Telemedicine applied to neuromuscular disorders: focus on the COVID-19 pandemic era.

Neuromuscular diseases are rare and usually chronic progressive disorders that require a multidisciplinary clinical evaluation and functional monitoring. The patient-physician relationship and therapies are also key elements to be provided. The COVID-19 pandemic dramatically changed the way patients' health was managed and national health care services underwent a radical reorganization. Telemedicine, with the use of Information and Communication Technology (ICT) by health professionals, became the main strategy to ensure the continuation of care. However, the experience regarding the use of Telemedicine in neuromuscular disorders is very limited and the scientific literature is extremely scarce. From the first experiences in the '50s, the development of Telemedicine has been supplemented and supported by the implementation of ICT to guarantee the secure and effective transmission of medical data. Italian national guidelines (2010-2020) describe the technical and professional guarantees necessary to provide Telemedicine services. Nevertheless, at the time the pandemic appeared, no guidelines for clinical evaluation or for the administration of functional scales remotely were available for neuromuscular diseases. This has been a critical point when clinical evaluations were mandatory also for the renewal of drug prescriptions. However, the common opinion that telemedicine basic services were important to overcome the change in medical practice due to COVID-19 in neuromuscular diseases, even in pediatric age, emerged. Moreover, alternative digital modalities to evaluate patients at home in a kind of virtual clinic were considered as a field of future development.

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.

Teleneurology in a center for neuromuscular diseases during the COVID-19 pandemic.

OBJECTIVE: The coronavirus disease 2019 (COVID-19) pandemic has urged the adoption of telemedicine services as a measure of greater patients' safety. This study aims to investigate efficacy and feasibility of teleneurology care for patients with neuromuscular diseases (NMD). METHODS: All patient visits from 4th March 2020 to 27th March 2021, the period of COVID-19 lockdown requiring partial transition to tele-consultation, were reviewed. Demographic data and medical records during the implementation of the "hybrid model" of both remote and in-person visits during COVID-19 era were analyzed and compared to those of the pre-pandemic era. RESULTS: A total of 2179 tele-consultation contacts for 182 patients (mean age 46.4 years, 42.3% female) were conducted. Tele-consultation was primarily performed by telephone calls. During that period, 1745 telephone calls were conducted, resulting in a 4.5 mean increase/patient compared to 2019. There was a 15.1% decrease in first time in-person consultations compared to 2019. However, the mean number of monitoring visits per person during pandemic was no different from previous years. With the exception of 3 patients with advanced stage of amyotrophic lateral sclerosis, no other patients required an unscheduled appointment or hospitalization for unforeseen reasons related to tele-consultation restrictions. CONCLUSION: Monitoring of NMD patients could benefit from tele-provided multidisciplinary services. The experience gained during COVID-19 pandemic could be useful for further development of tele-medicine.

Just Breathe: Tips and Highlights for Managing Pediatric Respiratory Distress and Failure.

Anatomically, the airway is ever changing in size, anteroposterior alignment, and point of most narrow dimension. Special considerations regarding obesity, chronic and acute illness, underlying developmental abnormalities, and age can all affect preparation and intervention toward securing a definitive airway. Mechanical ventilation strategies should focus on limiting peak inspiratory pressures and optimizing lung protective tidal volumes. Emergency physicians should work toward minimizing risk of peri-intubation hypoxemia and arrest. With review of anatomic and physiologic principles in the setting of a practical approach toward evaluating and managing distress and failure, emergency physicians can successfully manage critical pediatric airway encounters.

How to manage with telemedicine people with neuromuscular diseases?

INTRODUCTION: COVID-19 pandemic radically transformed our daily clinical practice, raising the need not to lose close contact with patients without being able to see them face-to-face. These issues are even more felt and evident in fragile patients, as those affected by neuromuscular disease. An important help came from new digital technologies that allow clinicians to remotely monitor health status and any deterioration of chronically ill patients. METHODS: In this mini-review, an initiative of the "Digital Technologies, Web and Social Media Study Group" of the Italian Society of Neurology, we propose to analyze the approach to neuromuscular patients by looking over raising evidence on the main cornerstones of Telemedicine (TM): clinician-patient interaction, remote clinical assessment, remote monitoring, and digital therapeutics. In particular, we explored the strategies developed by researchers and their impact on the physical and emotional status of the patients, with particular focusing on their adherence to the program of virtual monitoring. RESULTS: TM plays an important role in each of four stages of approach to neuromuscular disease, having demonstrated validity in keep close clinical patient interaction, clinical assessment, remote monitoring, and telerehabilitation. Nevertheless, there is no remote alternative to electrophysiological testing neither validate tools to assess disability. CONCLUSION: The role of TM in neuromuscular care is yet underestimated but is crucial, beyond the pandemic era. Further development of TM is advisable, through making specific apps, remotely controlled by clinicians, and making more engaging clinicians-patients interaction. Last, it is necessary to ensure adequate internet access to everyone.

Doctor-Should I get the COVID-19 vaccine? Infection and immunization in individuals with neuromuscular disorders.

The clinical course of neuromuscular disorders (NMDs) can be affected by infections, both in immunocompetent individuals, and in those with reduced immunocompetence due to immunosuppressive/immunomodulating therapies. Infections and immunizations may also trigger NMDs. There is a potential for reduced efficacy of immunizations in patients with reduced immunocompetence. The recent vaccination program for coronavirus disease-2019 (COVID-19) raises several questions regarding the safety and efficacy of this vaccine in individuals with NMDs. In this Practice Topic article, we address the role of vaccine-preventable infections in NMDs and the safety and efficacy of immunization in individuals with NMDs, with emphasis on vaccination against COVID-19.

[NPPV Treatment for Neuromuscular Diseases during the COVID-19 Pandemic].

A novel coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), emerged from Wuhan in December 2019 and resulted in a severe outbreak of pneumonia (COVID-19). SARS-CoV-2 is transmitted through respiratory droplets produced by coughs or sneezes, as well as aerosols containing viral particles. Noninvasive positive pressure ventilation (NPPV) is widely used to support respiration in patients with neuromuscular diseases. However, NPPV is also an aerosol-generating procedure. Without appropriate precautions, the risk of spreading the virus is high in NPPV users infected with SARS-CoV-2. At home, self-quarantine is effective in protecting caregivers of patients using NPPV, whereas in hospitals, using a negative pressure room is preferred. As SARS-CoV-2 can survive on surfaces for several days, disinfecting the NPPV machine and the items frequently touched in the room is essential. Setting viral filters is useful for preventing virus transmission and keeping the inside of the NPPV machine clean. Caregivers must wear appropriate personal protective equipment. Furthermore, it should be paid attention to the potential transmission from asymptomatic SARS-CoV-2 carriers. During the current pandemic, it is necessary to minimize the risk of transmission among patients using NPPV.

Respiratory management for patients with neuromuscular disorders during the COVID-19 pandemic.

The reviews of this paper are available via the supplemental material section.

Neurologic complications of COVID-19.

Patients with COVID-19 have a fairly high risk of neurologic complications, including encephalopathy, stroke, central nervous system infection, seizures, and neuromuscular diseases. Many report losing their senses of smell and taste, and many survivors report lingering neurocognitive impairment. The diagnosis and treatment of these complications does not differ from that in other patients, although sophisticated testing may not be readily available for a patient in intensive care and respiratory isolation. Clinicians should therefore be alert to these complications.

Estimating the impact of COVID-19 pandemic on services provided by Italian Neuromuscular Centers: an Italian Association of Myology survey of the acute phase.

INTRODUCTION: Since February 2020, the outbreak of COVID-19 in Italy has forced the health care system to undergo profound rearrangements in its services and facilities, especially in the worst-hit areas in Northern Italy. In this setting, inpatient and outpatient services had to rethink and reorganize their activities to meet the needs of patients during the "lockdown". The Italian Association of Myology developed a survey to estimate the impact of these changes on patients affected by neuromuscular disorders and on specialized neuromuscular centers during the acute phase of COVID-19 pandemic. METHODS: We developed an electronic survey that was sent to neuromuscular centers affiliated with the Italian Association of Myology, assessing changes in pharmacological therapies provision, outpatient clinical and instrumental services, support services (physiotherapy, nursing care, psychological support) and clinical trials. RESULTS: 40% of surveyed neuromuscular centers reported a reduction in outpatient visit and examinations (44.5% of centers in Northern regions; 25% of centers in Central regions; 50% of centers in Southern regions). Twenty-two% of centers postponed in-hospital administration of therapies for neuromuscular diseases (23.4% in Northern regions; 13.0% in Central regions; 20% in Southern regions). Diagnostic and support services (physiotherapy, nursing care, psychological support) were suspended in 57% of centers (66/43/44% in Northern, Central and Southern centers respectively) Overall, the most affected services were rehabilitative services and on-site outpatient visits, which were suspended in 93% of centers. Strategies adopted by neuromuscular centers to overcome these changes included maintaining urgent on-site visits, addressing patients to available services and promoting remote contact and telemedicine. CONCLUSIONS: Overall, COVID-19 pandemic resulted in a significant disruption of clinical and support services for patients with neuromuscular diseases. Despite the efforts to provide telemedicine consults to patients, this option could be promoted and improved further. A close collaboration between the different neuromuscular centers and service providers as well as further implementation of telehealth platforms are necessary to ensure quality care to NMD patients in the near future and in case of recurrent pandemic waves.

Management of patients with neuromuscular disorders at the time of the SARS-CoV-2 pandemic.

The novel Coronavirus disease-19 (COVID-19) pandemic has posed several challenges for neuromuscular disorder (NMD) patients. The risk of a severe course of SARS-CoV-2 infection is increased in all but the mildest forms of NMDs. High-risk conditions include reduced airway clearance due to oropharyngeal weakness and risk of worsening with fever, fasting or infection Isolation requirements may have an impact on treatment regimens administered in hospital settings, such as nusinersen, glucosidase alfa, intravenous immunoglobulin, and rituximab infusions. In addition, specific drugs for SARS-CoV2 infection under investigation impair neuromuscular function significantly; chloroquine and azithromycin are not recommended in myasthenia gravis without available ventilatory support and prolonged prone positioning may influence options for treatment. Other therapeutics may affect specific NMDs (metabolic, mitochondrial, myotonic diseases) and experimental approaches for Coronavirus disease 2019 may be offered "compassionately" only after consulting the patient's NMD specialist. In parallel, the reorganization of hospital and outpatient services may change the management of non-infected NMD patients and their caregivers, favouring at-distance approaches. However, the literature on the validation of telehealth in this subgroup of patients is scant. Thus, as the first wave of the pandemic is progressing, clinicians and researchers should address these crucial open issues to ensure adequate caring for NMD patients. This manuscript summarizes available evidence so far and provides guidance for both general neurologists and NMD specialists dealing with NMD patients in the time of COVID-19.

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.

Guidance for the care of neuromuscular patients during the COVID-19 pandemic outbreak from the French Rare Health Care for Neuromuscular Diseases Network.

In France, the epidemic phase of COVID-19 caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) began in February 2020 and resulted in the implementation of emergency measures and a degradation in the organization of neuromuscular reference centers. In this special context, the French Rare Health Care for Neuromuscular Diseases Network (FILNEMUS) has established guidance in an attempt to homogenize the management of neuromuscular (NM) patients within the French territory. Hospitalization should be reserved for emergencies, the conduct of treatments that cannot be postponed, check-ups for which the diagnostic delay may result in a loss of survival chance, and cardiorespiratory assessments for which the delay could be detrimental to the patient. A national strategy was adopted during a period of 1 to 2months concerning treatments usually administered in hospitalization. NM patients treated with steroid/immunosuppressants for a dysimmune pathology should continue all of their treatments in the absence of any manifestations suggestive of COVID-19. A frequently asked questions (FAQ) sheet has been compiled and updated on the FILNEMUS website. Various support systems for self-rehabilitation and guided exercises have been also provided on the website. In the context of NM diseases, particular attention must be paid to two experimental COVID-19 treatments, hydroxycholoroquine and azithromycin: risk of exacerbation of myasthenia gravis and QT prolongation in patients with pre-existing cardiac involvement. The unfavorable emergency context related to COVID-19 may specially affect the potential for intensive care admission (ICU) for people with NMD. In order to preserve the fairest medical decision, a multidisciplinary working group has listed the neuromuscular diseases with a good prognosis, usually eligible for resuscitation admission in ICU and, for other NM conditions, the positive criteria suggesting a good prognosis. Adaptation of the use of noninvasive ventilation (NIV) make it possible to limit nebulization and continue using NIV in ventilator-dependent patients.

COVID-19 and neuromuscular disorders.

The coronavirus 2019 (COVID-19) pandemic has potential to disproportionately and severely affect patients with neuromuscular disorders. In a short period of time, it has already caused reorganization of neuromuscular clinical care delivery and education, which will likely have lasting effects on the field. This article reviews (1) potential neuromuscular complications of COVID-19, (2) assessment and mitigation of COVID-19-related risk for patients with preexisting neuromuscular disease, (3) guidance for management of immunosuppressive and immunomodulatory therapies, (4) practical guidance regarding neuromuscular care delivery, telemedicine, and education, and (5) effect on neuromuscular research. We outline key unanswered clinical questions and highlight the need for team-based and interspecialty collaboration. Primary goals of clinical research during this time are to develop evidence-based best practices and to minimize morbidity and mortality related to COVID-19 for patients with neuromuscular disorders.