abstract objective autosomal recessive spinal muscular atrophy (sma) is, after cystic fibrosis, the second most common fatal monogenic disorder and the second most common hereditary neuromuscular disease after duchenne dystrophy. the disease is characterized by degeneration of anterior horn cells leading to progressive paralysis with muscular atrophy. depending on the clinical type (werdnig- ho...

how to cite this article: mohammadi m. clinical neurophysiology in pediatric neurometabolic disorders. iran j child neurol autumn 2014;8:4 (suppl.1):7. pls see pdf.

Surface Electromyography (EMG) is a standard method used in clinical practice and research to assess motor function in order to help with the diagnosis of neuromuscular pathology in human and animal models. EMG recorded from trunk muscles involved in the activity of breathing can be used as a direct measure of respiratory motor function in patients with spinal cord injury (SCI) or other disorde...

Researchers and clinicians routinely rely on interference electromyograms (EMGs) to estimate muscle forces and command signals in the neuromuscular system (e.g., amplitude, timing, and frequency content). The amplitude cancellation intrinsic to interference EMG, however, raises important questions about how to optimize these estimates. For example, what should the length of the epoch (time wind...

The effectiveness of EMG biofeedback training for tension headache has been well established. Previous studies evaluating changes in an average EMG activity score from pre- to posttreatment have not consistently found a relationship between a reduction in average EMG activity and headache improvement at posttreatment. The current study is a preliminary analysis of the utility of EMG variance as...

A surface EMG signal represents the linear transformation of motor neuron discharge times by the compound action potentials of the innervated muscle fibers and is often used as a source of information about neural activation of muscle. However, retrieving the embedded neural code from a surface EMG signal is extremely challenging. Most studies use indirect approaches in which selected features ...

Electromyography is a commonly used method to determine relative effort and neuromuscular drive to skeletal muscle. A limitation of the interpretation of EMG within the literature is the many methods used to determine the intensity of muscle activation. In the current study, ten healthy young adults performed a level walking task while EMG was recorded from the tibialis anterior, medial gastroc...

OBJECTIVE Cervical vestibular evoked myogenic potentials (cVEMPs) are short-latency electromyogram (EMG) evoked by high-level acoustic stimuli recorded from the activated sternocleidomastoid muscle and used to evaluate otolith organ function. The purpose of this study was to investigate the effects of aging on the cVEMP and on the sternocleidomastoid muscle EMG level. DESIGN A cross-sectional...