Ultrasound (US) is a patient-friendly imaging modality that is well suited for structural assessments of skeletal muscle and surrounding tissues, and its use for both diagnostic and monitoring in neuromuscular medicine is steadily expanding. Despite its advantages, the broader clinical implementation of muscle US is still hinder by the obtained images and their interpretation varying between operators. Recent advances in artificial intelligence (AI)-particularly in deep learning (DL)-have led to significant innovations in medical imaging. These developments hold considerable promise for addressing the inherent limitations of muscle US and for improving its clinical applicability. This review summarizes key muscle US biomarkers, encompassing both qualitative visual assessments and quantitative parameters. We discuss their utility in the diagnosis of neuromuscular disorders and their potential role as responsive biomarkers for monitoring disease progression, based on findings from previous studies. We also introduce foundational concepts of AI and DL and review recent studies that have applied these technologies in muscle US for the automated segmentation of muscle boundaries, feature extraction, and disease classification. Finally, we highlight current challenges and outline future directions needed to fully realize the potential of AI-enhanced muscle US as a standardized and widely applicable tool for assessing neuromuscular diseases.

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.

Citations

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  S. de Jonge, W.V. Potters, C. Verhamme
  Clinical Neurophysiology.2024; 159: 41.     CrossRef
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  Xiong Zeng, Ying Dong, Xiaohao Wang
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