DENTATE CEREBELLAR ATROPHY
\dˈɛnte͡ɪt sˈɛɹɪbˌɛləɹ ˈatɹəfi], \dˈɛnteɪt sˈɛɹɪbˌɛləɹ ˈatɹəfi], \d_ˈɛ_n_t_eɪ_t s_ˈɛ_ɹ_ɪ_b_ˌɛ_l_ə_ɹ ˈa_t_ɹ_ə_f_i]\
Sort: Oldest first
-
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)
By DataStellar Co., Ltd
