Term Name
neurodegeneration with brain iron accumulation
External Reference
Synonyms
neurodegeneration with brain iron accumulation, NBIA
Definition
Neurodegeneration with brain iron accumulation (NBIA, formerly Hallervorden-Spatz syndrome) encompasses a group of rare neurodegenerative disorders characterized by progressive extrapyramidal dysfunction (dystonia, rigidity, choreoathetosis), iron accumulation in the brain and the presence of axonal spheroids, usually limited to the central nervous system.