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Ontology Browser
Term:
olivopontocerebellar atrophy (DOID:14784)
Annotations: Rat: (10) Mouse: (10) Human: (12) Chinchilla: (10) Bonobo: (11) Dog: (10) Squirrel: (10) Pig: (10)
Parent Terms Term With Siblings Child Terms
aceruloplasminemia  
agenesis of the corpus callosum with peripheral neuropathy  
autosomal recessive spinocerebellar ataxia 19  
autosomal recessive spinocerebellar ataxia 2  
BRAIN ABNORMALITIES, NEURODEGENERATION, AND DYSOSTEOSCLEROSIS  
Cerebellar Atrophy with Seizures and Variable Developmental Delay  
CEREBELLAR ATROPHY, DEVELOPMENTAL DELAY, AND SEIZURES  
Cerebellar Atrophy, Visual Impairment, and Psychomotor Retardation  
Childhood-Onset Neurodegeneration with Ataxia, Dystonia, and Gaze Palsy  
Childhood-onset Neurodegeneration with Ataxia, Tremor, Optic Atrophy, and Cognitive Decline  
Childhood-Onset Neurodegeneration with Brain Atrophy  
Childhood-Onset Neurodegeneration with Cerebellar Atrophy  
Chronic Traumatic Encephalopathy 
Corneal Cerebellar Syndrome 
demyelinating disease +   
eye degenerative disease +   
eyelid degenerative disease +  
Feigenbaum Bergeron Richardson Syndrome 
FINCA Syndrome  
Friedreich ataxia +   
Galloway-Mowat syndrome +   
Growth Failure, Microcephaly, Mental Retardation, Cataracts, Large Joint Contractures, Osteoporosis, Cortical Dysplasia, and Cerebellar Atrophy 
hereditary ataxia +   
Hereditary Spinal Ataxia 
Huntington's disease-like 2  
Idiopathic Basal Ganglia Calcification 1  
Idiopathic Basal Ganglia Calcification 6  
Idiopathic Basal Ganglia Calcification 7  
infantile cerebellar-retinal degeneration  
LEUKOENCEPHALOPATHY, DEVELOPMENTAL DELAY, AND EPISODIC NEUROLOGIC REGRESSION SYNDROME  
motor neuron disease +   
Mousa Al din Al Nassar Syndrome 
multiple system atrophy +   
Multiple System Atrophy (MSA) with Orthostatic Hypotension 
myoclonic cerebellar dyssynergia +  
Nervous System Heredodegenerative Disorders +   
Nervous System Paraneoplastic Syndromes +   
neurodegeneration with brain iron accumulation +   
NEURODEGENERATION, EARLY-ONSET, WITH CHOREOATHETOID MOVEMENTS AND MICROCYTIC ANEMIA  
NEURODEGENERATION, INFANTILE-ONSET, BIOTIN-RESPONSIVE  
NEURODEVELOPMENTAL DISORDER WITH CEREBELLAR ATROPHY AND WITH OR WITHOUT SEIZURES  
Neuronal Intranuclear Inclusion Disease  
olivopontocerebellar atrophy +   
A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085)
PEHO syndrome  
plexopathy 
pontocerebellar hypoplasia +   
Posterior Column Ataxia 
postpoliomyelitis syndrome 
primary cerebellar degeneration +   
prion disease +   
Radiation Sensitivity Chromosome Instability Syndrome, Autosomal Dominant 
Recurrent Metabolic Crises with Variable Encephalomyopathic Features and Neurologic Regression  
secondary Parkinson disease +   
SEIZURES, EARLY-ONSET, WITH NEURODEGENERATION AND BRAIN CALCIFICATIONS  
Shy-Drager Syndrome  
Spastic Pseudosclerosis 
Spinocerebellar Ataxias +   
stress-induced childhood-onset neurodegeneration with variable ataxia and seizures  
striatonigral degeneration +   
Subacute Combined Degeneration  
synucleinopathy +   
tauopathy +   
TDP-43 Proteinopathies +   

Synonyms
Exact Synonyms: Dejerine Thomas syndrome ;   Familial Olivopontocerebellar Atrophies ;   Familial Olivopontocerebellar Atrophy ;   Idiopathic Olivopontocerebellar Atrophies ;   Idiopathic Olivopontocerebellar Atrophy ;   Inherited Olivopontocerebellar Atrophies ;   Inherited Olivopontocerebellar Atrophy ;   Nonfamilial Olivopontocerebellar Atrophies ;   Nonfamilial Olivopontocerebellar Atrophy ;   Olivo Ponto Cerebellar Atrophy ;   Olivo Ponto Cerebellar Degeneration ;   Olivo-Ponto-Cerebellar Degenerations ;   Olivopontocerebellar Degeneration ;   Olivopontocerebellar Degenerations ;   Olivopontocerebellar Hypoplasia ;   Pontoolivocerebellar Atrophies ;   Pontoolivocerebellar Atrophy ;   Presenile Ataxia ;   Thomas' syndrome ;   WADIA-SWAMI SYNDROME ;   olivopontocerebellar atrophies ;   presenile ataxias
Primary IDs: MESH:D009849 ;   RDO:0002378
Xrefs: NCI:C84947
Definition Sources: http://www.ninds.nih.gov/disorders/opca/opca.htm "DO", MESH:D009849

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