Submit Data |  Help |  Video Tutorials |  News |  Publications |  FTP Download |  REST API |  Citing RGD |  Contact   

Ontology Browser

Parent Terms Term With Siblings Child Terms
3-methylglutaconic aciduria type 3  
AIDS Dementia Complex  
Alexander Disease  
alexia +  
Alopecia, Hypogonadism, Extrapyramidal Disorder  
Alzheimer's disease +   
amnestic disorder +   
amyotrophic lateral sclerosis-parkinsonism/dementia complex 1  
anxiety disorder +   
Ataxia with Myoclonic Epilepsy and Presenile Dementia 
Auditory Perceptual Disorders +   
basal ganglia calcification +   
basal ganglia cerebrovascular disease +  
Benign Familial Chorea  
biotin-responsive basal ganglia disease  
Brain-Lung-Thyroid Syndrome  
Canavan disease  
Cerebral Amyloidosis with Spongiform Encephalopathy +   
Cerebrocortical Degeneration of Infancy 
childhood spinal muscular atrophy +   
Childhood-Onset Chorea with Psychomotor Retardation  
CHOPS Syndrome  
chorea gravidarum 
Choreoathetosis, Familial Inverted 
Cockayne syndrome +   
Cognitive Dysfunction +   
Cognitive Impairment with or Without Cerebellar Ataxia  
Congenital Muscular Dystrophy with Severe Central Nervous System Atrophy and Absence of Large Myelinated Fibers 
Consciousness Disorders +   
Creutzfeldt-Jakob disease +   
deafness-dystonia-optic neuronopathy syndrome  
dementia +   
Dementia/Parkinsonism with Non-Alzheimer Amyloid Plaques 
Diffuse Neurofibrillary Tangles with Calcification 
Dystonia Musculorum Deformans +   
Familial Amyloid Polyneuropathies +   
familial encephalopathy with neuroserpin inclusion bodies  
frontotemporal dementia +   
Frontotemporal Lobar Degeneration +   
Generalized Epilepsy and Paroxysmal Dyskinesia  
Gerstmann-Straussler-Scheinker syndrome  
Gilles de la Tourette syndrome +   
Hereditary Central Nervous System Demyelinating Diseases +   
Hereditary Optic Atrophies +   
hereditary sensory neuropathy +   
hereditary spastic paraplegia 75  
Huntington's disease +   
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
Huntington's disease-like 2  
Huntington's Disease-Like Syndrome  
ITM2B-related cerebral amyloid angiopathy 2  
juvenile amyotrophic lateral sclerosis with dementia 
Kennedy's disease  
Kluver-Bucy syndrome +  
Kohlschutter-Tonz syndrome  
Lafora disease  
Lewy body dementia +   
Lipodystrophy with Congenital Cataracts and Neurodegeneration  
MAST syndrome  
Meige syndrome 
Mental Retardation, X-Linked +   
mitochondrial DNA depletion syndrome 6  
mood disorder +   
Motor Neuron Disease with Dementia and Ophthalmoplegia 
motor peripheral neuropathy +   
multiple system atrophy +   
myotonia congenita +   
myotonic dystrophy type 1 +   
Neuhauser Eichner Opitz Syndrome 
neuroacanthocytosis +   
neurofibromatosis +   
neurogenic scapuloperoneal syndrome Kaeser type  
neuroleptic malignant syndrome 
neuronal ceroid lipofuscinosis +   
Opticocochleodentate Degeneration 
pantothenate kinase-associated neurodegeneration +   
Parkinsonian Disorders +   
paroxysmal nonkinesigenic dyskinesia 1  
paroxysmal nonkinesigenic dyskinesia 2 
PCWH syndrome  
Peripheral Neuropathy, Ataxia, Focal Necrotizing Encephalopathy, and Spongy Degeneration of Brain 
Premature Atherosclerosis with Deafness, Nephropathy, Diabetes Mellitus, Photomyoclonus, and Degenerative Neurologic Disease 
Presenile Dementia, Kraepelin Type 
primary cerebellar degeneration +   
Primary Progressive Aphasia +   
Progressive Pallidal Degeneration with Retinitis Pigmentosa 
Progressive Psychomotor Deterioration  
progressive supranuclear palsy +   
psychotic disorder +   
Remitting Chorea with Nystagmus and Cataracts 
Spastic Paraplegia with Associated Extrapyramidal Signs 
Spastic Paraplegia, Optic Atrophy, and Dementia 
spinal muscular atrophy with lower extremity predominance +   
Spongiform Encephalopathy with Neuropsychiatric Features  
subacute delirium +   
syndromic microphthalmia 10 
syndromic X-linked intellectual disability 5  
tuberous sclerosis +   
Unverricht-Lundborg syndrome +   
vascular dementia +   
Waisman syndrome  
Wilson disease +   
Woodhouse-Sakati Syndrome  
Wright Dyck Syndrome 

Exact Synonyms: HD ;   Huntington Chronic Progressive Hereditary Chorea ;   Huntington Disease ;   Huntington Disease, Akinetic Rigid Variant ;   Huntington chorea ;   Huntington's Chorea ;   Juvenile Huntington Disease ;   akinetic rigid variant of Huntington disease ;   juvenile onset Huntington disease ;   late onset Huntington disease
Primary IDs: MESH:D006816
Alternate IDs: OMIM:143100 ;   RDO:0002347
Xrefs: GARD:6677 ;   ICD9CM:333.4 ;   NCI:C82342
Definition Sources: MESH:D006816, url: "DO", url: "DO"

paths to the root


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