Severe pseudo-hypertrophic dystrophy in the early stage of walking clumsily, easy to fall, can not run and climb the stairs, standing spinal cord anteriorly convex, the abdomen out, the two feet aside, walking slowly swaying, a special “duck step” gait, when from the supine to stand up is very difficult, must be rolled over to the prone position, and then both hands to climb the edge of the knees, and gradually upward to support the rise. It can also be seen in the proximal limb muscles, quadriceps and brachialis muscles. How is severe pseudohypertrophic dystrophy diagnosed? Boys are affected, but individual girls may develop the disease due to inactivation of the other X chromosome in addition to carrying the mutated gene. The main manifestations of the disease include: 1. Progressive muscle weakness and regression of motor function. 2. The affected children’s motor development is basically normal at birth or early infancy, and a few of them have mild delayed motor development, or unsteady gait after independent walking and easy to fall. Symptoms generally begin to be obvious after the age of 5 years, hip girdle muscle weakness is becoming increasingly serious, walking wobble like a duck gait, falls more frequently, can not go up the stairs and jumping, shoulder girdle and the whole body spine force followed by progressive decline, most of the loss of the ability to walk independently at the age of 10 years old, most of them appear before the age of 20 years of pharyngeal muscles and respiratory muscle weakness, low voice, swallowing and respiratory difficulties, easy to die of secondary infections, such as aspiration pneumonia, BMD symptoms are milder, may survive until 40 years of age, and may be able to survive until 40 years of age. BMD symptoms are milder, may survive until about 40 years old. 2, Gower’s sign, due to the early weakness of the hip belt muscles, generally after the age of 3 years, the child can not stand up directly from the supine position, must be rolled over into the prone position, and then two feet apart, both hands first supported on the ground, then one hand support to the same side of the calf, and with the other hand alternately shifted to support on the knees and thighs, so as to make the repulsion from the depth of the bowing position gradually vertical, and finally into the standing position of the lumbar convexity. 3.Pseudo-muscular hypertrophy and extensive muscle atrophy. Early that is the pelvis and thigh progressive muscle atrophy, but the gastrocnemius due to fat and collagen soccer hyperplasia and pseudohypertrophy, and other parts of the muscle sit in sharp contrast, when the shoulder girdle muscle atrophy, raise the arm scapula away from the chest wall, the formation of “winged scapula”. When the child’s torso is lifted from the axilla, both arms are turned upward, with a tendency to slip out of the examiner’s hands. Spinal muscular atrophy can lead to spinal curvature deformity, muscle atrophy occurs in the late stage of the disease, causing knee, hip or upper arm flexion deformity. 4, most of the children have cardiomyopathy, and even heart failure occurs, but its severity is not consistent with skeletal muscle weakness. Almost all children have different degrees of intellectual impairment, which is also not parallel to the severity of muscle weakness, of which 2%-30% are more pronounced, with an IQ <70.