Becker muscular dystrophy

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 02/04/2015

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88 Becker muscular dystrophy

Instruction

Examine this patient’s muscles.

Salient features

History

• Know whether there is a family history of the condition

• Age of onset

• Ask about shortness of breath (heart failure as a result of cardiomyopathy)

• History of mental retardation.

Examination

• Young adult male (>15 years)

• Proximal weakness of the lower extremities (in later stages more generalized muscle involvement)

• Pseudohypertrophy of calves

• Facial muscle weakness is characteristically absent or insignificant

• Kyphoscoliosis in late stages.

Proceed as follows:

• Tell the examiner that you would like to check the IQ (mental retardation may be seen).

Diagnosis

This young patient has proximal muscle weakness and pseudohypertrophy (lesion) caused by Becker’s dystrophy (aetiology). The patient has mild disability and the condition is usually progressive (functional status).

Questions

What is the difference between Duchenne and Becker muscular dystrophy?

By definition, patients with Becker muscular dystrophy can ambulate beyond the age of 15 years. The onset in Becker is usually between the ages of 5 and 15 years, but onset can occur in the third or fourth decades or even later. The majority survive into the fourth or fifth decades. Becker muscular dystrophy is less common (3 per 100 000 live male births) and has a more variable presentation of skeletal muscle weakness (Fig. 88.1).

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