G11.11

Billable

Friedreich ataxia

HCC Category Mapping

V28HCC 200 — Cerebellar Ataxia and Other Degenerative Diseases of Nervous System

0.262

V24HCC 72 — Spinal Cord Disorders/Injuries

0.464

ESRDHCC 72 — Spinal Cord Disorders/Injuries

0.000

What This Code Means

Friedreich ataxia is an inherited genetic disorder that causes progressive damage to the nervous system, leading to loss of coordination, muscle weakness, and heart problems.

Coding Tips

•Document the genetic confirmation or family history pattern consistent with autosomal recessive inheritance
•Code associated cardiac complications (e.g., cardiomyopathy, arrhythmias) separately if present

Clinical Significance

Friedreich ataxia is the most common inherited ataxia, caused by GAA trinucleotide repeat expansion in the FXN gene, leading to progressive neurological and cardiac dysfunction. This diagnosis carries major implications for risk adjustment due to associated cardiomyopathy, scoliosis, and diabetes that drive significant healthcare costs and multisystem management needs.

Documentation Requirements

✓Genetic testing confirming GAA repeat expansion in FXN gene, or clinical criteria with family history
✓Autosomal recessive inheritance pattern documented
✓Age of onset (typically before age 25)
✓Progressive ataxia with neurological exam findings
✓Cardiac evaluation results (echocardiogram for hypertrophic cardiomyopathy)
✓Associated conditions documented separately (cardiomyopathy, scoliosis, diabetes mellitus)

Commonly Confused Codes

G11.10 — Early-onset cerebellar ataxia, unspecified: use only when Friedreich ataxia has been ruled out or not confirmed G11.19 — Other early-onset cerebellar ataxia: for confirmed non-Friedreich early-onset ataxias G11.2 — Late-onset cerebellar ataxia: Friedreich ataxia typically presents before age 25 G60.1 — Refsum disease: another hereditary sensorimotor neuropathy with ataxia but different genetic basis

Code Hierarchy

└G11Hereditary ataxia └G11.1Early-onset cerebellar ataxia └G11.11Friedreich ataxia

└G11.11Friedreich ataxia