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Physician Podcasts

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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.

Rapid screening for inflammatory neuropathies by standardized clinical criteria

    9/23/2016
Dr. Ted Burns interviews Dr. Chafic Karam about his article Rapid screening for inflammatory neuropathies by standardized clinical criteria. In this podcast, he discusses the Class IV evidence that 3 neuropathy characteristics—onset, distribution, and associated systemic features—accurately identify patients with inflammatory neuropathies. Rapid screening for inflammatory neuropathies by ODS clinical criteria is highly sensitive and has a high negative predictive value for noninflammatory neuropathies. ODS uses simple clinical criteria to rapidly screen for patients with a potentially treatable form of neuropathy and accelerate their diagnostic evaluation. Neurol Clin Pract 10.1212.

Sensory Neuronopathies

    9/19/2016
Dr. Justin Willer interviews Dr. Kelly Gwathmey about her article, Sensory Neuronopathies. The sensory neuronopathies (or ganglionopathies) are a small subcategory of neuropathies characterized by primary degeneration of the dorsal root ganglia and trigeminal ganglion sensory neurons, resulting in a distinctive clinical presentation. Patients typically have subacute onset of asymmetric, non–length-dependent sensory impairment and early ataxia. The etiologies of acquired sensory neuronopathies are rather limited. Early identification is imperative, as they may herald an underlying malignancy or an autoimmune condition such as Sjögren syndrome. This podcast reviews the Muscle and Nerve article on this subject. Muscle Nerve 53: 8–19, 2016

Critically re-evaluating a common technique: Accuracy, reliability, and confirmation bias of EMG

    3/25/2016
Dr. Stephen Goutman interviews Drs. Pushpa Narayanaswami and Seward Rutkove about their article Critically re-evaluating a common technique: Accuracy, reliability, and confirmation bias of EMG. This study supports that in evaluations of radiculopathy (1) serial EMG studies should be performed by the same electromyographer since intrarater reliability is better than interrater reliability; (2) knowledge of clinical information does not bias EMG interpretation substantially; (3) EMG has moderate diagnostic accuracy for radiculopathy with modest specificity and electromyographers should exercise caution interpreting mild abnormalities. Neurology. 2016 Jan 19;86(3):218-23. doi: 10.1212/WNL.0000000000002292. Epub 2015 Dec 23.

Can Mycophenolate Mofetil be Tapered Safely in MG? A Retrospective, Multicenter Analysis

    2/19/2016
Dr. Justin Willer interviews Dr. Lisa Hobson-Webb about her Muscle & Nerve article on safely tapering mycophenolate mofetil (MMF) in myasthenia gravis (MG) patients. MMF is frequently used to treat patients with MG but there is little information to guide clinicians on the safety of reducing the dose in well-controlled patients. Discussion includes standard dosages, determining clinical response to MMF and suggested goals in tapering MMF. Muscle Nerve. 2015 Aug;52(2):211-5. doi: 10.1002/mus.24694. Epub 2015 Jun 18.

Paraproteinemic Neuropathies

    2/8/2016
Dr. Justin Willer interviews Dr. Divisha Raheja about her Muscle & Nerve article on paraproteinemic neuropathies. When the neuropathy is the presenting feature, neuromuscular specialists can play a particularly important role, possibly uncovering clinical, laboratory, radiologic, electrodiagnostic, or biopsy findings that lead to identification of the underlying paraproteinemia. Dr. Raheja offers suggestions on when testing should be done, elaborating on the various types of testing as well as treatments. Muscle Nerve. 2015 Jan;51(1):1-13. doi: 10.1002/mus.24471.

Inherited Etiologies of Rhabdomyolysis

    10/21/2015
In this second part of the Clinical Presentation and Acquired Causes of Rhabdomyolysis podcast, Dr. Ted Burns interviews Drs. Jessica Nance and Andrew Mammen about their paper Diagnostic evaluation of rhabdomyolysis from the June 2015 issue of Muscle and Nerve. Rhabdomyolysis is characterized by severe acute muscle injury resulting in muscle pain, weakness, and/or swelling with release of myofiber contents into the bloodstream. Symptoms develop over hours to days after an inciting factor and may be associated with dark pigmentation of the urine. This podcast focuses on the inherited etiologies. For the article in Muscle and Nerve refer to Muscle Nerve 51: 793-810, 2015.

Pharmacotherapy for Neuropathic Pain in Adults: A Systematic Review and Meta-Analysis

    9/22/2015
Dr. Stephen Goutman interviews Dr. Nadine Attal about the article she coauthored in Lancet Neurology on pharmacotherapy for neuropathic pain in adults. Drs. Goutman and Attal discuss the evidence and limitations supporting each class of medication aimed at the treatment of neuropathic pain and how these recommendations can be incorporated into clinical practice. Lancet Neurol. 2015 Feb;14:162-173.

Clinical Presentation and Acquired Causes of Rhabdomyolysis

    9/8/2015
Dr. Ted Burns interviews Drs. Jessica Nance and Andrew Mammen about their paper Diagnostic evaluation of rhabdomyolysis from the June 2015 issue of Muscle and Nerve. Rhabdomyolysis is characterized by severe acute muscle injury resulting in muscle pain, weakness, and/or swelling with release of myofiber contents into the bloodstream. Symptoms develop over hours to days after an inciting factor and may be associated with dark pigmentation of the urine. This podcast focuses on the clinical presentation and acquired causes which can include substance abuse, medication or toxic exposures, electrolyte abnormalities, endocrine disturbances, and autoimmune myopathies. For the article in Muscle and Nerve refer to Muscle Nerve 51: 793-810, 2015.

Patterns of Clinical and EDX Abnormalities in early ALS

    8/11/2015
Noah Kolb, MD, interviews Neil Simon, MBBS, BSc, FRACP, about his article on patterns of clinical and electrodiagnostic abnormalities in early ALS, published in Muscle and Nerve with Drs. Lomen-Hoerth and Kiernan. Drs. Kolb and Simon discuss the distribution of clinical and neurophysiological abnormalities in patients with early ALS. Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the clinical region of onset. Findings suggest a pattern of disease spread in ALS. This study may serve to guide ongoing development of disease quantitation biomarkers and the targeting of future neuroprotective strategies. Muscle Nerve 50: 894–899, 2014

NCS Technique for Ulnar Neuropathy

    6/17/2015
An interview about ulnar neuropathy with the neurologist who invented ulnar inching, William W. Campbell, MD, and Mary Franklin, REEGT, CNCT. Have you ever wondered when to test the FDI with an ulnar nerve entrapment? Have you ever asked yourself what is ulnar mapping and when should I do this? Maybe you’ve wondered about the “10 centimeter” theory in measuring the ulnar nerve around the elbow or about the degree at which the elbow should be flexed and when to inch? If so, then this podcast is for you! It’s an excellent educational resource for technologists and physicians alike!

Neuralgic Amyotrophy (Parsonage-Turner): Etiology, Treatment, Natural History by Nens Van Alfen

    5/12/2015
Neuralgic amyotrophy--also known as Parsonage-Turner syndrome or brachial plexus neuritis--is a distinct and painful peripheral neuropathy that causes episodes of multifocal paresis and sensory loss in a brachial plexus distribution with concomitant involvement of other PNS structures (such as the lumbosacral plexus or phrenic nerve) in a large number of patients. The phenotype can be limited or extensive and the amount of disability experienced also varies between patients, but many are left with residual disabilities that affect their ability to work and their everyday life. Both idiopathic and hereditary forms exist. This podcast discusses etiology, treatment, and natural history.

Neuralgic Amyotrophy (Parsonage-Turner): presentation and diagnosis. Interview with Nens Van Alfen

    5/1/2015
Neuralgic amyotrophy--also known as Parsonage-Turner syndrome or brachial plexus neuritis--is a distinct and painful peripheral neuropathy that causes episodes of multifocal paresis and sensory loss in a brachial plexus distribution with concomitant involvement of other PNS structures (such as the lumbosacral plexus or phrenic nerve) in a large number of patients. The phenotype can be limited or extensive and the amount of disability experienced also varies between patients, but many are left with residual disabilities that affect their ability to work and their everyday life. Both idiopathic and hereditary forms exist.

Whole Exome Sequencing by Dr. Daniel MacArthur

    4/16/2015
Whole Exome Sequencing and the Clinical Impact of Sequencing Techniques by Dr. Daniel MacArthur, interviewed by Dr. Ted Burns.

Next Generation Sequencing and its Impact on Neuromuscular Medicine by Dr. Daniel MacArthur

    4/13/2015
Next Generation Sequencing and its Impact on Neuromuscular Medicine by Dr. Daniel MacArthur interviewed by Dr. Ted Burns.

ALS Onset is Influenced by the Burden of Rare Variants in Known ALS Genes by Drs. Baloh and Harms

    3/27/2015
ALS Onset is Influenced by the Burden of Rare Variants in Known ALS Genes by Drs. Robert Baloh and Matthew Harms, interviewed by Dr. Ted Burns. Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes.Ann Neurol. 2015 Jan;77(1):100-13. doi: 10.1002/ana.24306. Epub 2014 Nov 27.
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