Guillain-Barre syndrome following COVID-19 vaccination: a case report and an updated review

Background: Coronavirus disease-2019 (COVID-19) has caused a pandemic that has recently affected every aspect of life. Fortunately, many vaccines with high safety and efficacy profiles were developed timely to face this pandemic. In a very short time, billions of people were vaccinated. In the meantime, a wide range of neurological syndromes are being reported. Guillain-Barre syndrome (GBS) which is a rare immune-mediated post-infectious peripheral neuropathy was reported after both the COVID-19 infection itself and many types of its vaccines. Method(s): We are reporting a case of post-AstraZeneca vaccine GBS and reviewing the literature of all reported post-COVID-19 vaccines GBS till July 2021. Result(s): 29 adult patients were reported. Of them 58.6% were males. Their mean age is 58.2 years. The median time to clinical onset after vaccine administration was 13.2 days. 86.2% of patients had their symptoms following immunization with the 1st dose of AstraZeneca vector-based covid vaccine. Facial palsy was the most predominant single symptom in 75.8% of patients. Conclusion(s): Guillain-Barre syndrome is a well-recognized but still rare adverse event following vaccination against COVID-19. Although preliminary data incriminates viral vector-based vaccines more than the other types, active post-vaccination surveillance and more powerful statistics are mandatory to reach a solid conclusion regarding the presence of a causal relation.Copyright © 2022

Case Presentation Competition, 5 Back to Basics

Serotonin syndrome associated with clozapine withdrawal and concurrent selective serotonin reuptake inhibitor (SSRI) use has previously been reported. A 56-year-old female with schizophrenia was admitted for pyrexia, rigidity, and altered mental state after her second dose of clozapine restart. She had discontinued her long-term clozapine 2 weeks prior. She developed ventilatory failure, reduced consciousness, eye deviation, and worsening rigidity, requiring ICU support. Examination showed a right upper motor neurone syndrome with absent ankle reflexes. She had raised inflammatory markers and creatine kinase. Serum neuropathy, encephalitis screen, and COVID PCR were negative. Respiratory investigations were unfruitful. MRI head and spine did not show brain or cord signal change to correlate to signs. Lumbar puncture showed a quiet CSF, negative culture, viral PCR, and encephalitis antibodies. EEG showed bihemispheric background slowing. Despite clinical improvement, repeat examination showed persistent signs. She was diagnosed with serotonin syndrome after developing a bilateral tremor. Treatment with cyproheptadine correlated with an improvement in her signs, cognitive state, and EEG. Serotonin syndrome can present with reversible neuromuscular signs. With clozapine withdrawal, it can require a prolonged time course of recovery in contrast with classical serotonin syndrome. Cyprohepta- dine can cause agranulocytosis and this delays clozapine restart.

Experience with telemedicine in neuromuscular clinic during COVID-19 pandemic.

Objectives: The aim of the present study was to evaluate the feasibility and acceptability of telehealth for the care of neuromuscular patients during the COVID-19 pandemic. Methods: Neuromuscular patients or their caregivers, as well as health care providers (HCPs), who completed a televisit during the pandemic received an online survey, assessing satisfaction with the visit, quality of care, and experience with the televisit interference. Results: Surveys from 46 neuromuscular patients (including 18 with motor neuron disease [MND])/caregivers and 7 HCPs were completed. Several aspects of televisits including good communication, adequate time to discuss concern, provision of equal care, and telemedicine interference were rated favorably among participants. Telehealth was strongly satisfactory in 30 (65.22%) and satisfactory in 15 (32.61%) neuromuscular patients/caregivers. In 18 MND patients, this was 10 (55.56%) and 7 (38.89%), respectively. Moreover, 24 (52.17%) neuromuscular patients/caregivers would strongly agree and 18 (39.13%) would agree to participate again in televisits. This was 10 (55.56%) and 4 (33.33%) for MND cases, respectively. Various medical issues were addressed during the televisits including medication management, ordering tests/referrals, discussion of goals of care, and research. The predictive stepwise logistic model found younger age as a predicting factor for higher satisfaction from, or participation again in, televisits in neuromuscular patients. Limb onset location was also a predicting factor for strong satisfaction from televisits in MND cases. Conclusions: Telemedicine is feasible and highly effective at achieving personalized care that was rated satisfactory by the majority of neuromuscular patients/caregivers and HCPs during the COVID-19 pandemic.

Ultrasound-guided glenohumeral joint injections for shoulder pain in ALS: A case series.

Introduction: Shoulder pain is a common secondary impairment for people living with ALS (PALS). Decreased range of motion (ROM) from weakness can lead to shoulder pathology, which can result in debilitating pain. Shoulder pain may limit PALS from participating in activities of daily living and may have a negative impact on their quality of life. This case series explores the efficacy of glenohumeral joint injections for the management of shoulder pain due to adhesive capsulitis in PALS. Methods: People living with ALS and shoulder pain were referred to sports medicine-certified physiatrists for diagnostic evaluation and management. They completed the Revised ALS Functional Rating Scale and a questionnaire asking about their pain levels and how it impacts sleep, function, and quality of life at baseline pre-injection, 1-week post-injection, 1 month post-injection, and 3 months post-injection. Results: We present five cases of PALS who were diagnosed with adhesive capsulitis and underwent glenohumeral joint injections. Though only one PALS reported complete symptom resolution, all had at least partial symptomatic improvement during the observation period. No complications were observed. Conclusions: People living with ALS require a comprehensive plan to manage shoulder pain. Glenohumeral joint injections are safe and effective for adhesive capsulitis in PALS, but alone may not completely resolve shoulder pain. Additional therapies to improve ROM and reduce pain should be considered.

Community Non-Invasive Ventilation practitioner service utilisation for Motor Neurone Disease patients following the Covid-19 pandemic

Introduction: The community non-invasive ventilation (NIV) practitioner service is supported by two physiotherapists who provide out-reach domiciliary support to patients with Motor Neurone Disease (MND). The aim of this study was to look at service utilisation, including NIV set-ups and palliative care referrals for this cohort of patients following the Covid-19 pandemic. Method(s): During the UK national lockdowns and the periods of shielding for vulnerable patients, the service was run remotely via telephone support with home visits only undertaken if clinically indicated. We retrospectively studied all patients in the service with MND from January 2019 to December 2021, looking at number of new referrals to the service, NIV set-ups and referrals to palliative care. Result(s): 105 patients were identified with MND, 52% male, mean age 67 (SD 10). Between 2020 and 2021, new referrals increased by 35%, NIV set-ups by 56% and palliative care referrals also increased by 22%. A similar trend was observed for the data comparing 2019 and 2021 (see figure 1). Conclusion(s): This study identified an increase in community MND NIV practitioner service utilisation and palliative care referrals following the Covid-19 pandemic. Further work is needed to understand how the impact of the pandemic on access to healthcare and the shielding of MND patients may have contributed to these findings.

Post-Vaccination/Post-COVID Immune-Mediated Demyelination of the Brain and Spinal Cord: A Novel Neuroimaging Finding.

Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes coronavirus disease 2019 (COVID-19), which not only produces respiratory symptoms but is known to involve almost every system, and its neuroinvasive properties have been well demonstrated throughout the pandemic. Also, to combat the pandemic, there was rapid development and induction of various vaccination drives, following which many adverse events following immunization (AEFIs) have been reported, which include neurological complications as well. Method: We present a series of three cases, post vaccination, with and without a history of COVID illness that showed remarkably similar findings on magnetic resonance imaging (MRI). Result: A 38-year-old male presented with complaints of weakness of the bilateral lower limbs with sensory loss and bladder disturbance a day after receiving his first dose of ChadOx1 nCoV-19 (COVISHIELD) vaccine. A 50-year-old male with hypothyroidism characterized by autoimmune thyroiditis and impaired glucose tolerance experienced difficulty in walking 11.5 weeks after being administered with COVID vaccine (COVAXIN). A 38-year-old male presented with subacute onset progressive symmetric quadriparesis 2 months after their first dose of a COVID vaccine. The patient also had sensory ataxia, and his vibration sensation was impaired below C7. All three patients had typical pattern of involvement of the brain and spine on MRI with signal changes in bilateral corticospinal tracts, trigeminal tracts in the brain, and both lateral and posterior columns in the spine. Conclusion: This pattern of brain and spine involvement on MRI is a novel finding and is likely a result of post-vaccination/post-COVID immune-mediated demyelination.

Impact of the covid-19 pandemic on amyotrophic lateral sclerosis care in the UK.

The Covid-19 pandemic has impacted healthcare. Our aim was to identify how amyotrophic lateral sclerosis (ALS) care in the UK has been affected by the pandemic by exploring the experiences of people living with ALS (plwALS), healthcare professionals (HCPs) working with plwALS, and ALS care centers. Three surveys were carried out to explore the experiences of plwALS, HCPs and ALS care centers during the pandemic. Quantitative data were analyzed using descriptive and inferential statistics and triangulated with the qualitative data which were analyzed thematically. Responses from 53 plwALS, 73 HCPs and 23 ALS care centers were analyzed. Five main themes were identified: keeping safe, losses, negative emotions, delivering care and alternative care delivery in a pandemic. PlwALS and HCPs felt that care was sub-optimal as a result of the pandemic. Changes to care included longer waiting times and face-to-face appointments being canceled or replaced by virtual consultations. While benefits of virtual consultations were reported, concerns were raised about incomplete clinical assessments and the disruption of provision of testing and interventions. ALS care has changed as a result of the pandemic. Patients have had a lack of face-to-face contact with HCPs and have experienced delays to investigations and treatments. PlwALS and HCPs were concerned about the impact of this change, but the long-term implications remain unclear. We propose recommendations for HCPs caring for plwALS, that will promote continuity of evidenced based care in the context of a pandemic.

Primary lateral sclerosis natural history study - planning, designing, and early enrollment.

Introduction/Aims. Primary lateral sclerosis (PLS) is exceedingly rare and has been an enigmatic disease. Recent progress has drastically changed this perception, with early biomarkers being investigated and potential medications for PLS emerging at the preclinical stage. The aim of this paper is to describe a study of PLS natural history and discuss the limitations and proposed solutions to the study of a rare and slowly progressive disease. Methods. The PLS Natural History Study is a 30-site, 24-month, prospective study that is supported by multiple funding sources. The study aims to enroll 50 early PLS (disease duration ≤4 years) and 50 definite PLS (disease duration 4 to 15 years) participants using modified PLS Diagnostic Criteria. Smartphone-based assessments including semi-quantitative and quantitative measures and patient-reported outcomes are utilized. In-person quantitative measures are also completed during site visits. The change in the PLS Functional Rating Scale score is the primary outcome. The study utilizes the NeuroBANK® patient-centric data capture and management platform. The biostatistical analysis plan has been developed. Results. In one year, 28 participants have been recruited. Enrollment has been much slower than anticipated due to the COVID-19 pandemic, the rarity of PLS, and potential study competition for internal resources from ALS clinical trials. Discussion. We discuss the need for more innovative methods to enroll and study individuals with such rare diseases and propose a number of mechanisms by which more efficient enrollment could be facilitated.

A decision making tool and protocol for early cuff deflation and one way valve inline for patients who are ventilated with a tracheostomy - A case series report

Introduction: Early cuff deflation and one way valve placement inline for patients who are ventilated with a tracheostomy allows for the restoration of verbal communication with concomitant psychological benefits, and enables assessment of bulbar function, delirium, pain, airway patency and speech and language problems.1,2 Such an approach has not been shown to impact respiratory or ventilatory outcomes.3-5 Whilst the benefits may seem obvious, the practice of using one way valves inline has not been widely documented and there are a few published patient selection criteria or protocols, but no national guidelines. Our multidisciplinary team designed a novel decision making tool and protocol to improve specialist service provision. Objectives: This was a retrospective audit of i) our decision making tool and ii) our protocol for early cuff deflation, one way valve inline placement and ventilator adjustments in a specialist tertiary referral neuroscience intensive care unit. Methods: The decision making tool and protocol guides the selection of patients and the approach to early cuff deflation. We performed a retrospective analysis of medical and therapy electronic patient records on consecutive patients with whom we had used this tool and protocol over a two year period from December 2018 -December 2020. Data included diagnosis, primary mode of ventilation, aim of first cuff deflation, time tolerated for first cuff deflation (minutes), and number of days between first cuff deflation and decannulation. Results: Eighteen consecutive patients were selected for early cuff deflation using the decision making tool with the following diagnoses: Encephalitis (5), COVID pneumonitis (5), Guillain Barre Syndrome (4), Intracerebral haemorrhage (1), Posterior communicating artery aneurysm (1), Motor Neurone Disease (1), Syringomyelia (1). At the time of the initial assessment, the ventilation status was: 10 patients on CPAP/PS, 4 on SIMV, 2 on High Flow Oxygen Therapy and 2 on a period of self-ventilation. The decision making tool defined the aim of the initial trial as laryngeal wean for 10 patients and to facilitate communication for 8. The median time for one way valve use for the initial trial was 10 minutes (range 4-25). There were no deleterious effects from following the protocol. All patients received further one way valve inline trials, and seventeen were weaned from the ventilator without respiratory compromise. One patient with Motor Neurone Disease remained ventilator dependent. Seventeen patients were subsequently decannulated (median 26.5 days after initial cuff deflation, range 12-209). Conclusions: Eighteen neurointensive care patients were successfully able to use a one way valve inline in accordance with our decision making tool and protocol. It is hoped that our practice will prompt a wider discussion amongst different intensive care multidisciplinary teams about careful patient selection and judicious use of a one way valve inline. We plan to collect patient's experience of the practice and to update our protocol with emerging evidence around optimal ventilator settings for using one way valves inline.

The effect of energy conservation strategies on fatigue, function, and quality of life in adults with motor neuron disease: Randomized controlled trial

Background: Fatigue is one of the most frequent complaints in patients with motor neuron diseases (MNDs), with a significant impact on the quality of life (QOL). There is lack of enough evidence for current pharmacological or non-pharmacological treatments of fatigue in this population to be applied in clinical setting. Energy conservation strategies are one of the key interventions for fatigue management in chronic diseases. We aimed to investigate the effect of applying these techniques in the fatigue management of patients with MND. Methods: This randomized controlled trial (RCT) study was carried out on 28 patients with MND. Participants were randomly assigned to either the intervention or control group. In addition to routine treatment, patients in the intervention group participated in 3 weekly 1-hour energy conservation programs provided by an experienced occupational therapist. The Fatigue Severity Scale (FSS) score, 36-Item Short Form Survey (SF-36), and Canadian Occupational Performance Measure (COPM) were measured at baseline, immediately after the last intervention session, and one month later. Results: FSS and COPM significantly changed after the course in the intervention group (P < 0.001 and P = 0.001, respectively). Both FSS and COPM improved significantly toward the final assessment only in the intervention group. The SF-36 changes were not significant in each of the groups. Conclusion: According to the findings of the present study, using energy conservation strategies could lead to better mid-term fatigue management and occupational performance improvement, but it did not improve QOL in patients with MND.

Palliative Care Congress, Recovering, Rebounding, Reinventing

The proceedings contain 154 papers. The topics discussed include: cordotomy improves pain in palliative cancer patients receiving care from a specialist center, and continues to rebuild following temporary closure due to the COVID-19 pandemic;the impact on staff of providing non-invasive advanced respiratory support during the COVID-19 pandemic' a qualitative study in an acute hospital;levels of grief, support needs and risk factors among people bereaved during the COVID-19 pandemic: baseline results from a longitudinal UK online survey;the palliative care needs of patients with multiple sclerosis, Parkinson's related diseases and motor neuron disease: a secondary analysis of the optcare neuro trial data;a co-speciality cross-boundary model of supportive care: an innovative approach to caring for the palliative care needs of heart failure patients;creating equity of community palliative care in Manchester in a pandemic. does the 'Midhurst model' of care work?;palliative medicine specialist training: designing and implementing an oncology placement fit for the future;managing dying: multimedia end of life care simulation training for medical undergraduates;and use of a novel trigger tool for the identification and referral of patients in the last year of life pallitrigger.

Abstracts of the 12th Annual Meeting of the Italian Association for the study of the Peripheral Nervous System (ASNP)

The proceedings contain 69 papers. The topics discussed include: chemotherapy induced peripheral neurotoxicy: why should we care?;studying the caudal nerve anatomy and physiology to refine detection of peripheral nerve damage in rodent models;anxiety and depression in Charcot-Marie-tooth disease: data from the Italian CMT National Registry;fatigue in CMT: a web based survey from the Italian CMT National Registry;early molecular diagnosis of mutations on the transthyretin gene as a strategy to improve the prognosis of hereditary transthyretin-mediated amyloidosis - an update of the GENILAM project;THR124MET myelin protein zero mutation mimicking motor neuron disease;torsional neuropathy in parsonage turner syndrome following anti-COVID19 vaccination. how to detect and manage with it?;isolated musculocutaneous involvement in parsonage-turner syndrome associated with SARS-COV2 vaccination;neonatal FC receptor expression in patients with chronic dysimmune neuropathy. a feasibility study;and peripheral neuropathies after common organ transplantations. literature review and the use of electrophysiological tests and ultrasound.

THE IMPACT OF THE COVID-19 PANDEMIC ON THE MENTAL HEALTH OF MND PATIENTS

Background Motor neurone disease (MND) is an isolating condition that has many implications on patients mental health. Throughout the COVID-19 pandemic, patients with chronic health conditions, including those with MND, have been further removed from the support of their specialist teams. Objectives 1) To determine the impact of the COVID-19 pandemic on the mental health of the Salford Royal MND patients. 2) To improve the mental health support offered by the service by addressing patient concerns and feedback. Methods A survey was sent to patients who had been receiving care by the Salford Royal MND Team since the 1st of January, 2020. The answers were reviewed and measures were put in place to address concerns. Patients were resurveyed two months later and answers were compared. Results Patients reported a higher proportion of issues with their mental health during the pandemic compared to prior. The service was seen as doing adequately but areas were identified to address. Our interventions were largely successful with many patients reporting an increase in the support they receive from the Salford Royal MND Team. Conclusions Areas of improvement were identified to accommodate the change in the way the Salford Royal MND Team deliver their care.

Novel Selenium-based compounds with therapeutic potential for SOD1-linked amyotrophic lateral sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease as well as Lou Gehrig's disease, is a progressive neurological disorder selectively affecting motor neurons with no currently known cure. Around 20% of the familial ALS cases arise from dominant mutations in the sod1 gene encoding superoxide dismutase1 (SOD1) enzyme. Aggregation of mutant SOD1 in familial cases and of wild-type SOD1 in at least some sporadic ALS cases is one of the known causes of the disease. Riluzole, approved in 1995 and edaravone in 2017 remain the only drugs with limited therapeutic benefits. METHODS: We have utilised the ebselen template to develop novel compounds that redeem stability of mutant SOD1 dimer and prevent aggregation. Binding modes of compounds have been visualised by crystallography. In vitro neuroprotection and toxicity of lead compounds have been performed in mouse neuronal cells and disease onset delay of ebselen has been demonstrated in transgenic ALS mice model. FINDING: We have developed a number of ebselen-based compounds with improvements in A4V SOD1 stabilisation and in vitro therapeutic effects with significantly better potency than edaravone. Structure-activity relationship of hits has been guided by high resolution structures of ligand-bound A4V SOD1. We also show clear disease onset delay of ebselen in transgenic ALS mice model holding encouraging promise for potential therapeutic compounds. INTERPRETATION: Our finding established the new generation of organo-selenium compounds with better in vitro neuroprotective activity than edaravone. The potential of this class of compounds may offer an alternative therapeutic agent for ALS treatment. The ability of these compounds to target cysteine 111 in SOD may have wider therapeutic applications targeting cysteines of enzymes involved in pathogenic and viral diseases including main protease of SARS-Cov-2 (COVID-19). FUNDING: Project funding was supported by the ALS Association grant (WA1128) and Fostering Joint International Research (19KK0214) from the Ministry of Education, Culture, Sports, Science and Technology (MEXT), Japan.