Next Article in Journal
Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis
Next Article in Special Issue
Animal Models of the Neuromuscular Junction, Vitally Informative for Understanding Function and the Molecular Mechanisms of Congenital Myasthenic Syndromes
Previous Article in Journal
Articular Cartilage Aging-Potential Regenerative Capacities of Cell Manipulation and Stem Cell Therapy
Previous Article in Special Issue
Fundamental Molecules and Mechanisms for Forming and Maintaining Neuromuscular Synapses
Open AccessReview

Neurophysiological Assessment of Abnormalities of the Neuromuscular Junction in Children

Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children National Health Service (NHS) Foundation Trust, London WC1N 3JH, UK
Int. J. Mol. Sci. 2018, 19(2), 624; https://doi.org/10.3390/ijms19020624
Received: 24 October 2017 / Revised: 16 January 2018 / Accepted: 17 January 2018 / Published: 22 February 2018
(This article belongs to the Special Issue The Neuromuscular Synapse in Health and Disease)
The function of the neuromuscular junction in children is amenable to electrophysiological testing. Of the two tests available, repetitive nerve stimulation is uncomfortable and has a reduced sensitivity compared with single-fibre methodology. The latter is the method of choice, recording the variability in neuromuscular transmission as a value called jitter. It can be performed by voluntary activation of the muscle being examined, which is not suitable in children, or by stimulation techniques. A modification of these techniques, called Stimulated Potential Analysis with Concentric needle Electrodes (SPACE), is well tolerated and can be performed while the child is awake. It has a high sensitivity (84%) for the diagnosis of neuromuscular transmission disorders, the majority of which are myasthenic syndromes, and a moderate specificity (70%). The latter can be improved by the exclusion of neurogenic causes and the determination of the degree of jitter abnormality. Minor jitter abnormalities, under 115% of the upper limit of normal, are usually caused by myopathies with an associated neuromuscular transmission disorder, whereas levels higher than this value are usually associated with one of the myasthenic conditions. View Full-Text
Keywords: repetitive nerve stimulation; single fibre electromyography; Myasthenia; Paediatrics; neuromuscular junction repetitive nerve stimulation; single fibre electromyography; Myasthenia; Paediatrics; neuromuscular junction
Show Figures

Figure 1

MDPI and ACS Style

Pitt, M. Neurophysiological Assessment of Abnormalities of the Neuromuscular Junction in Children. Int. J. Mol. Sci. 2018, 19, 624.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Search more from Scilit
 
Search
Back to TopTop