RAMSAY HUNT DENTATE SYNDROME
\ɹˈamse͡ɪ hˈʌnt dˈɛnte͡ɪt sˈɪndɹə͡ʊm], \ɹˈamseɪ hˈʌnt dˈɛnteɪt sˈɪndɹəʊm], \ɹ_ˈa_m_s_eɪ h_ˈʌ_n_t d_ˈɛ_n_t_eɪ_t 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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