[Medline]. Orthopedist - The need for management of scoliosis and joint contractures are major indications for consultation with an orthopedist. A tendon release procedure, also called heel cord surgery, can treat ankle and other contractures while a person is still walking. Osteoporos Int. Braces, also called orthoses, can support just the ankle and foot or extend over the knee. Another form of muscular dystrophy caused by dystrophin deficiency is the Becker type. A doctor may prescribe steroid medications to help individuals remain able to walk for as long as possible. The aim of gene therapy is to deliver DNA encoding dystrophin or other therapeutic genes, such as utrophin, to muscle. Both Duchenne and Becker muscular dystrophies are caused by an abnormal gene carried on the X sex chromosome. Braces on the lower legs help keep the limbs stretched and flexible, delaying the onset of contractures. Health Qual Life Outcomes. The clinical course of Becker muscular dystrophy is variable. 2015. Prednisone is by far the most commonly prescribed corticosteroid for DMD in the United States. However, this is a general biomarker for muscle break down. Procedures, You are being redirected to Life expectancy and death from cardiomyopathy amongst carriers of Duchenne and Becker muscular dystrophy in Scotland. Part II. There's currently no cure for muscular dystrophy (MD), but a variety of treatments can help to manage the condition. BMD causes muscle cells to die, and results in the muscle becoming weak, small, and deformed. Young HK, Barton BA, Waisbren S, et al. Spinal fusion to correct scoliosis may be scheduled based on the progression of spinal deformity and the age of the patient. Progressive scoliosis and contracture formation may require surgical intervention. To see a presentation by a pulmonary medicine specialist, see the August 2012 video Lung Health in Neuromuscular Disease. 1955. Becker muscular dystrophy is less severe than Duchenne muscular dystrophy. Part II. Females rarely develop symptoms. Risk of fracture in patients with muscular dystrophies. Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Physical Medicine and RehabilitationDisclosure: Nothing to disclose. The treatment of BMD focuses on lessening the symptoms associated with it. Treatment is supportive and includes: 1. Intervention/treatment Phase ; Becker Muscular Dystrophy: Drug: (+)-Epicatechin: Phase 1: Detailed Description: The safety and tolerability of three escalating doses of (+)-epicatechin will be assessed and early effectiveness measured by changes in plasma biomarkers, tissue biomarkers from muscle biopsies, cardiac imaging, and on clinical function assessments of participants' muscle … Mol Ther. 1994 Jul. Some patients may need placement of a feeding tube. If not treated, these can become severe, causing discomfort and restricting mobility and flexibility. [Full Text]. For boys and men who use power wheelchairs, who aren’t very active, or who take prednisone (steroidal drug), excessive weight gain can occur. Becker muscular dystrophy is similar in many ways to duchenne muscular dystrophy but it is less serious. The disease is found mostly in boys. It primarily affects males. [Medline]. 7 (7):410-4. Avoid obesity. 64(9):1635-7. Investigations. It is a genetic condition and it is caused by a fault in a gene called dystrophin. Specific adaptations (to aid, for instance, dressing and bathroom skills) may be provided. Treatment for muscular dystrophy. Orthopedic appliances such as braces and wheelchairs may improve mobility and self-care. Becker muscular dystrophy occurs in about 3 to 6 out of every 100,000 births. There is currently no treatment available targeting the underlying genetic cause of Becker muscular dystrophy. Dysphagia concerns may be evaluated by a speech therapist. Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy. In some cases, heart involvement (cardiomyopathy) is the first sign. Becker muscular dystrophy. Becker Muscular Dystrophy Treatment . Am J Hum Genet. ike Duchenne MD, Becker MD mostly affects boys. Arch Psychiatr Nervenkr Z Gesamte Neurol Psychiatr. Revising Cardiac Care in Muscular Dystrophies, DMD Clinical Research Network Studying Dystrophin-Deficient Heart, Cardiac Complications and Management in BMD, Nutritional Considerations While on Corticosteroids, Nutritional Needs in Neuromuscular Disease, Functional Considerations: Occupational and Physical Therapy. Almost exclusively affect boys. But many over age 50 can walk without help or by using a cane, crutches, or walker. Preservation of the C-terminus of dystrophin molecule in the skeletal muscle from Becker muscular dystrophy. Appropriate cautions must be taken to avoid medications that may precipitate malignant hyperthermia. As the patient's weakness progresses, appropriate equipment and … 2013 Sep. 23(9):855-64. Physical and occupational therapy can help to strengthen muscles and complete daily tasks. Duchenne and Becker muscular dystrophy: Treatment & Research. What are the treatments for Becker muscular dystrophy? [Medline]. BMD is caused by dys … Current and Emerging Therapies in Becker Muscular Dystrophy (BMD) Acta Myol. Grootenhuis MA, de Boone J, van der Kooi AJ. A combination of immobility and weak abdominal muscles can lead to severe constipation, so the diet should be high in fluid and fiber, with fresh fruits and vegetables dominant. [28, 29]. [Medline]. Segura LG, Lorenz JD, Weingarten TN, Scavonetto F, Bojanic K, Selcen D, et al. There is currently no treatment for the condition. The age of onset and rate of progression can vary. Muscular dystrophy treatment has improved in recent years and there is a great deal of research into future treatments and possible cures. 2007 Oct 11. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. 313417-overview (Orthoses also are known as orthotics.). The impact of BMD can be significantly minimized by keeping the body as flexible, upright, and mobile as possible. A study by Yamada et al found that when patients with BMD were matched by physical function status to patients with DMD, both groups had similar swallowing problems. J Phys Conf Ser. Muscle cramps may be helped by night splints, massage or compression treatment using air-filled boots. 2007 Dec 3. Becker muscular dystrophy This type of muscular dystrophy also more commonly affects boys. A device known as a cough assist can help with this problem. 1259041-overview Data for or against the use of corticosteroids in BMD are lacking. Pediatrics. See MDA updates on COVID-19. Becker muscular dystrophy (BMD) is a condition which causes weakness in the muscles. [Medline]. Ann Neurol. This disease is caused by a mutation in the gene that encodes a protein called dystrophin. Pouwels S, de Boer A, Leufkens HG, Weber WE, Cooper C, van Onzenoort HA, et al. Schwartz M, Hertz JM, Sveen ML, et al. As with DMD, there is muscle wasting and weakness which is mainly proximal. Recent evidence suggests that exercise training is beneficial. LGMD2I presenting with a characteristic Duchenne or Becker muscular dystrophy phenotype. Thanks to general medical advances, particularly in cardiology, people with Becker muscular dystrophy (BMD) are living longer in the 21st century than in previous decades. Eur Heart J Cardiovasc Imaging. The evolution is variable. It's particularly important that limbs be stretched. Becker muscular dystrophy is very similar to Duchenne muscular dystrophy.The main difference is that it gets worse at a much slower rate and it is less common. Becker and Kiener initially described Becker muscular dystrophy (BMD) in 1955. It causes muscles to weaken and waste over time, leading to increasing and often severe disability. Functional Treatment Considerations: Occupational and Physical Therapy. The disease mainly affects boys and usually start to manifest between age 10 and 15. Becker muscular dystrophy (DIS-trah-fee), or BMD, is a genetic disease affecting different groups of muscles in the body. There is not a cure for Becker muscular dystrophy at present. Treatment: Physical therapy, braces, corrective surgery, assisted ventilation: Prognosis: ... Becker muscular dystrophy (BMD) is a less severe variant of Duchenne muscular dystrophy and is caused by the production of a truncated, but partially functional form of dystrophin. Because no cure exists for BMD, treatment is focused on controlling a patient's symptoms. Restoration of the Dystrophin-associated Glycoprotein Complex After Exon Skipping Therapy in Duchenne Muscular Dystrophy. Becker muscular dystrophy is similar to Duchenne muscular dystrophy, but it’s less severe. 193(4):427-48. Identifying mosaicism in the mother or father, as well as determining the risk of transmission, is another topic to consider in nonheterozygote carrier families or in isolated cases. Muscular Dystrophy Association National Office, 800-572-1717 | ResourceCenter@mdausa.org. A second, much rarer type of the disorder is Becker muscular dystrophy. [Medline]. Becker muscular dystrophy (BMD) has onset usually in childhood, frequently by 11 years. Treatment. J Neurol Sci. Living with muscular dystrophy: health related quality of life consequences for children and adults. 2008 Feb. 18(2):100-6. Becker dystrophy has later onset and causes milder symptoms. Kids usually get diagnosed with the condition after age 7. When taken at relatively high doses for long periods of time, it can have significant side effects, such as weight gain, short stature, delayed puberty, decreased bone density, vertebral bone fractures, behavioral abnormalities, and cataracts. Speech therapy may help if one experiences a difficulty with swallowing. Physical activity is important for those who have this disease, as bed rest can cause BMD to worsen. Koenig M, Beggs AH, Moyer M, et al. Some men need a wheelchair, while others may only need walking aids such as canes or braces. There is no cure, but treatments are available to help with symptoms and maximize muscle function. Curr Opin Mol Ther. [3] 2. There is no cure for BMD, and treatment is symptomatic. [Full Text]. Share cases and questions with Physicians on Medscape consult. Due to dystrophin gene deficiency, its severity is unpredictable. Clinical-molecular correlation in 104 mild X-linked muscular dystrophy patients: characterization of sub-clinical phenotypes. Please confirm that you would like to log out of Medscape. Muscle weakness occurs primarily in your arms and legs, with symptoms appearing in between age 11 and 25. 2011 Nov 8. No definitive evidence demonstrates that steroids are effective against BMD. Inactivity, such as bed rest can make the muscle disease worse. A physical therapy program is usually part of the treatment for BMD. As your symptoms develop, the healthcare professionals treating you will advise on the options. Heart. The evolution is variable. Improvements in cardiac care, attention to respiratory function, and judicious use of spinal correction surgery have led to increased survival in the DMD population. Although some look at these devices as symbols of disability, most users find using devices allow one to actually be more mobile, energetic and independent than when trying to walk on very weak legs. Becker PE, Kiener F. [A new x-chromosomal muscular dystrophy.]. Medical research on muscular dystrophy and myopathy. ©2021, Muscular Dystrophy Association Inc. All rights reserved. The goal of treatment is to control symptoms to maximize the person's quality of life. There is no cure for BMD, and treatment is symptomatic. Muscle transfers, such as with the posterior tibialis muscle, also may be considered to preserve functional mobility. Mobility concerns are addressed, including the need for devices to assist with mobility, such as a scooter or a fully adapted wheelchair with a custom seat and back, custom supports, and electric power. Access and mobility concerns in the community invariably touch upon the adjustment issues faced by individuals with a progressive disability. Becker MD also affects similar areas of the body to Duchenne MD, although the symptoms tend to be less severe. Hum Mol Genet. [Medline]. It's important that the surgical team know about that a patient has BMD so that complications can be avoided or quickly treated. Phase 1 Gene Therapy for Duchenne Muscular Dystrophy Using a Translational Optimized AAV Vector. Generally, walking difficulties begin after the age of 16. Recurrent aspiration pneumonias from progressive dysphagia may eventually cause mortality in BMD. Range-of-motion exercises, performed on a regular schedule, help delay contractures by … Medications belonging to a group known as corticosteroids – or glucocorticoids –  have been found effective in slowing the course of DMD. J Child Neurol. [Medline]. Cirak S, Feng L, Anthony K, Arechavala-Gomeza V, Torelli S, Sewry C, et al. Another commonly used corticosteroid for DMD is deflazacort (Emflaza), which was approved by the US Food and Drug Administration (FDA) in February 2017 to treat the disease. People with muscular dystrophy should be monitored throughout their lives. Referral to a psychiatrist or psychologist may be needed. Stephen Kishner, MD, MHA Professor of Clinical Medicine, Physical Medicine and Rehabilitation Residency Program Director, Louisiana State University School of Medicine in New Orleans Comprehensive evaluation of structural and functional myocardial impairments in Becker muscular dystrophy using quantitative cardiac magnetic resonance imaging. Read about the promise of stem cell research for muscular dystrophy patients, and successful treatments tested in dogs. Caloric intake should be restricted to keep weight down, as obesity puts greater stress on already weakened skeletal muscles and the heart. 4(4):349-58. [Medline]. Currently there is no known cure for Becker muscular dystrophy. Cardiac transplantation may be offered as an option to patients with BMD with severe cardiomyopathy and limited or skeletal (voluntary) muscle disease. Specific planning for avocational needs and desires may be coordinated with a recreational therapist. 3. BMD can present in several ways such as waddling gait, exercise related cramps with or without myoglobinuria. Becker muscular dystrophy (DIS-trah-fee), or BMD, is a genetic disease affecting different groups of muscles in the body. Inherited neuromuscular disorders: Pathway to diagnosis. 6. A genetic disease is one that you are born with and you may have inherited from your family. As of 2019, most therapies are supportive in nature, although truly disease-modifying therapies are the subject of intense research. Nicolas A, Raguenes-Nicol C, Ben Yaou R, et al. The dystrophinopathies Duchenne and Becker muscular dystrophies (DMD and BMD) represent the most common inherited disorders of muscle. This type of muscular dystrophy likewise more typically impacts young boys, according to iytmed.com. What are the treatments for Becker muscular dystrophy? Becker muscular dystrophy symptoms and effects develop slowly, with symptoms appearing between ages 5 and 15. Physical and occupational therapy can help to strengthen muscles and complete daily tasks. 5:31. Benjamin R Mandac, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Physical Medicine and RehabilitationDisclosure: Nothing to disclose. It does not provide medical advice, diagnosis, or treatment. It has been shown that prednisone therapy can be helpful for patients diagnosed with BMD as well.2 However, little is known about the effect of the benefits of glucocorticosteroid (a class of corticosteroid) for BMD patients. Some experts recommend swimming and water exercises (aquatic therapy) as a good way to keep muscles as toned as possible without causing undue stress on them. Inactivity (such as bed rest) or sitting down for too long can worsen the muscle disease. Becker muscular dystrophy usually affects boys and men. Progressive weakness toward the end of the disease process may lead to dysphagia and an increased risk of aspiration pneumonia. BMD patients that present dysphagia (difficulty with swallowing) should be referred to a speech and language therapist for swallowing assessment. Limb-girdle dystrophy, Facio-scapulo-humeral dystrophy and Becker dystrophy are some other types of the problem that can commonly affect boys and girls. BMD can present in several ways such as waddling gait, exercise related cramps with or without myoglobinuria. In some people with BMD, particularly as they age, breathing muscles can weaken, resulting in less-than-optimal breathing, particularly during sleep. This type is characterized by progressive weakness and loss of muscle mass in the cardiac and skeletal muscles. Heart muscle also is commonly affected, making cardiac problems a prominent feature of the disease. Activity is encouraged. This can limit your movement and even lead to pain. Simultaneous mutation scanning for gross deletions, duplications and point mutations in the DMD gene. Some people with Becker MD begin using a wheelchair in their twenties. This can be treated by a noninvasive strategy known as bilevel positive airway pressure (BiPAP). There is no cure for muscular dystrophy and no way to stop it from getting worse. Your MDA Care Center physician will refer you to a physical therapist for a thorough evaluation and recommendations. Scooters and wheelchairs are especially valuable when covering long distances. It also affects boys but the symptoms start later -- between ages 11 and 25. Becker muscular dystrophy severity is linked to the structure of dystrophin. Most people who have Becker muscular dystrophy (BMD) receive treatments similar to those people who have Duchenne muscular dystrophy (DMD). Becker Muscular Dystrophy Treatment . There are several ways to minimize and postpone contractures. Much of the pioneering research into a treatment for Duchenne muscular dystrophy may also benefit those with Becker muscular dystrophy. 24 (5):1267-79. When contractures have advanced, surgery may be performed to relieve them. Genetic diagnosis of Duchenne and Becker muscular dystrophy using next-generation sequencing technology: comprehensive mutational search in a single platform. Some doctors prescribe steroids to help keep a patient walking for as long as possible. Becker Muscular Dystrophy. The onset of this condition is slower and the symptoms start later in childhood. Mavrogeni S, Markousis-Mavrogenis G, Papavasiliou A, Kolovou G. Cardiac involvement in Duchenne and Becker muscular dystrophy. Therapy & Treatment. Muscle weakness occurs primarily in your arms and legs, with symptoms appearing in between age 11 and 25. It will also depend on how severe the condition is. Doctors have found that a low-calorie diet doesn’t have any harmful effect on the muscles. Constipation may be an associated problem, given poor fluid intake and progressive difficulty with commode transfers. 2008 Jan. 16(1):53-61. Rarely cardiomyopathy might be the presenting feature. Appropriate consultations may include the following: Anesthesiologist - Preoperative management and planning for appropriate anesthesia are key reasons for consultation with an anesthesiologist. [23]. Elizabeth A Moberg-Wolff, MD Medical Director, Pediatric Rehabilitation Medicine Associates [24, 25] Dantrolene sodium is probably the best medication to use if malignant hyperthermia arises. 2005 Dec. 116(6):1569-73. Geneticist - Consultation regarding the carrier status of the patient's mother and siblings is important. Duchenne and Becker muscular dystrophy in adolescents: current perspectives. 2015:141856. Physical therapy may be helpful to maintain muscle strength. [Medline]. For Duchenne and Becker muscular dystrophies, protein and DNA tests can identify carriers, and DNA probes can provide prenatal diagnosis. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Depending on local services, any one of a wide range of health care professionals can serve as a coordinator of clinical care. Becker muscular dystrophy. 3.2. [Medline]. The buoyancy of the water helps protect against certain kinds of muscle strain and injury. For Duchenne and Becker muscular dystrophies, muscle biopsy may show whether dystrophin, a muscle protein, is missing or abnormal, and DNA testing is used to analyze the condition of the related gene. Supplementing vitamin D and calcium is also recommended. Some doctors prescribe steroids to help keep a patient walking for as long as possible. 2018. Menezes MP, North KN. 2017 Mar 13. Diseases & Conditions, encoded search term (Becker Muscular Dystrophy) and Becker Muscular Dystrophy, Physical Medicine and Rehabilitation for Limb-Girdle Muscular Dystrophy, Certifying Medical Cannabis: What APRNs Need to Know, FDA OKs Viltolarsen (Viltepso) for Duchenne Muscular Dystrophy, Ataluren Delays Disease Milestones in Patients With Nonsense Mutation DMD, Myopathies: Muscling Your Way to the Diagnosis, Convicted Ex-Surgeon Pleads Guilty to New Fraud Charges, FDA Approves Implant as Alternative to ACL Reconstruction, Epidural Corticosteroid Injections for Sciatica, Increased Risk of Bone Fractures in Non-meat Eaters, Spinal Epidural Abscess: Diagnosis, Management, and Outcomes. Becker muscular dystrophy (BMD) is an inherited condition that causes progressive weakness and wasting of the skeletal and cardiac (heart) muscles. Gurvich OL, Tuohy TM, Howard MT, et al. 1962 Sep-Dec. 21:551-66. BMD is an inherited disease with a male distribution pattern and a clinical picture similar to that of Duchenne muscular dystrophy (DMD). Such adaptations range from methods of buttoning and zippering clothes to grab bars and raised toilet seats in the bathroom. Becker muscular dystrophy follows x-linked recessive inheritance so it mostly affects males, but some … Weakness progresses, and emergencies related to cardiac and respiratory symptoms are hallmarks of advance in the disease process. Kat Kolaski, MD Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine 1 A defining feature of BMD is a high degree of variability in symptoms— including onset, severity, and cor- 2013. Jenkins HM, Stocki A, Kriellaars D, Pasterkamp H. Breath stacking in children with neuromuscular disorders. There is currently no cure for muscular dystrophy. 2015 Mar 1. For an August 2012 video on this topic, see Functional Considerations: Occupational and Physical Therapy. Myoblast treatment, as well as the use of stem cells, also may be alternative modalities if proven successful. It is therefore important for people with Becker muscular dystrophy to have regular heart monitoring by a specialist. Genetic testing will confirm the diagnosis of Duchenne and Becker and show the exact mutation in the gene. It has been shown that early treatment with perindopril delayed the onset and progression of prominent left ventricle dysfunction in children with DMD.1. Rev Can Biol. Muscular Dystrophy News is strictly a news and information website about the disease. Educational institutions, from public schools to community colleges and universities, may have resources that can be utilized. Neurology. 2013 Aug 16. [Medline]. Duchenne and Becker muscular dystrophy: Treatment & Research. Adolesc Health Med Ther. Steroids have been reported to show benefit in patients with DMD, but there are conflicting reports. Becker's muscular dystrophy is an X-linked inherited muscular dystrophy with the disorder occurring at locus Xp21.2. [Medline]. Glucocorticoid treatment should be stopped progressively to avoid adverse reactions. Pediatr Pulmonol. Dis Markers. Muscle weakness usually starts in the legs and hips. [Medline]. Arahata K, Beggs AH, Honda H, et al. The risk of malignant hyperthermia is significant, given the intrinsic muscle disorder. However, this is a general biomarker for muscle break down. Although there's no cure for any form of muscular dystrophy, treatment for some forms of the disease can help extend the time a person with the disease can remain mobile and help with heart and lung muscle strength. [Medline]. Diagnosis is suggested clinically and is confirmed by genetic testing or analysis of the protein product (dystrophin) of the mutated gene. Medscape Education, Case Discussions in the Early Recognition of Duchenne Muscular Dystrophy (DMD), 2002 [Medline]. Treatments … 1976 Oct. 10(4):189-201. Ankle-foot orthoses are sometimes prescribed for night wear to keep feet from pointing downward and keep the Achilles tendon stretched. Becker muscular dystrophy (BMD) generally leads to slowly worsening disability, but the amount of disability can vary among affected people. Videofluoroscopic evaluation may be performed to demonstrate the risk of aspiration. How is Becker muscular dystrophy treated in a child? It does not provide medical advice, diagnosis or treatment. Early interventions may focus on stretching tight muscles (which may initially be the only therapy goal). Investigations. Ashton EJ, Yau SC, Deans ZC, et al. A doctor may prescribe steroid medications to help individuals remain able to walk for as long as possible. Becker Muscular Dystrophy Treatment. The goal of treatment is to keep muscles as strong as possible to allow a child to function independently. It typically impacts males, with onset occurring between ages five and fifteen. Duchenne muscular dystrophy and Becker muscular dystrophy are X-linked recessive disorders characterized by progressive proximal muscle weakness caused by muscle fiber degeneration. Marty B, Gilles R, Toussaint M, et al. The primary goals of physical therapy are to allow greater motion in the joints and to prevent contractures and scoliosis (spinal curvature). Loss of balance and coordination. for: Medscape. Often the first test is a blood test measuring CK (creatine kinase), which is extremely elevated in DMD and BMD. Privacy Policy | Terms of Use | State Fundraising Notices. 2011 Nov 3. Emflaza has been proven to improve motor function, strength, and pulmonary function and to reduce the risk of lumbar spine deviations and loss of walking ability. How is Becker Muscular Dystrophy treated? Muscle weakness usually becomes apparent between the ages of 5 and 15. [Medline]. Yoga for muscular dystrophy can also effectively help you manage your symptoms and slow down the progress of muscular dystrophy. Becker muscular dystrophy. Patients are given supportive care, leg braces and physiotherapy to help them function in daily life. In some cases, Becker muscular dystrophy can lead to life-threatening health problems, as heart and breathing muscles weaken. It is a less severe from of muscular dystrophy than Duchenne's muscular dystrophy. Symptoms of Becker MD usually begin in childhood, but they're often relatively mild at this point. To view a presentation by an occupational therapist, see the August 2012 video Functional Treatment Considerations: Occupational and Physical Therapy. [Medline]. People with BMD may have unexpected adverse reactions to certain types of anesthesia. Becker's muscular dystrophy (BMD) is similar to the more common muscular dystrophy - Duchenne muscular dystrophy (DMD) - but the clinical course is milder.