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Term:
neurodegeneration with brain iron accumulation (DOID:0110734)
Annotations: Rat: (13) Mouse: (13) Human: (13) Chinchilla: (13) Bonobo: (13) Dog: (13) Squirrel: (13) Pig: (13)
Parent Terms Term With Siblings Child Terms
aceruloplasminemia  
agenesis of the corpus callosum with peripheral neuropathy  
BRAIN ABNORMALITIES, NEURODEGENERATION, AND DYSOSTEOSCLEROSIS  
cerebral folate receptor alpha deficiency  
Childhood-Onset Neurodegeneration with Ataxia, Dystonia, and Gaze Palsy  
Childhood-Onset Neurodegeneration with Brain Atrophy  
Childhood-Onset Neurodegeneration with Cerebellar Atrophy  
Chronic Traumatic Encephalopathy 
demyelinating disease +   
eye degenerative disease +   
eyelid degenerative disease +  
Feigenbaum Bergeron Richardson Syndrome 
FINCA Syndrome  
Heme Oxygenase 1 Deficiency  
hemosiderosis +   
hereditary ataxia +   
Hunter Carpenter Macdonald Syndrome 
Huntington's disease-like 2  
hyperferritinemia-cataract syndrome  
Idiopathic Basal Ganglia Calcification 1  
Idiopathic Basal Ganglia Calcification 6  
Idiopathic Basal Ganglia Calcification 7  
infantile cerebellar-retinal degeneration  
iron deficiency anemia +   
Iron Overload +   
Karak Syndrome  
L-Ferritin Deficiency  
Maccario Mena Weir Syndrome 
Metabolic Crises, Recurrent, with Variable Encephalomyopathic Features and Neurologic Regression  
motor neuron disease +   
multiple system atrophy +   
myoclonic cerebellar dyssynergia +  
Nervous System Heredodegenerative Disorders +   
neurodegeneration with brain iron accumulation +   
A neurodegenerative disease characterized by progressive iron accumulation in the basal ganglia and other regions of the brain, resulting in extrapyramidal movements, such as parkinsonism and dystonia. (DO)
NEURODEGENERATION, CHILDHOOD-ONSET, STRESS-INDUCED, WITH VARIABLE ATAXIA AND SEIZURES  
NEURODEGENERATION, EARLY-ONSET, WITH CHOREOATHETOID MOVEMENTS AND MICROCYTIC ANEMIA  
Neuronal Intranuclear Inclusion Disease  
olivopontocerebellar atrophy +   
Osteopetrosis and Infantile Neuroaxonal Dystrophy 
pantothenate kinase-associated neurodegeneration +   
Paraneoplastic Syndromes, Nervous System +   
PEHO syndrome  
Pick's disease  
plexopathy 
pontocerebellar hypoplasia +   
postpoliomyelitis syndrome 
primary cerebellar degeneration +   
prion disease +   
Radiation Sensitivity Chromosome Instability Syndrome, Autosomal Dominant 
Recurrent Metabolic Encephalomyopathic Crises with Rhabdomyolysis, Cardiac Arrhythmias, and Neurodegeneration  
Schindler Disease, Type I  
secondary Parkinson disease +   
Spastic Pseudosclerosis 
Subacute Combined Degeneration  
synucleinopathy +   
tauopathy +   
TDP-43 Proteinopathies +   

Synonyms
Exact Synonyms: NBIA ;   Neurodegeneration with brain iron accumulation (NBIA)
Narrow Synonyms: Neuroaxonal dystrophy, PLA2G6-related ;   Neurodegeneration with brain iron accumulation 1, atypical
Related Synonyms: Iron Accumulation In Brain
Primary IDs: MESH:C538421
Alternate IDs: OMIA:002105 ;   RDO:0004395
Xrefs: GARD:11899 ;   OMIM:PS234200 ;   ORDO:385
Definition Sources: https://rarediseases.info.nih.gov/diseases/11899/index, https://www.ncbi.nlm.nih.gov/pubmed/18981035, https://www.ninds.nih.gov/Disorders/All-Disorders/Neurodegeneration-Brain-Iron-Accumulation-Information-Page

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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.