Friedreich ataxia

This code represents Friedreich ataxia, a rare, progressive, neurodegenerative disorder characterized by degeneration of the spinal cord, cerebellum, and peripheral nerves. It primarily affects coordination, balance, and speech, leading to gait ataxia, dysarthria, and often cardiomyopathy.

Use this code when documenting a confirmed diagnosis of Friedreich ataxia. Supporting documentation should include neurological examination findings consistent with the disease, genetic testing confirming the FXN gene mutation, or a definitive clinical diagnosis by a neurologist.

• Ensure documentation specifies "Friedreich ataxia" to differentiate from other hereditary ataxias.
• Code any associated manifestations, such as cardiomyopathy (I42.x), diabetes mellitus (E10.x), or scoliosis (M41.x), as secondary diagnoses.
• Do not confuse with other hereditary ataxias like cerebellar ataxia (G11.x) or spastic ataxia (G11.4).