X-linked recessive is the manner in which this condition is inherited
Specialty
Neurology
Symptoms
Severe upper extremity muscle weakness,[2] Toe-walking[3]
Causes
Mutations in DMD gene[4]
Diagnostic method
Neurological exam, muscle exam[3]
Treatment
No current cure, Physical therapy [3]
Becker muscular dystrophy (BMD) is an X-linked recessive inherited disorder characterized by slowly progressing muscle weakness of the legs and pelvis. It is a type of dystrophinopathy.[5][3] The cause is mutations and deletions in any of the 79 exons encoding the large dystrophin protein, essential for maintaining the muscle fiber's cell membrane integrity.[6] Becker muscular dystrophy is related to Duchenne muscular dystrophy in that both result from a mutation in the dystrophin gene, however the hallmark of Becker is milder in-frame deletions.[4] and hence has a milder course, with patients maintaining ambulation till 50–60 years if detected early.[7][8]
While there is no known cure, management strategies such as physical therapy, braces, and corrective surgery may alleviate symptoms.[9] Assisted ventilation may be required in those with weakness of breathing muscles.[6] Several drugs designed to address the root cause are currently available including gene therapy (Elevidys).[6]
Other medications used include glucocorticoids (Deflazacort, Vamorolone); calcium channel blockers (Diltiazem); to slow skeletal and cardiac muscle degeneration, anticonvulsants to control seizures and some muscle activity, and Histone deacetylase inhibitors (Givinostat) to delay damage to dying muscle cells.[9][6] These patients do not require antisense drugs (Ataluren, Eteplirsen etc.) as certain percentage of dystrophin is already expressed.[6]
^"Becker muscular dystrophy: MedlinePlus Medical Encyclopedia". medlineplus.gov. Archived from the original on 15 March 2017. Retrieved 30 July 2019.
^Cite error: The named reference pat was invoked but never defined (see the help page).
^ abcd"Becker muscular dystrophy". NIH. Archived from the original on 7 April 2016. Retrieved 17 April 2016.
^ ab"Becker muscular dystrophy | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". Archived from the original on 2016-04-28. Retrieved 2016-04-19.
^"Duchenne and Becker muscular dystrophy". NIH.gov. NIH. Archived from the original on 24 March 2017. Retrieved 17 April 2016.
^ abcdeCite error: The named reference NIH2016Re was invoked but never defined (see the help page).
Beckermusculardystrophy (BMD) is an X-linked recessive inherited disorder characterized by slowly progressing muscle weakness of the legs and pelvis...
Duchenne musculardystrophy (DMD) is a severe type of musculardystrophy predominantly affecting boys. The onset of muscle weakness typically begins around...
MuscularDystrophy Association (MDA) is an American nonprofit organization dedicated to supporting people living with musculardystrophy, ALS, and related...
referred to as musculardystrophy. The deletions of one or several exons of the dystrophin DMD gene cause Duchenne and Beckermusculardystrophies. The large...
is remembered for his studies of muscular dystrophies. Becker'smusculardystrophy (OMIM 300376) and Becker myotonia (OMIM 255700) are named after him...
Facioscapulohumeral musculardystrophy (FSHD) is a type of musculardystrophy, a group of heritable diseases that cause degeneration of muscle and progressive...
glycogenosis related to dystrophy gene deletion: patient has a previously undescribed myopathy associated with both Beckermusculardystrophy and a glycogen storage...
provide for research with respect to various forms of musculardystrophy, including Duchenne, Becker, limb girdle, congenital, facioscapulohumeral, myotonic...
conditions include amyotrophic lateral sclerosis, myasthenia gravis, and musculardystrophy.[citation needed] The first step before insertion of the needle electrode...
itself remains. For instance, some individuals with Duchenne and Beckermusculardystrophy may start with true muscle hypertrophy, but later develop into...
infiltration of fat or other tissue). For instance, Duchenne and Beckermusculardystrophy may start as true muscle hypertrophy, but later develop into pseudohypertrophy...
and Genetics. While in Children, Neuromuscular diseases such as Beckermusculardystrophy, including X-linked genetic disorder, are directly linked with...
A (20 June 2018). "Skeletal Muscle Metabolism in Duchenne and BeckerMuscularDystrophy-Implications for Therapies". Nutrients. 10 (6): 796. doi:10.3390/nu10060796...
with dystrophin, the protein associated with Duchenne musculardystrophy and Beckermusculardystrophy. The name comes from the Greek word syntrophos, meaning...
related to Duchenne musculardystrophy is known as Pradhan Sign, and the others associated with facioscapulohumeral musculardystrophy (FSHD) and similar...
a vehicle for gene therapy, in the treatment of Duchenne and Beckermusculardystrophy. As mutations in the gene which codes for the protein dystrophin...
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