neurodegeneration with brain iron accumulation
MONDO:0018307Mondo
Findings
No curated finding names neurodegeneration with brain iron accumulation yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0018307), read 2026-09-29. CC BY 4.0.
Where it sits
- A kind of
- Narrower terms (14)
- aceruloplasminemia
- early-onset progressive encephalopathy-spastic ataxia-distal spinal muscular atrophy syndrome
- fatty acid hydroxylase-associated neurodegeneration
- Kufor-Rakeb syndrome
- neurodegeneration with brain iron accumulation 4
- neurodegeneration with brain iron accumulation 5
- neurodegeneration with brain iron accumulation 6
- neurodegeneration with brain iron accumulation 7
- neurodegeneration with brain iron accumulation 8
- neurodegeneration with brain iron accumulation 9
- neuroferritinopathy
- pantothenate kinase-associated neurodegeneration
- PLA2G6-associated neurodegeneration
- Woodhouse-Sakati syndrome
Other names
1 name
Resolves to: neurodegeneration with brain iron accumulation
- Also called
- NBIA