Becker muscular dystrophy (BMD) was first described in 1953 by Emile Becker as a benign variant of Duchenne muscular Dystrophy (DMD). Compared with DMD, BMD is clinically more heterogeneous, with initial presentation in the teenage years and loss of ambulation beyond the age of 16 and a wide spectrum of clinical presentations, ranging from only myalgias and muscle cramps to exercise intolerance...

Protein-truncating mutations in the dystrophin gene lead to the progressive muscle wasting disorder Duchenne muscular dystrophy, whereas in-frame deletions typically manifest as the milder allelic condition, Becker muscular dystrophy. Antisense oligomer-induced exon skipping can modify dystrophin gene expression so that a disease-associated dystrophin pre-mRNA is processed into a Becker muscula...

The impacts of potentially treatable psychological parameters on quality life are relatively unreported in adults with Facioscapulohumeral, Becker and Limb-girdle muscular dystrophy. purpose this study was to compare life, parameters, physical function between dystrophy controls, examine relationships among these Twenty-one (n = 7 Becker, n 8 6 Limb-girdle) ten age-matched controls participated...

A total of 278 families of probands with Duchenne or Becker muscular dystrophy has been ascertained and offered genetic counselling. Linkage studies have been performed in these families using polymorphic DNA markers identifying loci linked to Duchenne and Becker muscular dystrophy. The clinical features of the probands are discussed: there was marked intrafamilial resemblance in the severity o...

© Springer Science+Business Media New York 2015 S. Grossbard, The Marriage Motive: A Price Theory of Marriage, DOI 10.1007/978-1-4614-1623-4_2 This chapter and the following are adapted from “A Theory of Allocation of Time in Markets for Labor and Marriage,” Economic Journal, Vol. 94, pp. 863–882, December 1984. While writing it I benefited from helpful comments from Gary Becker, Jerry Green (f...

1. Mostacciuolo M, Pastorello E, Vazza G et al. Facioscapulohumeral muscular dystrophy: epidemiological and molecular study in a north-east Italian population sample. Clin Genet 2009: 75: 550–555. 2. Lemmers RJLF, Van der Vliet PJ, Klooster R et al. A unifying genetic model for fascioscapulohumeral muscular dystrophy. Science 2010: 329: 1650–1653. 3. Scionti I, Greco F, Ricci G et al. Large-sca...

Duchenne muscular dystrophy (DMD) is the most common inherited neuromuscular disease. After identification of the mutation in the index patient, family members can be reliably investigated. Carriers should be informed about their risk of having offspring with the disease and about their own risk for cardiomyopathy for which regular cardiac surveillance is recommended. In a small country like th...

Background Duchenne (DMD) and Becker (BMD) muscular dystrophy (MD) are characterized by progressive peripheral muscular damage. The cardiac involvement is high and often complicated by severe heart failure and high mortality. Cardiovascular magnetic resonance (CMR) can identify early stages of cardiomyopathy, as presence of myocardial fibrosis (MF). Several studies have shown negative correlati...

Copyright © 2016 by the American Academy of Pediatrics In September 2015, the American Academy of Pediatrics endorsed the following publication: 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...