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Term:
primary cerebellar degeneration (DOID:9277)
Annotations: Rat: (218) Mouse: (217) Human: (233) Chinchilla: (206) Bonobo: (213) Dog: (217) Squirrel: (207) Pig: (213)
Parent Terms Term With Siblings Child Terms
aceruloplasminemia  
agenesis of the corpus callosum with peripheral neuropathy  
Alexander Disease  
amyotrophic lateral sclerosis +   
Arima Syndrome 
baylisascariasis 
Behrens Baumann Dust Syndrome 
BRAIN ABNORMALITIES, NEURODEGENERATION, AND DYSOSTEOSCLEROSIS  
Canavan disease  
Cerebellar Degeneration-Related Autoantigen 3 
Cerebellar Neoplasms +   
Cerebellar Vermis Aplasia with Associated Features suggesting Smith-Lemli-Opitz Syndrome and Meckel Syndrome 
CEREBELLAR, OCULAR, CRANIOFACIAL, AND GENITAL SYNDROME  
Cerebelloparenchymal Disorder VI 
Cerebrocortical Degeneration of Infancy 
Childhood-Onset Neurodegeneration with Ataxia, Dystonia, and Gaze Palsy  
Childhood-Onset Neurodegeneration with Brain Atrophy  
Childhood-Onset Neurodegeneration with Cerebellar Atrophy  
Chronic Traumatic Encephalopathy 
Cockayne syndrome +   
Congenital Muscular Dystrophy with Severe Central Nervous System Atrophy and Absence of Large Myelinated Fibers 
Dandy-Walker syndrome +   
demyelinating disease +   
Dykes Markes Harper Syndrome 
Dystonia Musculorum Deformans +   
Dystonia with Cerebellar Atrophy 
epidural abscess 
eye degenerative disease +   
eyelid degenerative disease +  
Familial Amyloid Polyneuropathies +   
familial encephalopathy with neuroserpin inclusion bodies  
Feigenbaum Bergeron Richardson Syndrome 
FINCA Syndrome  
Gerstmann-Straussler-Scheinker syndrome  
Gilles de la Tourette syndrome +   
GLYCOSYLPHOSPHATIDYLINOSITOL BIOSYNTHESIS DEFECT 15  
hereditary ataxia +   
Hereditary Central Nervous System Demyelinating Diseases +   
Hereditary Optic Atrophies +   
hereditary sensory neuropathy +   
Huntington's disease +   
Huntington's disease-like 2  
Huntington's Disease-Like Syndrome  
Idiopathic Basal Ganglia Calcification 1  
Idiopathic Basal Ganglia Calcification 6  
Idiopathic Basal Ganglia Calcification 7  
infantile cerebellar-retinal degeneration  
Joubert syndrome +   
Kennedy's disease  
L-2-hydroxyglutaric aciduria  
Lafora disease  
Leukoencephalomyelopathy  
Lipodystrophy with Congenital Cataracts and Neurodegeneration  
Megalencephaly with Thick Corpus Callosum, Cerebellar Atrophy, and Intellectual Disability  
Mental Retardation, X-Linked +   
Metabolic Crises, Recurrent, with Variable Encephalomyopathic Features and Neurologic Regression  
Microphthalmia, Syndromic 10 
Miller Fisher syndrome 
mitochondrial DNA depletion syndrome 6  
motor neuron disease +   
motor peripheral neuropathy +   
multiple system atrophy +   
myelitis +   
myelomeningocele +   
myoclonic cerebellar dyssynergia +  
myotonia congenita +   
myotonic dystrophy type 1 +   
Nervous System Heredodegenerative Disorders +   
neuroacanthocytosis +   
neurodegeneration with brain iron accumulation +   
NEURODEGENERATION, CHILDHOOD-ONSET, STRESS-INDUCED, WITH VARIABLE ATAXIA AND SEIZURES  
NEURODEGENERATION, EARLY-ONSET, WITH CHOREOATHETOID MOVEMENTS AND MICROCYTIC ANEMIA  
neurofibromatosis +   
neuronal ceroid lipofuscinosis +   
Neuronal Intranuclear Inclusion Disease  
neuroschistosomiasis 
olivopontocerebellar atrophy +   
Opticocochleodentate Degeneration 
pantothenate kinase-associated neurodegeneration +   
Paraneoplastic Cerebellar Degeneration 
Paraneoplastic Syndromes, Nervous System +   
PCWH syndrome  
PEHO syndrome  
Peripheral Neuropathy, Ataxia, Focal Necrotizing Encephalopathy, and Spongy Degeneration of Brain 
Pick's disease  
plexopathy 
Pneumorrhachis 
pontocerebellar hypoplasia +   
Porencephaly Cerebellar Hypoplasia Malformations 
PORETTI-BOLTSHAUSER SYNDROME  
postpoliomyelitis syndrome 
Premature Atherosclerosis with Deafness, Nephropathy, Diabetes Mellitus, Photomyoclonus, and Degenerative Neurologic Disease 
primary cerebellar degeneration +   
A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. Sporadic and inherited subtypes occur. Inheritance patterns include autosomal dominant, autosomal recessive, and X-linked.
prion disease +   
Progressive Microcephaly with Seizures and Cerebral and Cerebellar Atrophy  
progressive muscular atrophy  
Radiation Sensitivity Chromosome Instability Syndrome, Autosomal Dominant 
Scapuloperoneal Syndrome, Neurogenic, Kaeser Type  
secondary Parkinson disease +   
Spastic Pseudosclerosis 
Spinal Cord Injuries +   
spinal cord lipoma +  
Spinal Cord Neoplasms +   
Spinal Cord Vascular Diseases +   
Spinal Intradural Arachnoid Cysts 
spinal muscular atrophy +   
Spinal Muscular Atrophy, Lower Extremity-Predominant, 2A, Autosomal Dominant  
Spinal Muscular Atrophy, Lower Extremity-Predominant, 2B, Autosomal Dominant  
Spongiform Encephalopathy with Neuropsychiatric Features  
Stevenson-Carey Syndrome 
Stiff-Person syndrome  
Subacute Cerebellar Degeneration 
Subacute Combined Degeneration  
survival motor neuron spinal muscular atrophy +   
synucleinopathy +   
syringomyelia +   
tabes dorsalis +  
tauopathy +   
TDP-43 Proteinopathies +   
tertiary neurosyphilis +  
tethered spinal cord syndrome 
tuberous sclerosis +   
Unverricht-Lundborg syndrome  
vascular myelopathy 
Wilson disease +   

Synonyms
Exact Synonyms: Corticostriatal-Spinal Degenerations ;   Early Onset Cerebellar Ataxia ;   Familial Spinocerebellar Degeneration ;   Familial Spinocerebellar Degenerations ;   Hereditary Ataxia ;   Hereditary Ataxias ;   Hereditary Oligophrenic Cerebello Lental Degeneration ;   Hereditary Spinocerebellar Degeneration ;   Hereditary Spinocerebellar Degenerations ;   Inherited Spinocerebellar Degeneration ;   Inherited Spinocerebellar Degenerations ;   Late Onset Cerebellar Ataxia ;   MSS ;   Marie Cerebellar Ataxia ;   Marie's Cerebellar Ataxia ;   Primary Cerebellar Degenerations ;   Spino Cerebellar Degeneration ;   Spino Cerebellar Degenerations ;   Spinocerebellar Degeneration ;   corticostriatal spinal degeneration ;   spinocerebellar degenerations ;   spinocerebellar disease ;   spinocerebellar diseases
Narrow Synonyms: SENSORIMOTOR NEUROPATHY
Primary IDs: MESH:D013132
Alternate IDs: OMIM:248800 ;   RDO:0000109
Definition Sources: MESH:D013132

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