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)

Annotation

do not confuse entry term RAMSAY HUNT CEREBELLAR SYNDROME with RAMSAY HUNT AURICULAR SYNDROME see HERPES ZOSTER OTICUS or RAMSAY HUNT PARALYSIS SYNDROME see PARKINSONIAN DISORDERS

DUI

D002527 MeSH Browser

CUI

M0003859

Previous indexing

Cerebellar Diseases (1966-1969)

History note

2000(1970); use CEREBELLAR ATAXIA 1970-1990

Public note

2000; see CEREBELLAR DYSSYNERGIA 1991-1999; see CEREBELLAR ATAXIA 1970-1990