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Unformatted text preview: squatting position due to proximal myopathy) pseudohypertrophy of calves Muscle wasting (those muscles affected) Later in the progression of DMD abnormal bone development may lead to skeletal deformities, including scoliosis. Progressive proximal muscle weakness of the legs and pelvis associated with a loss of muscle mass is observed first. Eventually this weakness spreads to the arms, neck, and other areas. Often, patients are wheelchair-bound by 12 and the condition is frequently fatal by the age of 20. Investigations Diagnosis is often made on clinical grounds alone. Creatinine phosphokinase levels in the blood are elevated to levels 100-200 times normal. EMG shows weakness caused by muscular tissue degeneration rather than neural damage. Muscle biopsy shows: characteristic variation in muscle fibre size fibre necrosis fat replacement absence of dystrophin on immunochemical staining...
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- Fall '10