DENTATE CEREBELLAR ATAXIA
\dˈɛnte͡ɪt sˈɛɹɪbˌɛləɹ atˈe͡ɪksi͡ə], \dˈɛnteɪt sˈɛɹɪbˌɛləɹ atˈeɪksiə], \d_ˈɛ_n_t_eɪ_t s_ˈɛ_ɹ_ɪ_b_ˌɛ_l_ə_ɹ a_t_ˈeɪ_k_s_iə]\
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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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