Pantothenate kinase-associated neurodegeneration
ICD-10 Code
G23.0
Pantothenate kinase-associated neurodegeneration
Definition
Pantothenate kinase-associated neurodegeneration (PKAN) is the most common type of neurodegeneration with brain iron accumulation (NBIA), a rare neurodegenerative disorder characterized by progressive extrapyramidal dysfunction (dystonia, rigidity, choreoathetosis), iron accumulation on the brain and axonal spheroids in the central nervous system.
Also known as Hallervorden-Spatz disease, Hallervorden-Spatz syndrome, NBIA1, neurodegeneration with brain iron accumulation 1, neurodegeneration with brain iron accumulation type 1, pantothenate kinase-associated neurodegeneration, pigmentary pallidal degeneration, PKAN — per MONDO