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Neuromuscular transmission defects in myopathies: Rare but worth searching for
Author(s) -
Elahi Behzad,
Laughlin Ruple S.,
Litchy William J.,
Milone Margherita,
Liewluck Teerin
Publication year - 2019
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.26393
Subject(s) - myopathy , medicine , congenital myopathy , neuromuscular transmission , pyridostigmine , repetitive nerve stimulation , hydroxychloroquine , weakness , inflammatory myopathy , myasthenia gravis , surgery , muscle biopsy , biopsy , disease , covid-19 , infectious disease (medical specialty)
: Decremental responses in repetitive nerve stimulation have been reported in a few hereditary myopathies. We examined the frequency of decrement in a cohort of myopathy patients. Methods : We reviewed all patients referred for myopathy who underwent repetitive nerve stimulation between January 2007 and May 2017. We included patients with decrement (>10%) and either a pathological or molecular diagnosis of myopathy. Results : Among 157 patients with myopathies, 4 patients had decrement (2 hydroxychloroquine‐associated vacuolar myopathy, 1 centronuclear myopathy, and 1 distal myopathy). One hydroxychloroquine‐associated vacuolar myopathy patient also had inflammatory myopathy. Pyridostigmine improved weakness in the centronuclear myopathy patient, but not in the distal myopathy patient. No patient with an acquired myopathy received pyridostigmine. Conclusions : Despite the rare occurrence of decrement in myopathy, its presence may urge consideration of pharmacological intervention. Muscle Nerve 59:475–478, 2019