Neurodegeneration with brain iron accumulation
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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.
Synonyms: NBIA
This is just here as a test because I lose it
Term information
database cross reference
- ICD-10:G23.0 (NTBT (ORPHA code's Narrower Term maps to a Broader Term))
- ICD-10:G23.0 (Attributed (The ICD10 code is attributed by Orphanet))
- MeSH:C538421 (E (Exact mapping: the two concepts are equivalent))
- UMLS:C2931845 (E (Exact mapping: the two concepts are equivalent))
Term relations
Subclass of:
- Genetic neurodegenerative disease with dementia
- Neurometabolic disease
- Miscellaneous movement disorder due to genetic neurodegenerative disease
- Miscellaneous movement disorder due to neurodegenerative disease
- Neurodegenerative disease with dementia
- has_inheritance some X-linked dominant
- present_in some Europe and has_point_prevalence_range some 1-9 / 1 000 000 and has_point_prevalence_average_value value 0.2
- has_age_of_onset some adolescent
- has_inheritance some autosomal recessive
- has_inheritance some autosomal dominant
- has_age_of_onset some childhood
- has_age_of_onset some infancy
- has_age_of_onset some adult
Related from:
part_of
- Woodhouse-Sakati syndrome
- Fatty acid hydroxylase-associated neurodegeneration
- Kufor-Rakeb syndrome
- Beta-propeller protein-associated neurodegeneration
- Aceruloplasminemia
- Early-onset progressive encephalopathy-spastic ataxia-distal spinal muscular atrophy syndrome
- COASY protein-associated neurodegeneration
- Mitochondrial membrane protein-associated neurodegeneration
- Neuroferritinopathy
- Pantothenate kinase-associated neurodegeneration