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Podcasts

Podcasts can be listened to by clicking the description below or by searching for “AANEM” in iTunes or Apple’s Podcast app.  


 
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Clinical features of LRP4/agrin-antibody–positive Myasthenia Gravis

    11/3/2021
Dr. Elahi Behzad interviews Dr. Michael H. Rivner on the clinical features of LRP4/agrin-antibody–positive Myasthenia Gravis.

Approach to Dysphagia With Focus on Neuromuscular Disorders

    9/10/2021
Dr. Elie Naddaf interviews Dr. Georgia Malandraki and Dr. Kendrea Garand on the approach to dysphagia, with focus on neuromuscular disorders.

Porphyric Neuropathy Part II

    7/23/2021
Dr. Priya Dhawan interviews Drs. Mohamed Kazamel & Robert Desnick on their article, Porphyric Neuropathy Part II.

Porphyric Neuropathy Part I

    7/23/2021
Dr. Priya Dhawan interviews Drs. Mohamed Kazamel & Robert Desnick on their article, Porphyric Neuropathy Part I.

COVID-19-Associated Guillain-Barre Syndrome: The Early Pandemic Experience

    4/21/2021
Dr. Willer interviews Dr. Caress on his article, COVID-19-Associated Guillain-Barre Syndrome: The Early Pandemic Experience. Please review these papers that were mentioned in this podcast: "Epidemiological and cohort study finds no association between COVID-19 and Guillain-Barre´ syndrome." "COVID-19 vaccine and Guillain-Barre´ syndrome let’s not leap to associations."

Autonomic Neuropathies

    4/15/2021
Dr. Patel interviews Dr. Stino on his article, Autonomic Neuropathies.

Guidelines on clinical presentation and management of nondystrophic myotonias

    3/17/2021
Dr. Waheed interviews Dr. LoRusso and Dr. Statland on thier article, Guidelines on clinical presentation and management of nondystrophic myotonias.

COVID-19 and neuromuscular disorders.

    3/1/2021
Dr. Elie Naddaf interviews Dr. Amanda Guidon and Dr. Anthony Amato regarding coronavirus 19 (COVID19) and Neuromuscular disorders. This podcaste reviews the potential neuromuscular complications of COVID-19, impact of COVID-19 on patients with preexisting neuromuscular disease, and guidance for management of immunosuppressive and immunomodulatory therapies and timing vaccination. We also review the impact of COVID19 on training in neuromuscular and electrodiagnostic medicine

Electrodiagnostic approach to patients presenting with respiratory symptoms

    10/28/2020
Dr. Elie Naddaf interviews Dr. Andrea Boon about the approach to patients presenting to the EMG laboratory with respiratory symptoms, to evaluate for underlying neuromuscular cause of these symptoms. Dr Boon describes the role of phrenic nerve conduction studies and needle electromyography of the diaphragm and the technical limitations of these studies. Dr Boon also details the role of diaphragm ultrasound to enhance the accuracy and safety of diaphragm electromyography, and improve the diagnostic yield of EDX testing, including an overview of the diaphragm ultrasound technique.

Anterior interosseous nerve syndrome

    9/30/2020
Dr. Sandra Hearn interviews Dr. Darryl Sneag, Dr. Scott Wolfe, and Dr. Joseph Feinberg about their work in advancing our understanding of anterior interosseous nerve syndrome, based on their article: Sneag DB, Arányi Z, Zusstone EM, Feinberg JH, Queler SC, Nwawka OK, Lee SK, Wolfe SW. Fascicular constrictions above elbow typify anterior interosseous nerve syndrome. Muscle & Nerve. 2020 Mar;61(3):301-10. The authors describe how severe anterior interosseous nerve syndrome (AINS) reveals fascicular constrictions of the AIN fascicles within the median nerve, typically localizing proximal to the elbow. The findings support an evolving understanding that many idiopathic mononeuropathies may arise from inflammatory (rather that compressive/entrapment) etiologies.

“Rehabilitative approach to ALS patients” with Dr. M.Elliott highlighting his article “Eye-controlled, power wheelchair performs well for ALS patients.”

    9/3/2020
Katarzyna Ibanez, MD interviewing Dr. M.Elliott on “Rehabilitative approach to ALS patients” while also highlighting his article “Eye-controlled, power wheelchair performs well for ALS patients.”

Immunomodulatory and Immunosuppressive Therapy for Neuromuscular Disorders Part II

    7/24/2020
Dr. Justin Willer interviews Dr. Constantine Farmakidis on his article, Immunomodulatory and Immunosuppressive Therapy for Neuromuscular Disorders Part II. While traditional immunosuppressive and immunomodulatory therapies remain the cornerstone of immune-mediated neuromuscular disease management, new and novel agents including antigen-specific, monoclonal antibody drugs, have emerged as important treatment options. This article is the second of a two-part series that reviews immune-based therapies in neuromuscular diseases. The first article provides an update on the use of traditional immune-based therapies such as corticosteroids, plasma exchange, steroid-sparing immunosuppressive drugs, and intravenous immunoglobulin G. This second article focuses on new and novel immune-based therapies, including eculizumab, a complement inhibitor approved for acetylcholine receptor antibody-positive myasthenia gravis; rituximab, a B-cell depletion therapy with evolving indications in neuromuscular diseases; and the subcutaneous formulation of immunoglobulin G that gained approval for use in chronic inflammatory demyelinating polyradiculoneuropathy in 2018. Finally, several novel antigen-specific drugs at different stages of investigation in neuromuscular disease are also reviewed.

Immunomodulatory and Immunosuppressive Therapy for Neuromuscular Disorders Part I

    7/24/2020
Dr. Justin Willer interviews Dr. Constantine Farmakidis on his article, Immunomodulatory and Immunosuppressive Therapy for Neuromuscular Disorders Part I. While traditional immunosuppressive and immunomodulatory therapies remain the cornerstone of immune-mediated neuromuscular disease management, new and novel agents including antigen-specific, monoclonal antibody drugs, have emerged as important treatment options. This article is the second of a two-part series that reviews immune-based therapies in neuromuscular diseases. The first article provides an update on the use of traditional immune-based therapies such as corticosteroids, plasma exchange, steroid-sparing immunosuppressive drugs, and intravenous immunoglobulin G. This second article focuses on new and novel immune-based therapies, including eculizumab, a complement inhibitor approved for acetylcholine receptor antibody-positive myasthenia gravis; rituximab, a B-cell depletion therapy with evolving indications in neuromuscular diseases; and the subcutaneous formulation of immunoglobulin G that gained approval for use in chronic inflammatory demyelinating polyradiculoneuropathy in 2018. Finally, several novel antigen-specific drugs at different stages of investigation in neuromuscular disease are also reviewed.

Nutritional Neuropathies

    5/26/2020
Dr. Priya Dhawan interviews Dr. Kelly Gwathmey on her article, Nutritional Neuropathies.

Quality improvement projects in the EMG laboratory

    4/6/2020
Dr. Elie Naddaf interviews Dr. Andrea Leep Hunderfund on her article, Quality improvement projects in the EMG laboratory.

Long-term Safety and Efficacy of Eculizumab in Generalized Myasthenia Gravis

    12/18/2019
Dr. Elahi Behzad interviews Dr. Srikanth Muppidi on his article, Long-term Safety and Efficacy of Eculizumab in Generalized Myasthenia Gravis. Eculizumab is effective and well tolerated in patients with antiacetylcholine receptor antibody-positive refractory generalized myasthenia gravis (gMG; REGAIN; NCT01997229). We report an interim analysis of an open-label extension of REGAIN, evaluating eculizumab's long-term safety and efficacy. Eculizumab (1,200 mg every 2 weeks for 22.7 months [median]) was administered to 117 patients. The safety profile of eculizumab was consistent with REGAIN; no cases of meningococcal infection were reported during the interim analysis period. Myasthenia gravis exacerbation rate was reduced by 75% from the year before REGAIN (P < 0.0001). Improvements with eculizumab in activities of daily living, muscle strength, functional ability, and quality of life in REGAIN were maintained through 3 years; 56% of patients achieved minimal manifestations or pharmacological remission. Patients who had received placebo during REGAIN experienced rapid and sustained improvements during open-label eculizumab (P < 0.0001). These findings provide evidence for the long-term safety and sustained efficacy of eculizumab for refractory gMG. Muscle Nerve 2019.

Ultrasound-Guided Treatment of Peripheral Entrapment Neuropathies

    10/30/2019
Dr. Sandra Hearn interviews Dr. John Norbury on his article, Ultrasound-Guided Treatment of Peripheral Entrapment Neuropathies. The advent of high‐resolution neuromuscular ultrasound (US) has provided a useful tool for conservative treatment of peripheral entrapment mononeuropathies. US‐guided interventions require careful coordination of transducer and needle movement along with a detailed understanding of sonoanatomy. Preprocedural planning and positioning can be helpful in performing these interventions. Corticosteroid injections, aspiration of ganglia, hydrodissection, and minimally invasive procedures can be useful nonsurgical treatments for mononeuropathies refractory to conservative care. Technical aspects as well as the current understanding of the indications and efficacy of these procedures for common entrapment mononeuropathies are reviewed in this study. Muscle & Nerve, 2019.

Effect of shoulder and elbow position on ulnar nerve conduction

    9/24/2019
The aims of this study were to determine the effect of shoulder and elbow position on ulnar nerve conduction studies (NCS), and the length of reserve that asymptomatic individuals have in their ulnar nerves. Healthy subjects (n = 22) underwent ulnar NCS in 4 positions of shoulder abduction and elbow flexion. The mean increase in nerve conduction velocity (NCV) from the slack position of shoulder adducted and elbow extended to the stretch position of shoulder abducted and elbow flexed was 2.9 m/s (P < 0.01). Using this difference, the mean length of reserve in the ulnar nerve along the limb was found to be 28.3 (SD=13.8) mm. Shoulder abduction increased the mean NCV 0.8 m/s (P = 0.03) independent of elbow flexion. This study demonstrates an ulnar nerve reserve in healthy patients that is taken up with shoulder abduction and elbow flexion and suggests shoulder position should be standardized during ulnar NCS. Muscle Nerve, 2019.

Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis

    7/17/2019
Dr. Justin Willer interviews Dr. Michael Hehir on his article, Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis. A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)‐antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in anti–muscle‐specific kinase (MuSK)‐MG. Methods: The therapeutic impact of thymectomy was evaluated from data collected for a multicenter, retrospective blinded review of rituximab in MuSK‐MG. Results: Baseline characteristics were similar between thymectomy (n = 26) and nonthymectomy (n = 29) groups, including treatment with rituximab (42% vs. 45%). At last visit, 35% of thymectomy subjects reached the primary endpoint, a Myasthenia Gravis Foundation of America (MGFA) post‐intervention status (PIS) score of minimal manifestations (MM) or better, compared with 55% of controls (P = 0.17). After controlling for age at onset of MG, rituximab, prednisone, and intravenous immunoglobulin/plasma exchange treatment, thymectomy was not associated with greater likelihood of favorable clinical outcome (odds ratio = 0.43, 95% confidence interval 0.12–1.53, P = 0.19). Discussion: Thymectomy was not associated with additional clinical improvement in this multicenter cohort of MuSK‐MG patients. Muscle Nerve 59:404–410, 2019

Satisfactory Response With Achieving Maintenance Low-Dose Prednisone in Generalized Myasthenia Gravis

    7/3/2019
Dr. Elie Naddaf interviews Dr. Mazen Dimachkie on his article, Satisfactory Response With Achieving Maintenance Low-Dose Prednisone in Generalized Myasthenia Gravis. To estimate the satisfactory response rate (SR%) with achieving maintenance, low-dose prednisone in acetylcholine receptor antibody-positive generalized myasthenia gravis. In this retrospective study, we estimate the SR% as defined by (remission/minimal manifestations status for at least 6 months using 7.5 mg or less of prednisone daily, for maintenance treatment at 2, 4, and 6 years after symptoms onset) for patients who were not taking steroid-sparing immunosuppressant (SSI) as a primary outcome and for patients taking an SSI as a secondary outcome. Forty-five patients were not taking an SSI at 2 years, 34 patients at 4 years, and 17 patients at 6 years; SR% was 44.4%, 64.7%, and 58.8%, respectively. Thirty-six patients were taking an SSI at 2 years, 22 patients at 4 years, and 15 patients at 6 years; the SR% was 50.0%, 45.4%, and 66.7%, respectively. Nearly half of the generalized myasthenia gravis patients who were not taking an SSI achieved an SR.

Clinical and Electrodiagnostic Features Of Nontraumatic Sciatic Neuropathy

    6/4/2019
Dr. Justin Willer interviews Dr. Yuebing Li about his article, Clinical and Electrodiagnostic Features Of Nontraumatic Sciatic Neuropathy. This study sought to characterize etiologies and features of sciatic neuropathy unrelated to penetrating nerve trauma. Methods: This investigation was a retrospective review of 109 patients with electrodiagnostically confirmed sciatic neuropathies. Results: Hip replacement surgery represented the most common (34.9%) etiology, whereas inflammatory sciatic neuropathy was seen in 7.3%. Electrodiagnostic testing revealed an axonal neuropathy in 95.4% and a demyelinating neuropathy in 4.6%. Predominant involvement of the peroneal division was seen in 39.4% and was tibial in 5.5%. Nine of 31 (29.0%) patients who had MRI or neuromuscular ultrasound study showed abnormalities within the sciatic nerve. At the final visit, 46.4% of patients required assistance for ambulation. Young age, lack of severe initial weakness, and presence of tibial compound muscle action potential or sural sensory nerve action potential were predictors of favorable outcome. Discussion: Sciatic neuropathies are usually axonal on electrodiagnostic testing, affect preferentially the peroneal division, and are commonly associated with incomplete recovery. Muscle Nerve 59:309–314, 2019.

Needle Electromyography and Histopathologic Correlation in Myopathies

    4/24/2019
Dr. Behzad Elahi interviews Dr. Devin I. Rubin on his article, Needle Electromyography and Histopathologic Correlation in Myopathies. Fibrillation potentials and short-duration MUPs predict pathologic changes of muscle fiber necrosis, splitting, and/or vacuolar changes (as seen with inflammatory myopathies and muscular dystrophies). Absence of fibrillation potentials suggests other myopathologic changes (e.g., congenital myopathy). Muscle Nerve 59:315-320, 2019.

Muscle Biopsy EMG Correlation

    2/11/2019
Dr. Scott Speelziek interviews Dr. Elie Naddaf on his article, Muscle Biopsy EMG Correlation. In myopathies, the correlation of individual electromyographic and histopathologic findings remains poorly explored, as most previous studies have focused on the ability of muscle biopsy and electromyography to distinguish the neuropathic vs. myopathic nature of the underlying neuromuscular disease. By demonstrating a clear correlation of various electromyographic and histopathologic findings, this study improves interpreting electrodiagnostic testing in myopathies, and serves as the basis to further assess the correlation between clinical, electromyographic, and histopathologic findings.

Long‐term neuromuscular outcomes of west nile virus infection: A clinical and electromyographic evaluation of patients with a history of infection

    8/31/2018
Dr. Willer interviews Dr. Athar Parveen on her article, Long‐term neuromuscular outcomes of west nile virus infection: A clinical and electromyographic evaluation of patients with a history of infection. The study found that 33% of these patients (10 of 30) showed abnormalities on nerve conduction and/or needle electromyography due to primary or secondary outcomes of WNV infection. Most common electrodiagnostic findings and causes of long-term disability were related to anterior horn cell poliomyelitis (WNV poliomyelitis). Electrical data on these patient populations were similar to those observed in chronic poliomyelitis. With more than 16,000 cases of WNV neuroinvasive disease reported across the USA since 1999, understanding clinical outcomes from infection will provide a resource for physicians managing long-term care of these patients. Muscle Nerve 57: 77-82, 2018.

Electrodiagnostic Errors Contribute to Chronic Inflammatory Demyelinating Polyneuropathy Misdiagnosis

    7/23/2018
Dr. Willer interviews Dr. Allen on their article, Electrodiagnostic Errors Contribute to Chronic Inflammatory Demyelinating Polyneuropathy Misdiagnosis.
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