Update in electromyography

Abstract

It is easy to forget the contribution of electromyography (EMG) to the investigation of paediatric peripheral neuromuscular disease, and this review highlights its continued importance. The discovery that Brown-Vialetto-van Leare disease, when associated with disorder of riboflavin metabolism, may be treatable has raised awareness of the importance of EMG for its early detection. Unexpected discovery of motor neuronopathy, which may be useful for the definition of the phenotype of several conditions, now has an added significance. The investigation of disorders of peripheral nerve cannot proceed without nerve conduction studies but particular interest has been shown in its role in the management of obstetric brachial plexus palsy, with investigation within 1 month now recommended. The key role of neurophysiology in identifying abnormalities of the neuromuscular junction, and therefore leading investigators to a diagnosis of myasthenia, is once again highlighted. EMG in muscle disease continues to have a role, particularly when identifying myotonia. Paediatric EMG, while a daunting technical challenge to some practitioners, remains a valuable investigative tool for the specialists in paediatric neuromuscular disorders and will continue to deliver important diagnostic information, often as quickly and accurately as other more recent innovations.