DENTATE NUCLEUS SYNDROME, RAMSAY HUNT
\dˈɛnte͡ɪt njˈuːklɪəs sˈɪndɹə͡ʊm], \dˈɛnteɪt njˈuːklɪəs sˈɪndɹəʊm], \d_ˈɛ_n_t_eɪ_t n_j_ˈuː_k_l_ɪ__ə_s s_ˈɪ_n_d_ɹ_əʊ_m]\
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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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Nearby Words
- dentate gyrus
- dentate layer
- dentate leaf
- dentate ligament
- dentate nucleus
- Dentate Nucleus Syndrome, Ramsay Hunt
- dentate suture
- dentate, dentated
- dentate-ciliate
- dentate-sinuate
- dentated