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POMT2-related limb-girdle muscular dystrophy R14

ORPHA:206559· ICD-10 G71.0

Definition

A form of limb-girdle muscular dystrophy characterized by proximal weakness (manifesting as slowness in running) presenting in infancy, along with calf hypertrophy, mild lordosis, scapular winging and normal intelligence (or mild intellectual disability).

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy