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BMJ Case Rep ; 14(4)2021 Apr 21.
Article in English | MEDLINE | ID: covidwho-1197246


A previously fit and well 72-year-old man was referred to the acute medical unit with acute shortness of breath and confusion. He had presented 6 months earlier to his General Practitioner with a 6-month history of weight loss and lethargy. Despite CT imaging and extensive blood tests, no cause was found. He was having ongoing outpatient investigations, including a respiratory review leading up to his admission; the deterioration in his condition also coincided with the implementation of the COVID-19 lockdown. On admission, he was found to be in acute-on-chronic type 2 respiratory failure; examination revealed scattered fasciculations. Further inpatient electromyography (EMG) and nerve conduction study (NCS) confirmed motor neuron disease (MND). This case highlighted the importance of considering neuromuscular causes for acute respiratory failure in acute presentations and demonstrated the challenges in the diagnosis of MND in those presenting atypically with non-specific symptoms and the limitations of remote consultations in complex cases.

Motor Neuron Disease , Respiratory Insufficiency , Weight Loss , Aged , COVID-19 , Humans , Male , Motor Neuron Disease/complications , Motor Neuron Disease/diagnosis , Respiratory Insufficiency/etiology
Front Neurol ; 12: 607790, 2021.
Article in English | MEDLINE | ID: covidwho-1178007


The coronavirus disease 2019 (COVID-19) pandemic has prompted a rapid and unprecedented reorganization of medical institutions, affecting clinical care for patients with chronic neurological diseases. Although there is no evidence that patients with neuromuscular disorders (NMD) confer a higher infection risk of COVID-19, NMD and its associated therapies may affect the patient's ability to cope with infection or its systemic effects. Moreover, there is a concern that patients with chronic NMD may be at increased risk of manifesting severe symptoms of COVID-19. In particular, as respiratory compromises account for the major cause of mortality and morbidity in NMD patients, newly emerging data also show that the risk of exacerbation caused by COVID-19 accumulates in this particular patient group. For example, patients with motor neuron disease and dystrophinopathies often have ventilatory muscle weakness or cardiomyopathy, which may increase the risk of severe COVID-19 infection. Thus, the COVID-19 pandemic may severely affect NMD patients. Several neurological associations and neuromuscular networks have recently guided the impact of COVID-19 on patients with NMD, especially in managing cardiopulmonary involvements. It is recommended that patients with moderate- to high-risk NMD be sophisticatedly monitored to reduce the risk of rapid decline in cardiopulmonary function or potential deterioration of the underlying NMD. However, limited neuromuscular-specific recommendations for NMD patients who contract COVID-19 and outcome data are lacking. There is an urgent need to properly modify the respiratory care method for NMD patients, especially during the COVID-19 pandemic. Conclusively, COVID-19 is a rapidly evolving field, and the practical guidelines for the management of NMD patients are frequently revised. There must be a close collaboration in a multidisciplinary care team that should support their hospital to define a standardized care method for NMD patients during the COVID pandemic. This article reviews evidence-based practical guidelines regarding care delivery, modification, and education, highlighting the need for team-based and interspecialty collaboration.

BMJ Open ; 11(3): e044996, 2021 03 23.
Article in English | MEDLINE | ID: covidwho-1148166


INTRODUCTION: Motor neuron disease (MND) is a rapidly progressive and fatal neurodegenerative disorder with limited treatment options. The Motor Neuron Disease Systematic Multi-Arm Randomised Adaptive Trial (MND-SMART) is a multisite UK trial seeking to address the paucity in effective disease-modifying drugs for people with MND (pwMND). Historically, neurological trials have been plagued by suboptimal recruitment and high rates of attrition. Failure to recruit and/or retain participants can cause insufficiently representative samples, terminated trials or invalid conclusions. This study investigates patient-specific factors affecting recruitment and retention of pwMND to MND-SMART. Improved understanding of these factors may improve trial protocol design, optimise recruitment and retention. METHODS AND ANALYSIS: PwMND on the Scottish MND Register, Clinical Audit Research and Evaluation of MND (CARE-MND), will be invited to participate in a prospective observational cohort study that investigates factors affecting trial participation and attrition. We hypothesise that patient-specific factors will significantly affect trial recruitment and retention. Participants will complete the Hospital Anxiety and Depression Scale, 9-Item Patient Health Questionnaire and State-Trait Anxiety Inventory-Form Y to evaluate neuropsychiatric symptoms, the ALS-Specific Quality of Life Questionnaire-Brief Form and Centre for Disease Control and Prevention-Health-Related Quality of Life for quality of life and a novel study-specific questionnaire on Attitudes towards Clinical Trial Participation (ACT-Q). Clinical data on phenotype, cognition (Edinburgh Cognitive and Behavioural ALS Screen) and physical functioning (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised) will also be collated. Caregivers will complete the Brief Dimensional Apathy Scale. After 12 months, a data request to MND-SMART will evaluate recruitment and retention. Descriptive statistics will summarise and compare assessments and participants reaching impairment thresholds. Variable groupings: attitudes, quality of life, cognition, behaviour, physical functioning, neuropsychiatric and phenotype. Univariate and multivariable logistic regression will explore association with participation/withdrawal in MND-SMART; presented as ORs and 95% CIs. ETHICS AND DISSEMINATION: Ethical approval was provided by the West of Scotland Research Ethics Committee 3 (20/WS/0067) on 12 May 2020. The results of this study will be published in a peer-reviewed journal, presented at academic conferences and disseminated to participants and the public.

Amyotrophic Lateral Sclerosis , Motor Neuron Disease , Amyotrophic Lateral Sclerosis/therapy , Humans , Observational Studies as Topic , Prospective Studies , Quality of Life , Scotland