Myoclonic Cerebellar Dyssynergia

**Definition:** 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)

**Tree numbers:** - C10.228.140.252.700.250 - C10.228.854.787.500 - C10.574.500.825.250 - C16.320.400.780.500

**Synonyms:** - Dentate Cerebellar Atrophy - Dentate Nucleus Syndrome, Ramsay Hunt - Dyssynergia Cerebellaris Myoclonica - Dyssynergia Cerebellaris Progressiva - Ramsay Hunt Cerebellar Syndrome - Ramsay Hunt Dentate Syndrome - Dentate Cerebellar Ataxia - Cerebellar Dyssynergia