Ramsay Hunt Dentate Syndrome

《英文msh詞典》Ramsay Hunt Dentate Syndrome
[入口詞] Ramsay Hunt Dentate Syndrome
[主題詞] Myoclonic Cerebellar Dyssynergia
[英文釋義] 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)