the service. Johnny comes back wounded in many ways, one of them being an amputee with phantom limb syndrome. Boots tries to help his owner as best he can by scratching the prosthetic leg that seems to pain Johnny so, and ponders the winters long ago when they used to play in the snow together. After our global campaign against COVID, I couldn't help but feel the poem had a renewed sense of meaning to many of the walking wounded who served their communities in the face of COVID. So it is a poem I hope in memorial to veterans of wars military and medical.
{"title":"Boots","authors":"Michael Stanley","doi":"10.17161/rrnmf.v2i5.15966","DOIUrl":"https://doi.org/10.17161/rrnmf.v2i5.15966","url":null,"abstract":"\"Boots\" is a lyrical, narrative poem from the perspective of a Maine Coon Cat rescued one winter by a boy, Johnny, who grows up to be a young man enlisting in the service. Johnny comes back wounded in many ways, one of them being an amputee with phantom limb syndrome. Boots tries to help his owner as best he can by scratching the prosthetic leg that seems to pain Johnny so, and ponders the winters long ago when they used to play in the snow together. After our global campaign against COVID, I couldn't help but feel the poem had a renewed sense of meaning to many of the walking wounded who served their communities in the face of COVID. So it is a poem I hope in memorial to veterans of wars military and medical.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"75 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":"121038131","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}
Pub Date : 2022-03-11DOI: 10.17161/rrnmf.v3i1.15536
Bakri H. Elsheikh, Obinna Moneme, M. Freimer, J. Kissel, W. Arnold
Introduction: There are conflicting reports of depression prevalence in myasthenia gravis (MG). The influence of somatic symptoms on screening assessments is not apparent. We investigated the frequency of somatic and non-somatic symptoms of depression in MG. We also explored the relationship between depression and MG using disease severity and quality of life measures. Methods: Three cohorts of participants (MG, healthy and disease controls) were prospectively assessed with the Beck Depression Inventory 2 (BDI-II) and BDI-Primary Care (BDI-PC) surveys, modified Rankin Scale, MGFA classification, MG-MMT, MG-ADL and MG-QOL15. Results: A total of 31 MG, 29 disease controls, and 30 healthy controls were enrolled. Depression frequency indicated by BDI-II in MG 48% (15/31) and disease control 31% (9/29) was not significantly different [p=0.17]. However, we found a significantly higher frequency than healthy controls 10% (3/30) [p=0.001]. In contrast, depression frequency indicated by BDI-PC was similar in the MG 29% (9/31) and disease controls MG, 24% (7/29)[p=0.77] as well as the healthy controls 10% (3/30) [p=0.08]. Using the BDI-II scale, participants with MG who were depressed had higher scores on MG-MMT, MG-ADL, and MG-QOL15 than those who were not depressed. The difference in MG-ADL and MG-QOL15 scores remained significant using the BDI-PC score. Discussion: These findings suggest depression screening assessments that include physical symptoms overestimate depression in MG and chronic autoimmune neuromuscular disorders. A higher frequency of self-reported depression is associated with increasing disease severity and low quality of life.
{"title":"Screening for depression in myasthenia gravis","authors":"Bakri H. Elsheikh, Obinna Moneme, M. Freimer, J. Kissel, W. Arnold","doi":"10.17161/rrnmf.v3i1.15536","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.15536","url":null,"abstract":"Introduction: There are conflicting reports of depression prevalence in myasthenia gravis (MG). The influence of somatic symptoms on screening assessments is not apparent. We investigated the frequency of somatic and non-somatic symptoms of depression in MG. We also explored the relationship between depression and MG using disease severity and quality of life measures. Methods: Three cohorts of participants (MG, healthy and disease controls) were prospectively assessed with the Beck Depression Inventory 2 (BDI-II) and BDI-Primary Care (BDI-PC) surveys, modified Rankin Scale, MGFA classification, MG-MMT, MG-ADL and MG-QOL15. Results: A total of 31 MG, 29 disease controls, and 30 healthy controls were enrolled. Depression frequency indicated by BDI-II in MG 48% (15/31) and disease control 31% (9/29) was not significantly different [p=0.17]. However, we found a significantly higher frequency than healthy controls 10% (3/30) [p=0.001]. In contrast, depression frequency indicated by BDI-PC was similar in the MG 29% (9/31) and disease controls MG, 24% (7/29)[p=0.77] as well as the healthy controls 10% (3/30) [p=0.08]. \u0000Using the BDI-II scale, participants with MG who were depressed had higher scores on MG-MMT, MG-ADL, and MG-QOL15 than those who were not depressed. The difference in MG-ADL and MG-QOL15 scores remained significant using the BDI-PC score. Discussion: These findings suggest depression screening assessments that include physical symptoms overestimate depression in MG and chronic autoimmune neuromuscular disorders. A higher frequency of self-reported depression is associated with increasing disease severity and low quality of life.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"39 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":"123538670","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}
Pub Date : 2022-03-11DOI: 10.17161/rrnmf.v3i1.15770
Mukaish Kumar, B. Banik, R. Govindarajan
Background: COVID-19 related Guillain-Barré syndrome has a broad spectrum of presentation. In most reported cases, respiratory symptoms preceded by neurological deficits by one to two weeks, suggesting that the clinical course is mostly post-infectious. In this case report, we present a para-infectious case of GBS with COVID-19. Case presentation: A 37-year-old male patient presented with fever, chills, myalgia, cough, and anosmia. COVID-19 test came positive. He was managed conservatively. On the 7th day of follow-up, he recovered except for a persistent loss of smell and taste. Two weeks after his initial presentation, he reported low back pain and bilateral lower extremity weakness and had a repeat COVID-19 test, which returned positive. His history, physical exam, CSF analysis, nerve conduction, and electromyography test revealed Guillain-Barre Syndrome. We managed GBS with supportive treatment in the hospital, and on follow-up of three months, he recovered fully. Conclusion: In our case, we report a para-infectious case of GBS with C0VID-19, and we managed this case without intravenous immunoglobulin or plasmapheresis. The decision to treat a COVID-19 related GBS case with a traditional GBS treatment option (intravenous immunoglobulin or plasmapheresis) should be taken in conjunction with co-morbidities and a tailored case of case basis.
{"title":"Guillain-Barre Syndrome Secondary to COVID-19: A case report and short review of other published cases","authors":"Mukaish Kumar, B. Banik, R. Govindarajan","doi":"10.17161/rrnmf.v3i1.15770","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.15770","url":null,"abstract":"Background: \u0000COVID-19 related Guillain-Barré syndrome has a broad spectrum of presentation. In most reported cases, respiratory symptoms preceded by neurological deficits by one to two weeks, suggesting that the clinical course is mostly post-infectious. In this case report, we present a para-infectious case of GBS with COVID-19. \u0000Case presentation: \u0000A 37-year-old male patient presented with fever, chills, myalgia, cough, and anosmia. COVID-19 test came positive. He was managed conservatively. On the 7th day of follow-up, he recovered except for a persistent loss of smell and taste. Two weeks after his initial presentation, he reported low back pain and bilateral lower extremity weakness and had a repeat COVID-19 test, which returned positive. His history, physical exam, CSF analysis, nerve conduction, and electromyography test revealed Guillain-Barre Syndrome. We managed GBS with supportive treatment in the hospital, and on follow-up of three months, he recovered fully. \u0000Conclusion: \u0000In our case, we report a para-infectious case of GBS with C0VID-19, and we managed this case without intravenous immunoglobulin or plasmapheresis. The decision to treat a COVID-19 related GBS case with a traditional GBS treatment option (intravenous immunoglobulin or plasmapheresis) should be taken in conjunction with co-morbidities and a tailored case of case basis.","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"90 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":"121387428","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}
Pub Date : 2022-03-11DOI: 10.17161/rrnmf.v3i1.14989
J. Morena, B. Jiang, M. Freimer, J. Hoyle, Bakri H. Elsheikh, D. Arnold, S. Lorusso
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. Methods: 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. Results: 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. Conclusion: 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.
{"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}
Pub Date : 2022-03-11DOI: 10.17161/rrnmf.v3i1.16747
R. Govindarajan, S. Iyadurai, Alex Karenevich, L. Herbelin, J. Statland, R. Barohn
{"title":"EE2: 3,4-Diaminopyridine Phosphate for AAL- The EEDAPP-ALS Trial","authors":"R. Govindarajan, S. Iyadurai, Alex Karenevich, L. Herbelin, J. Statland, R. Barohn","doi":"10.17161/rrnmf.v3i1.16747","DOIUrl":"https://doi.org/10.17161/rrnmf.v3i1.16747","url":null,"abstract":"","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":"39 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":"124631937","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}
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