Muscular Dystrophy - Duchenne Muscular Dystrophy

Duchenne Muscular Dystrophy

It is the most common and most serious form of muscular dystrophy. The condition is named after the French neurologist Guillaume Benjamin Amand Duchenne, who first described the disease in 1868. This form of muscular dystrophy occurs only in males. Approximately 1 in 3,500 to 4000 males develop this condition. The disease is prevalent in approximately 63 people per million, worldwide.

Cause

It occurs due to a defect in the gene that is responsible for production of dystrophin, a protein that helps in maintaining the strength and integrity of muscles. It is disease linked to the X chromosome, in which defective genes are passed from mothers who carry the defective gene. Therefore, father to son-transmission of the disease is not seen. Although mothers who transmit the gene are mostly unaffected, their father, brothers or uncles may be affected.