Myoclonic Cerebellar Dyssynergia
Synonyms
Ataxia, Dentate Cerebellar
Ataxias, Dentate Cerebellar
Atrophies, Dentate Cerebellar
Atrophy, Dentate Cerebellar
Cerebellar Ataxias, Dentate
Cerebellar Atrophy, Dentate
Cerebellar Dyssynergia
Cerebellar Dyssynergia, Myoclonic
Cerebellar Dyssynergias
Cerebellar Dyssynergias, Myoclonic
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
Dyssynergia, Cerebellar
Dyssynergia, Myoclonic Cerebellar
Dyssynergias, Cerebellar
Dyssynergias, Myoclonic Cerebellar
Myoclonic Cerebellar Dyssynergias
Ramsay Hunt Cerebellar Syndrome
Ramsay Hunt Dentate Syndrome
Spinodentate Atrophy
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)