G71.0349

HCC Category Mapping

What This Code Means

A genetic muscle weakness disorder affecting the hip and shoulder muscles caused by dysfunction of sarcoglycan proteins other than alpha or beta subtypes.

Coding Tips

• •Use this code for gamma, delta, or epsilon sarcoglycan dysfunction when specifically identified
• •Document which sarcoglycan subtype is affected in the medical record for clarity

Clinical Significance

Limb girdle muscular dystrophy due to other sarcoglycan dysfunction captures gamma-sarcoglycan (LGMD2C), delta-sarcoglycan (LGMD2F), and epsilon-sarcoglycan deficiencies. These rarer sarcoglycanopathies have variable severity but can be as severe as Duchenne muscular dystrophy. Precise identification of the sarcoglycan subtype is important for genetic counseling and emerging gene therapy approaches.

Documentation Requirements

• ✓Genetic testing identifying the specific sarcoglycan mutation (gamma, delta, or epsilon)
• ✓Muscle biopsy showing specific sarcoglycan protein deficiency when available
• ✓Clinical presentation: severity, distribution of weakness, age of onset
• ✓Cardiac and respiratory function assessments
• ✓Documentation specifying which sarcoglycan subtype (other than alpha or beta) is affected
• ✓Family history and inheritance pattern documentation

Commonly Confused Codes