G71.035

HCC Category Mapping

What This Code Means

A genetic muscle weakness disorder affecting the hip and shoulder muscles caused by anoctamin-5 protein dysfunction, which is involved in muscle calcium regulation.

Coding Tips

• •Confirm anoctamin-5 gene mutation through genetic testing before coding this specific subtype
• •Note that anoctamin-5 deficiency can present with variable muscle involvement patterns

Clinical Significance

Limb girdle muscular dystrophy due to anoctamin-5 dysfunction (LGMD2L/LGMDR12) is an increasingly recognized form of limb girdle dystrophy involving a calcium-activated chloride channel protein. It typically presents in adulthood with milder proximal weakness and elevated creatine kinase. Accurate coding is important as this subtype has distinct prognostic features and potential therapeutic implications.

Documentation Requirements

• ✓Genetic testing confirming ANO5 gene mutation
• ✓Clinical presentation: adult-onset proximal weakness, elevated creatine kinase
• ✓Age of onset and progression pattern
• ✓Muscle biopsy findings when available
• ✓Functional assessment: walking ability, arm strength, respiratory function
• ✓Provider's explicit diagnosis specifying anoctamin-5 dysfunction

Commonly Confused Codes