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Journal of Clinical Neuromuscular Disease 2014 September
In this issue, we review recent reports of anti-agrin antibodies as a cause of myasthenia gravis (MG) and of the clinical and electrophysiologic responses to acetylcholinesterase inhibitors in muscle specific kinase (MuSK) MG. We highlight recommendations from a working group in the United Kingdom regarding pregnancy and MG and review a report of the use of maintenance plasma exchange and intravenous immunoglobulin in juvenile MG. Regarding articles on peripheral neuropathy, there is a recent report of inflammatory neuropathy after hip surgery and a review of transthyretin amyloidosis. Two reports of hereditary neuropathy with liability to pressure palsies include atypical and typical presentations, early onset, and electrodiagnostic and pathologic feature. Quality measures for distal sensory polyneuropathy, including more quantitative assessment of pain and potential fall risk, are outlined. Myopathy is discussed as a complication of radiation therapy, and reports on outcomes of critical illness myopathy with or without neuropathy, autoimmune inflammatory myopathies, and inclusion body myositis are reviewed. Brugada syndrome, as a possible fatal consequence of spinal bulbar muscular atrophy, is discussed along with clinically relevant topics in amyotrophic lateral sclerosis, namely bladder dysfunction and venous thromboembolism.
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