How to define and enhance diagnostic and assistance pathways in neuromuscular diseases during the COVID-19 pandemic: the concept of network.

The main consequence of the COVID-19 pandemic has been to increase the distance between patients and their doctors and to limit the opportunities to compare experiences and clinical cases in the medical community. Based on this, we adopted a strategy to create networks with the ambition to break down these distances and to unify the process of care and management. Here we report the results and perspectives of our efforts and studies. A summary of the presentations on the topic, held during the webinars organized for macro-areas by the Italian Association of Myology with the aim of raising awareness among "non-expert doctors" who deal with neuromuscular disorders in the era of COVID-19 was collected and here reported. Although the macro-areas responded in different way to the problems of neuromuscular patients in the era of COVID-19, they all have tried to create a network between doctors and opportunity for education and information, with the secondary outcome to have shared process of care and management. Telemedicine, virtual meetings and the strengthening of national and international networks, through research projects, were the nodal and common points. Due to their complexity, neuromuscular diseases had already taught clinicians the importance of multidisciplinary confrontation. COVID-19 has further strengthened the need to create links between clinicians and experts, even of different nationalities, in order to guarantee to patients the best possible care, but above all, access and continuity of care even in critical periods. Adequate answers have been given to these problems, though there is still a lot to improve.

A post pandemic roadmap toward remote assessment for neuromuscular disorders: limitations and opportunities.

Recent advances in technology and expanding therapeutic opportunities in neuromuscular disorders has resulted in greater interest in and development of remote assessments. Over the past year, the rapid and abrupt COVID-19 shutdowns and stay-at-home orders imposed challenges to routine clinical management and clinical trials. As in-person services were severely limited, clinicians turned to remote assessments through telehealth to allow for continued care. Typically, disease-specific clinical outcome assessments (COAs) for neuromuscular disorders (NMD) are developed over many years through rigorous and iterative processes to fully understand their psychometric properties. While efforts were underway towards developing remote assessments for NMD before the pandemic, few if any were fully developed or validated. These included assessments of strength, respiratory function and patient-reported outcomes, as well as wearable technology and other devices to quantify physical activity and function. Without many choices, clinicians modified COAs for a virtual environment recognizing it was not yet known how they compared to standard in-person administration. Despite being able to quickly adapt to the demands of the COVID-19 pandemic, these experiences with remote assessments uncovered limitations and opportunities. It became clear that existing COAs required modifications for use in a virtual environment limiting the interpretation of the information gathered. Still, the opportunity for real-world evaluation and reduced patient burden were clear benefits to remote assessment and may provide a more robust understanding and characterization of disease impact in NMD. Hence, we propose a roadmap navigating an informed post-pandemic path toward development and implementation of safe and successful use of remote assessments for patients with NMD.

Early effects of the COVID-19 pandemic on the acute flaccid paralysis surveillance in East and Southern African countries.

INTRODUCTION: the World health organisation (WHO) African Region reported the first confirmed COVID-19 case caused by the SARS-CoV-2 on 25th February 2020, and the first case for the East Southern Africa (ESA) sub-region was on 5th March 2020. Almost all countries in the ESA sub region implemented the WHO-recommended preventive measures variably after the notification of community transmission of the COVID-19 disease. This resulted in the disruption of the outpatient, immunization surveillance, and the related supply chain activities. METHODS: a comparative analysis study design of secondary acute flaccid paralysis (AFP) surveillance data received from the East and Southern Africa sub-region countries to evaluate the effect of the COVID-19 pandemic in the AFP field surveillance for the same time period of March to December 2019 and 2020. RESULTS: we observed that 52.4% of second stool samples were received in the laboratory within 72 hours from March to December 2019, and only 48.1% in the same period of 2020. A 4.3% decline with a p-value of <0.0001 (95% CI, ranges from 2.326% to 6.269%). Similarly, we noted a 4.7% decline in the number of reported AFP cases in the ESA sub-region for March to December 2020 compared to the same period in 2019, a p-value of less than 0.001 (95% CI ranges from 2.785 to 6.614). For the percentage of stool adequacy, we observed a 3.37% decline for April in 2020 compared to April 2019 with a p-value of less than 0.001 (95% CI ranges from 2.059 to 4.690). CONCLUSION: we observed a decline in the core AFP surveillance (non polio) NP-AFP rate, and percentage of stool adequacy in countries severely affected by the COVID-19 disease. These countries implemented stringent transmission prevention measures such as lock-down and international transportation restrictions.

Impact of COVID-19 on the quality of life of patients with neuromuscular disorders in the Lombardy area, Italy.

INTRODUCTION: /AIMS: Patients with neuromuscular disorders (NMDs), including many elderly, immunosuppressed, and disabled individuals, may have been particularly affected during the coronavirus disease 2019 (COVID-19) pandemic in Lombardy, a COVID-19 high-incidence area between February and May 2020. We aimed to evaluate the effects of the COVID-19 pandemic on the quality of life (QoL) and perceived disease burden of this group of patients. METHODS: We conducted a cross-sectional phone-based survey study between June 1 and June 14, 2020, on a sample of 240 NMD patients followed at our clinic in Milan, Italy. We asked about perceived NMD burden and QoL before and during the COVID-19 pandemic. We collected responses on access to outpatient care and ancillary services. We investigated the presence of symptoms suggestive of COVID-19 infection and confirmed cases. RESULTS: We collected 205 responses: 53 patients (25.9%) reported a subjective worsening of the underlying NMD. QoL measures showed a significant worsening between pre and pandemic time frames (odds ratio, 2.14 95%; confidence interval, 1.82-2.51). Outpatient visits were postponed in more than half of cases (57.1%), with 104 patients (50.7%) experiencing a cancellation of scheduled diagnostic tests. 79 patients (38.5%) reported at least one symptom attributable to COVID-19 infection. Among the 10 patients tested with nasopharyngeal swabs, 6 tested positive and 3 died from respiratory failure, including 2 patients on corticosteroid/ immunosuppressive therapy. DISCUSSION: The COVID-19 pandemic affected QoL and limited access to outpatient care and ancillary services of NMD patients in Lombardy between February and May 2020.

[Neuromuscular complications of SARS-CoV-2 infection-Part 2: muscle disorders]. / Neuromuskuläre Komplikationen einer SARS-CoV-2-Infektion ­ Teil 2: Erkrankungen der Muskulatur.

Apart from disorders and diseases of the peripheral nerves, symptoms and disorders of the musculature and the neuromuscular transmission have also been described in association with coronavirus disease 2019 (COVID-19). In the second part of our review we provide an overview about frequently reported symptoms, such as myalgia as well as defined disorders, such as rhabdomyolysis, myositis, myasthenia and intensive care unit (ICU)-acquired weakness, which have been described during severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections or COVID-19.Furthermore, the criteria for a causality, such as association strength, plausibility, time course, and experimental evidence for a causal association that should be applied for the COVID-19-asssociated neuromuscular conditions described in the two parts of the review are discussed. At present, in addition to anosmia, which is also known in the lay press, myalgia in particular as a nonspecific symptom are frequent sequelae of a symptomatic SARS-CoV­2 infection. Other neuromuscular complications seem to be principally plausible (considering the pathogenesis) but apparently rare consequences of a SARS-CoV­2 infection. Prospective or cohort studies are necessary to confirm a causality and assess the risk.

Ethical decision-making for children with neuromuscular disorders in the COVID-19 crisis.

The sudden appearance and proliferation of coronavirus disease 2019 has forced societies and governmental authorities across the world to confront the possibility of resource constraints when critical care facilities are overwhelmed by the sheer numbers of grievously ill patients. As governments and health care systems develop and update policies and guidelines regarding the allocation of resources, patients and families affected by chronic disabilities, including many neuromuscular disorders that affect children and young adults, have become alarmed at the possibility that they may be determined to have less favorable prognoses due to their underlying diagnoses and thus be assigned to lower priority groups. It is important for health care workers, policymakers, and government officials to be aware that the long-term prognoses for children and young adults with neuromuscular disorders are often more promising than previously believed due to a better understanding of the natural history of these diseases, benefits of multidisciplinary supportive care, and novel molecular therapies that can dramatically improve the disease course. Although the realities of a global pandemic have the potential to require a shift from our usual, highly individualistic standards of care to crisis standards of care, shifting priorities should nonetheless be informed by good facts. Resource allocation guidelines with the potential to affect children and young adults with neuromuscular disorders should take into account the known trajectory of acute respiratory illness in this population and rely primarily on contemporary long-term outcome data.