Benign pseudohypertrophic muscular dystrophy; Becker's dystrophy
Becker muscular dystrophy is an inherited disorder that involves slowly worsening muscle weakness of the legs and pelvis.
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. This disease is caused by a mutation in the gene that encodes a protein called dystrophin.
The disorder is passed down through families (inherited). Having a family history of the condition raises your risk.
Becker muscular dystrophy occurs in about 3 to 6 out of every 100,000 births. The disease is found mostly in boys.
Females rarely develop symptoms. Males will develop symptoms if they inherit the defective gene. Symptoms most often appear in boys between ages 5 and 15, but may begin later.
Muscle weakness of the lower body, including the legs and pelvis area, slowly gets worse, causing:
Other symptoms may include:
The health care provider will do a nervous system (neurological) and muscle exam. A careful medical history is also important, because symptoms are similar to those of Duchenne muscular dystrophy. However, Becker muscular dystrophy gets worse much more slowly.
An exam may find:
Tests that may be done include:
There is no known cure for Becker muscular dystrophy. However there are many new drugs currently undergoing clinical testing that show significant promise in treating the disease.The current goal of treatment is to control symptoms to maximize the person's quality of life. Some providers prescribe steroids to help keep a patient walking for as long as possible.
Activity is encouraged. Inactivity (such as bed rest) can make the muscle disease worse. Physical therapy may be helpful to maintain muscle strength. Orthopedic appliances such as braces and wheelchairs may improve movement and self-care.
Abnormal heart function may require the use of a pacemaker.
Genetic counseling may be recommended. Daughters of a man with Becker muscular dystrophy will very likely carry the defective gene and could pass it on to their sons.
Becker muscular dystrophy leads to slowly worsening disability. However, the amount of disability varies. Some people may need a wheelchair. Others may only need to use walking aids such as canes or braces.
Lifespan is most often shortened if there are heart and breathing problems.
Complications may include:
Call your provider if:
Genetic counseling may be advised if there is a family history of Becker muscular dystrophy.
Amato AA. Disorders of skeletal muscle. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 110.
Bharucha-Goebel DX. Muscular dystrophies. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 627.
Gloss D, Moxley RT III, Ashwal S, Oskoui M. Practice guideline update summary: corticosteroid treatment of Duchenne muscular dystrophy: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2016;86(5):465-472. PMID: 26833937 pubmed.ncbi.nlm.nih.gov/26833937/.
Selcen D. Muscle diseases. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 393.BACK TO TOP
Review Date: 2/4/2020
Reviewed By: Amit M. Shelat, DO, FACP, FAAN, Attending Neurologist and Assistant Professor of Clinical Neurology, Stony Brook University School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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