Hallervorden-Spatz disease

This code represents Pantothenate Kinase-Associated Neurodegeneration (PKAN), a rare, inherited neurodegenerative disorder characterized by progressive dystonia, parkinsonism, spasticity, and cognitive decline. It is a form of neurodegeneration with brain iron accumulation (NBIA), specifically linked to mutations in the PANK2 gene.

Use this code for patients diagnosed with Hallervorden-Spatz disease, now more accurately termed Pantothenate Kinase-Associated Neurodegeneration (PKAN). Documentation should clearly indicate a diagnosis of PKAN, often supported by genetic testing confirming PANK2 mutations or characteristic MRI findings (e.g., "eye-of-the-tiger" sign).

• Ensure documentation specifies "Hallervorden-Spatz disease" or "Pantothenate Kinase-Associated Neurodegeneration (PKAN)" to support this specific code.
• Do not confuse this with other forms of Neurodegeneration with Brain Iron Accumulation (NBIA); this code is specific to PKAN.
• Consider sequencing this code as the primary diagnosis when it is the main reason for the encounter, especially for diagnostic workup or management of the disease itself.