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Physician Podcasts

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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.

Amyloid TTR treatment studies

    7/23/2018
Dr. Burns interviews Dr. Litchy and Dr. Jim & Peter Dyck on their article, Amyloid TTR treatment studies.

Subcutaneous versus intravenous immunoglobulin for chronic autoimmune neuropathies. A meta-analysis

    6/13/2018
Dr. Speelziek interviews Dr. Dr. Kimpinski and Dr. Sposato on their article, Subcutaneous versus intravenous immunoglobulin for chronic autoimmune neuropathies. A meta-analysis.

Complex and simple clinical reaction times are associated with gait, balance, and major fall injury in older subjects with diabetic peripheral neuropathy.

    3/27/2018
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 (R/P = 0.653/0.004), and decreased step width variability and range (R/P = -0.696/0.001 and -0.782/<0.001, respectively) 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.

Acute Nutritional Axonal Neuropathy

    3/27/2018
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 is one syndrome, caused by micronutrient deficiencies. Muscle Nerve 57: 33-39, 2018.

The Value Transformation of Health Care: Impact on Neuromuscular and Electrodiagnostic Medicine.

    1/19/2018
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 potential to reshape health care delivery in the United States, with implications for neuromuscular and electrodiagnostic (EDX) specialists. Meaningful quality measures are required for neuromuscular and EDX specialists. Muscle Nerve 56: 679-683, 2017.

Hereditary Myopathies With Early Respiratory Insufficiency in Adults.

    1/19/2018
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.

The thoracic outlet syndromes: Part 1 and 2. The arterial, venous, neurovascular, and disputed thoracic outlet syndromes

    10/26/2017
Dr. Willer interviews Dr. Mark Ferrante about the article The thoracic outlet syndromes: Part 1 and 2. The arterial, venous, neurovascular, and disputed thoracic outlet syndromes.

Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy

    8/24/2017
Dr. Nicholas Silvestri interviews Dr. Thomas Brannagan about the article Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy. Reasons for therapeutic failure in CIDP are inadequate immunotherapy and alternative diagnoses. Certain clinical and electrophysiological features help to distinguish true CIDP from mimics. Once CIDP is confirmed, optimization of IVIg dosing, addition of corticosteroids, plasmapheresis, or chemotherapy results in consistent improvement. Caution is advised when using response to therapy to diagnose CIDP. Muscle Nerve 55: 476-482, 2017.

International consensus guidance for management of myasthenia gravis: Executive summary

    8/24/2017
Dr. Nicholas Silvestri interviews Dr. Donald Sanders about the article International consensus guidance for management of myasthenia gravis: Executive summary. In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. Guidance statements were developed for symptomatic and immunosuppressive treatments, IV immunoglobulin and plasma exchange, management of impending and manifest myasthenic crisis, thymectomy, juvenile MG, MG associated with antibodies to muscle-specific tyrosine kinase, and MG in pregnancy.

Optimizing muscle selection for electromyography in amyotrophic lateral sclerosis

    6/22/2017
Dr. Justin Willer interviews Dr. Yuebing Li about optimizing muscle selection for electromyography in amyotrophic lateral sclerosis. He was part of a group that compared the yield of limb and thoracic paraspinal muscle examination for revealing lower motor neuron (LMN) dysfunction on electromyography (EMG) in amyotrophic lateral sclerosis (ALS). Distal limb muscles showed the highest electrodiagnostic sensitivities of LMN dysfunction in ALS regardless of onset region and diagnostic certainty at the time of diagnosis. Electrodiagnostic yield is higher in muscles from the onset limb. Noncontiguous spread of lower motor neuron degeneration is present in ALS. Optimally selected 6 upper and 5 lower extremity muscles yielded >98% of potential positive cervical or lumbosacral segments. An algorithmic approach to needle EMG in ALS based on pretest probability of individual muscles optimizes electrodiagnostic yield, thus possibly minimizing test duration and patient discomfort. Muscle Nerve. 2016 Oct 19. doi: 10.1002/mus.25444.

Transthyretin amyloid neuropathy has earlier neural involvement but better prognosis than primary

    3/3/2017
Dr. Ted Burns interviews Drs. Adam Loavenbruck and Phillip Low about their article "Transthyretin amyloid neuropathy has earlier neural involvement but better prognosis than primary amyloid counterpart: an answer to the paradox?" One hundred one cases of amyloidosis with peripheral neuropathy were identified, 60 primary and 41 transthyretin. Twenty transthyretin cases were found to have Val30Met mutations; 21 had other mutations. Compared to primary cases, transthyretin cases had longer survival, longer time to diagnosis, higher composite autonomic severity scale scores, greater reduction of upper limb nerve conduction study amplitudes, more frequent occurrence of weakness, and later non-neuronal systemic involvement. Four systemic markers (cardiac involvement by echocardiogram, weight loss > 10 pounds, orthostatic intolerance, fatigue) in combination were highly predictive of poor survival in both groups. Their findings suggest that transthyretin has earlier and greater predilection for neural involvement and more delayed systemic involvement. The degree and rate of systemic involvement is most closely related to prognosis. Ann Neurol 2016;80:401-411.

Association of inclusion body myositis with T cell large granular lymphocytic leukaemia

    1/27/2017
Dr. Ted Burns interviews Dr. Stephen Greenberg about his article about the association of inclusion body myositis with T cell large granular lymphocytic leukaemia. Iinclusion body myositis and T cell large granular lymphocytic leukaemia are rare diseases involving pathogenic cytotoxic CD8+ T cells. After encountering four patients with both disorders, we prospectively screened 38 patients with inclusion body myositis for the presence of expanded large granular lymphocyte populations by standard clinical laboratory methods (flow cytometry, examination of blood smears, and T cell receptor gene rearrangements), and performed muscle immunohistochemistry for CD8, CD57, and TIA1. Most (22/38; 58%) patients with inclusion body myositis had aberrant populations of large granular lymphocytes in their blood meeting standard diagnostic criteria for T cell large granular lymphocytic leukaemia. These T cell populations were clonal in 20/20 patients and stably present on follow-up testing in 15 patients a median of 350 days later. Cross-sectional data suggested more aggressive disease in patients with such expansions than without. Muscle immunohistochemistry demonstrated invasion of large granular lymphocytes into muscle in 15/15 inclusion body myositis patients but in only 1/28 patients with dermatomyositis or polymyositis. The extent of CD8+ and CD57+ cells in inclusion body myositis muscle correlated with the size of blood large granular lymphocyte populations. Myofibre-invading cells expressed CD57, a marker of persistent T cell exposure to antigen and T cell aggressiveness. Brain. 2016 May;139(Pt 5):1348-60. doi: 10.1093/brain/aww024. Epub 2016 Feb 26.

POEMS vs. CIDP

    12/21/2016
POEMS, which is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes, is a rare disorder that often looks like CIDP when encountered in the neuromuscular or neurology clinic. Dr. Ted Burns interviews Drs. Michelle Maurermann and P. James B. Dyck about three articles about the topic. The articles are: Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy (Muscle Nerve. 2015 Oct;52:658-9), Nerve pathologic features differentiate POEMS syndrome from CIDP (Acta Neuropathol Commun. 2016 Oct 31;4:116.), and Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP (J Neurol Neurosurg Psychiatry. 2012 May;83:480-6).

Gene editing in monogenic diseases with CRISPR. Interview with Eric Olson, PhD

    12/7/2016
Dr. Ted Burns interviews Dr. Eric Olson about gene editing in monogenic diseases with CRISPR

Presymptomatic ALS genetic counseling and testing: Experience and recommendations

    11/28/2016
Dr. Ted Burns interviews Dr. Michael Benatar about his article, Presymptomatic ALS genetic counseling and testing: Experience and recommendations. Remarkable advances in our understanding of the genetic contributions to amyotrophic lateral sclerosis (ALS) have sparked discussion and debate about whether clinical genetic testing should routinely be offered to patients with ALS. A related, but distinct, question is whether presymptomatic genetic testing should be offered to family members who may be at risk for developing ALS. Existing guidelines for presymptomatic counseling and testing are mostly based on small number of individuals, clinical judgment, and experience from other neurodegenerative disorders. Over the course of the last 8 years, we have provided testing and 317 genetic counseling sessions (including predecision, pretest, posttest, and ad hoc counseling) to 161 first-degree family members participating in the Pre-Symptomatic Familial ALS Study (Pre-fALS), as well as testing and 75 posttest counseling sessions to 63 individuals with familial ALS. Based on this experience, and the real-world challenges we have had to overcome in the process, we recommend an updated set of guidelines for providing presymptomatic genetic counseling and testing to people at high genetic risk for developing ALS. These recommendations are especially timely and relevant given the growing interest in studying presymptomatic ALS. Neurology. 2016 Jun 14;86(24):2295-302

Randomized Trial of Thymectomy in Myasthenia Gravis

    11/4/2016
Dr. Ted Burns interviews Dr. Gil Wolfe about his article Randomized Trial of Thymectomy in Myasthenia Gravis. Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. A multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone was done. The results showed thymectomy improved clinical outcomes over a 3-year period in patients with nonthymomatous myasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.). N Engl J Med. 2016 Aug 11;375(6):511-22.

Normative Data Task Force: Establishing High-Quality Reference Values for NCS

    10/18/2016
Dr. Ted Burns interviews Dr. Timothy Dillingham about the Normative Data Taskforce (NDTF) report on establishing high quality reference values for nerve conduction studies (NCS). Because there are not uniform standards for NCS in the United States, the NDTF developed consensus-based methodological criteria for examining NCS and applied this quality criteria to a systematic review of the published literature. The NDTF has done the difficult work of scouring the literature and identifying high quality articles for our members. Nerve conduction testing techniques and normative reference values from these studies that met a high quality standard are now presented for our membership to use in an easy to follow tabular format. Two reports on the work of the NDTF were published in the September issue of Muscle and Nerve: Muscle Nerve. 2016 Sep;54(3):366-70. doi: 10.1002/mus.25204 and Muscle Nerve. 2016 Sep;54(3):371-7.
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