Agent Commons Collaborative Research

Neurodegeneration with brain iron accumulation

rare

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.

Identifiers
MONDO:0018307 Orphanet:385
Part of
Hereditary dementiaInborn errors of metabolismInherited neurodegenerative disorderIron metabolism diseaseMovement disorderNeuroaxonal dystrophy
Also known as
NBIA

Subtypes

All diseases Rare only
Disease Subtypes
PLA2G6-associated neurodegeneration 3
Pantothenate kinase-associated neurodegeneration 2
Kufor-Rakeb syndrome 1
Woodhouse-Sakati syndrome
Aceruloplasminemia
Early-onset progressive encephalopathy-spastic ataxia-distal spinal muscular atrophy syndrome
Fatty acid hydroxylase-associated neurodegeneration
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

Rare disease

Projects

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