Welcome to the WCN 2023 Interactive Program

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IDIOPATHIC INFLAMMATORY MYOPATHIES: WHICH DIAGNOSTIC TOOLS SHOULD WE USE?

The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of diseases, sharing symptoms of progressive muscle weakness and muscle inflammation. Other organs are frequently involved, such as the skin, lungs and heart. Based on clinical features, muscle pathology, and serological findings IIM are classified in four subsets: Dermatomyositis (DM), Immune-mediated necrotizing myopathy (IMNM), Anti-synthetase syndrome (ASyS), and Inclusion-Body-Myositis (IBM). Polymyositis (PM) is a disputed entity and there are two other groups of IIM, i.e., overlap myositis (myositis associated with connective tissue disorders) and a heterogeneous group, called non-specific myositis which are as yet not clearly delineated.

Serum creatine kinase activity is always measured, but may be normal in active disease, e.g. DM and IBM. Whether an EMG is useful has as yet not been established. A cornerstone in the diagnostic process is the muscle biopsy to identify inflammation and to exclude myopathies which may also be associated with inflammation. There is an ongoing debate whether a muscle biopsy may be replaced by assessment of MSAs, in particular in DM. Muscle MRI or muscle ultrasound is often used in the initial work-up, to aid in the diagnosis of IIM, to distinguish subtypes, and to target muscle biopsy to maximize yield. The use of machine learning algorithms trained on transcriptomics data to classify muscle biopsies from patients with different mysositis subtypes will be discussed. The diagnostic procedures should also include a search for extramuscular manifestations and screening for malignancy in DM and IMNM.

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Telemedicine use for neuromuscular clinics had a niche existence for years in servicing geographically remote areas, although conventional clinics in neuromuscular centres predominated, even for patients with severely reduced mobility. The COVID-19 pandemic demonstrated how fragile the system of multidisciplinary clinics had become, when physical attendance became unfeasible, and some patients even reported significant deterioration of their health status. For example, outreach monitoring visits for complex treatments such home ventilation were cancelled, and in many cases remote assessment and monitoring were rudimentary. This is surprising, because remote institution of treatments and monitoring of vital functions has been possible for some time. The need for in-person visits can be minimised by structured video interviews and physical assessments, and the patients may gain in their own control of their condition. In many cases, this would mean that access to the neuromuscular centre could be simplified and improved for many patients even after the end of the pandemic. There are significant health economic benefits with minor change to the economic model required. This talk discusses the options for Telemedicine applications in neuromuscular medicine, both with respect to the recent pandemic, and beyond with scope for improving patient care.

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In addition to reviewing the latest trends in Myasthenia Gravis (MG) management, I will use a few illustrative scenarios to discuss therapeutic approaches to problems which may be encountered in a busy MG clinic. These clinical problems will include cases with treatment refractory fatigue and weakness and the current thinking around MG-associated ophthalmoplegia and its management. Comorbidities such as those who are also living with HIV infection, or who may come from high Tuberculosis prevalent areas, may impact the management of MG. My focus will be on practical approaches and with cognisance of the cost implications in choosing therapies.