Which clinical history findings are characteristic of autosomal-dominant limb-girdle muscular dystrophy 1I (LGMD1I)

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

LGMD1I (LGMDD4 Calpain-3; 15q15)

Similar syndrome to LGMD2A (calpainopathy), but milder.

Later age of onset (mid-30s).

Milder weakness, but similar pattern (proximal leg [glutei/hamstring], paraspinal, medial gastrocnemius).

Myalgia in 50%.

CK may be markedly elevated.


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