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AANEM Presents Nerve and Muscle Junction

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AANEM Presents Nerve and Muscle Junction is discourse on recent publications in neuromuscular and electrodiagnostic medicine literature, featuring interviews with the authors and other experts. Presented by the American Association of Neuromuscular and Electrodiagnostic Medicine.
135 Episodes
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Dr. Justin Willer interviews Dr. Michael Hehir on his article, Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis.
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.
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.
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.
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.
Dr. Justin 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 either nerve conduction 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.
Dr. Justin Willer interviews Dr. Jeffrey Allen on his article, Electrodiagnostic Errors Contribute to Chronic Inflammatory Demyelinating Polyneuropathy Misdiagnosis.
Dr. Ted Burns interviews Dr. William Litchy and Drs. Jim and Peter Dyck on their articles, Amyloid TTR treatment studies. The articles are Inotersen Treatment for Patients with Hereditary Transthyretin Amyloidosis (N Engl J Med. 2018 Jul 5;379(1):22-31. doi: 10.1056/NEJMoa1716793) and Patisiran, an RNAi Therapeutic, for Hereditary Transthyretin Amyloidosis (N Engl J Med. 2018 Jul 5;379(1):11-21. doi: 10.1056/NEJMoa1716153.)
Dr. Speelziek interviews Dr. Kurt Kimpinski and Dr. Luciano Sposato on their article, Subcutaneous versus intravenous immunoglobulin for chronic autoimmune neuropathies. A meta-analysis. High-dose intravenous immunoglobulin (IVIg) is an evidence-based treatment for multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP). Recently, subcutaneous immunoglobulin (SC-Ig) has received increasing attention. The authors performed a meta-analysis of reports of efficacy and safety of SC-Ig versus IVIg for inflammatory demyelinating polyneuropathies.The efficacy of SC-Ig is similar to IVIg for CIDP and MMN and has a significant safety profile. Muscle Nerve 55: 802-809, 2017.
Dr. Sandra Hearn interviews Dr. James Richardson on his article, Complex and simple clinical reaction times are associated with gait, balance, and major fall injury in older subjects with diabetic peripheral neuropathy. In the diabetic peripheral neuropathy subjects, the ratio of complex RTclin accuracy to simple RTclin latency was strongly associated with longer UST, and decreased step width variability and range on an uneven surface. Additionally, the 2 DPN subjects sustaining major injuries had lower complex RTclin accuracy:simple RTclin latency than those without.The ratio of complex RTclin accuracy:simple RTclin latency is a potent predictor of UST and frontal plane gaitvariability in response to perturbations and may predict major fall injury in older subjects with DPN. These short latency neurocognitive measures may compensate for lower limb neuromuscular impairments and provide a more comprehensive understanding of balance and fall risk. Am J Phys Med Rehabil. 2017 Jan;96(1):8-16.
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