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Neurophysiological testing in congenital myasthenic syndromes: A systematic review of published normal data
Author(s) -
Kosac Ana,
Gavillet Erika,
Whittaker Roger G.
Publication year - 2013
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.23929
Subject(s) - medicine , repetitive nerve stimulation , electromyography , neuromuscular transmission , medline , neurophysiology , neuromuscular disease , physical medicine and rehabilitation , pediatrics , disease , psychiatry , political science , law
: Congenital myasthenic syndromes are a heterogeneous group of inherited neuromuscular disorders. Neurophysiological testing has a major role in screening suspected cases and guiding genetic testing. We performed a systematic literature review to ascertain whether sufficient age‐specific normative data exist for these techniques. Methods : We searched Ovid Medline (1948 to October 2012) for citations on repetitive nerve stimulation (RNS) or single fiber electromyography (SFEMG) in children. Results : We identified 5 articles containing data on 48 normal children studied with RNS and 1 article containing data on SFEMG in 20 normal children. A variety of techniques were used, including a variety of stimulation frequencies. Nevertheless, the data identify significant differences in the response to RNS in children as compared with adults, emphasizing the need for adequate age‐appropriate normal values. Conclusions : We found a paucity of published normal data for pediatric neuromuscular transmission studies. Further studies using standardized techniques are needed to expand the currently limited pool of available data. Muscle Nerve 48:711–715, 2013