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Dictionary » Dentate nucleus syndrome, Ramsay Hunt
 

Dentate nucleus syndrome, Ramsay Hunt

Introduction: Dentate nucleus syndrome, Ramsay Hunt

Description of Dentate nucleus syndrome, Ramsay Hunt

Dentate nucleus syndrome, Ramsay Hunt: 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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