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Term:
neurodegeneration with brain iron accumulation (DOID:0110734)
Annotations: Rat: (12) Mouse: (12) Human: (12) Chinchilla: (12) Bonobo: (12) Dog: (12) Squirrel: (12)
Parent Terms Term With Siblings Child Terms
aceruloplasminemia  
agenesis of the corpus callosum with peripheral neuropathy  
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 +   
Hereditary Hyperferritinemia with Congenital Cataracts  
Hunter Carpenter Macdonald Syndrome 
Huntington's disease +   
Huntington's disease-like 2  
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  
Lafora disease  
Maccario Mena Weir Syndrome 
Metabolic Crises, Recurrent, with Variable Encephalomyopathic Features and Neurologic Regression  
METABOLIC ENCEPHALOMYOPATHIC CRISES, RECURRENT, WITH RHABDOMYOLYSIS, CARDIAC ARRHYTHMIAS, AND NEURODEGENERATION  
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  
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 
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.