Respiratory muscle weakness caused by neuromuscular disease can lead to both acute and chronic respiratory failure. Respiratory failure caused by Guillain-Barré syndrome and myasthenia gravis can potentially improve with disease-specific therapy. However, respiratory failure in amyotrophic lateral sclerosis is irreversible, and it may be necessary to provide full-time ventilation support along with additional assistance. Noninvasive ventilation is recommended for managing acute or acute-on-chronic hypercapnic respiratory failure caused by neuromuscular disease. It has also been effective in weaning patients off invasive mechanical ventilation. Although noninvasive ventilation offers numerous benefits over invasive mechanical ventilation, it is crucial to consider the specific contraindications and limitations of noninvasive ventilation and ensure its appropriate usage based on the patient's condition and needs. The timely recognition of neuromuscular respiratory failure is critical, as early intervention can be life-saving. This review focused on the clinical assessment and management of acute respiratory failure in neuromuscular diseases.

Clinical evaluations, nerve conduction studies, and electromyography play major complementary roles in electrophysiologic diagnoses. Electromyography can be used to assess pathologic changes and localize lesions occurring in locations ranging from motor units to anterior-horn cells. Successfully performing electromyography requires knowledge of the anatomy, physiology, and pathology of the peripheral nervous system as well as sufficient skill and interpretation ability. Electromyography techniques include acquiring data from visual/auditory signals and performing needle positioning, semiquantitation, and interpretation. Here we introduce the basic concepts of electromyography to guide clinicians in performing electromyography appropriately.

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Nerve conduction study (NCS) is an electrophysiological tool to assess the overall function of cranial and peripheral nervous system, therefore NCS has been diagnostically helpful in the identification and characterization of disorders involving nerve roots, peripheral nerves, muscle and neuromuscular junction, and are frequently accompanied by a needle Electromyography. Furthermore, NCS could provide valuable quantitative and qualitative results into neuromuscular function. Usually, motor, sensory, or mixed nerve studies can be performed with using NCS, stimulating the nerves with the recording electrodes placed over a distal muscle, a cutaneous sensory nerve, or the entire mixed nerve, respectively. And these findings of motor, sensory, and mixed nerve studies often show different and distinct patterns of specific abnormalities indicating the neuromuscular disorders. The purpose of this special article is to review the neurophysiologic usefulness of NCS, to outline the technical factors associated with the performance of NCS, and to demonstrate characteristic NCS changes in the setting of various neuromuscular conditions.

Citations

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Background
Floppy infant syndrome has a number of different etiologies. Methods: One hundred twenty-three consecutive patients of floppy infant syndrome were included in this study. We reviewed all the electrophysiologic tests of these patients and the medical record of patients showing abnormalities in the electrophysiologic studies.
Results
Of the 123 patients, twenty-six (21.1%) showed definite abnormalities in electrophysiologic tests; 8 myopathies, 14 neuropathies and 4 unclassified. The neuropathy was further classified as 5 neuronopathies and 9 sensorimotor polyneuropathies. With muscle or sural nerve biopsy and genetic test, a final diagnosis was made of Duchenne muscular dystrophy in 4, Becker muscular dystrophy in 1, spinal muscular atrophy in 2, and metachromatic leukodystrophy in 1.
Conclusions
About 21% of patients presented with floppy infant syndrome showed abnormalities in the neuromuscular system. The electrophysiologic test is valuable to guide further investigations in diagnosing the cause of floppy infant syndrome.

In the normal neuromuscular junction, the nerve action potential(NAP) opens the P-type calcium channels, and resultant influx of calciumions produces the simultaneous exocytosis of

Electrodiagnostic studies are valuable in confirming the diagnosis of a disorder of neuromuscular transmission. They are used to distinguish presynaptic and postsynaptic abnormalities. These studies provide an objective measure of the severity of the illness and may be useful in assessing the response to therapy. This article reviews the electrodiagnostic techniques that are commonly used today and highlights their specificity, sensitivity, and pitfalls. Repetitive nerve stimulation test (RNST) and single-fiber electromyography (SFEMG) are the most available electrophysiologic test in the diagnosis of neuromuscular junction disorders. RNS showing 10% decrement in amplitude from the first to fourth or fifth intravolley waveform while stimulating at 2~5 Hz is valid for the diagnosis of MG. The degree of increment needed for the diagnosis of LEMS is at least 25% but most accurate when greater than 100%. Abnormal jitter or impulse blocking are the appropriate criteria for diagnosis of NMJ disorders when using SFEMG. SFEMG is more sensitive than RNS for the diagnosis of disorders of neuromuscular transmission, especially in MG but may be less specific or may not be available.