RESUMO
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.
Assuntos
Glucocorticoides , Neurologistas , Doenças Neuromusculares , Humanos , Doenças Neuromusculares/tratamento farmacológico , Glucocorticoides/uso terapêutico , Glucocorticoides/efeitos adversos , Canadá , Inquéritos e Questionários , Masculino , Padrões de Prática Médica/estatística & dados numéricos , Padrões de Prática Médica/normas , Feminino , Adulto , Gerenciamento ClínicoRESUMO
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.
Assuntos
Condução Nervosa , Percepção da Dor , Masculino , Humanos , Feminino , Pessoa de Meia-Idade , Condução Nervosa/fisiologia , Percepção da Dor/fisiologia , Eletromiografia , Dor/diagnóstico , Exame NeurológicoRESUMO
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.
Assuntos
Betacoronavirus , Infecções por Coronavirus/transmissão , Transmissão de Doença Infecciosa/prevenção & controle , Eletromiografia/normas , Pandemias , Pneumonia Viral/transmissão , Guias de Prática Clínica como Assunto , Telemedicina/métodos , COVID-19 , Canadá/epidemiologia , Infecções por Coronavirus/epidemiologia , Atenção à Saúde/organização & administração , Humanos , Pacientes Ambulatoriais , Pneumonia Viral/epidemiologia , Encaminhamento e Consulta , SARS-CoV-2 , Triagem , Estados Unidos/epidemiologiaRESUMO
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.
Assuntos
Agendamento de Consultas , Infecções por Coronavirus/epidemiologia , Eletrodiagnóstico/métodos , Higiene das Mãos , Equipamento de Proteção Individual , Pneumonia Viral/epidemiologia , Assistência Ambulatorial , Betacoronavirus , COVID-19 , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/prevenção & controle , Descontaminação , Eletromiografia , Contaminação de Equipamentos , Humanos , Controle de Infecções , Máscaras , Programas de Rastreamento , Condução Nervosa , Pandemias/prevenção & controle , Pneumonia Viral/diagnóstico , Pneumonia Viral/prevenção & controle , SARS-CoV-2 , Estados Unidos/epidemiologiaRESUMO
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.
Assuntos
Isquemia Encefálica , Procedimentos Endovasculares , Acidente Vascular Cerebral , Idoso , Isquemia Encefálica/cirurgia , Feminino , Humanos , Masculino , Probabilidade , Estudos Retrospectivos , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/terapia , Trombectomia , Resultado do TratamentoRESUMO
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.
Assuntos
Betacoronavirus , Infecções por Coronavirus/terapia , Exame Neurológico/normas , Pneumonia Viral/terapia , Guias de Prática Clínica como Assunto/normas , Telemedicina/normas , Gravação em Vídeo/normas , COVID-19 , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Humanos , Exame Neurológico/métodos , Neurologistas/normas , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , SARS-CoV-2 , Telemedicina/métodosAssuntos
Eletrodiagnóstico , Dor , Humanos , Dor/diagnóstico , Ansiedade/diagnóstico , Ansiedade/etiologia , Transtornos de AnsiedadeRESUMO
BACKGROUND AND PURPOSE: Stroke is a relatively common and challenging condition in hospitalized patients. Previous studies have shown delays in recognition and assessment of inpatient strokes leading to poor outcomes. The goal of this quality improvement initiative was to evaluate an in-hospital code stroke algorithm and educational program aimed at reducing the response times for inpatient stroke. METHODS: An inpatient code stroke algorithm was developed, and an educational intervention was implemented over 5 months. Data were recorded and compared between the 36-month period before and the 15-month period after the intervention was implemented. Outcome measures included time from last seen normal to initial assessment and from last seen normal to brain imaging. RESULTS: During the study period, there were 218 inpatient strokes (131 before the intervention and 87 after the intervention). Inpatient strokes were more common on cardiovascular wards (45% of cases) and occurred mainly during the perioperative period (60% of cases). After implementation of an inpatient code stroke intervention and educational initiative, there were consistent reductions in all timed outcome measures (median time to initial assessment fell from 600 [109-1460] to 160 [35-630] minutes and time to computed tomographic scan fell from 925 [213-1965] to 348.5 [128-1587] minutes). CONCLUSIONS: Our study reveals the efficacy of an inpatient code stroke algorithm and educational intervention directed at nurses and allied health personnel to optimize the prompt management of inpatient strokes. Prompt assessment may lead to faster stroke interventions, which are associated with better outcomes.
Assuntos
Codificação Clínica/normas , Conhecimentos, Atitudes e Prática em Saúde , Hospitalização , Melhoria de Qualidade/normas , Acidente Vascular Cerebral/diagnóstico , Tempo para o Tratamento/normas , Idoso , Algoritmos , Codificação Clínica/tendências , Competência Clínica/normas , Feminino , Hospitalização/tendências , Humanos , Masculino , Pessoa de Meia-Idade , Melhoria de Qualidade/tendências , Acidente Vascular Cerebral/terapia , Tempo para o Tratamento/tendênciasRESUMO
INTRODUCTION: Pneumothorax is a potentially serious complication of electromyography (EMG). Data on the frequency of pneumothorax after EMG are lacking. The purpose of this study was to determine the frequency, timing, and risk factors for iatrogenic pneumothorax after EMG. METHODS: Cases of pneumothorax after EMG were reviewed for clinical, electrophysiological, and radiological data. RESULTS: Of 64,490 EMG studies, 7 patients had an association between the EMG and pneumothorax. All patients were symptomatic and presented within 24 hours of EMG. Sampling of serratus anterior and diaphragm was causative in 1 patient each. In 5 patients, multiple high-risk muscles were sampled. The highest frequency of pneumothorax was observed with examination of serratus anterior (0.445%) and diaphragm (0.149%). CONCLUSIONS: The frequency of symptomatic iatrogenic pneumothorax after EMG appears to be low, and examinations of serratus anterior and diaphragm carry the highest risk. Electromyographers should be aware of the risk of pneumothorax and should counsel patients accordingly.
Assuntos
Eletromiografia/efeitos adversos , Pneumotórax/diagnóstico , Pneumotórax/etiologia , Adolescente , Idoso de 80 Anos ou mais , Feminino , Humanos , Doença Iatrogênica , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiologia , Fatores de Risco , Adulto JovemRESUMO
INTRODUCTION: Adult polyglucosan body disease (APBD) usually presents with progressive spastic paraparesis, neurogenic bladder, and distal lower limb sensory abnormalities. It is caused by mutations in the glycogen branching enzyme gene (GBE1). METHODS: We describe a woman with an unusual phenotype manifesting as progressive left brachial more than lumbosacral plexopathies, with central sensory and corticospinal tract involvement. RESULTS: Magnetic resonance imaging of the brain and cervical spine showed abnormal T2 signal within the ventral pons and medulla bilaterally, involving the pyramidal tracts and the medial leminisci. There was also medullary and cervical spine atrophy. On nerve biopsy, large polyglucosan bodies were noted in the endoneurium. The patient was found to be compound heterozygous for 2 novel mutations in GBE1. Peripheral blood leukocyte GBE activity was markedly reduced to 7% of normal, confirming the diagnosis of APBD. CONCLUSIONS: In this report we describe a new phenotype of APBD associated with 2 novel mutations. Muscle Nerve 53: 976-981, 2016.
Assuntos
Progressão da Doença , Lateralidade Funcional/fisiologia , Doença de Depósito de Glicogênio/fisiopatologia , Doenças do Sistema Nervoso/fisiopatologia , Condução Nervosa/fisiologia , Análise Mutacional de DNA , Feminino , Sistema da Enzima Desramificadora do Glicogênio/genética , Doença de Depósito de Glicogênio/diagnóstico por imagem , Doença de Depósito de Glicogênio/genética , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/diagnóstico por imagem , Doenças do Sistema Nervoso/genética , RNA Mensageiro/metabolismo , Tempo de Reação/fisiologia , Nervo Sural/patologiaRESUMO
INTRODUCTION: The aim of this consensus statement is to provide a recommendation from AANEM experts on the clinical utility of genetic testing. It is not meant to recommend or endorse any specific genetic testing methodology or algorithm. METHODS: The AANEM Professional Practice Committee reached a consensus based on expert opinion on the utility of genetic testing in neuromuscular diseases and made recommendations on factors that physicians and patients should consider when deciding whether to proceed with such testing. RESULTS: Despite the costs of genetic testing, these tests can be both valuable and beneficial in the diagnosis and treatment of neuromuscular diseases in many situations. CONCLUSIONS: The AANEM believes that performing genetic testing to arrive at a specific molecular diagnosis is a critical step in providing high-quality care to neuromuscular patients. The cost of testing should not be a deterrent, as there are important clinical, safety, psychosocial, and research benefits. Muscle Nerve 54: 1007-1009, 2016.
Assuntos
Consenso , Testes Genéticos , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/genética , Humanos , Sociedades Médicas/normas , Estados UnidosAssuntos
Esclerose Múltipla , Miosite , Miosite Orbital , Diplopia , Humanos , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico por imagem , Miosite/complicações , Miosite/diagnóstico por imagem , Músculos Oculomotores/diagnóstico por imagem , Miosite Orbital/complicações , Miosite Orbital/diagnóstico por imagemRESUMO
INTRODUCTION: In acid maltase deficiency (AMD), electrical myotonia (EM) may be restricted to paraspinal muscles. A comprehensive description of the electromyographic (EMG) findings in AMD is lacking. The purpose of this study is to describe the EMG features in adult-onset AMD, focusing on the distribution of EM. METHODS: A retrospective chart review of AMD patients diagnosed at Mayo Clinic over age 18 years. RESULTS: Thirty-seven patients were included. Twenty-eight (76%) had EM in at least 1 muscle, and EM was more common in paraspinal and proximal limb muscles. The tensor fasciae latae (TFL) was equally sensitive to the paraspinals for EM. Three of 4 patients had EM identified in the diaphragm. CONCLUSIONS: Approximately three-quarters of adult-onset AMD patients display EM on EMG. The paraspinal muscles and TFL are the most likely to demonstrate EM, and EM can be detected in the diaphragm of adult onset AMD patients.
Assuntos
Eletromiografia , Doença de Depósito de Glicogênio Tipo II/complicações , Miotonia/diagnóstico , Miotonia/etiologia , Adolescente , Adulto , Idade de Início , Diafragma/fisiopatologia , Feminino , Doença de Depósito de Glicogênio Tipo II/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Miotonia/epidemiologia , Músculos Paraespinais/fisiopatologia , Estudos Retrospectivos , Adulto JovemAssuntos
Betacoronavirus , Infecções por Coronavirus , Pandemias , Pneumonia Viral , COVID-19 , Humanos , SARS-CoV-2RESUMO
Background and Objectives: The COVID-19 pandemic forced a shift to virtual care in several neurologic care settings. Little is known about the validity of the virtual neurologic examination (VNE) for clinical decision making when compared with the in-person neurologic examination (IPNE). The objective of this study was to investigate the utility of the VNE in arriving at an accurate localization and diagnosis in comparison with the traditional IPNE in an ambulatory outpatient setting. Methods: A retrospective chart review of patients examined virtually and in-person within 4 months at outpatient general neurology and neuromuscular clinics from 2 tertiary academic care centers during the COVID-19 pandemic was conducted. The Cohen kappa coefficient was calculated to test agreement between virtual and in-person assessment results, and descriptive statistical methods were used to compare accuracy, localization, and diagnosis. Results: A total of 81 patients met the inclusion criteria. Overall, there was fair agreement between VNE and IPNE (64% agreement, p = 0.003). Substantial agreement between VNE and IPNE was observed for gait abnormalities; moderate agreement for extraocular movements, facial weakness, dysarthria, fasciculation, and lower limb weakness; and fair agreement for bulk, upper limb weakness, and sensation. No agreement between VNE and IPNE was seen for hypokinetic or hyperkinetic movements and cerebellar signs. Compared with the IPNE, specificity of the VNE was 86% and sensitivity was 56%. Some cases demonstrated a consistent localization (44%) and diagnosis (57%) after virtual and in-person assessments. The localization was changed in 15% and refined in 41% of cases between visits. The diagnosis was changed in 14% and refined in 30% of cases. Discussion: The high rates of agreement in detecting an abnormality on the VNE and IPNE for some maneuvers and resultant clinical impressions may support the validity of the VNE for initial consultation depending on the clinical scenario. The VNE seems to be a good surrogate evaluation compared with the IPNE for certain chief complaints. The low sensitivity suggests that a normal VNE should warrant further in-person clinical correlation, especially in the context of a highly concerning history. The IPNE is more sensitive in detecting subtle abnormalities on examination, and a low threshold should be used to bring a patient in for an IPNE if the VNE is normal in certain clinical contexts.
RESUMO
INTRODUCTION: The relationship between sleep and neuromuscular fatigue is understood poorly. The goal of this study was to evaluate the effects of napping on quantitative measures of neuromuscular fatigue in patients with myasthenia gravis (MG). METHODS: Eight patients with mild to moderate MG were recruited. Patients underwent maintenance of wakefulness tests (MWT) and multiple sleep latency tests (MSLT). The Quantitative Myasthenia Gravis Score (QMGS) was measured before nap and after each nap to examine the effects of napping and sleep on neuromuscular weakness. RESULTS: Results showed that QMGS improves only after naps where patients slept more than 5 min but not where patients did not sleep or slept less than 5 min. CONCLUSIONS: Daytime napping mitigates neuromuscular fatigue in patients with MG, especially if patients slept for more than 5 min.
Assuntos
Fadiga Muscular/fisiologia , Miastenia Gravis/fisiopatologia , Junção Neuromuscular/fisiopatologia , Sono/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polissonografia/métodos , Estudos Prospectivos , Índice de Gravidade de Doença , Método Simples-Cego , Fatores de Tempo , Vigília/fisiologiaAssuntos
Autoanticorpos/metabolismo , Encéfalo/patologia , Miastenia Gravis/patologia , Receptores Proteína Tirosina Quinases/metabolismo , Receptores Colinérgicos/metabolismo , Encéfalo/fisiopatologia , Feminino , Humanos , Imunoglobulinas/administração & dosagem , Imunoglobulinas/farmacologia , Pessoa de Meia-Idade , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatologia , Alta do PacienteRESUMO
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.