Familial dysautonomia [Riley-Day]

This code identifies a rare, inherited neurological disorder affecting the autonomic nervous system. It primarily impacts sensory perception, blood pressure regulation, tear production, and temperature control, leading to a wide range of systemic dysfunctions. Patients often present with absent fungiform papillae of the tongue, diminished pain and temperature sensation, and labile blood pressure.

Assign this code for patients diagnosed with familial dysautonomia, also known as Riley-Day syndrome. Documentation should clearly indicate the genetic basis or the constellation of classic clinical signs and symptoms consistent with this specific autonomic neuropathy. This code is appropriate for both initial diagnosis and ongoing management of the condition.

• Ensure documentation specifies "familial dysautonomia" or "Riley-Day syndrome" to differentiate from other autonomic neuropathies.
• Consider sequencing this code as a primary diagnosis when it is the focus of the encounter, or as a secondary diagnosis when managing complications.
• Do not confuse with other generalized dysautonomias (e.g., G90.8) unless the specific familial form is ruled out.