Neuromuscular disorders : NMD
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Myasthenia gravis is characterized by muscle weakness and fatigue. As sustained muscle use increases the weakness, the value of physical training programs has previously been questioned. This is a review to clarify the safety and usefulness of systematic training in myasthenia gravis, based on a systematic search in available databases using the relevant key words. ⋯ This can improve both muscle strength and daily function. Type and intensity of systematic training should be adapted in the individual patient. A minimum of 150 min of exercise per week is recommended for myasthenia gravis patients with mild and moderate disease.
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Neuromuscul. Disord. · Jul 2020
Review Case ReportsAChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review.
Muscle-specific tyrosine kinase antibody (MuSK-Ab) and acetylcholine receptor antibody (AChR-Ab) coexistence in myasthenia gravis (MG) is very rare. In this report, two children with AChR-Ab switching to double antibody positive MG (DP-MG) or MuSK-Ab positive MG (MuSK-MG) are described. Six similar cases were found in the literature via online database search. ⋯ The average age of onset was 7.25 ± 5.95 years. Four AChR-MG patients switched to DP-MG with no known precipitating factor and four switched after thymectomy (two to MuSK-MG and two to DP-MG). After the serological switch, the patients transitioned to the phenotype of MuSK-MG and responded poorly to cholinesterase inhibitors and well to corticosteroids and plasma exchange.
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Motor neuron diseases as amyotrophic lateral sclerosis and post-polio syndrome are characterized by prominent muscular weakness and severe motor disability. The prevalence of fatigue in motor neuron diseases is remarkably high, and may contribute significantly to patients' disability and a poor quality of life. Despite its high prevalence, fatigue is an understudied clinical problem in motor neuron diseases and is often overlooked. The present review will attempt to explain the pathogenic mechanisms of muscle fatigue in amyotrophic lateral sclerosis and post-polio syndrome, to cover the various methods of quantification of fatigue that measure both subjective and physiological components, and to summarize the evidence regarding therapeutic interventions that can be used in order to minimize this symptom.
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Animal models in biomedical research are important for understanding the pathological mechanisms of human diseases at a molecular and cellular level. Several aspects of mammalian animals, however, may limit their use in modelling neuromuscular disorders. ⋯ This review focuses on a number of key studies using the zebrafish to model hereditary muscle diseases with additional emphasis on recent advances in zebrafish functional genomics and drug discovery. Increasing research in zebrafish disease models, combined with knowledge from mammalian models, will bring novel insights into the disease pathogenesis of neuromuscular disorders, as well as facilitate the development of effective therapeutic strategies.
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The purpose of this review is to alert non-anaesthesiologists to the various complications from which patients with neuromuscular disorders and those susceptible to malignant hyperthermia can suffer during anaesthesia. The patient's outcome correlates with the quality of consultation between anaesthesiologists, surgeons, neurologists and cardiologists. ⋯ This review provides a synopsis of pre-operative anaesthetic considerations and adverse drug effects on skeletal, cardiac and smooth muscle tissue. It describes the pathogenetic aspects of typical complications and introduces anaesthetic procedures for the various neuromuscular disorders, including regional anaesthesia for patients in whom a restriction of respiratory and/or cardiac function is predicted.