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Ontology Browser
Term:
myoclonic cerebellar dyssynergia (DOID:12707)
Annotations: Rat: (0) Mouse: (0) Human: (0) Chinchilla: (0) Bonobo: (0) Dog: (0) Squirrel: (0) Pig: (0)
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 +   
myoclonic cerebellar dyssynergia +  
A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal SEIZURES, spasticity, and DYSKINESIAS. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (From Joynt, Clinical Neurology, 1991, Ch37, pp60-1)
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 CEREBELLAR ATROPHY AND WITH OR WITHOUT SEIZURES  
Neurodevelopmental Disorder with Epilepsy, Spasticity, and Brain Atrophy  
Neuronal Intranuclear Inclusion Disease  
olivopontocerebellar atrophy +   
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  
Spastic Pseudosclerosis 
Spinocerebellar Ataxias +   
stress-induced childhood-onset neurodegeneration with variable ataxia and seizures  
Subacute Combined Degeneration  
synucleinopathy +   
tauopathy +   
TDP-43 Proteinopathies +   

Synonyms
Exact Synonyms: Cerebelloparenchymal Disorder V ;   Dentate Cerebellar Ataxia ;   Dentate Cerebellar Ataxias ;   Dentate Cerebellar Atrophies ;   Dentate Cerebellar Atrophy ;   Dentate Nucleus Syndrome, Ramsay Hunt ;   Dyssynergia Cerebellaris Myoclonica ;   Dyssynergia Cerebellaris Myoclonica Of Hunt ;   Dyssynergia Cerebellaris Progressiva ;   Myoclonic Cerebellar Dyssynergias ;   Ramsay Hunt Dentate Syndrome ;   Ramsay Hunt cerebellar syndrome ;   cerebellar dyssynergia ;   cerebellar dyssynergias ;   progressive cerebellar tremor ;   spinodentate atrophy
Primary IDs: MESH:D002527
Alternate IDs: OMIM:213400 ;   RDO:0005155
Xrefs: GARD:9256 ;   ICD10CM:G11.1
Definition Sources: MESH:D002527

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