Neuropathy in association with hereditary ataxia

This code signifies a peripheral neuropathy that occurs as a secondary manifestation or complication of a genetically inherited ataxia. It describes the nerve damage (neuropathy) that is directly linked to an underlying hereditary disorder affecting coordination and balance (ataxia).

Use this code when documentation clearly indicates a diagnosis of peripheral neuropathy in a patient with a confirmed hereditary ataxia. This typically requires a neurologist's assessment, genetic testing results, and nerve conduction studies or electromyography confirming the neuropathy.

• Always code the specific type of hereditary ataxia first, if known and documented, as the primary diagnosis.
• Ensure documentation specifies "hereditary" ataxia; do not use for acquired or idiopathic ataxia with neuropathy.
• Distinguish from other forms of neuropathy not associated with hereditary ataxia, such as diabetic neuropathy or inflammatory neuropathies.