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)

Synonyms: Ramsay Hunt Dentate Syndrome, Dyssynergia Cerebellaris Myoclonica Of Hunt, Spinodentate Atrophy, Cerebellar Ataxias, Dentate, Cerebellar Dyssynergia, Dyssynergias, Cerebellar, Dentate Cerebellar Atrophy, Dentate Nucleus Syndrome, Ramsay Hunt, Ataxias, Dentate Cerebellar, Dyssynergias, Myoclonic Cerebellar, Dentate Cerebellar Ataxia, Cerebelloparenchymal Disorder V, Myoclonic Cerebellar Dyssynergias, Dentate Cerebellar Atrophies, Ataxia, Dentate Cerebellar, Cerebellar Dyssynergias, Dyssynergia, Myoclonic Cerebellar, Cerebellar Dyssynergias, Myoclonic, Atrophies, Dentate Cerebellar, Cerebellar Atrophy, Dentate, Atrophy, Dentate Cerebellar, Dyssynergia, Cerebellar, Myoclonic Cerebellar Dyssynergia, Ramsay Hunt Cerebellar Syndrome, Dentate Cerebellar Ataxias, Dyssynergia Cerebellaris Myoclonica, Dyssynergia Cerebellaris Progressiva, Cerebellar Dyssynergia, Myoclonic