Aanem Presents Nerve And Muscle Junction

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.

Dr. Willer interviews Dr. Johanna Hamel and Dr. Eric Logigian about their article Acute Nutritional Axonal Neuropathy. This study describes clinical, laboratory, and electrodiagnostic features of a severe acute axonal polyneuropathy common to patients with acute nutritional deficiency in the setting of alcoholism, bariatric surgery (BS), or anorexia. Thirteen patients were identified with a severe, painful, sensory or sensorimotor axonal polyneuropathy that developed over 2-12 weeks with sensory ataxia, areflexia, variable muscle weakness, poor nutritional status, and weight loss, often with prolonged vomiting and normal cerebrospinal fluid protein. Vitamin B6 was low in half and thiamine was low in all patients when obtained before supplementation. Patients improved with weight gain and vitamin supplementation, with motor greater than sensory recovery. The authors suggest that acute or subacute axonal neuropathy in patients with weight loss or vomiting associated with alcohol abuse, BS, or dietary deficiency

Dr. Scott Speelziek interviews Dr. Pushpa Narayanaswami and Dr. Lyell K Jones about the article, The Value Transformation of Heath Care: Impact on Neuromuscular and Electrodiagnostic Medicine. Beginning in 2017, most physicians who participate in Medicare are subject to the Medicare Access and CHIP Reauthorization Act (MACRA), the milestone legislation that signals the US health care system's transition from volume-based to value-based care. Here we review emerging trends in development of value-based healthcare systems in the US. MACRA and the resulting Quality Payment Program create 2 participation pathways, the Merit-based Incentive Payment System (MIPS) and the Advanced Alternative Payment Model (AAPM) pathway. Although there are several program incentives for AAPM participation, to date there have been few AAPM options for specialists. MIPS and its widening bonus and penalty window will likely be the primary participation pathway in the early years of the program. Value-based payment has the potentia

Dr. Ted Burns interviews Dr. Elie Naddaf and Dr. Margherita Milone about the article Hereditary Myopathies With Early Respiratory Insufficiency in Adults. Hereditary myopathies with early respiratory insufficiency as a predominant feature of the clinical phenotype are uncommon and underestimated in adults. The authors highlighted the most common hereditary myopathies associated with early respiratory insufficiency as the predominant clinical feature, and underscored the importance of a timely diagnosis for patient care. Muscle Nerve 56: 881-886, 2017.

Thoracic Outlet Syndrome Part 1 and Part 2

Evaluation of Patients With Refractory Chronic Inflammatory Demyelinating Polyneuropathy

International Consensus Guidance for Management of Myasthenia Gravis: Executive Summary

Optimizing Muscle Selection for Electromyography in Amyotrophic Lateral Sclerosis

Association of Inclusion Body Myositis With T Cell Large Granular Lymphocytic Leukaemia

Transthyretin Amyloid Neuropathy has Earlier Neural Involvement but Better Prognosis than Primary

POEMS vs CIDP

Gene Editing in Monogenic Diseases With CRISPR by Eric Olson, PhD

Presymptomatic ALS Genetic Counseling and Testing: Experience and Recommendations

Randomized Trial of Thymectomy in Myasthenia Gravis by Dr. Gil Wolfe