Becker's muscular dystrophy

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A rare and relatively benign form of muscular dystrophy of pelvis-girdle type with better prognosis than Duchenne's dystrophy.

Description

A rare and relatively benign form of muscular dystrophy of pelvis-girdle type with better prognosis than Duchenne's dystrophy. It is characterized by weakening and greater enlargement of calves during adolescence or young adulthood, but later onset and slower rate of progression. Frequently associated with colour blindness. Cardiomyopathy is common. Many have benign cardiomyopathy and live to old age, as distinct from the Duchenne form of muscular atrophy. Onset in the second and third decades of life. Etiology unknown. Inheritance is recessive X-chromosomal. The mutated gene is the same as in Duchenne's muscular dystrophy, but the mutation is different.

In 1955 Becker investigated a German family with an X-linked form of muscular dystrophy. This condition differed from the classical Duchenne dystrophy by virtue of late onset and a benign course. Becker examined five affected family members and elicited the information that six persons in the kindred had reproduced. The fact that persons with Duchenne dystrophy never procreate was further evidence for syndromic identity. With Franz Kiener Becker published a brief account in 1955, and in 1957 expanded his description. In 1962 Becker wrote a detailed account of a second family in which 10 males were affected. In this article he presented data concerning another kindred, which he had found in the archives of the Max Planck Institute, Munich. Becker also mentioned that he had reviewed the literature and recognised the condition in previous descriptions of a family in the Bonn area and another living near Wesel in North Rhine-Westphalia. Becker's dystrophy constitutes about 10 percent of all X-linked muscular dystrophy.

We thank Fergus Moore for information submitted.

Bibliography

  • P. E. Becker, F. Kiener:
    Eine neue x-chromosomale Muskeldystrophie.
    Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1955, 193: 427-448.
  • P. E. Becker:
    Neue Ergebnisse der Genetik der Muskeldystrophien.
    Acta genetica et statistica medica, 1957, 7: 303-310.

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