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Myoclonic Cerebellar Dyssynergia MeSH Descriptor Data 2025
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)
Entry Term(s)
Cerebellar Dyssynergia
Cerebelloparenchymal Disorder V
Dentate Cerebellar Ataxia
Dentate Cerebellar Atrophy
Dentate Nucleus Syndrome, Ramsay Hunt
Dyssynergia Cerebellaris Myoclonica
Dyssynergia Cerebellaris Myoclonica Of Hunt
Dyssynergia Cerebellaris Progressiva
Ramsay Hunt Cerebellar Syndrome
Ramsay Hunt Dentate Syndrome
Spinodentate Atrophy
Previous Indexing
Cerebellar Diseases (1966-1969)
Public MeSH Note
2000; see CEREBELLAR DYSSYNERGIA 1991-1999; see CEREBELLAR ATAXIA 1970-1990
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)