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Transthyretin Amyloid Neuropathy has Earlier Neural Involvement but Better Prognosis than Primary
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
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
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.
Mutations in SEPT9 Cause Heredic Neuralgic Amyotrophy by Phillip Chance, MD and Mark Hannibal, MD
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.
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