The diagnostic and referral workflow for children with neuromuscular disorders is evolving, particularly as newborn screening programs are expanding in tandem with novel therapeutic developments. However, for the children who present with symptoms and signs of potential neuromuscular disorders, anatomic localization, guided initially by careful history and physical examination, continues to be the cardinal initial step in the diagnostic evaluation. It is important to consider whether the localization is more likely to be in the lower motor neuron, peripheral nerve, neuromuscular junction, or muscle. After that, disease etiologies can be divided broadly into inherited versus acquired categories. Considerations of localization and etiologies will help generate a differential diagnosis, which in turn will guide diagnostic testing. Once a diagnosis is made, it is important to be aware of current treatment options, as a number of new therapies for some of these disorders have been approved in recent years. Families are also increasingly interested in clinical research, which may include natural history studies and interventional clinical trials. Such research has proliferated for rare neuromuscular diseases, leading to exciting advances in diagnostic and therapeutic technologies, promising dramatic changes in the landscape of these disorders in the years to come.
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http://dx.doi.org/10.1016/j.pediatrneurol.2023.08.034 | DOI Listing |
ARP Rheumatol
January 2024
Department of Neuromuscular Diseases & Centre for Rheumatology, University College London, London, UK.
Am J Hum Genet
January 2025
Institute of Medical Genetics and Applied Genomics, University of Tübingen, 72076 Tübingen, Germany; Center for Rare Disease, University of Tübingen, 72076 Tübingen, Germany; Genomics for Health in Africa (GHA), Africa-Europe Cluster of Research Excellence (CoRE).
Inborn errors of selenoprotein expression arise from deleterious variants in genes encoding selenoproteins or selenoprotein biosynthetic factors, some of which are associated with neurodegenerative disorders. This study shows that bi-allelic selenocysteine tRNA-specific eukaryotic elongation factor (EEFSEC) variants cause selenoprotein deficiency, leading to progressive neurodegeneration. EEFSEC deficiency, an autosomal recessive disorder, manifests with global developmental delay, progressive spasticity, ataxia, and seizures.
View Article and Find Full Text PDFArch Orthop Trauma Surg
January 2025
Orthopedics Research Center, Mashhad University of Medical Science, Mashhad, Iran.
Introduction: There exist conflicting electrodiagnostic reports between diagnosing mild carpal tunnel syndrome (CTS) and normal results, depending on the interpretation methods used by electrodiagnosticians. This underscores the necessity for precise clinical guidelines. This study aims to assess how the variation between mild and normal electrophysiological reports impacts (1) subsequent clinical outcomes in patients diagnosed with CTS and (2) physicians' decision-making.
View Article and Find Full Text PDFRheumatol Int
January 2025
Department of Pediatric Rheumatology, Istanbul Medeniyet University, Istanbul, Turkey.
Chronic non-bacterial osteomyelitis (CNO) is an inflammatory bone disease, usually diagnosed in childhood. It is characterized by the presence of multifocal or unifocal osteolytic lesions that can cause bone pain and soft tissue swelling. CNO is known to have soft tissue involvement.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
January 2025
Neurology Department, LR18SP03, Razi University Hospital, Tunis, Tunisia.
Amyotrophic Lateral Sclerosis(ALS) has traditionally been managed as a neuromuscular disorder. However, recent evidence suggests involvement of non-motor domains. This study aims to evaluate the impact of APOE and MAPT genotypes on the cognitive features of ALS.
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