Friedreich's ataxia

This code represents Friedreich's ataxia, a rare, inherited, progressive neurodegenerative disorder primarily affecting the spinal cord and cerebellum. It is characterized by progressive gait and limb ataxia, dysarthria, and often includes cardiac involvement (hypertrophic cardiomyopathy) and diabetes mellitus.

Use this code for patients diagnosed with Friedreich's ataxia based on clinical presentation, genetic testing confirming FXN gene mutations, and neurological examination findings. Documentation should clearly indicate the diagnosis of Friedreich's ataxia as the primary or secondary reason for the encounter.

• Always confirm the diagnosis with genetic testing results if available, though clinical diagnosis can suffice.
• Be aware of associated conditions like hypertrophic cardiomyopathy (425.1) or diabetes mellitus (250.xx) and code them separately as secondary diagnoses.
• This is a chronic, progressive condition; expect to see this code used repeatedly for ongoing management and monitoring.