Neuropathic heredofamilial amyloidosis

This code describes a genetic disorder characterized by the abnormal deposition of amyloid protein in nerves and other tissues, leading to progressive peripheral neuropathy and autonomic dysfunction. It encompasses various forms of inherited amyloidosis primarily affecting the nervous system.

Use this code for patients diagnosed with a hereditary form of amyloidosis where the predominant clinical manifestation is neuropathy. Documentation should clearly state a diagnosis of "heredofamilial amyloidosis with neuropathy," "familial amyloid polyneuropathy," or a specific genetic type like ATTRv amyloidosis with neurological involvement.

• Do not use this code for acquired or localized amyloidosis; ensure the documentation specifies a hereditary form.
• Consider additional codes for specific manifestations, such as cardiac involvement (I43) or renal failure (N18.x), if documented.
• Always review genetic testing results or family history when available to confirm the hereditary nature of the amyloidosis.