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By definition, all limb girdle muscular dystrophies (LGMD) cause progressive proximal weakness, [3] meaning weakness of the muscles on or close to the torso that worsens over time. Explicitly, LGMD preferentially affects muscles of the hip girdle, thigh, shoulder girdle, and/or upper arm. [8] [6] The muscle weakness is generally symmetric. [11]
Weakness is symmetric, progressive, and proximal (on or close to the torso), usually affecting the hip girdle and shoulder girdle muscles. [1] [3] Hip weakness can manifest as a waddling gate. [1] Shoulder weakness can manifest as winged scapulas. [1] Muscle contractures, especially of the Achilles tendon, and scoliosis can also occur. [1]
Muscle weakness can also be classified as either "proximal" or "distal" based on the location of the muscles that it affects. Proximal muscle weakness affects muscles closest to the body's midline, while distal muscle weakness affects muscles further out on the limbs. Proximal muscle weakness can be seen in Cushing's syndrome [18] and ...
Gowers's sign is a medical sign that indicates weakness of the proximal muscles, namely those of the lower limb. The sign describes a patient that has to use their hands and arms to "walk" up their own body from a squatting position due to lack of hip and thigh muscle strength. It is named after William Richard Gowers. [1] [2]
Characteristic of IBM is weakness of finger flexion, knee extension, and ankle dorsiflexion. [6] Other inflammatory myopathies cause a proximal muscle weakness pattern, such as weakness of hip flexion, abduction, and extension, as well as shoulder abduction. [6] IBM and other inflammatory myopathies both cause bicep/tricep weakness. [6]
Problems with gripping objects, tying shoe laces, and using utensils can all be brought on by upper limb involvement. Proximal limb weakness is a fundamental clinical characteristic that sets apart chronic inflammatory demyelinating polyneuropathy from the vast majority of distal polyneuropathies, which are far more common.