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Term:
Childhood-onset Neurodegeneration with Ataxia, Tremor, Optic Atrophy, and Cognitive Decline (DOID:9002043)
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Parent Terms Term With Siblings Child Terms
Ataxia +     
optic atrophy +     
Tremor +     
3-methylglutaconic aciduria type 3  
Abetalipoproteinemia Neuropathy  
aceruloplasminemia  
adult-onset ataxia and polyneuropathy  
agenesis of the corpus callosum with peripheral neuropathy  
Al Gazali Khidr Prem Chandran Syndrome 
Al Gazali Sabrinathan Nair Syndrome 
Arts syndrome  
Ataxia with Fasciculations 
Ataxia with Myoclonic Epilepsy and Presenile Dementia 
Ataxia, Deafness, and Cardiomyopathy 
ATAXIA, PROGRESSIVE SEIZURES, MENTAL DETERIORATION, AND HEARING LOSS  
ATAXIA, SPASTIC, CHILDHOOD-ONSET, AUTOSOMAL RECESSIVE, WITH OPTIC ATROPHY AND MENTAL RETARDATION  
Ataxia-Microcephaly-Cataract Syndrome 
Ataxia-Oculomotor Apraxia 4  
ataxic cerebral palsy 
Atonic-Astatic Syndrome of Foerster 
Bangstad Syndrome 
Behr syndrome  
Benign Childhood Paroxysmal Tonic Upgaze with Ataxia 
Bhaskar Jagannathan Syndrome 
BRAIN ABNORMALITIES, NEURODEGENERATION, AND DYSOSTEOSCLEROSIS  
CANOMAD Syndrome 
CAPOS Syndrome  
Carnitine Acetyltransferase Deficiency  
Cataract Ataxia Deafness 
Cataracts, Ataxia, Short Stature, and Mental Retardation 
Cerebroretinal Microangiopathy with Calcifications and Cysts +   
Childhood-Onset Neurodegeneration with Ataxia, Dystonia, and Gaze Palsy  
Childhood-onset Neurodegeneration with Ataxia, Tremor, Optic Atrophy, and Cognitive Decline  
An autosomal recessive progressive disorder with onset of symptoms in the first decade.
Childhood-Onset Neurodegeneration with Brain Atrophy  
Childhood-Onset Neurodegeneration with Cerebellar Atrophy  
Christianson syndrome  
Chronic Traumatic Encephalopathy 
COACH syndrome +   
coenzyme Q10 deficiency disease +   
Congenital Myopathy with Tremor  
Deafness Hyperuricemia Neurologic Ataxia 
deafness-dystonia-optic neuronopathy syndrome  
demyelinating disease +   
Diaminopentanuria 
DYSTONIA, CHILDHOOD-ONSET, WITH OPTIC ATROPHY AND BASAL GANGLIA ABNORMALITIES  
Episodic Mitochondrial Myopathy with or without Optic Atrophy and Reversible Leukoencephalopathy  
Erythrokeratodermia with Ataxia 
essential tremor +   
eye degenerative disease +   
eyelid degenerative disease +  
familial isolated deficiency of vitamin E  
Feigenbaum Bergeron Richardson Syndrome 
FINCA Syndrome  
fragile X-associated tremor/ataxia syndrome  
Gait Ataxia +   
Geniospasm 
glaucomatous atrophy of optic disc 
Global Developmental Delay, Progressive Ataxia, and Elevated Glutamine  
hereditary ataxia +   
Hereditary Optic Atrophies +   
Huntington's disease-like 2  
hypomyelinating leukodystrophy 7 with or without oligodontia and-or hypogonadotropic hypogonadism  
HYPOTONIA, ATAXIA, AND DELAYED DEVELOPMENT SYNDROME  
HYPOTONIA, ATAXIA, DEVELOPMENTAL DELAY, AND TOOTH ENAMEL DEFECT SYNDROME  
Hypotonia, Congenital Nystagmus, Ataxia and Abnormal Auditory Brainstem Response 
Idiopathic Basal Ganglia Calcification 1  
Idiopathic Basal Ganglia Calcification 6  
Idiopathic Basal Ganglia Calcification 7  
infantile cerebellar-retinal degeneration  
Joubert syndrome 7  
Leukoencephalopathy with Ataxia  
LEUKOENCEPHALOPATHY, DEVELOPMENTAL DELAY, AND EPISODIC NEUROLOGIC REGRESSION SYNDROME  
Mental Retardation with Optic Atrophy, Facial Dysmorphism, Microcephaly, and Short Stature 
Mitochondrial Myopathy, and Ataxia  
motor neuron disease +   
multiple system atrophy +   
Muscular Atrophy, Ataxia, Retinitis Pigmentosa, and Diabetes Mellitus 
myoclonic cerebellar dyssynergia +  
Myokymia 1  
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 Impaired Intellectual Development, Hypotonia, and Ataxia   
NEURODEVELOPMENTAL DISORDER WITH SPASTIC QUADRIPLEGIA, OPTIC ATROPHY, SEIZURES, AND STRUCTURAL BRAIN ANOMALIES  
Neuronal Intranuclear Inclusion Disease  
olivopontocerebellar atrophy +   
optic atrophy 10  
Optic Atrophy, Hearing Loss, and Peripheral Neuropathy, Autosomal Dominant 
OPTIC ATROPHY-ATAXIA-PERIPHERAL NEUROPATHY-GLOBAL DEVELOPMENTAL DELAY SYNDROME  
partial optic atrophy 
Partington syndrome  
PEHO syndrome  
Peripheral Neuropathy, Ataxia, Focal Necrotizing Encephalopathy, and Spongy Degeneration of Brain 
PHARC syndrome  
plexopathy 
pontocerebellar hypoplasia +   
Posterior Column Ataxia with Retinitis Pigmentosa  
postpoliomyelitis syndrome 
primary cerebellar degeneration +   
primary optic atrophy 
Primary Orthostatic Tremor 
prion disease +   
Progressive Encephalopathy with Amyotrophy and Optic Atrophy  
progressive myoclonus epilepsy 1B  
Radiation Sensitivity Chromosome Instability Syndrome, Autosomal Dominant 
Reardon Wilson Cavanagh Syndrome 
Recurrent Metabolic Crises with Variable Encephalomyopathic Features and Neurologic Regression  
Richards-Rundle Syndrome 
secondary Parkinson disease +   
SEIZURES, EARLY-ONSET, WITH NEURODEGENERATION AND BRAIN CALCIFICATIONS  
Sensory Ataxia, Autosomal Dominant  
Silengo Lerone Pelizza Syndrome 
spastic ataxia +   
Spastic Ataxia with Congenital Miosis 
Spastic Paraplegia, Ataxia, and Mental Retardation 
Spastic Pseudosclerosis 
Spinocerebellar Ataxias +   
SPOAN syndrome  
spondyloepimetaphyseal dysplasia, Genevieve-type  
stress-induced childhood-onset neurodegeneration with variable ataxia and seizures  
Subacute Combined Degeneration  
synucleinopathy +   
Tapetoretinal Degeneration with Ataxia 
tauopathy +   
TDP-43 Proteinopathies +   
Treft Sanborn Carey Syndrome 
Tremor of Intention, Ataxia, and Lipofuscinosis 
Tryptophanuria with Dwarfism 
Warburg micro syndrome +   
X-Linked Mental Retardation Gustavson Type 

Synonyms
Exact Synonyms: CONATOC
Primary IDs: OMIM:618868
Definition Sources: OMIM:618868

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