Some forms progress over a normal lifespan. Dr. Ayah Elmaghrabi answered. BMD is inherited in an X-linked pattern. [17] The drug prednisone contributes to an increased production of the protein utrophin which closely resembles dystrophin, the protein that is defective in BMD. Muscular dystrophy can be inheirited, or occur for the first time in an individual. Respiratory failure is another cause of death in most types of muscular dystrophy. A minority of females with the mutation are manifesting carriers, who usually have a mild form of the disorder. The gene mutation causes the dystrophin protein to … Eye problems can vary based on the type of muscular dystrophy. [medical citation needed]. [20] According to a review by Bushby, et al. Becker muscular dystrophy is related to Duchenne muscular dystrophy in that both result from a mutation in the dystrophin gene, but has a milder course. Genes contain codes, or recipes, for proteins, which are important biological components in all forms of life. There are nine types of muscular dystrophy, all of which cause weakness and disability. BMD causes muscle cells to die, and results in the muscle becoming weak, small, and deformed. Becker muscular dystrophy is a muscle-wasting condition, first described in 1956, which usually affects only males. The risk to the siblings of an affected individual depends upon the carrier status of the mother. Muscle weakness also occurs in the arms, neck, and other areas, but not as noticeably severe as in the lower half of the body. Terms of Use | State Fundraising Notices. Some forms of muscular dystrophy are … Among the exams/tests performed are:[14][15], There is no known cure for Becker muscular dystrophy yet. 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.). Becker muscular dystrophy affects the muscles of the hips, pelvic area, thighs and shoulders, as well as the heart.Becker muscular dystrophy (BMD) is one of nine types of muscular dystrophy, a group of genetic, degenerative diseases primarily affecting voluntary muscles. Orthopedic appliances such as braces and wheelchairs may improve mobility and self-care. For asymptomatic carriers, it is recommended to repeat cardiac assessments every three to five years; however, for carriers who develop symptoms, it is recommended to go through more frequent cardiac assessments.3, Muscular Dystrophy Association National Office, 800-572-1717 | ResourceCenter@mdausa.org. In advanced cases of BMD, the diaphragm may be affected and function improperly. Carrier females have a 50% chance of passing the DMD mutation in each pregnancy. [21], The progression of Becker muscular dystrophy is highly variable—much more so than Duchenne muscular dystrophy. Darras, B. T., Program, N., Miller, D. T. & Urion, D. K. Dystrophinopathies - GeneReviews - NCBI Bookshelf. Becker muscular dystrophy is caused by abnormalities (mutations) in the DMD gene that is responsible for the production of the dystrophin protein. There is not a cure for Becker muscular dystrophy at present. 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. ... Types of MD inherited in this way include Duchenne MD and Becker MD, which is why these conditions are more common and more severe in males. Muscular dystrophy (MD) refers to a group of more than 30 genetic diseases that cause muscle degeneration, progressive weakness and chronic or permanent shortening of tendons and muscles. It is important that a team that includes all of these subspecialists provides Duchenne and/or Becker care. Becker muscular dystrophy (BMD) is caused by specific mutations in the DMD gene. The disorder is passed down through families (inherited). ©2021, Muscular Dystrophy Association Inc. All rights reserved. There is currently no cure for Duchenne or Becker muscular dystrophy. The gene is located on the X chromosome. Symptoms usually appear in men at about ages 8–25, but may sometimes begin later. Muscle weakness occurs mostly in your arms and … Becker muscular dystrophy is very similar to Duchenne muscular dystrophy. 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. [8] Becker muscular dystrophy (BMD) also demonstrates the following: The disorder is inherited with an X-linked recessive inheritance pattern. BMD is less severe than DMD. It is named after German doctor Peter Emil Becker, who first described this variant of Duchenne muscular dystrophy (DMD) in the 1950s. Every boy inherits an X chromosome from his mother and a Y chromosome from his father, which is what makes him male. Stay informed. Some may even need a wheelchair or other mobility aids. Becker muscular dystrophy can cause cardiomyopathy, a weakening of the heart muscles, which, if unaddressed, can lead to heart failure and the need for a transplant. That means the gene that sometimes contains a mutation causing these diseases is on the X chromosome. Because of the partially functional dystrophin, muscles don't degenerate as badly or quickly as they do in patients diagnosed with DMD, who don’t produce functional dystrophin at all. [23], Becker muscular dystrophy is named after the German doctor Peter Emil Becker who published an article about it in 1955.[24][25]. One study showed that there may be two distinct patterns of progression in Becker muscular dystrophy. Duchenne and Becker muscular dystrophy. But with assistive devices, independence can be maintained. (Because this mutation isn’t in the mother’s blood cells, it’s impossible to detect by standard carrier testing). It is a type of dystrophinopathy. Becker muscular dystrophy is related to Duchenne muscular dystrophy in that both result from a mutation in the dystrophin gene,[4] but has a milder course.[6]. Calf muscles initially enlarge during the ages of 5-15 (an attempt by the body to compensate for loss of muscle strength), but the enlarged muscle tissue is eventually replaced by fat and connective tissue (pseudohypertrophy) as the legs become less used (with use of wheelchair). The DMD gene gives the body instructions to make a protein called dystrophin. 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. Dystrophin plays a role in keeping muscle cells intact; lack of dystrophin causes muscle cells to be fragile and easily damaged. 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. 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. 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. BMD can run in a family, even if only one person in the biological family has it. Signs and symptoms are similar to those of Duchenne muscular dystrophy, but tend to be milder and progress more slowly. There are two possible explanations: If a mother gives birth to a child with BMD, there’s always the possibility that more than one of her egg cells has a dystrophin gene mutation, putting her at higher-than-average risk for passing the mutation to another child. Manifesting carriers may have heart problems, which can show up as shortness of breath or inability to do moderate exercise. Girls get two X chromosomes, one from each parent. 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. Cause of Becker muscular dystrophy. Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are often discussed together because they cause similar patterns of weakness and are inherited in the same way. Myotonic and Facioscapulohumeral muscular dystrophy manifest once puberty has passed, as well as in early adulthood . Respiratory failure can occur from acute pneumonia due to aspiration, restrictive pulmonary function due to respiratory muscle weakness. The causes are genetic. Becker's muscular dystrophy is a less severe form of the disease. 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. Others cause severe muscle weakness and loss of functional disability in a relatively quick time frame. The first symptoms are often mobility problems affecting the hip girdle. Treatment. A genetic disease is one that you are born with and you may have inherited from your family. In some cases, Becker muscular dystrophy can lead to life-threatening health problems, as heart and breathing muscles weaken. [19], The investigational drug Debio-025 is a known inhibitor of the protein cyclophilin D, which regulates the swelling of mitochondria in response to cellular injury. Becker muscular dystrophy (BMD) is a rare inherited disorder of the muscles. ... Spontaneous gene mutations can cause MD to develop in people who don't have a family history of the condition. Inactivity (such as bed rest) or sitting down for too long can worsen the muscle disease. There are more than 30different types and manifestations of muscular dystrophy. if a primary protein is not functioning properly then maybe another protein could take its place by augmenting it. The DMD gene provides instructions for making a protein called dystrophin.This protein is located primarily in skeletal and cardiac muscle, where it helps stabilize and protect muscle fibers. If she's found to be a carrier, regular strength evaluations and close cardiac monitoring can help her manage any symptoms that may arise. “Becker Muscular Dystrophy (for Parents).” Edited by Mena T. Scavina, This page was last edited on 24 November 2020, at 21:59. [18], The cardiac problems that occur with EDMD and myotonic muscular dystrophy may require a pacemaker. Privacy Policy | Terms of Use | State Fundraising Notices. This would lead to easy fatigue with even minimal activity. The most common types of muscular dystrophy include:1 1. Symptoms generally begin in the teens but may not occur until the mid-20s or even later. For these women, a dystrophin deficiency may result in weaker muscles that fatigue easily in the back, legs, and arms. It usually appears between the ages of 2 and 16 but can appear as late as age 25. Once the new mutation has been passed to a son or daughter, he or she can pass it to the next generation. 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