Severe pseudohypertrophic dystrophy, almost exclusively seen in boys, if the mother is a gene carrier, 50% of male offspring, often starting at the age of 2-8 years, the initial sense of walking clumsy, easy to fall, can not run and climb buildings, standing spinal cord convexity, abdomen out, both feet aside, walking slowly swaying, a special “duck gait” gait When standing, it is very difficult to walk from supine, and must first turn over prone, then climb the two knees with both hands and gradually support upward (Gower’s sign). It can also be seen in the proximal limb muscles, quadriceps and brachialis. How to check for severe pseudohypertrophic dystrophy? The main manifestations of the disease include: 1. Progressive muscle weakness with motor regression. The child’s motor development is basically normal at birth or in early infancy, and a few have mild motor development delay, or unstable gait after independent walking and easy to fall. Most of them lose the ability to walk independently after the age of 10, and most of them develop weakness of throat muscles and respiratory muscles before the age of 20, with low voice, difficulty in swallowing and breathing, and easily die from secondary infections such as aspiration pneumonia. Milder, may survive until about 40 years of age. 2. Gower’s sign. Due to the early weakness of the hip belt muscles, children generally cannot stand up directly from the supine position after the age of 3 years, but must first turn over into a prone position, then spread the feet apart, support both hands on the ground first, then one hand to support the ipsilateral calf, and alternately shift with the other hand to support the knee and thigh, so that the drive from a deep bowing position gradually upright, and finally into a standing position with waist convexity. 3, pseudomuscular hypertrophy and extensive muscle atrophy. Early that there is pelvic and thigh progressive muscle atrophy, but gastrocnemius due to fat and collagen soccer hyperplasia and pseudohypertrophy, and other parts of the muscle critical sitting contrast, when the shoulder girdle muscle atrophy, raise the arm when the inner side of the scapula away from the chest wall, forming a “winged scapula”. When the child’s torso is lifted from the axilla, the child’s arms go up and have a tendency to slip out of the examination hands. Spinal muscle atrophy can lead to spinal bending deformity, and muscle atrophy occurs later in the disease, causing knee, hip or upper arm flexion deformity. 4. Most children have cardiomyopathy and even heart failure, but its severity is not consistent with skeletal muscle weakness. Almost all children have varying degrees of intellectual impairment, which also does not parallel the severity of skeletal muscle weakness, of which 2-30% are more pronounced, with IQ <70. Clinical manifestations are divided into the following types: 1. Pseudohypertrophy. Duchenne-type dystrophy (DMD): almost exclusively seen in boys, the initial sense of walking clumsy, easy to fall, can not run and climb the building, standing when the spinal cord convex, the abdomen out, both feet skewed, a special "duck gait" gait, when the supine walk to stand very difficult, must first turn over prone, and then hands climbing the two knees When the body is on its back, it is very difficult to stand up. Becker type (BMD): The first symptom is weakness of the pelvic girdle and femoral muscles, with slow progression and long duration. 2, Facioscapulohumeral muscular dystrophy. 3.Limb band type myotonic dystrophy. 4.Other types are quadriceps type, distal type, progressive extraocular muscle paralysis type, oculo-pharyngeal muscle type, etc., which are extremely rare.