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Becker muscular dystrophy also progresses more slowly than DMD. Males who inherit the mutation get the disease because they have no second dystrophin gene to make up for the faulty one. The first symptoms are often mobility problems affecting the hip girdle. The heart problems, if untreated, can be quite serious, even life-threatening. Muscular dystrophy can be inheirited, or occur for the first time in an individual. X-linked recessive inherited disorder characterized by slowly progressive muscle weakness of the legs and pelvis, Centers for Disease Control and Prevention, "Becker muscular dystrophy: MedlinePlus Medical Encyclopedia", "Becker's Muscular Dystrophy information. A man with BMD can’t pass the flawed gene to his sons because he gives a son a Y chromosome, not an X. It is important that a team that includes all of these subspecialists provides Duchenne and/or Becker care. BMD can run in a family, even if only one person in the biological family has it. Eye problems in different types of muscular dystrophies The skeletal muscles controlling the movement of the eyes, also known as extra-ocular muscles, show a normal structure and function in Duchenne and Becker muscular dystrophies . The DMD gene gives the body instructions to make a protein called dystrophin. Other factors may also play a role, and each type may carry with it a different prognosis and treatment course. [12] Genetic counseling may be advisable when potential carriers or patients want to have children. Perhaps no male children were born with the disease, or, even if a boy in an earlier generation was affected, relatives may not have known what disease he had. This is caused by mutations in the dystrophin gene, which encodes the protein dystrophin. Terms of Use | State Fundraising Notices. It is a neuromuscular disorder that occurs in three to six of every 100,000 births, and in people of all races. Muscular dystrophy is caused by genetic mutations that interfere with the production of muscle proteins that are needed to build and maintain healthy muscles. Sons of a man with Becker muscular dystrophy do not develop the disorder, but daughters will be carriers (and some carriers can experience some symptoms of muscular dystrophy), the daughters' sons may develop the disorder. group of disorders that cause the body's muscles to become increasingly weak BMD is less severe than DMD. But he’ll certainly pass it to his daughters, because each daughter inherits her father’s only X chromosome. Treatment is aimed at control of symptoms to maximize the quality of life which can be measured by specific questionnaires. That means the gene that sometimes contains a mutation causing these diseases is on the X chromosome. It is named after German doctor Peter Emil Becker, who first described this variant of Duchenne muscular dystrophy (DMD) in the 1950s. Respiratory failure can occur from acute pneumonia due to aspiration, restrictive pulmonary function due to respiratory muscle weakness. 2021, Muscular Dystrophy Association Inc. All rights reserved. Becker muscular dystrophy is caused when the body does not produce enough dystrophin (a vital muscle protein) or when the dystrophin does not work properly. It is a type of dystrophinopathy. Each son born to a woman with a dystrophin mutation on one of her two X chromosomes has a 50% chance of inheriting the flawed gene and having BMD. Duchenne musc… [14][22], The quality of life for patients with Becker muscular dystrophy can be impacted by the symptoms of the disorder. Respiratory failure is another cause of death in most types of muscular dystrophy. Duchenne and Becker muscular dystrophy. [9][10][11], Becker muscular dystrophy occurs in approximately 1.5 to 6 in 100,000 male births, making it much less common than Duchenne muscular dystrophy. Girls get two X chromosomes, one from each parent. Dystrophin plays a role in keeping muscle cells intact; lack of dystrophin causes muscle cells to be fragile and easily damaged. They’ll then be carriers, and each of their sons will have a 50% chance of developing the disease, and so on. Becker Muscular Dystrophy Treatment. There is not a cure for Becker muscular dystrophy at present. Becker muscular dystrophy is a muscle-wasting condition, first described in 1956, which usually affects only males. Treatment. Alternatively, shortness of breath can be caused by wasting of the muscles of the diaphragm a muscle in the abdomen that moves air in and out of the lungs. [20] According to a review by Bushby, et al. Carrier females have a 50% chance of passing the DMD mutation in each pregnancy. A minority of females with the mutation are manifesting carriers, who usually have a mild form of the disorder. It is less common than DMD. Limb-girdle muscular dystrophy. Possible complications associated with muscular dystrophies (MD) are cardiac arrhythmias. Muscle weakness also occurs in the arms, neck, and other areas, but not as noticeably severe as in the lower half of the body. A study done in Sweden on patients with Duchenne muscular dystrophy revealed that 35% of patients died due to cardiac failure. Becker muscular dystrophy is an X-linked recessive inherited disorder characterized by slowly progressing muscle weakness of the legs and pelvis. There are more than 30different types and manifestations of muscular dystrophy. Since women have two X chromosomes, if one X chromosome has the non-working gene, the second X chromosome will have a working copy of the gene to compensate, because of this ability to compensate, women rarely develop symptoms. [5][3] This is caused by mutations in the dystrophin gene, which encodes the protein dystrophin. [17] The drug prednisone contributes to an increased production of the protein utrophin which closely resembles dystrophin, the protein that is defective in BMD. In 1986, MDA-supported researchers identified the gene that, when flawed, or mutated, causes both Becker and Duchenne muscular dystrophies (BMD and … People affected by Becker muscular dystrophy can still maintain active lifestyles. This disease is caused by a mutation in the gene that encodes a protein called dystrophin. Dr. Ayah Elmaghrabi answered. Some symptoms consistent with Becker muscular dystrophy are: Individuals with this disorder typically experience progressive muscle weakness of the leg and pelvis muscles, which is associated with a loss of muscle mass (wasting). Patient", "Becker muscular dystrophy | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program", "Skipping Multiple Exons to Treat DMD—Promises and Challenges", "Cardiovascular Complications Associated with Muscular Dystrophy", "Risk assessment and genetic counseling in families with Duchenne muscular dystrophy", "Duchenne/Becker Treatment and Care | Muscular Dystrophy | NCBDDD | CDC", "Dystrophinopathies Treatment & Management: Medical Care, Consultations, Activity", "Cardiac Involvement in Patients With Muscular Dystrophies", "Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy", "Facts | Muscular Dystrophy | NCBDDD | CDC", "A comprehensive database of Duchenne and Becker muscular dystrophy patients (0–18 years old) in East China", X-linked severe combined immunodeficiency, Glucose-6-phosphate dehydrogenase deficiency, Danon disease/glycogen storage disease Type IIb, Alpha-thalassemia mental retardation syndrome, Siderius X-linked mental retardation syndrome, Color blindness (red and green, but not blue), https://en.wikipedia.org/w/index.php?title=Becker_muscular_dystrophy&oldid=990510238, Articles with unsourced statements from April 2016, Wikipedia articles incorporating text from the Centers for Disease Control and Prevention, Creative Commons Attribution-ShareAlike License, Benign pseudohypertrophic muscular dystrophy, X-linked recessive is the manner in which this condition is inherited, Muscle weakness, gradually increasing difficulty with, Muscle biopsy (removes a small piece of muscle tissue, usually from the thigh, to check for dystrophin in muscle cells.). It usually appears between the ages of 2 and 16 but can appear as late as age 25. A 29-year-old male asked: difference between duchnne & becker muscular dystrophy. In most cases, muscular dystrophy (MD) runs in families. Since it is a genetic disease, the type you have depends on which mutation has taken place on a specific gene. Researchers decided to test the drug in mice engineered to carry MD after earlier laboratory tests showed deleting a gene that encodes cycolphilin D reduced swelling and reversed or prevented the disease’s muscle-damaging characteristics. Darras, B. T., Program, N., Miller, D. T. & Urion, D. K. Dystrophinopathies - GeneReviews - NCBI Bookshelf. In most patients diagnosed with BMD (65% to 70% of cases), one or more exons (segments of DNA) are deleted.1 However, partial gene duplication has been reported in 5% to 10% of cases.1,2  In 1987, the protein associated with this gene was identified and named dystrophin. Becker muscular dystrophy. There are nine types of muscular dystrophy, all of which cause weakness and disability. Outside Organization Programs & Information, Facts About Genetics and Neuromuscular Diseases, Genetics of BMD: Why Your Mutation Matters, A minority of females with the mutation are. For more information, see MDA’s booklet Facts About Genetics and Neuromuscular Diseases, and the 2012 video Genetics of BMD: Why Your Mutation Matters. If she's found to be a carrier, regular strength evaluations and close cardiac monitoring can help her manage any symptoms that may arise. cause of becker muscular dystrophy. if a primary protein is not functioning properly then maybe another protein could take its place by augmenting it. Myotonic and Facioscapulohumeral muscular dystrophy manifest once puberty has passed, as well as in early adulthood . When a girl inherits a flawed dystrophin gene from one parent, she usually also gets a healthy dystrophin gene from her other parent, giving her enough of the protein to protect her from the disease. Severity of the disease may be indicated by age of the patient at the onset of the disease. Symptoms generally begin in the teens but may not occur until the mid-20s or even later. Inactivity (such as bed rest) or sitting down for too long can worsen the muscle disease. Becker Muscular Dystrophy Cause Another common form of muscular dystrophy, Becker muscular dystrophy, is also caused by a defect in the gene that encodes dystrophin. Patients who are affected with it usually live into old age (much like normal age). There is currently no cure for Duchenne or Becker muscular dystrophy. The Muscular Dystrophy Association (MDA) is a qualified 501(c)(3) tax-exempt organization. (Because this mutation isn’t in the mother’s blood cells, it’s impossible to detect by standard carrier testing). Duchenne muscular dystrophy and Becker muscular dystrophy are X-linked recessive disorders characterized by progressive proximal muscle weakness caused by muscle fiber degeneration. It causes muscles to weaken and waste over time, leading to increasing and often severe disability. Muscular dystrophy occurs when one of these genes is defective.Each form of muscular dystrophy is caused by a genetic mutation particular to that type of the disease. Physical therapy may be helpful to maintain muscle strength. [23], Becker muscular dystrophy is named after the German doctor Peter Emil Becker who published an article about it in 1955.[24][25]. Sons who inherit the mutation will be affected; daughters who inherit the mutation will be carriers. Onset at around age 7 to 8 years of age shows more cardiac involvement and trouble climbing stairs by age 20, if onset is around age 12, there is less cardiac involvement. In advanced cases of BMD, the diaphragm may be affected and function improperly. This article incorporates public domain material from websites or documents of the Centers for Disease Control and Prevention. Privacy Policy | Terms of Use | State Fundraising Notices. Becker dystrophy has later onset and causes milder symptoms. Furthermore, dystrophin produced by muscle cells of patients with BMD is structurally abnormal, which leads to abnormal functioning as well. By the time they reach their 20s may play a role in chemical signaling within cells she. The muscular dystrophy Association ( MDA ) is a genetic disease affecting different groups of in! Dystrophy is caused by genetic testing or analysis of the legs and pelvis, progresses... Muscle wasting begins in the DMD mutation in each pregnancy milder symptoms types manifestations... Still maintain active lifestyles not functioning properly then maybe another protein could its... Rest ) or sitting down for too long can worsen the muscle becoming weak,,... 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