Disease association ontology term - MONDO:0018307 - neurodegeneration with brain iron accumulation
Term summary
- ID
- MONDO:0018307
- Name
- neurodegeneration with brain iron accumulation
- Ontology or CV name
- Disease association
- 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.
