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.

A prospective study of benign fasciculation syndrome and anxiety.

INTRODUCTION: Benign fasciculations are common. Despite the favorable prognosis of benign fasciculation syndrome (BFS), patients are often anxious about their symptoms. In this study, we prospectively followed 35 patients with BFS over a 24-month period. METHODS: We conducted serial questionnaires to assess anxiety, associated symptoms, and duration. RESULTS: 71.4% of patients were men, and 34.4% were employed in the medical field. Most reported anxiety, but only 14% were anxious as measured by the Zung self-rating anxiety scale. Fasciculations were most common in the calves and persisted in 93% of patients. Anxiety levels did not change over time. Associated symptoms (subjective weakness, sensory symptoms, and cramps) were common and resolved to varying degrees. No patients developed motor neuron disease. DISCUSSION: BFS is a benign disorder that usually persists over time. Commonly associated symptoms include subjective weakness, sensory symptoms, and cramps. BFS is usually not associated with pathologic anxiety. Muscle Nerve 58:852-854, 2018.

Investigating the relationship between participation in the building infrastructure leading to diversity (BUILD) initiative and intent to pursue a science career: A cross-sectional analysis.

This paper presents an analysis of survey data to examine the association between supervised structured mentoring and students' intent to pursue a career in science. Data were collected from students in the 10 Building Infrastructure Leading to Diversity (BUILD) research training programs, developed through grants from the National Institutes of Health. Propensity score matching and multinomial logistic regression demonstrated that exposure to BUILD programs-meaning participation in undergraduate research, receipt of mentoring from a primary mentor, and/or participation as a funded scholar and/or associate of each BUILD site's training program-was associated with increased intent to pursue a science career. These findings have implications for STEM program evaluation and practice in higher education.

Developing Situated Measures of Science Instruction Through an Innovative Electronic Portfolio App for Mobile Devices: Reliability, Validity, and Feasibility.

Adoption of new instructional standards in science demands high-quality information about classroom practice. Teacher portfolios can be used to assess instructional practice and support teacher self-reflection anchored in authentic evidence from classrooms. This study investigated a new type of electronic portfolio tool that allows efficient capture of classroom artifacts in multimedia formats using mobile devices. We assess the psychometric properties of measures of quality instruction in middle school science classrooms derived from the contents of portfolios collected using this novel tool-with instruction operationalized through dimensions aligned to the Next Generation Science Standards. Results reflect low rater error and adequate reliability for several dimensions, a dominant underlying factor, and significant relations to some relevant concurrent indicators. Although no relation was found to student standardized test scores or course grades, portfolio ratings did relate to student self-efficacy perceptions and enjoyment of science. We examine factors influencing measurement error, and consider the broader implications of the results for assessing the validity of portfolio score interpretations, and the feasibility and potential value of this type of tool for summative and formative uses, in the context of large-scale instructional improvement efforts.

Pediatric sciatic neuropathy associated with neoplasms.

Seven children with sciatic neuropathy associated with an underlying neoplasm are reported. Clinical presentation, electrophysiological data, imaging, pathology, and/or autopsy results are described. Pain and weakness, primarily foot drop, were the most common presenting symptoms. The mechanism of sciatic neuropathy was varied and included: nerve infiltration by the adjacent neoplasm (neuroblastoma, rhabdomyosarcoma, and leukemic or lymphomatous infiltration); an expanding, intrinsic neurogenic tumor (perineurioma); or intraoperative stretch injury (osteosarcoma resection). The prognosis for sciatic nerve recovery was good among children who survived their associated cancer. Three children died from the cancer or complications of treatment. One child with perineurioma remained clinically stable, and two children improved after treatment of their neoplasm.

Immune-mediated necrotizing myopathy associated with statins.

We report patients from two neuromuscular centers who were evaluated between the years 2000 and 2008 and met the following criteria: (1) proximal muscle weakness occurring during or after treatment with statins; (2) elevated serum creatine kinase (CK); (3) persistence of weakness and elevated CK despite discontinuation of the statin; (4) improvement with immunosuppressive agents; and (5) muscle biopsy showing necrotizing myopathy without significant inflammation. Twenty-five patients fulfilled our inclusion criteria. Twenty-four patients required multiple immunosuppressive agents. Fifteen patients relapsed after being tapered off immunosuppressive therapy. Exposure to statins prior to onset was significantly higher in patients with necrotizing myopathy (82%) as compared to those with dermatomyositis (18%), polymyositis (24%), and inclusion-body myositis (38%) seen in the same time period. The lack of improvement following discontinuation of statins, the need for immunosuppressive therapy, and frequent relapse when treatment was tapered suggest an immune-mediated etiology for this rare, statin-associated necrotizing myopathy.

Worsening of anti-Hu paraneoplastic neurological syndrome related to anti-PD-1 treatment: Case report and review of literature.

We present an illustrative case of a 62-year-old woman with small cell lung cancer who developed progressive worsening of pre-existing anti-Hu antibody associated sensory neuronopathy after treatment with programmed cell death-1 (PD-1) inhibitor, nivolumab. We review the literature and identify 6 reported cases to understand the clinical outcomes of patients with anti-Hu paraneoplastic neurologic syndrome (PNS) treated with anti-PD-1 treatment. The PNS clinical spectrum comprised of encephalitis, a combination of sensory neuronopathy and anti-NMDAR encephalitis, isolated sensory neuronopathy, and encephalomyelitis. Immune checkpoint inhibitor have the potential to worsen pre-existing anti-Hu PNS and may promote the development of anti-Hu PNS.

Peripheral Vasculitic Neuropathy Associated With Minocycline Use.

We describe 2 patients presenting with multiplex mononeuritis, associated with skin manifestation, secondary to minocycline-induced vasculitis. One of the cases is associated neither with lupus nor polyarteritis nodosa. An extensive laboratory workup ruled out any possible underlying immunologic disorder. Electrodiagnostic studies were conducted to show axonal neuropathy in patchy and multifocal distribution consistent with multiplex mononeuritis. This diagnosis was confirmed with nerve biopsy. Withdrawing from the offending medication, minocycline, improved the patients' clinical condition and the quantitative serological measures.

Barraquer-Simons Syndrome.

BACKGROUND: Barraquer-Simons syndrome (BSS) is a rare acquired lipodystrophy characterized by gradually symmetric subcutaneous fat loss in a craniocaudal distribution, associated with hypocomplementemia, diabetes and hypertriglyceridemia. Few investigators have studied body fat distribution with cross-sectional imaging techniques. METHODS: We present 2 cases of BSS with emphasis on phenotypic analysis through cross-sectional imaging. RESULTS: For the first time, we demonstrate bone marrow involvement and deep cervical and axillary fat sparing of Barraquer-Simons using magnetic resonance imaging. CONCLUSION: Phenotypic analysis in lipodystrophies such as Barraquer-Simons is an essential guide for future experiments. Therefore, careful analysis of cross-sectional imaging should be conducted in future studies as areas of involvement or fat sparing may be overlooked. The major contributions of our work are that this is the first time that deep cervical or nuchal and axillary fat sparing and bone marrow involvement has been documented in BSS.

Myopathies.

Electrodiagnostic studies are an important adjunct to the clinical examination of a patient with a suspected myopathy; however, the clinical examination is crucial in making an accurate diagnosis, because electrodiagnostic studies have only a limited role in delineating with certainty the underlying myopathic disorder. Hereditary and acquired myopathies are reviewed in this article, with particular emphasis on distinguishing clinical and electrodiagnostic features. The hereditary myopathies that are discussed include the muscular dystrophies and the congenital distal mitochondrial, and metabolic myopathies. Acquired myopathies, including inflammatory, endocrine, and toxic myopathies, as well as those associated with systemic illness, are briefly reviewed.

Lambert-Eaton syndrome, an unrecognized treatable pediatric neuromuscular disorder: three patients and literature review.

BACKGROUND: Lambert-Eaton myasthenic syndrome, a presynaptic neuromuscular junction autoimmune disorder, rarely occurs in children. Patients typically present with proximal lower extremity weakness with areflexia. METHODS: We report three children presenting between ages 9 and 10 years diagnosed with Lambert-Eaton myasthenic syndrome 2 years, 1 year, and 5 months later, respectively. Their clinical attributes are correlated with nine other pediatric Lambert-Eaton myasthenic syndrome patients found in our literature review. RESULTS: These patients were identified as having Lambert-Eaton myasthenic syndrome during their evaluation for proximal weakness. Low-amplitude compound muscle action potentials classically facilitating >100% with voluntary exercise and/or 50 Hz stimulation were essential to diagnosis. Three of the 12 children had associated malignancies, two of them had lymphoproliferative disorders with onset of symptoms more rapid than the rest, and the third had neuroblastoma. The nine nonparaneoplastic Lambert-Eaton myasthenic syndrome patients responded to immunomodulatory therapy with close return to their baseline function. Complete remission no longer necessitating medication was reported in two patients. Follow-up up to 17 years was available on two patients previously reported. CONCLUSION: Lambert-Eaton myasthenic syndrome is a diagnosis that must be considered in children presenting with unidentified proximal muscle weakness. In most children, Lambert-Eaton myasthenic syndrome is a primary autoimmune disorder that is treatable. Nevertheless, a search for malignancy is recommended.

Rituximab and mycophenolate combination therapy in refractory dermatomyositis with multiple autoimmune disorders.

We report a case of dermatomyositis associated with rheumatoid arthritis, Hashimoto thyroiditis, and diabetes mellitus responsive only to combination of rituximab with mycophenolate. A 42-year-old woman presented with proximal muscle weakness, myalgias, fever, night sweats, and shortness of breath. Creatinine kinase was 8155 IU/L, and muscle biopsy was diagnostic of dermatomyositis. She was started on glucocorticoids; her systemic symptoms improved, but her muscle weakness persisted. She was serially treated with intravenous immunoglobulin, azathioprine, and mycophenolate mofetil without improvement in her weakness. She responded dramatically to combination therapy with rituximab and mycophenolate, with improvement in strength and normalization of creatinine kinase. She has been well controlled on rituximab infusion every 6 months and maintenance mycophenolate mofetil.

Achalasia, chronic sensory neuropathy, and N-type calcium channel autoantibodies: beneficial response to IVIG.

Autoimmune gastrointestinal dysmotility (AGID) can result from paraneoplastic onconeuronal antibodies. Patients may present with regional hypomotility anywhere along the gastrointestinal tract. We report a case of a woman who developed an insidious sensory neuropathy and achalasia. She was found to have a high-titer of N-type voltage gated-calcium channel (VGCC) antibodies. She demonstrated clinical and electrophysiological improvement of her neuropathy, as well as improvement of her swallowing and gait, after treatment with intravenous immunoglobulins.

Sensory polyneuropathy associated with paclitaxel-eluting coronary stent.

Paclitaxel is a microtubule-stabilizing chemotherapeutic agent used in ovarian and breast cancer; its principal adverse effect is sensory neuropathy. We describe the occurrence of sensory polyneuropathy after multiple paclitaxel-eluting stents in a patient who may have sub-clinical Sjogrens syndrome.

Pediatric sciatic neuropathies due to unusual vascular causes.

Four cases of pediatric sciatic neuropathies due to unusual vascular mechanisms are reported. Pediatric sciatic neuropathies were seen after umbilical artery catheterization, embolization of arteriovenous malformation, meningococcemia, and hypereosinophilic vasculitis. Electrophysiologic studies demonstrated abnormalities in motor studies of peroneal and tibial nerves. Sensory studies demonstrated abnormalities of sural and superficial peroneal nerves. Results of needle electromyography were abnormal in sciatic-innervated muscles. Prognosis was variable and depended on the severity of the initial nerve injury.