Differential diagnosis of benign pseudohypertrophic myotonic dystrophy

       Baker-type progressive myotonic dystrophy progresses slowly (the disease can last more than 25 years, and often can still walk after 20 years of age); it is not accompanied by myocardial involvement or only mildly involved, and has a good prognosis, also known as benign type. The disease is a genetic disorder, multi-linked recessive inheritance, individual for chromosomal recessive inheritance.  Baker type progressive myotonic dystrophy often starts after 10 years of age, the first symptom is weakness of the pelvic girdle and femoral muscles, the progression is slow, the course of the disease is long, 25 years or more after the onset of symptoms before being unable to walk, most of them do not become paralyzed at the age of 30-40 years, the prognosis is better. Duchenne type dystrophy: also known as severe pseudohypertrophy 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 skimming, walking slowly swaying, a special When standing, the spinal cord is protruding, the abdomen is out, the feet are splayed out, and the walk is slow and swaying, with a special “duck stance” gait, and it is very difficult to stand up from a supine position. It can also be seen in the proximal limb muscles, quadriceps and brachialis.