Chronic glucocorticoid management in neuromuscular disease: A survey of neuromuscular neurologists.

INTRODUCTION/AIMS: Glucocorticoids (GC) are first-line therapy for many neuromuscular diseases. There is a lack of guidelines regarding the prevention and management of GC complications in the context of neuromuscular disease, introducing the potential for practice variation, that may compromise quality of care. Our aim was to evaluate the practice patterns among Canadian adult neuromuscular neurologists on the screening, management, and treatment of GC-related complications and to identify variances in practice. METHODS: A web-based anonymous questionnaire was disseminated to 99 Canadian adult neuromuscular neurologists. Questions addressed patterns of screening, prevention, monitoring, and treatment of GC-induced adverse events, including infection prophylaxis, vaccination, bone health, hyperglycemia, and other complications. RESULTS: Seventy-one percent completed the survey. Of those, 52% perform screening blood work prior to initiating GC, 56% screen for infections, and 18% for osteoporosis. The majority monitor glycemic control and blood pressure (>85%). Thirty-two (46%) reported that they do not primarily monitor GC complications, but rather provide recommendations to the primary care physician. Pneumocystis jiroveci pneumonia prophylaxis was never used by 29%, and 29% recommend vaccinations prior to GC initiation. Calcium supplementation was recommended by 80% to prevent osteoporosis. Only 36% were aware of any existing guidelines for preventing GC complications, and 91% endorsed a need for neurology-specific guidelines. DISCUSSION: There is substantial variability in the management of GC adverse effects among neuromuscular neurologists, often not corresponding to limited published literature. Our results support the need for improved education and neurology-specific guidelines to help standardize practice and improve and prevent complications.

Pain perception during electrodiagnostic studies and the impact of learners.

INTRODUCTION/AIMS: Electrodiagnostic testing (EDX) may be perceived as painful, which may influence patient expectations and test completion. Our aim was to characterize which component of the EDX was more painful, and to determine if any factors, particularly performance by a learner, influenced this perception. METHODS: Participants were prospectively recruited and completed a brief questionnaire to rate their perception of pain before and after each component of the EDX. Demographic information and information about the test itself was collected. RESULTS: A total of 251 participants were recruited, 55.3% female, with a mean age of 52.9 y. Most participants rated pain after nerve conduction studies (NCS) and needle electromyography (EMG) as mild to moderate with a similar number rating each component as more painful than the other. There was no effect of sex on overall ratings, although females felt that the test was more painful than anticipated more often than males. If a learner performed the EMG, it was more likely that the test would be rated as moderately to severely painful, and more likely for the EMG to be rated as more painful than the NCS (p < .05). DISCUSSION: The finding that NCS and EMG perceived pain were similar may help accurately inform patients of test expectations, guide test planning and help reduce the likelihood of incomplete or canceled testing. More effort might be required to help mitigate EMG pain when learners are involved.

Identifying gaps in the management of atrial fibrillation with anticoagulation for stroke prevention (GIANTS): a quality improvement initiative.

OBJECTIVES: Direct oral anticoagulants (DOACs) are indicated for preventing ischemic stroke in 95% of patients with atrial fibrillation (AF). However, DOACs are largely underutilized or inappropriately dosed. This study aimed to explore the rates of appropriate DOAC use between repatriated and non-repatriated patients with AF who presented with an acute ischemic stroke not deemed candidates for revascularization therapies. MATERIALS AND METHODS: Data were collected via electronic medical records and patients were contacted via telephone to obtain medication information and other missing clinical information. Multivariate logistic regression analyses were conducted to determine associations between appropriate use of a DOAC and repatriation status adjusting for age, sex, and comorbidities. RESULTS: A total of 49 patients were included in the study with a mean follow-up of 31.5 (± 11.9) months. Eleven patients (22.4%) died, and 4 (8.2%) patients were lost to follow-up. Overall, 9 (26.5%) patients (3 non-repatriated and 6 repatriated) were found to be on suboptimal or inappropriate anticoagulation. Repatriation was associated with over six fold (OR 6.53; 95% CI 1.20-35.7) likelihood of suboptimal or inappropriate anticoagulation. CONCLUSIONS: Awareness of this finding is critical to overcome therapeutic inertia in the use of DOACs for ischemic stroke patients with AF. With stroke care becoming increasingly nuanced, peripheral centers may lack the ability to guide anticoagulation in a timely and effective manner. Creation of tertiary short-term follow-up stroke clinics with cardiology follow-up may be an effective strategy to ensure standard of care. Research into systems contributors affecting the appropriate use of DOACs should be elucidated.

Patient preference for virtual versus in-person visits in neuromuscular clinical practice.

INTRODUCTION/AIMS: It is unknown if patients with neuromuscular diseases prefer in-person or virtual telemedicine visits. We studied patient opinions and preference on virtual versus in-person visits, and the factors influencing such preferences. METHODS: Telephone surveys, consisting of 11 questions, of patients from 10 neuromuscular centers were completed. RESULTS: Five hundred and twenty surveys were completed. Twenty-six percent of respondents preferred virtual visits, while 50% preferred in-person visits. Sixty-four percent reported physical interaction as "very important." For receiving a new diagnosis, 55% preferred in-person vs 35% reporting no preference. Forty percent were concerned about a lack of physical examination vs 20% who were concerned about evaluating vital signs. Eighty four percent reported virtual visits were sufficiently private. Sixty eight percent did not consider expenses a factor in their preference. Although 92% were comfortable with virtual communication technology, 55% preferred video communications, and 19% preferred phone calls. Visit preference was not significantly associated with gender, diagnosis, disease severity, or symptom management. Patients who were concerned about a lack of physical exam or assessment of vitals had significantly higher odds of selecting in-person visits than no preference. DISCUSSION: Although neither technology, privacy, nor finance burdened patients in our study, more patients preferred in-person visits than virtual visits and 40% were concerned about a lack of physical examination. Interactions that occur with in-person encounters had high importance for patients, reflecting differences in the perception of the patient-physician relationship between virtual and in-person visits.

Virtual Specialist Care During the COVID-19 Pandemic: Multimethod Patient Experience Study.

BACKGROUND: Transitioning nonemergency, ambulatory medical care to virtual visits in light of the COVID-19 global pandemic has been a massive shift in philosophy and practice that naturally came with a steep learning curve for patients, physicians, and clinic administrators. OBJECTIVE: We undertook a multimethod study to understand the key factors associated with successful and less successful experiences of virtual specialist care, particularly as they relate to the patient experience of care. METHODS: This study was designed as a multimethod patient experience study using survey methods, descriptive qualitative interview methodology, and administrative virtual care data collected by the hospital decision support team. Six specialty departments participated in the study (endoscopy, orthopedics, neurology, hematology, rheumatology, and gastroenterology). All patients who could speak and read English and attended a virtual specialist appointment in a participating clinic at St. Michael's Hospital (Toronto, Ontario, Canada) between October 1, 2020, and January 30, 2021, were eligible to participate. RESULTS: During the study period, 51,702 virtual specialist visits were conducted in the departments that participated in the study. Of those, 96% were conducted by telephone and 4% by video. In both the survey and interview data, there was an overall consensus that virtual care is a satisfying alternative to in-person care, with benefits such as reduced travel, cost, time, and SARS-CoV-2 exposure, and increased convenience. Our analysis further revealed that the specific reason for the visit and the nature and status of the medical condition are important considerations in terms of guidance on where virtual care is most effective. Technology issues were not reported as a major challenge in our data, given that the majority of "virtual" visits reported by our participants were conducted by telephone, which is an important distinction. Despite the positive value of virtual care discussed by the majority of interview participants, 50% of the survey respondents still indicated they would prefer to see their physician in person. CONCLUSIONS: Patient experience data collected in this study indicate a high level of satisfaction with virtual specialty care, but also signal that there are nuances to be considered to ensure it is an appropriate and sustainable part of the standard of care.

Mitochondrial Neurogastrointestinal Encephalopathy Disease: A Rare Disease Diagnosed in Siblings with Double Vision.

Mitochondrial neurogastrointestinal encephalopathy disease (MNGIE) is a rare autosomal recessive condition characterized by gastrointestinal dysmotility, external ophthalmoplegia, leukoencephalopathy, and sensorimotor neuropathy. A 31-year-old man was referred for a 1-year history of horizontal diplopia related to a large exotropia from chronic progressive external ophthalmoplegia. MRI revealed a diffuse leukoencephalopathy and his 3-year history of chronic intermittent diarrhea, cachexia, and diffuse sensory more than motor peripheral neuropathy led to a unifying clinical diagnosis of MNGIE. This was later confirmed with genetic testing, which revealed a homozygous pathogenic mutation in the thymidine phosphorylase (TYMP) gene. His younger brother had an identical clinical syndrome and was similarly diagnosed. MNGIE diagnosis is important to establish to avoid unnecessary invasive testing for gastrointestinal, ophthalmological, and neurological symptoms and to ensure patients receive appropriate nutritional and genetic counselling. Gene therapy offers a potential future therapy for patients with this condition.

Modification to Neurology Residency Training: The Toronto Neurology COVID-19 Pandemic Experience.

We describe the University of Toronto Adult Neurology Residency Program's early experiences with and response to the coronavirus disease 2019 pandemic, including modifications to the provision of neurologic care while upholding neurology education and safety. All academic and many patient-related activities were virtualized. This maintained physical distancing while creating a city-wide videoconference-based teaching curriculum, expanding the learning opportunities to trainees at all academic sites. Furthermore, we propose a novel split-team model to promote resident safety through physical distancing of teams and to establish a capacity to rapidly adapt to redeployment, service needs, and trainee illness. Finally, we developed a unique protected code stroke framework to safeguard staff and trainees during hyperacute stroke assessments in this pandemic. Our shared experiences highlight considerations for contingency planning, maintenance of education, sustainability of team members, and promotion of safe neurologic care. These interventions serve to promote trainee safety, wellness, and resiliency.

Fracture Risk in Patients with Myasthenia Gravis: A Population-Based Cohort Study.

BACKGROUND: Prednisone is a common treatment for myasthenia gravis (MG), and osteoporosis is a known potential risk of chronic prednisone therapy. OBJECTIVE: Our aim was to evaluate the risk of serious fractures in a population-based cohort of MG patients. METHODS: An inception cohort of patients with MG was identified from administrative health data in Ontario, Canada between April 1, 2002 and December 31, 2015. For each MG patient, we matched 4 general population comparators based on age, sex, and region of residence. Fractures were identified through emergency department and hospitalization data. Crude overall rates and sex-specific rates of fractures were calculated for the MG and comparator groups, as well as rates of specific fractures. Adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using Cox regression. RESULTS: Among 3,823 incident MG patients (followed for a mean of 5 years), 188 (4.9%) experienced a fracture compared with 741 (4.8%) fractures amongst 15,292 matched comparators. Crude fracture rates were not different between the MG cohort and matched comparators (8.71 vs. 7.98 per 1000 patient years), overall and in men and women separately. After controlling for multiple covariates, MG patients had a significantly lower risk of fracture than comparators (HR 0.74, 95% CI 0.63-0.88). CONCLUSIONS: In this large, population-based cohort of incident MG patients, MG patients were at lower risk of a major fracture than comparators. The reasons for this finding are unclear but may highlight the importance osteoporosis prevention.

Diagnostic modelling and therapeutic monitoring of immune-mediated necrotizing myopathy: role of electrical myotonia.

Delayed diagnosis of immune-mediated necrotizing myopathy leads to increased morbidity. Patients with the chronic course without 3-hydroxy-3-methylglutaryl-coenzyme-A reductase-IgG or signal recognition particle-IgG are often challenging to diagnose. Immunotherapy response can also be difficult to assess. We created a statistical model to assist immune-mediated necrotizing myopathy diagnosis. Electrical myotonia versus fibrillations were reviewed as biomarkers for immunotherapy treatment response. Identified were 119 immune-mediated necrotizing myopathy cases and 938 other myopathy patients. Inclusion criteria included all having electrophysiological evaluations, muscle biopsies showing inflammatory/necrotizing myopathies, comprehensively recorded neurological examinations, and creatine kinase values. Electrical myotonia was recorded in 56% (67/119) of retrospective and 67% (20/30) of our validation immune-mediated necrotizing myopathy cohorts, and significantly (P < 0.001) favoured immune-mediated necrotizing myopathy over other myopathies: sporadic inclusion body myositis (odds ratio = 4.78); dermatomyositis (odds ratio = 10.61); non-specific inflammatory myopathies (odds ratio = 8.46); limb-girdle muscular dystrophies (odds ratio = 5.34) or mitochondrial myopathies (odds ratio = 14.17). Electrical myotonia occurred in immune-mediated necrotizing myopathy seropositive (3-hydroxy-3-methylglutaryl-coenzyme-A reductase-IgG 70%, 37/53; signal recognition particle-IgG 29%, 5/17) and seronegative (51%, 25/49). Multivariate regression analysis of 20 variables identified 8 (including electrical myotonia) in combination accurately predicted immune-mediated necrotizing myopathy (97.1% area-under-curve). The model was validated in a separate cohort of 30 immune-mediated necrotizing myopathy cases. Delayed diagnosis of cases with electrical myotonia occurred in 24% (16/67, mean 8 months; range 0-194). Half (8/19) had a chronic course and were seronegative, with high model prediction (>86%) at the first visit. Inherited myopathies were commonly first suspected in them. Follow-up evaluation in patients with electrical myotonia on immunotherapy was available in 19 (median 21 months, range 2-124) which reduced from 36% (58/162) of muscles to 7% (8/121; P < 0.001). Reduced myotonia correlated with immunotherapy response in 64% (9/14) as well as with median creatine kinase reduction of 1779 U/l (range 401-9238, P < 0.001). Modelling clinical features with electrical myotonia is especially helpful in immune-mediated necrotizing myopathy diagnostic suspicion among chronic indolent and seronegative cases. Electrical myotonia favours immune-mediated necrotizing myopathy diagnosis and can serve as an adjuvant immunotherapy biomarker.

Cardiac and Respiratory Complications of Necrotizing Autoimmune Myopathy.

OBJECTIVE: To characterize the cardiorespiratory abnormalities in patients with necrotizing autoimmune myopathy (NAM). PATIENTS AND METHODS: Cardiopulmonary features of patients with NAM evaluated in our neuromuscular clinic (January 1, 2004, to September 20, 2018) were reviewed retrospectively with respect to autoantibody status and history of cardiac disease. Clinical characteristics and laboratory findings were compared among patient subgroups. RESULTS: We identified 109 patients with NAM: 36 anti-3-hydroxy-3-methylglutaryl coenzyme A reductase autoantibody (anti-HMGCR Ab)-positive, 18 anti-signal recognition particle antibody (anti-SRP Ab)-positive (3 dual anti-HMGCR/anti-SRP Ab-positive), and 58 seronegative. Median age at diagnosis was 60 years (range, 18-86 years). Forty-three patients had dyspnea at presentation and 32 patients had preexisting risk for cardiac disease (10 coronary artery disease and 28 hypertension). The electrocardiogram was abnormal in 55 of 86 patients (33 without cardiac risk factors), including prolonged corrected QT interval (QTc) (n=31), conduction blocks (n=19), and atrial or ventricular ectopic beats (n=10). Echocardiography was abnormal in 34 of 72 patients, including 19 of 45 without preexisting cardiac disease risks. Echocardiographic abnormalities included left ventricular diastolic dysfunction (n=31) and systolic dysfunction (n=8). The left ventricular diastolic dysfunction improved in 4 of 11 patients after treatment. Pulmonary function testing showed changes suggestive of neuromuscular respiratory muscle weakness in 51 of 66 patients and reduced carbon monoxide diffusing capacity in 11 of 35 patients. However, only 6 patients had radiographic evidence of interstitial lung disease (2 anti-HMGCR Ab-positive and 4 seronegative). Overnight oximetry revealed desaturations in 24 of 38 patients. Six patients required mechanical ventilation and 7 required noninvasive ventilatory support. CONCLUSION: Most patients with NAM exhibited cardiac and respiratory muscle dysfunction. Immunotherapy can improve echocardiographic abnormalities. Interstitial lung disease was rarely identified. Formal evaluation of cardiac and respiratory status should be integral in assessment of patients with NAM.

Delayed Thrombectomy Center Arrival is Associated with Decreased Treatment Probability.

BACKGROUND: Endovascular thrombectomy (EVT) is effective in reducing disability in selected patients with stroke and large vessel occlusion (LVO), but access to this treatment is suboptimal. AIM: We examined the proportion of patients with LVO who did not receive EVT, the reasons for non-treatment, and the association between time from onset and probability of treatment. METHODS: We conducted a retrospective cohort study of consecutive patients with acute stroke and LVO presenting between January 2017 and June 2018. We used multivariable log-binomial models to determine the association between time and probability of treatment with and without adjustment for age, sex, dementia, active cancer, baseline disability, stroke severity, and evidence of ischemia on computerized tomography. RESULTS: We identified 256 patients (51% female, median age 74 [interquartile range, IQR 63.5, 82.5]), of whom 59% did not receive EVT. The main reasons for not treating with EVT were related to occlusion characteristics or infarct size. The median time from onset to EVT center arrival was longer among non-treated patients (218 minutes [142, 302]) than those who were treated (180 minutes [104, 265], p = 0.03). Among patients presenting within 6 hours of onset, the relative risk (RR) of receiving EVT decreased by 3% with every 10-minute delay in arrival to EVT center (adjusted RR 0.97 CI95 [0.95, 0.99]). This association was not found in the overall cohort. CONCLUSIONS: The proportion of patients with acute stroke and confirmed LVO who do not undergo EVT is substantial. Minimizing delays in arrival to EVT center may optimize the delivery of this treatment.

Guidance for resumption of routine electrodiagnostic testing during the COVID-19 pandemic.

As the world accommodates to the coronavirus disease 2019 (COVID-19) pandemic, routine in-person medical services are resuming. The resumption of non urgent electrodiagnostic (EDX) testing faces unique challenges due to the long duration of the procedure and direct close contact with patients, including studies with risk of exposure to oropharyngeal secretions. We provide consensus guidance for resumption of EDX testing, addressing scheduling, patient arrival and registration, use of personal protective equipment, COVID-19 screening and testing, the performance of EDX testing in outpatient and inpatient settings, cleaning and maintenance of the EDX equipment and laboratory, balancing trainee safety and training requirements, and patient care issues. These are broad recommendations that need to be adapted to local COVID-19 risks, institutional guidelines and policies, and changing federal, state, and local regulations, and to changes in the pandemic over time.

The Virtual Neurologic Exam: Instructional Videos and Guidance for the COVID-19 Era.

OBJECTIVE: To outline features of the neurologic examination that can be performed virtually through telemedicine platforms (the virtual neurological examination [VNE]), and provide guidance for rapidly pivoting in-person clinical assessments to virtual visits during the COVID-19 pandemic and beyond. METHODS: The full neurologic examination is described with attention to components that can be performed virtually. RESULTS: A screening VNE is outlined that can be performed on a wide variety of patients, along with detailed descriptions of virtual examination maneuvers for specific scenarios (cognitive testing, neuromuscular and movement disorder examinations). CONCLUSIONS: During the COVID-19 pandemic, rapid adoption of virtual medicine will be critical to provide ongoing and timely neurological care. Familiarity and mastery of a VNE will be critical for neurologists, and this article outlines a practical approach to implementation.

Practical guidance for managing electromyography requests and testing during the COVID-19 pandemic.

The COVID-19 pandemic has necessitated cancelation of elective or nonurgent contact with the healthcare system, including nonurgent nerve conduction studies and electromyography (electrodiagnostic [EDX] studies). The definitions of elective and nonurgent are physician judgments, and often are not straightforward. Clinical care must be provided to help our patients in a timely manner, while keeping them, healthcare personnel, and the community safe. Benefit/risk stratification is an important part of this process. We have stratified EDX studies into three categories: Urgent, Non-urgent, and Possibly Urgent, in an effort to help clinicians triage these referrals. For each category, we provide a rationale and some examples. However, each referral must be reviewed on a case-by-case basis, and the clinical situation will evolve over time, necessitating flexibility in managing EDX triaging. Engaging the referring clinician and, at times, the patient, may be useful in the triage process.

Validity evidence for Quality Improvement Knowledge Application Tool Revised (QIKAT-R) scores: consequences of rater number and type using neurology cases.

OBJECTIVES: To develop neurology scenarios for use with the Quality Improvement Knowledge Application Tool Revised (QIKAT-R), gather and evaluate validity evidence, and project the impact of scenario number, rater number and rater type on score reliability. METHODS: Six neurological case scenarios were developed. Residents were randomly assigned three scenarios before and after a quality improvement (QI) course in 2015 and 2016. For each scenario, residents crafted an aim statement, selected a measure and proposed a change to address a quality gap. Responses were scored by six faculty raters (two with and four without QI expertise) using the QIKAT-R. Validity evidence from content, response process, internal structure, relations to other variables and consequences was collected. A generalisability (G) study examined sources of score variability, and decision analyses estimated projected reliability for different numbers of raters and scenarios and raters with and without QI expertise. RESULTS: Raters scored 163 responses from 28 residents. The mean QIKAT-R score was 5.69 (SD 1.06). G-coefficient and Phi-coefficient were 0.65 and 0.60, respectively. Interrater reliability was fair for raters without QI expertise (intraclass correlation = 0.53, 95% CI 0.30 to 0.72) and acceptable for raters with QI expertise (intraclass correlation = 0.66, 95% CI 0.02 to 0.88). Postcourse scores were significantly higher than precourse scores (6.05, SD 1.48 vs 5.22, SD 1.5; p < 0.001). Sufficient reliability for formative assessment (G-coefficient > 0.60) could be achieved by three raters scoring six scenarios or two raters scoring eight scenarios, regardless of rater QI expertise. CONCLUSIONS: Validity evidence was sufficient to support the use of the QIKAT-R with multiple scenarios and raters to assess resident QI knowledge application for formative or low-stakes summative purposes. The results provide practical information for educators to guide implementation decisions.