Neuromuscular evaluation





The history of telemedicine


Telemedicine is the remote diagnosis and treatment of patients by means of telecommunications technology. However, this simple definition does not reflect the history of its development, which has been integrated with technology, and innovation. Like many technological advances that are now common to our daily lives and professions, telemedicine was birthed alongside the birth and growth of the internet in the 1960s. One of its earliest uses in medicine was the use of the satellite ‘Early Bird’ which allowed Dr Michael Debakey to conduct an aortic valve replacement while engaging with surgeons in the Director-General of the WHO and other surgeons in Geneva Switzerland. Fast forward to 2020, and the marriage of the internet and technology has created a diverse and broad telecommunication platform accessible not only to governments, world organizations, and institutions, but also to civilians, from the clinician and systems-based providers to the individual health care consumer.


Telemedicine in neurology


However, it was the use of telemedicine in stroke care that actually paved the way for the application of telemedicine in neurology practice settings. Telestroke services have been an essential part of acute stroke care for more than a decade, integrated with improved access, quality of care, and treatment rates with evidence of equitable outcomes. Until recently, with the advent of the COVID-19 pandemic, the advances witnessed with telestroke services have been limited in use for other neurological conditions.


Prior to the COVID-19 pandemic and its impact on society, and more specifically the delivery of quality health care, discussion of telemedicine use in neurology was limited to a minority of ambulatory practices with great variability in how it was used among clinicians and their practices. Most publications discussed the potential of telemedicine across different subspecialties and approaches to delivery, from multidisciplinary care clinics to rural isolated practices. Most publications reported noninferiority of telemedicine evaluations to in-person evaluations in regards to disease outcomes as well as patient and clinician satisfaction related to increased access, decreased travel, and costs per visit. Yet there has been minimal evidence showing utility across various settings and cultures, and even less evidence regarding its impact upon clinical outcomes in large cohorts, including that of neuromuscular medicine.


Telemedicine in neuromuscular disease


Neuromuscular conditions include a wide range of disorders with a broad range of etiopathogeneses, diagnostics approaches, and treatments. Some are rare, listed among the rare orphan diseases, such as amyotrophic lateral sclerosis. Others are more common, such as diabetic polyneuropathy. Thus, the approach to the diagnosis and management of these conditions also varies in terms of the types of care settings where neuromuscular patients receive care.


Telemedicine in ALS


Amyotrophic lateral sclerosis is a diagnosis of exclusion requiring a detailed examination and thorough testing. In addition, the disease requires a comprehensive multidisciplinary approach, typically at a tertiary center or practice with such an affiliation. In contrast, diabetic polyneuropathy and headache patients are managed across a variety of clinical care settings (urban, rural, academic, private), by generalists and specialists alike. Similar to other subspecialties, there is a paucity of randomized, blinded studies of telemedicine in neuromuscular medicine, with small, less robust studies focusing on patient and caregiver perception and satisfaction.


Neuromuscular disorders like ALS require subspecialized care, in-depth history and examination, interdisciplinary care, and extensive assessments and testing. These disorders also present special challenges in accessing health care due to impairments in mobility and function. This is further complicated in that the majority care is provided at tertiary medical centers with up to 25% of patients (e.g., ALS) living more than 100 miles away from such centers. Nonetheless, although there has been limited experience utilizing telemedicine for neuromuscular disorders across practice settings, there are reported benefits to its use in this patient population.


Amyotrophic lateral sclerosis is a neurodegenerative disorder of predominant motor neurons resulting in progressive motor disability of limbs and bulbar and respiratory functions. ALS patients benefit from the care of multidisciplinary care teams in terms of quality of life and survival, making the disease amenable to telemedicine in order to address the needs of the patient who is unable to access health care consistently in person (distance and disability). The application of telemedicine in ALS attempts to bridge the gap in care for such patients, who are often lost to follow-up, or transition their care to single provider clinics or home health agencies that provide physician or physician equivalent care.


One of the two applications of telehealth is by direct clinical video teleconferencing with the ALS care team. Van De Rijn et al. conducted a retrospective review of 136 videos for nearly 100 ALS patients living 10–3136 miles from the care center. The team provided televisits to both ambulating patients (> 50%) as well as more advanced patients on invasive ventilation (23%) and those receiving home hospice care (11%). Although limited by the retrospective design, notable benefits included convenience for patients at all stages of disease, extension of care to more advanced patients, and opportunities to engage other care providers in the encounter who otherwise might not have attended an in-person visit. Limitations identified include variability in the adoption of technology among patients and even providers, as well inconsistency of reimbursement at the time of the study.


In terms of outcomes, most reported no difference in disease progression for patients evaluated at regular intervals via televisits compared to patients seen in-person. Most reported high patient acceptance and satisfaction of telehealth services, only preferring in-person visits for psychosocial issues that may require in depth disclosure.


Another application of telehealth for ALS patients is the monitoring of ventilator compliance to manage parameters and use by accessing stored data from a data chip uploaded to a central site.


These interventions are associated with decreased emergency room visits and admissions. Similarly, a review of these studies found an overall benefit associated with decreased time and cost of travel by patients and increased patient satisfaction, as well as better management of palliative care.


Despite the benefits of telemedicine in the care of ALS patients, obvious challenges and questions remain as to its use and implementation in providing multidisciplinary care for this population. Logistical issues exist regarding assembling a team of providers for the encounter, as well as for the patient, who may not have resources or ability to access the technology. Furthermore, the vulnerability of the platform to technical difficulties, both external (e.g., weather) or internal (system support disruptions) remain as variables that are difficult to control.


Care centers have taken a variety of approaches to address this issue. Some have provided telecare that is primarily led by the physician or physician extender, who triages the patient needs or issues to team members who evaluate the patient at a separate time, either virtually, telephone or video, or in person if indicated. Prior to the COVID-19 pandemic of 2020, others used telehealth in combination with a home visit program in which the physician/physician extender remained remote and teleconferenced with team members who assessed the patient at their home. This latter approach addresses the limitation of performing a virtual examination as well as ensuring the accuracy and standardization of assessment and measures of disease for clinical care and research.


In 2019, Pulley et al. reported on the store and forward method for multidisciplinary ALS care, in which a patient is assessed by a trained provider, such as a nurse, at the patient’s home. The assessment is recorded and reviewed by individual team providers who send their recommendations to the clinic director within a week of the home visit. These recommendations are communicated to the patient by the clinical director via videoconferencing, or telephone communication, with the nurse present during a second home visit. The authors reported uniform patient satisfaction, although there was some variable response by providers regarding time to report recommendations and satisfaction with depending upon a proxy evaluator. This approach allowed patients to remain at home, including those who would otherwise be lost to follow-up due to advanced disease. However, in addition to limited to no reimbursement for team members, the authors noted challenges with managing the storage of large data files and quality of data capturing, as well as variable provider satisfaction with technology.


Another approach is to incorporate telemedicine encounters with in-person multidisciplinary clinics, thus allowing the homebound and those living significant distances away, the opportunity to be evaluated by the team of providers. This approach has been shown effective both before the restrictions of the pandemic and in response to the pandemic, with many providers across specialties providing care via a hybrid of in-person and telemedicine encounters within a multidisciplinary clinic.


It is also important to acknowledge that the use of telemedicine in the multidisciplinary care of neuromuscular patients is not restricted to a model that is solely focused on the neurologist as the primary provider. Telemedicine can also provide patients access to subspecialized therapy services including pulmonary and respiratory services, neurorehabilitation, assistive technology, and durable medical equipment evaluation, as well as neuropsychology, caregiver support, and palliative care.


In reference to home monitoring of non-invasive positive pressure ventilation and invasive ventilation, telemedicine has provided an avenue for providers to acquire measures of respiratory function and oxygenation that are incorporated into pulmonary rehabilitation, which was first documented in lung transplant patients in the 1990s.


Neurorehabilitation and home safety evaluations via telemedicine provide continuity of care for neuromuscular patients who would otherwise require serial visits. This was first pioneered by the Veterans Health Administration and has been associated with improvements in mobility, cognition, and quality of life, with superior results on functional outcomes compared to standard care. These benefits have also been seen with customized seating for wheelchair via telehealth wheelchair seating clinics.


Cognitive screening and complete neuropsychological assessments have been performed and are associated with improvements in coping skills, mood, and quality of life, especially when combined with behavioral therapy and patient support and networking. Similar benefits have been reported with programs that address caregiver wellness, although these studies were not specific to caregivers of neuromuscular patients.


Telemedicine in ALS and neuromuscular disorders clearly addresses issues related to patient access, cost, and burden associated with in-person care. It also provides opportunities for remote monitoring and management of related functions of respiration, rehabilitation, cognition, and well-being. Patient satisfaction is consistently rated as high among patients and providers, with some relative variability among the latter. Limitations include the lack of an examination, variability in technology access and support, as well as the ever-moving target of reimbursement, and state-specific limitations of practice.


Despite an increasing number of studies of the use of telemedicine in neurology and neuromuscular disorders, the breadth of studies in neuromuscular medicine lack sufficient methodology to evaluate robustly its impact on disease outcomes including mortality, function, quality of care, cost, and the impact on emergent evaluation of the acute patient.


Further research is indicated but is challenged by issues related to blinding of patients and providers to the intervention, inherent selection bias for patients who can access and navigate the platform, as well as the lack of standardization of care and outcome measures across diseases and practice settings. Furthermore, such studies need expansion to other chronic neuromuscular disorders, which would likely benefit from improved access, subspecialized care, and improved outcomes. Just as important is the need to examine the role of telehealth for those disorders with a more subacute or fluctuating clinical course, such as myasthenia gravis, and its impact on outcomes of costs, functional outcomes, quality of life, and satisfaction.


Telemedicine in neurology in the COVID-19 era


As the COVID-19 pandemic impacted the globe and the Unites States, the focus of health care centered on managing acute cases of COVID-19 in the setting of strained resources and health care providers, with an upswing in investigations of established and experimental therapies. With restrictions of social distancing and imposed isolation, a significant gap was created in providing care for ambulatory patients, and the provision of elective surgical and nonsurgical assessments and interventions. This gap required a rapid increase in telehealth applications across the broad spectrum of practices and settings due to the COVID-19 emergency. As a direct result, telemedicine visits increased by 50-98% across institutions and specialties.


Rapid transition to telehealth required expansion of coverage by federally supported and private payers, a temporary loosening of restrictions under the Health Insurance Portability and Accountability Act, expansion of physician licensing across state lines, and a rapid expansion of telehealth capacity. These measures allowed the ramp-up needed to provide care for patients for continuity of care, to address and triage emergent issues, and to provide a sense of support during an unsettling time.


The pandemic created a steep learning curve for neurologists whose knowledge of telemedicine was limited to its application in acute stroke care and a small number of academic and private settings. In a few months, neurologist learned to navigate existing platforms and technology and translate the components of the history and examination from a face-to-face encounter to a virtual encounter.


The speed and efficiency by which practitioners were able to make this transition was dependent upon the specialty and practice setting, as well as the presence of an existing telemedicine platform, and the bandwidth of the technology and that of its users.


The field of neurology includes an increasing number of subspecialties, which cover a subset of disorders and assessments that differ in reliance upon the examination or procedural assessments and treatments. Therefore, for subspecialties in which the history is central to the encounter, the transition to telemedicine actually provided a seamless path for providing continuity of care. This has been the experience for epileptologists, for whom the remote monitoring and assessment of seizures was already established for most practices. In epilepsy, both patient and providers have experienced benefits from the ease of scheduling follow-up encounters for ongoing patient issues without the burden of travel from home. Telemedicine for these patients seems to work well when paired with existing remote electrophysiologic monitoring, although it may not be adequate for new patient evaluation.


Telemedicine in neuromuscular medicine in the COVID-19 era


In neuromuscular medicine, transitioning to telemedicine was associated with unexpected benefits for both practitioner and patient, as well as challenges. Some shared by all of neurology, and some unique to the specialty.


Similar to previously published reports prior to COVID-19, our patients report high satisfaction with telemedicine due to it enabling safe sheltering, and involving less burden and cost associated with travel for face-to-face encounters. Visualization allowed patients to demonstrate their home environment as well as issues related to medical devices. Furthermore, family members and caregivers were able to participate in the encounter and assist with assessments if needed. Physician satisfaction was based on the ability to provide assessment from remote locations, providing continuity of care for those patients with relatively stable disease, nonemergent medicine adjustments, and triaging patients for additional testing or in-person assessments.


However, because assessments of a patient with neuromuscular disease often rely on a thorough history and examination, the telemedicine neuromuscular encounter has required clinicians to translate the neuromuscular examination virtually. While certain aspects of the exam can be assessed, albeit superficially, through visualization, other aspects of the exam (motor power, reflexes, fundoscopic, muscle tone) can not be performed virtually.


Neurologists have had to adopt new approaches to acquiring examination information from their patients without prior training or certification, often focusing on examination components that are considered more objective, easily demonstrated or instructed to the patient, and clearly visualized (oculomotor function, muscle bulk, symmetry of movement, speech, coordination, mentation, speech/language).


During this COVID-19 era, neuromuscular specialists consider this less-than-optimal assessment acceptable in the context of a patient with relatively stable disease. However, for a newly referred neuromuscular patient or a patient with unstable or active clinical disease, the virtual neuromuscular examination cannot replace an in-depth neuromuscular in-person evaluation.


Reliance upon telehealth has required creative approaches to the televisit by both provider and patient. Similar to the in-person encounter, patients’ bandwidth to prepare for the telehealth visit also varies. For some patients, comfort and competence with virtual technology remains a significant barrier to accessing telehealth. This has ranged from an inability to connect properly—reducing a visualized encounter to a limited telephone encounter—to a lack of “production” skill, with poor or misdirected placement of camera view or audio settings. In contrast, other patients may prepare for their telehealth visit well in advance, including ensuring proper connectivity and access, providing vitals via wearable or home devices, and ensuring proper positioning and visualization for adequate assessment. In addition, it is important to consider the impact of other social determinants of health, such as education, culture, race, and age, that historically have impacted access to care, also apply to telemedicine in both similar and novel ways.


The Virtual Examination of the Neuromuscular Patient


The approach to the virtual examination by neuromuscular specialists varies according to the patient and sub-specialty. Some specialists depend on straightforward visualization and observations that require little of the patient in terms of maneuvers (facial symmetry, speech, extraocular movements, and observed upper body range of motion), whereas other specialists may provide the patient with validated patient surveys for objective assessment or written and/or visual aids that provide instruction on the examination, or ask the patient to enlist a capable family member/spouse to assist with the examination ( on Expert Consult).


The incorporation of existing validated surveys and scales has provided objective assessments of function, disease progression, and symptom burden in neuromuscular medicine. Prior to COVID-19, use of these tools for diseases like amyotrophic lateral sclerosis (ALSFRS, CNS-Lability scale), myasthenia gravis (MG -ADL, MG QoL15), and neuropathy (VAS, INCAT) was limited to patients receiving care in specialized or multidisciplinary clinics involved in translational or clinical trial research. Although the current use of these tools in telemedicine for neuromuscular conditions has not been quantified, many of these measures have become an integral part of the telemedicine neuromuscular examination based on ease of administration by patients, caregivers, or providers. As practitioners continue to navigate delivery of care during the pandemic, it is likely that such tools and possible newly developed tools will become an integral part of the neuromuscular assessment across practice settings and neuromuscular disorders, including for respiratory function.


Wilson et al. constructed a neuropathy scale from 10 existing validated assessments ( Fig. 11.1 ). The investigators measured concordance between the newly constructed Veterans Affairs Neuropathy Scale (VANS) with documentation of neuropathy status based on chart review as well as the reliability of results between in-person vs. remote grading by board-certified neurologist vs. nonneurologist evaluators (telemedicine technician, medical assistant, and nurse), respectively. The VANS incorporates assessment of neuropathy findings as well as those for balance, mobility, and skin integrity.


Oct 30, 2021 | Posted by in NEUROLOGY | Comments Off on Neuromuscular evaluation
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