Comparison of Immediate Adverse Effects of COVID-19 Vaccine (COVAXIN BBV152) in Pregnant vs Nonpregnant Women

Aims and objectives: The aim of this study was to compare the immediate adverse effects of the coronavirus disease 2019 (COVID-19) vaccine (COVAXIN) in a pregnant woman with that of a nonpregnant woman. Material(s) and Method(s): It is a prospective observational study done at Vanivilas Hospital, Bangalore Medical College & Research Institute (BMCRI) for 2 months. The sample size was 100 pregnant and 100 nonpregnant women. Telephonically, patients were followed-up, and details of the side/adverse effects were collected in a proforma after 2 and 14 days. Data collected from both groups were analyzed using the Chi-square test or Fisher's exact test. Result(s): The majority of women were in the age group of <=25 years (64.0% and 36.0%, respectively) with a mean age of 25.01 +/- 3.71 years among the pregnant and 28.52 +/- 6.00 years among nonpregnant women. About 25.0% of pregnant women and 38.0% of nonpregnant women reported side effects. About 15.0% and 22.0% had taken treatment for side effects among pregnant women and nonpregnant women, respectively. Among the pregnant women, the common side effects reported were injection site pain (17) followed by fever (5), fatigue (4), and myalgia (03). Whereas among the nonpregnant women, the common side effects reported were injection site pain (28) followed by fever (6), myalgia (3), headache (2), and fatigue (1). Conclusion(s): Side effects reported following the administration of Covaxin in pregnant and nonpregnant women are fever, fatigue, injection site pain, myalgia, and headache. The proportion of side effects was not significantly different in the pregnant and nonpregnant women following Covaxin administration. Clinical significance: Covaxin is an inactivated killed vaccine against COVID-19 by Bharat Biotech. The vaccine has been recommended for pregnant women by the Government of India during corona pandemic. Studies are lacking regarding the difference in adverse events in pregnant versus nonpregnant women, after vaccine administration.Copyright © The Author(s).

Life Experiences in Neuromuscular Tracheotomized Patients in Times of Covid-19.

BACKGROUND: The management of tracheotomy at home can be very complex, affecting the patient's quality of life. OBJECTIVES: This case series study aimed to explore the experiences of patients affected by neuromuscular diseases (NMD) concerning tracheostomy and Invasive Mechanical Ventilation (IMV) management at home during the COVID-19 health emergency in Italy. METHODS: Semi-structured interviews and the following instruments were used: Connor and Davidson Resilience Scale (CD-RISC-25); Acceptance and Action Questionnaire-II (AAQ-II); State-Trait Anxiety Inventory (STAI); Langer Mindfulness Scale (LMS). Descriptive analyses, correlations, and qualitative analyses were carried out. RESULTS: 22 patients [50% female, mean age = 50.2 (SD = 21.2)] participated in the study. Participants who showed high levels of dispositional mindfulness in terms of novelty-seeking (r = 0.736, p = 0.013) and novelty production (r = 0.644, p = 0.033) were those with higher resilience. The main emotion that emerged was the fear of contagion (19 patients, 86.36%), due to the previous fragile condition, leading to a significant sense of abandonment. The tracheostomy's perception is seen in extremes as a lifesaver or a condemnation. The relationship with the health professionals moves from satisfaction to a feeling of abandonment with a lack of preparation. CONCLUSIONS: The relationship between resilience, flexibility, state anxiety and dispositional mindfulness offers ways to reinforce tracheostomy management at home, even in critical periods when going to the hospital may be difficult.

[Antibody production capacity after COVID-19 vaccination in immune-mediated neuromuscular diseases under immunotherapy].

The post-vaccination antibody response in patients with immune-mediated neuromuscular diseases under immuno-suppressive therapy has not been sufficiently verified. The Japanese Society of Neurology has stated that coronavirus disease 2019 (COVID-19) vaccination should be given priority in patients with immunotherapy-associated neuromuscular diseases; however, data on antibody production to a novel mRNA vaccine are scarce in these patients. In this study, we aimed to measure residual antibody titers after the second dose and produced antibodies after the third dose of SARS-CoV-2 mRNA vaccine in 25 patients with neuromuscular diseases under immuno-suppressive therapy (disease group). We compared the disease group antibody titers with those of 829 healthy employees in our hospital (control group). The disease group included 17 patients with myasthenia gravis, 4 with multiple sclerosis, 3 with inflammatory muscle disease, and 1 with chronic inflammatory demyelinating polyneuropathies. Seven cases of the disease group showed negative antibody levels (<15.0 s/co) before the third vaccination, and antibody titers in the positive cases ranged from 16.9 to 4,589.0 s/co. Three of the seven antibody-negative cases turned positive after the third vaccination, and all but one of the antibody-positive cases showed a booster effect, with antibody titers after the third dose ranging from 245.1 to 85,374.0 s/co (1.0 to 885.0 times higher than those before vaccination). Although the immune response in the disease group was modest compared to the control group, in which antibody titers after the third vaccination ranged from 67.8 to 150,000 s/co (0.9 to 5,402.1 times higher than those before vaccination), the result indicated that a constant immune response was achieved under immuno-suppressive therapy. Even in the control group, three participants tested negative for residual antibody before the third inoculation, and four of the antibody-positive participants (27.7-24,054.0 s/co) lacked a booster effect after the third vaccination.

Management of Metastatic CA (Cancer) Pancreas Pain in Post Chemotherapy, Radiotherapy Patient With Combination of Neural Interventions (Oral Neuromodulators, Peripheral Nerve Blocks) and Muscular Intervention USGDN (Ultrasonography Guided Dry Needling): A Case Report

Aims: To describe combined neural and muscular interventions in post covid exacerbations of cancer pain with disabilities.To present a new perspective of neuromyopathy to explain Intractable CA pancreas pain. Introduction: Additional challenges in cancer pain management are due to cancer treatment complications (chemotherapy, radiotherapy). CA Pancreas pains routinely addressed with oral neuromodulators, opioids neurolytic coeliac plexus block (NCPB) or splanchnic nerve radiofrequency ablation (SRF). 75 years male, CA pancreas with spine, pelvic bone metastasis, post chemotherapy radiotherapy. 6 months bedridden with post covid exacerbations in pain (vas 10/10) received prior painkillers. Started oral pregabalin 75mg od, ultracet bd, myospaz bd. Result(s): With 15 days medications vas 6/10, patient could sit on wheelchair. Given sciatico-femoral block, pain reduced vas 2/10 but recurred in 7 day vas 5/10. Started USGDN of tight back and lower limb muscles with 32G solid needles.post3 sessions vas 2/10, able to walk with support after 4 weeks. Discussion(s): Viscerosomatic convergence at the dorsal horn neurons produces visceral pain referred to back and abdominal muscles led to muscle spasm with generation of myofascial trigger points(MTrPs)and pain. USGDN addresses MTrPs. Needle insertion produces local twitch reflex (LTR) followed by muscle relaxation with pain relief. Neural interventions addresses only visceral nociceptive afferents from celiac plexus which forms 10% of total spinal cord afferent input which sensitizes peripheral and central motor nociceptive pathway processing neuromyopathy. Conclusion(s): Viscerosomatic convergence with muscles involvement (neuromyopathy) proved to be effectively managed by using combined approaches, neuromoduation and USGDN in Ca pancreas pain with disabilities.Copyright © 2023

A Smart and Home-based Telerehabilitation Tool for Patients with Neuromuscular Disorder

Over fourteen million people suffer from neuromuscular diseases in the UK such as strokes, spinal cord injuries, and Parkinson's disease etc. That means at least one in six people in the UK are living with one or more neurological conditions. In order for patients to return to normal life sooner, a rigorous rehabilitation process is needed. In hospitals, physiotherapists and neurological experts prescribe specific neurorehabilitation exercises. In most cases, patients need to schedule an appointment to receive treatment in a hospital or to have physiotherapists visit them at home. The number of neuromuscular patients has increased, resulting in longer hospital waiting times. In particular, during COVID-19, patients were not allowed to visit hospitals or have physiotherapists visit them due to government restrictions. Online guides for personalised and custom rehabilitation therapy for joint spasticity and stiffness are also not available. This paper reports the development of an IoT-based prototype system that monitors and records joint movements using sensory footwear (consisting of FSR and IMU sensors) and Kinect sensors. In addition, a prototype web portal is also being developed to record performance data during exercises at home and interact with clinicians remotely. A pilot study has been conducted with six healthy individuals and test results show that there is a strong correlation between Kinect data and FSR data in terms of coordination between joint movements. © 2022 IEEE.

Dutch Myositis Working Group (Dmwg)

Introduction. The poster of the Dutch Myositis Working Group (DMWG) aims to inform people about her goals, activities and ambitions. The group is run by seven patients, representing all types of myositis, supported by Spierziekten Nederland, the umbrella patient organization for neuromuscular disorders in The Netherlands and 4 myositis specialists as medical advisors. Chair: Ingrid de Groot. Contact email: myositis@spierziekten.nl Goals and ambitions of the Dutch myositis working group: * I n collaboration with medical advisors to provide information about IIM (idiopathic inflammatory myopathies) or myositis to newly diagnosed patients and their families: IIM types, symptoms, diagnosis, (new) treatment options, prognosis, inform them about the myositis expertise centres etc. * To connect and support people with all types of IIM: dermatomyositis (DM), polymyositis (PM), Anti Synthetase Syndrome (ASyS), immune mediated necrotizing myopathy (IMNM), juvenile dermatomyositis (JDM), overlap myositis. * To raise awareness of myositis among the public, health care professionals and researchers, pharmaceutical companies? * To collaborate with clinicians, researchers and funds on a national and international level with the aim to improve (clinical) care and research. * To stimulate and participate in the development and conducting of clinical trials. * To collaborate with myositis working groups and patient organisations abroad. * To represent the patient perspective within in the Myositis Network Netherlands and (inter)national myositis study groups. * Patient advocacy. Activities and services: * In person or online meetings aiming to offer moral support and an opportunity to share experiences, concerns etc. or just to socialize. Three times a year we organize separate meetings for people with IBM, for people with other IIM and for caregivers. * Website updates on treatment, guidelines, (inter)national research, activities and actualities (e.g. Covid situation). * Supply patients with brochures for GP/ family doctor, physiotherapist etc. * Online (secured) platform for members. * Annual patient conference with diagnosis specific scientific programs. * Monthly newsletters: these are personalized which means they contain mainly news on the receivers type of IIM (e.g. IBM or ASyS) and information on general topics concerning all people with IIM or neuromuscular disorder. * In person meetings and / or online webinars on general topics e.g. living with a chronic condition, work, pain, fatigue. * Annual meetings with medical advisors: the working group pays a visit to all medical advisors in their respective hospitals. * Representation at (inter)national conferences. * Representation in projects such as guidelines development. * Collaboration in (inter)national studies leading to enrolling Dutch patients, researchers and clinicians in multi-centre studies, (co-) authorships in publications and to presentations during conferences (Treat NMD, IMACS, MNN). * To advise and recommend on research proposals from patient perspective. * To advise decision makers on continuation of expert centres from patient perspective. Collaborations: * Myositis Network Netherlands: patient representation on the board. * OMERACT (Outcome Measures in Rheumatology): Patient Research Partner of the Myositis Working Group. * IMACS (International Myositis Assessment and Clinical Studies Group): steering committee member of Exercise & Rehabilitation Group, led by Helene Alexanderson, ass.prof PhD, RPT). * ENMC (European Neuromuscular Centre): patient representation in myositis workshops. * EULAR (European League against Rheumatism): member of PARE and Patient Research Partner. * GCOM. * ERN - NMD (European Reference Network for Neuromuscular Diseases): member of NMD working group led by em. prof. dr. Marianne de Visser. * Patient organizations for people living with myositis . We are in this together Since myositis is a (very) rare disease, the 'myositis community' is a small one although we're happy to say that it is expanding quite rapidly. Through our inte sive involvement in several national and international studies and research projects we now have close contacts with many myositis experts across the globe, which makes it easier to keep up with actualities and developments concerning research, treatment etc. and to disseminate this knowledge to our members. This helps us to inform, support and advocate for the Dutch people living with myositis and their families and at the same time it offers opportunities to give something back: by sharing with the research community and clinicians our experiential knowledge of the consequences of myositis on everyday life. That way we can contribute to more meaningful research. We can only go forward if we do this together! That is why we are very ambitious in our efforts to contribute to myositis research. Here we list our collaborative efforts: * In 2019 the Myositis Network Netherlands of clinicians and researchers with expertise in IIM was established in which the DMWG is representing the patient perspective by a member on the board. * In OMERACT Myositis Working Group a member of the DMWG is one of the two Patient Research Partners and as such an equal partner of this study aiming to define a set of core patient reported domains with regard to the quality of life and respective instruments for use in IIM. The involvement of the DMWG has led to the opportunity for Dutch patients to participate in Delphi surveys and to an opportunity for Dutch myositis clinics to collaborate in the longitudinal study that emerged from this. * The IMACS network is an important part of our international network. One of our DMWG members is member of the Executive Committee of the Exercise & Rehabilitation Group and as such can facilitate for Dutch patients to become involved in the current study with the ultimate objective to develop recommendations for exercise in all types of IIM. * Members of the DMWG participated in several ENMC workshops on IIM as patient representatives and will continue to do so in the future. * Through a PARE membership in EULAR and membership of the study group of 'collaborative research' the DMWG hopes to raise awareness of myositis within the influential EULAR community and to speak up on behalf of the patients in Europe living with IIM. * One of our members is member of the GCOM committee responsible for the patient program of GCOM and shares the ambitions of this GCOM committee to increase the involvement of patients in this very important IIM conference. * One DMWG member joined the ERN- Neuromuscular Disease group and as such represents the people with IIM living throughout Europe. * DMWG has ambitions to empower people living with IIM and to connect with them, crossing borders by doing so. We have close and amicable relationships with patient organisations in Australia, Czech Republic, Germany, Sweden, UK and USA. * Empowering patients is one of our goals and we accomplished this for instance in Sweden. On invitation by prof. dr. Ingrid Lundberg our chair visited the Karolinska Institute, spent a week with their myositis team and in return was one of the speakers on the annual patient meeting and helped the Swedish patients establish their own myositis working group.

Child neurology telemedicine: Analyzing 14 820 patient encounters during the first year of the COVID-19 pandemic

Aim: To determine the long-term impact of telemedicine in child neurology care during the COVID-19 pandemic and with the reopening of outpatient clinics. Method(s): We performed an observational cohort study of 34 837 in-person visits and 14 820 telemedicine outpatient visits across 26 399 individuals. We assessed differences in care across visit types, time-period observed, time between follow-ups, patient portal activation rates, and demographic factors. Result(s): We observed a higher proportion of telemedicine for epilepsy (International Classification of Diseases, 10th Revision G40: odds ratio [OR] 1.4, 95% confidence interval [CI] 1.3-1.5) and a lower proportion for movement disorders (G25: OR 0.7, 95% CI 0.6-0.8;R25: OR 0.7, 95% CI 0.6-0.9) relative to in-person visits. Infants were more likely to be seen in-person after reopening clinics than by telemedicine (OR 1.6, 95% CI 1.5-1.8) as were individuals with neuromuscular disorders (OR 1.6, 95% CI 1.5-1.7). Self-reported racial and ethnic minority populations and those with highest social vulnerability had lower telemedicine participation rates (OR 0.8, 95% CI 0.8-0.8;OR 0.7, 95% CI 0.7-0.8). Interpretation(s): Telemedicine continued to be utilized even once in-person clinics were available. Pediatric epilepsy care can often be performed using telemedicine while young patients with neuromuscular disorders often require in-person assessment. Prominent barriers for socially vulnerable families and racial and ethnic minorities persist.Copyright © 2022 Mac Keith Press.

Remote monitoring and virtual follow-up ventilation clinics in the age of Covid-19

Background: To review the efficacy of long-term non-invasive ventilation (LTV) with virtual follow-up, using assistive technologies and remote monitoring. Objective(s): Compliance greater than 4 hours, correction of hypercapnia and improvements in sleep and quality of life (QoL) were the objectives in improving patient outcome. Method(s): We conducted a retrospective cohort study including patients established on LTV over an 18 month period from the start of the pandemic. Initiation of LTV was established as an inpatient (n= 17;35%), outpatient (n= 28;57%) or from the patient's home (n= 4;8%) and follow-up was by telephone, video consultation or from the patient's own home. LTV was considered successful if compliance achieved was > 4 hours using remote monitoring, corrected hypercapnia (tCO2 < 6.5Kpa) and patient-reported improvements in sleep and QoL. Result(s): Forty-nine patients were included. LTV was initiated for: neuromuscular disease (n=19;39%), obesity hypoventilation or overlap (n=12;24%), chronic obstructive pulmonary disease (n=11;22%) and chest wall deformity (n=6;14%). Compliance > 4 hours was achieved in 69% with effective correction of hypercapnia (tCO2 < 6.5Kpa) in 67% and reported improved sleep and QoL at 63%. Overall success in all 3 objectives was 61%. Patient preference for future follow-up was 45% requesting face-to-face consultations and 33% to continue remotely. A proportion of the most vulnerable (14%) would prefer to be seen at home. Conclusion(s): Successful LTV can be achieved with remote compliance data and transcutaneous carbon dioxide monitoring to support virtual clinics. Patient preference for face-to-face consultations remains important in overall satisfaction.

Nocturnal hypoventilation and respiratory muscle/cough weakness in COVID-19 ICU survivors

Background: The impact of severe COVID-19 in nocturnal hypoventilation and respiratory muscles/cough strength is unknown. Aim(s): Characterize respiratory function in post-ICU COVID-19 patients and correlate these findings with COVID-19 associated outcomes. Method(s): Retrospective study with 55 post-ICU COVID-19 patients admitted to a rehabilitation center (RC). Clinical data were collected and patients performed arterial blood gas analysis, nocturnal oxy-capnography, maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) and peak cough flow (PCF) at admission. Result(s): Mean age was 64.4+/-10.2 years, 39 (70.9%) were male. Co-morbidities were present in 50 (90.9%): 39 (70.9%) hypertension;20 (36.4%) diabetes;3 (5.5%) OSA;1 (1.8%) COPD. None had neuromuscular disorders. Median of ICU length was 25.5 (min 5;max 190) days. Fifty-three (96.4%) needed mechanical and invasive ventilation, 6 (10.9%) ECMO and 22 (40%) tracheostomy (all decannulated before admission to our RC). Eighteen (32.7%) had criteria of nocturnal hypoventilation, 17 (30.9%) inspiratory, 16 (29.1%) expiratory and 17 (30.9%) cough weakness. Sepsis correlated with increased length of ICU stay (p=0.007) and with lower PCF values (p=0.048). Neurologic disfunction was associated with lower minimum SpO2 and more time of SpO2<88% (p=0.032;p=0.035). Renal disfunction was associated with higher values of mean and maximum TcCO2 (p=0.003;p=0.011). Patients with previously diagnosed OSA had higher values of MIP and PCF (p=0.006;p<0.001) at evaluation. Conclusion(s): In COVID-19 ICU survivors, nocturnal hypoventilation, inspiratory/expiratory muscle and cough weakness are common and may have an impact in rehabilitation outcomes.

Management of Spinal Muscular Atrophy New coordinated platform between patient and clinician in COVID-19 pandemic

Iran has a population of ~83 million people with marked cultural and economic differences. Due to culture and consanguineous marriage, inherited neuromuscular diseases (NMDs) are more common than some other countries. Iranian registry of SMA patients has been started with the onset of the outbreak of Covid-19. Due to the multi-organ involvement in COVID-19 infection and higher risks of mortality and morbidity for the neuromuscular disorders, ofering rehabilitation and management of these patients especially SMA can be highly effective in the complications of the disease as well as the number of hospitalizations. With considering this situation, new platform for registry and management of SMA has been developed. In this platform, Tele rehabilitation as well as the creation of parent organizations, are contributing to the increased quality of the management of SMA patients. Over the past months, this care has provided new experiments. This article describes the outcome of this joint efort and shares the experience gained in this registry.

SEROPOSITIVE MYASTHENIA GRAVIS AS THE CAUSE OF MULTIPLE FAILED EXTUBATIONS AND A PROLONGED ICU STAY FOR COVID-19 PNEUMONIA: A CASE REPORT AND BRIEF LITERATURE REVIEW

SESSION TITLE: Autoimmune Disorders: Both Primary and Secondary SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Neurologic sequelae of COVID infection appear to be common. The infection may present with neurologic symptoms, unmask neurologic disease, or worsen established disease. Myasthenia gravis (MG) is an autoimmune neuromuscular disease (NMD) that does not appear to be a usual COVID sequela. We present an elderly veteran with COVID pneumonia who struggled to wean from mechanical ventilation (MV) secondary to neuromuscular weakness. He was ultimately diagnosed with seropositive MG. CASE PRESENTATION: A 79 year old male with a history of prior COVID infection complicated by need for mechanical ventilation (MV) complained of progressive cough and shortness of breath. He was admitted for treatment of community-acquired pneumonia. On hospital day 8, he developed respiratory failure, was intubated, and was transferred to the intensive care unit (ICU). He was diagnosed with COVID a second time. After antibiotics and supportive treatment, he successfully completed a spontaneous breathing trial and was extubated. Within 24 hours, he developed hypercapnia, necessitating reintubation. Given his need for repeat intubations, we ordered myositis titers and MG autoantibodies. After a fourth failed extubation, a tracheostomy was placed. On hospital day 32, his acetylcholine receptor binding antibody returned positive at 30.0, suggesting seropositive MG. His MG composite score was 11 (for ptosis and ventilator dependence). For further work-up, a CT chest excluded thymoma;a focused neurological exam was limited by sedation, and inpatient electrodiagnostics were not feasible. He received 5 days of intravenous immune globulin (40 mg), a Prednisone taper, and Rivastigmine 60 mg thrice daily. His symptoms improved and he was transferred to the floor. DISCUSSION: It is well established that coronaviruses exhibit neurotropism. However, it is unclear whether the novel coronavirus SARS-CoV-2 unmasks underlying neurologic illness or creates de novo disease. Critical care physicians are often tasked with making an initial diagnosis of neuromuscular disease (NMD). NMD is a known cause of complicated extubations. When the diaphragm and accessory respiratory muscles fatigue, respiratory decompensation ensues as full MV support is removed. In many cases, underlying illness is unmasked during this process of extubation. In our case, it is unknown whether infectious insult led to molecular mimicry and development of autoantibodies or unmasked latent neuromuscular disease. If the infection did cause his disease, it would be one of the first cases of COVID-associated MG to be published. Our case is a reminder that NMD is a secondary cause of extubation failure and may suggest MG as a cause of MV weaning failure secondary to COVID. CONCLUSIONS: Critical care physicians should be aware of this potential neuromuscular complication of COVID infection as it may complicate MV weaning, increase vent days, and prolong ICU stays. Reference #1: Collantes MEV, Espiritu AI, Sy MCC, Anlacan VMM, Jamora RDG. Neurological manifestations in covid-19 infection: A systematic review and meta-analysis. Can J Neurol Sci. 2021 Jan;48(1):66-76. Doi: 10.1017/cjn.2020.146. Epub 2020 Jul 15. PMID: 32665054. Reference #2: Huber M, Rogozinski S, Puppe W, Framme C, Hoglinger G, Hufendiek K, Wegner F. Postinfectious onset of myasthenia gravis in a COVID-19 patient. Front Neurol. 2020 Oct 6;11:576153. Doi: 10.3389/fneur.2020.576153. eCollection 2020. PMID: 33123081. Reference #3: Muralidhar Reddy Y, B SK, Osman S, Murthy JMK. Temporal association between SARS-CoV-2 and new-onset myasthenia gravis: Is it causal or coincidental? BMJ Case Rep. 2021 Jul 21;14(7):e244146. Doi: 10.1136/bcr-2021-244146. PMID: 34290032. DISCLOSURES: No relevant relationships by Jeffrey Li No relevant relationships by Anupa Nadkarni No relevant relationships by Justin Owens No relevant relationships by Jennifer Perry no disclosure on file for Hayley Sp res;

COVID-19 PNEUMONIA COMPLICATED BY EXACERBATION OF NEW DIAGNOSIS OF MYASTHENIA GRAVIS

SESSION TITLE: COVID-19 Case Report Posters 1 SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: COVID-19 infection with concurrent myasthenia gravis (MG) presents a unique diagnostic challenge for the clinician. We report the case of a patient presenting with respiratory failure secondary to COVID-19 Pneumonia and being diagnosed with MG. CASE PRESENTATION: Patient is a 72-year-old male without any significant medical history presented with progressively worsening shortness of breath, associated dysphagia and fatigue for 1-week duration. Laboratory data and imaging findings were suggestive of COVID-19 pneumonia. Patient's respiratory status deteriorated requiring mechanical ventilation. His oxygenation improved with steroid and anti-viral therapy. However, he was unable to be liberated from the ventilator secondary to neuromuscular weakness. Further work up confirmed MG. Patient was treated with high-dose steroids, pyridostigmine and intravenous immunoglobulin therapy. Patient was unable to be extubated and required a tracheostomy placement. DISCUSSION: Observational studies have suggested the rate of MG exacerbation to be 10-15% in COVID-19 infection. Mortality was found to be significantly higher in these patients compared to patients without MG. Several case reports have also shown exacerbation of previously asymptomatic MG with COVID-19 infection. Studies have indicated underlying MG to be an independent prognostic risk factor in COVID-19 infection. Treatment involves achieving symptomatic improvement with use of pyridostigmine, corticosteroids and long-term steroid sparing agents, in addition to administering usual treatment for COVID-19 infection. CONCLUSIONS: Our patient presents as a case of COVID 19 infection complicated with a possibly induced or previously undiagnosed case of MG complicating the clinical course. During evaluation of patients with respiratory failure secondary to COVID-19 infection, history focused on symptoms that may indicate underlying neuromuscular diseases should be obtained for early diagnosis and proper management. Given the high co-existence, it is important for clinicians to be aware of the association and treatment strategies in such patients. Reference #1: Galassi G, Marchioni A. Myasthenia gravis at the crossroad of COVID-19: focus on immunological and respiratory interplay. Acta Neurol Belg. 2021;121(3):633-642. doi:10.1007/s13760-021-01612-6 Reference #2: Sriwastava S, Tandon M, Kataria S, Daimee M, Sultan S. New onset of ocular myasthenia gravis in a patient with COVID-19: a novel case report and literature review. J Neurol. 2021;268(8):2690-2696. doi:10.1007/s00415-020-10263-1 Reference #3: Rodrigues CL, de Freitas HC, Lima PRO, et al. Myasthenia gravis exacerbation and myasthenic crisis associated with COVID-19: case series and literature review. Neurol Sci. 2022;43(4):2271-2276. doi:10.1007/s10072-021-05823-w DISCLOSURES: No relevant relationships by Asad Chohan No relevant relationships by Saiara Choudhury No relevant relationships by Rahul Dadhwal No relevant relationships by Rene Franco No relevant relationships by Ahsan Syed No relevant relationships by Pahnwat Taweesedt No relevant relationships by Abhay Vakil

PROGRESSIVE ASCENDING WEAKNESS AFTER THE PFIZER/BIONTECH COVID-19 VACCINE

SESSION TITLE: Autoimmune Disorders: Both Primary and Secondary SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Myasthenia gravis (MG) occurs sporadically with no known causes. We present a rare case of new onset MG s/p COVID-19 vaccination. CASE PRESENTATION: A healthy 46-year-old female presented with progressing LE weakness for 3 months. Symptoms started 5 days after her initial Pfizer COVID-19 vaccine. Her workup showed negative neuroimaging, bland basic CSF studies from LP, with negative MS profile and AChR Ab. She presented again in 1 month with difficulty rising from a seated position, raising her arms above her head with blurry vision. Exam showed bilateral ptosis that improved with an ice pack test, weakness is worst in proximal muscles, but normal reflexes. Workup was again negative. Pyridostigmine was added after discharge (DC). 2 months after, she was admitted to the ICU for acute progressive fatiguability and dyspnea. EMG/NCS of the ulnar nerve showed 60-70% electrical decrement. She underwent therapeutic PLEX. Prednisone was added at DC followed by mycophenolate. 2 weeks later, she was again admitted with myasthenic crisis. She again underwent PLEX with improvement and intubation was avoided. Biweekly PLEX was started at DC. Testing for AChR, MuSK, and LRP4 Abs were initially negative, but AChR Abs were present 6 months later. She then underwent thymectomy showing hyperplasia. DISCUSSION: MG exacerbations have been attributable to infections (50%) and medications (30%). This has worsened during the COVID-19 pandemic especially when medications such as azithromycin were used to treat acute infections. While vaccine-induced flares or onset of autoimmune diseases have been described in literatures, new onset MG following vaccines is rare, limited to 1 to 3 case reports. No case, to our knowledge, correlated to the 1st dose like our patient. The temporal relationship between the COVID-19 vaccination and onset of MG symptoms in our patient could represent a correlation, but does not prove causality. Perhaps a more plausible theory is that the vaccine may have unmasked a previously unrecognized disease in high-risk patient. We ask if the COVID vaccine induces a similar cytokine storm, which hyperstimulates the immune system to a point that breaks immunologic self-tolerance. Interestingly, our patient was initially seronegative, but the presence of AChR Ab was confirmed after sensitive cell-based assays testing. Our patient may have had pre-existing self-antigens to the AChR that were released after receiving the Pfizer COVID-19 vaccine. CONCLUSIONS: The rate of COVID-19 vaccinations will soon surpass that of infections placing vulnerable individuals at risk for MG onset. Recognizing this risk will open discussions about vaccine safety. In doing so, we can begin to formulate new parameters for post-vaccination monitoring. The risks of and complications from acute COVID-19 still outweigh the rare adverse events from vaccines;thus, eligible patients should be offered the COVID-19 vaccine. Reference #1: Guidon AC, Amato AA. COVID-19 and neuromuscular disorders. Neurology. 2020 Jun 2;94(22):959-969. doi: 10.1212/WNL.0000000000009566. Epub 2020 Apr 13. PMID: 32284362. Reference #2: Tagliaferri AR, Narvaneni S, Azzam MH, Grist W. A Case of COVID-19 Vaccine Causing a Myasthenia Gravis Crisis. Cureus. 2021;13(6):e15581. Published 2021 Jun 10. doi:10.7759/cureus.15581 Reference #3: Chavez A, Pougnier C. A Case of COVID-19 Vaccine Associated New Diagnosis Myasthenia Gravis. Journal of Primary Care & Community Health. January 2021. doi:10.1177/21501327211051933 DISCLOSURES: No relevant relationships by andrew bui No relevant relationships by Sharonya Shrivastava

Clinical Features of the UK Myotonic Dystrophy Patient Registry

The UK Myotonic Dystrophy Patient Registry is a patient self-enrolling online database collecting clinical and genetic information about myotonic dystrophy type 1 (DM1) and type 2 (DM2). The registry was established in May 2012 with support from Muscular Dystrophy UK and the Myotonic Dystrophy Support Group and is coordinated Newcastle University. The registry aims to facilitate academic and clinical research, better characterise and understand DM, and disseminate information relating to upcoming studies and research advancements. The registry is used to capture longitudinal, selfreported data through an online portal available to patients and clinicians. Where specialised clinical or genetic information is required, the neuromuscular specialist involved in the patient's care can be invited to provide some additional information and the patient can select them from a pre-populated list at the registration stage. The registry is a Core Member of the TREAT-NMD Global Registries Network for DM1. Between May 2012 and January 2022, there were 834 patient registrations. On average there are 5 new registrations per month. For those reporting a clinical diagnosis, 96% have DM1 (of which 14% have a diagnosis of congenital DM) and 4% have DM2. Overall, 40% of patients have had genetic confirmation of their condition provided. The registry has previously supported almost 30 research enquiries to date. Since 2020, the registry has facilitated 11 enquiries including an industry enquiry, three COVID-19 surveys, and various surveys capturing information on dysphagia, pregnancy, patient preferences for future treatments and the patient/ caregiver experience. The registry continues to be a versatile, cost-effective research tool, helping facilitate and advance a range of DM research. Additional work continues to be done to improve reporting of genetic information on the registry and there are future data linkage plans between the registry and the Newcastle Research Biobank for Rare and Neuromuscular Diseases.

Clinical Features of the UK FSHD Patient Registry

The UK Facioscapulohumeral Muscular Dystrophy (FSHD) Patient Registry is a patient self-enrolling online database collecting clinical and genetic information about FSHD type 1 (FSHD1) and type 2 (FSHD2). The registry was established in May 2013 with support from Muscular Dystrophy UK and is coordinated by Newcastle University. The registry aims to facilitate academic and clinical research, better characterise and understand FSHD, and disseminate information relating to upcoming studies and research advancements. The registry is used to capture longitudinal, selfreported data through an online portal available to patients and clinicians. Where specialised clinical or genetic information is required, the neuromuscular specialist involved in the patient's care can be invited to provide some additional information and the patient can select them from a pre-populated list at the registration stage. The registry is a Core Member of the TREAT-NMD Global Registries Network for FSHD. Between May 2013 and January 2022, there were 1,074 patient registrations, with 84% based in the UK. On average, there are 9 new registrations per month. For those reporting a clinical diagnosis, 97% have FSHD or FSHD1, and 3% have FSHD2. Overall, 46% of patients have had genetic confirmation of FSHD1 provided. The registry has previously supported almost 30 registry enquiries to date. Since 2020, the registry has facilitated 12 enquiries including, three COVID-19 surveys, and various surveys capturing information on dysphagia, pregnancy, sleep and the patient/caregiver experience. The registry is currently one of the largest national FSHD patient registries and is an example of a versatile, cost-effective research tool, helping facilitate and advance a wide range of FSHD research. Additional work continues to be done to improve reporting of genetic information on the registry and there are future data linkage plans between the registry and the Newcastle Research Biobank for Rare and Neuromuscular Diseases.

Humoral Responses Following SARSCoV- 2 Vaccination in Patients with Commonly Used Immunosuppressants in Neuromuscular Disorders

Background: Disease-specific studies have reported impaired humoral responses after SARS-CoV-2 vaccination in patients with immune-mediated inflammatory disorders (IMIDs) treated with specific immunosuppressants and immunomodulating agents. The objective of this study is to investigate the humoral immune response after SARS-CoV-2 vaccination in patients using immunosuppressive and immunomodulating mono- and combination therapies, focussing on frequently prescribed therapies for inflammatory neuromuscular diseases. Methods: National prospective observational cohort study in selected patients with prevalent IMIDs including neuromuscular disease, and immunosuppressive or immunomodulating monotherapy (n=1273), combination therapies (n=419), patients without immunosuppressants (n=473), and healthy controls (n=174). Anti-RBD IgG responses and neutralisation capacity were monitored following standard vaccination regimens and a three-vaccination regimen in subgroups. Hybrid immune responses, i.e. vaccination after previous SARS-CoV-2 infection, were studied as a proxy for recall responses. Findings: Sera from 1869 participants without and 470 participants with previous SARS-CoV-2 infection were analysed. We included 168 (7 2%) patients with inflammatory neuropathies and myopathies, and 127 (5 4%) patients with myasthenia gravis. Humoral responses did not differ between disorders. Anti-CD20 therapy and mycophenolate mofetil combined with corticosteroids were associated with lower relative risks (RR) for reaching seroconversion following standard vaccination (RR: 0 32 and 0 61 respectively). The monotherapies corticosteroids, purine antagonists, methotrexate, mycophenolate mofetil and IVIg were not associated with a lower RR for reaching seroconversion (RR: 0 97, 0 98, 1 01, 0 86, and 0 99, respectively). Similarly, corticosteroids combined with either methotrexate or purine antagonists was not associated with a lower RR for reaching seroconversion (RR 0 89). A third vaccination increased seroconversion for mycophenolate mofetil combination treatments but not for anti-CD20 therapies. Most immunosuppressant groups showed moderately reduced antibody titres after standard vaccination that, in subgroups, did not increase after a third vaccination, although seroconversion rates and neutralisation capacity were unaffected. In participants with previous SARS-CoV-2 infection, SARS-CoV-2 antibodies were boosted after vaccination, regardless of immunosuppressive treatment. Interpretation: Humoral responses following vaccination are impaired by specific immunosuppressants, most relevant for neuromuscular diseases being anti-CD20 and mycophenolate mofetil combination treatments. After standard vaccination regimens most immunosuppressants show equal seroconversion to controls although antibody titres may be moderately reduced. As neutralisation capacity and recall responses are also preserved in these patients, this is not likely to translate in loss of (short term) protection. Alternatively, in immunosuppressants showing poor humoral responses after standard vaccination regimens such as, a third vaccination resulted in additional seroconversion in mycophenolate mofetil combination treatments whereas the effect for anti-CD20 therapy was limited.

Disease Activity after SARS-CoV-2 Vaccination and Infection in Patients with Immune-Mediated Neuromuscular Diseases

Importance: Viral infection or vaccination has the potential to increase disease activity in immune-mediated neuromuscular diseases. Objective: We aimed to evaluate whether SARSCoV- 2 vaccination and infection leads to increase of disease activity in patients with immune-mediated neuromuscular diseases. Methods: This is an interim analysis of a subset of patients from an ongoing prospective multi-center cohort study on SARS-CoV-2 vaccination in patients with various immune mediated inflammatory diseases in the Netherlands, the Target to-B!-COVID study (T2B!). Patients received digital questionnaires every two months from study entry to assess disease activity compared to previous visit using a 5-point Likert scale. In addition, in case of SARS CoV-2 infection (prior to vaccination) patients received an extra questionnaire to assess disease activity in the four weeks after infection. In cases of self-reported increase of disease activity, medical files were used to assess whether disease activity was reported by the treating physician, and whether changes were made in type or dose of immunosuppressive or immunomodulating treatment. Results: In total, we included 303 patients with immune-mediated neuromuscular disease of which 127 patients with inflammatory neuropathies, 133 patients with myasthenia gravis, and 43 patients with myositis. In the four months after completed vaccination, 67 (22.1%) patients indicated an increase in disease activity, of which 62 (93%) was reported as worse and 5 (7%) as much worse. In 10 (3.3%) of the cases with self-reported increase, disease activity was also reported by the treating physician in the medical chart. In 4 (1.3%) of patients with self-reported increase disease activity treatment was adjusted because of the increase in disease activity. A SARS-CoV-2 infection prior to vaccination occurred in 24 (8%) patients, from which 3 (12.5%) indicated an increase in disease activity, not leading to change in treatment. Conclusion: Increase of disease activity after SARS-CoV-2 vaccination or infection was reported infrequently, and was self-limiting in most cases. Findings from our cohort may help physicians in neuromuscular disease to adequately inform patients on the risk of increased disease activity due to SARS-CoV-2 vaccination or infection. Full and verified results will be reported at the ICNMD 2022.

Neuromuscular Diseases Associated with COVID-19 Vaccines

Several vaccine strategies are in use against the SARS-CoV2 virus which causes COVID-19. There are three major approaches to vaccine development: Using the whole virus (live virus, inactivated virus, or viral vector vaccines), using immunogenic parts of the virus, or using the genetic material of the virus. As of April 29, 2022, 10 vaccines with different mechanisms of action have received EUL by WHO.1 In the United States, 3 vaccines have received either emergency use authorization or FDA approval.2 The timing and number of injections in the primary series and timing and need for booster doses of these vaccines depend on the age of the person, underlying immunocompromised status.4 The vaccines have overall been demonstrated to be safe and effective in preventing COVID-19 infection and also in preventing serious COVID-19 infection. However they are rarely associated with some serious side effects. Thrombosis with thrombocytopenia syndrome (TTS) after Johnson & Johnson/ Janssen adenoviral vector vaccine is a rare complication that appears to affect women between the ages of 30 and 49 years. It has also been described with the Oxford/Astra Zeneca vaccine. Based on the available data there is no increased risk for TTS after mRNA vaccination, although cases of immune thrombocytopenia have bene reported after the Pfizer/ BioNTech and Moderna vaccines.3,4 An increased risk of Guillain-Barré syndrome has also been associated with this the Janssen vaccine.3 Myocarditis and pericarditis, particularly in male adolescents and young adults have been associated with the Pfizer and Moderna mRNA vaccines.5 Currently, antibody testing either post infection or post vaccination are not recommended for various reasons.6, 7 Preexposure prophylaxis with a combination of 2 long-acting antibodies, tixagevimab-cilgavimab target the receptor binding domain of the SARS-CoV-2 spike protein and inhibit virus attachment. In December 2021, the US FDA provided emergency use authorization for preexposure prophylaxis of COVID- 19 in patients 12 years or older weighing at least 40 kg, not currently infected with COVID-19 or have a known recent exposure and either have moderate to severe immunocompromise due to a medical condition or receipt of immunosuppressive treatments and may not mount an adequate immune response to COVID-19 vaccines, or in whom available vaccines are not recommended due to prior severe adverse reaction.8 This consists of 2 consecutive intramuscular injections at the same visit and the effi cacy appears to last for up to 6 months. It was granted marketing authorization in the EU in March 2022.