Abstract
Muscle atrophy and weakness of upper and lower extremity muscles are well characterized in neuromuscular diseases, but involvement of the axial muscles is poorly understood. Since the axial musculature stabilizes and moves the trunk and provides stability for movement of the extremities, knowledge about the axial musculature in patients with neuromuscular diseases is important. Studies have shown that the axial muscles are affected in a number of myopathies, such as RYR1-myopathy and facioscapulohumeral muscular dystrophy. However, axial myopathy has not been investigated in patients with limb-girdle and Becker muscular dystrophy, and Charcot-Marie-Tooth disease. The purpose of the study is; (1) to investigate the involvement of axial muscles in patients with neuromuscular diseases, (2) to evaluate psychometric properties of stationary dynamometry in patients with axial myopathy, and (3) to investigate the relationship between axial weakness and rigidity, posture, balance and pain. The design is an exploratory cross-sectional study. Fifty adult patients with limb-girdle and Becker muscular dystrophies and Charcot-Marie-Tooth disease and 50 healthy controls, matched for age, sex and body mass index, will be recruited. Patients will be examined in relation to range of motion, manual muscle test, hand-held dynamometry, isometric stationary dynamometry (Biodex System 4 Pro), magnetic resonance imaging, photographic posture analysis, Clinical Test of Sensory Integration and Balance (Biosway), Trunk Control Test, Fatigue Severity Scale, Low Back Pain Rating scale, and The Danish SF-36 Health Survey. This study will increase the understanding of axial muscle involvement in patients with neuromuscular diseases and contribute to diagnostic specificity. The evidence-basing of stationary dynamometry to measure trunk muscle strength in patients with axial myopathy is needed for clinical practice and clinical trial preparedness. Preliminary results will be presented at the poster.
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