Which clinical history findings are characteristic of autosomal-dominant limb-girdle muscular dystrophy 1B (LGMD1B)

Updated: Aug 15, 2019
  • Author: Monica Saini, MD, MBBS, MRCP(UK); Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

LGMD1B (laminopathy, allelic with autosomal dominant and autosomal recessive Emery-Dreifuss muscular dystrophy; excluded from the new classification).

Onset can be from childhood (< 10 y) to mid-30s.

LGMD1B results in proximal weakness with slow progression.

Distal limb and facial weakness may be late manifestations.

Cardiac disease begins by the 30s–50s and affects two thirds of patients. Atrioventricular (AV) block progresses from first degree to complete. Dilated cardiomyopathy and ventricular arrhythmias may also be present.


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