1/2/2023 0 Comments Bontempi pm 64 manualsDefinition and classification of axial myopathyWe included myopathies with sole or significant affection of the paraspinal musculature ( ), with or without more widespread muscle involvement. This update attempts to raise awareness about myopathies with axial weakness, and will describe conditions with paraspinal involvement, and methods for assessment. There is a need to focus on axial myopathies to characterize disease expression, consequences and develop methods for clinical assessment and treatment strategies. Conversely, dropped head or camptocormia may have many different aetiologies, complicating the diagnosis ( ). Paraspinal affection may be the first complaint in myopathies known for other typical manifestations such as facioscapulohumeral muscular dystrophy (FSHD1) or inclusion body myositis (sIBM). New disease entities with selective axial muscle involvement have also been described ( ), but overall the presence of axial myopathy among muscle diseases is unexplored. , IntroductionClassically, myopathies are categorized according to limb or cranial nerve muscle affection, but with the growing use of MRI it has become evident that many well-known myopathies have significant involvement of the axial musculature ( ). This article provides an overview of the present knowledge on axial myopathy with the aim to increase awareness and spur interest among clinicians and researchers in the field. Very sparse literature evaluating the validity of clinical assessment methods, electromyography, muscle biopsy and magnetic resonance imaging was identified and reference material is generally missing. Even in diseases named after a certain pattern of non-axial muscle affection, such as facioscapulohumeral and limb girdle muscular dystrophies, affection of the axial musculature was often severe and early, compared to other muscle groups.
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