Other terms
Cerebellar Dyssynergia, Myoclonic,Cerebellar Dyssynergias, Myoclonic,Dyssynergia, Myoclonic Cerebellar,Dyssynergias, Myoclonic Cerebellar,Myoclonic Cerebellar Dyssynergias,Dentate Cerebellar Ataxia,Ataxia, Dentate Cerebellar,Ataxias, Dentate Cerebellar,Cerebellar Ataxias, Dentate,Dentate Cerebellar Ataxias,Dentate Cerebellar Atrophy,Atrophies, Dentate Cerebellar,Atrophy, Dentate Cerebellar,Cerebellar Atrophy, Dentate,Dentate Cerebellar Atrophies,Ramsay Hunt Dentate Syndrome,Dyssynergia Cerebellaris Myoclonica,Dyssynergia Cerebellaris Progressiva,Ramsay Hunt Cerebellar Syndrome,Cerebellar Dyssynergia,Cerebellar Dyssynergias,Dyssynergia, Cerebellar,Dyssynergias, Cerebellar,Dentate Nucleus Syndrome, Ramsay Hunt
Description
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)
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