CONGENITAL ABSENCE OF CEREBRAL HEMISPHERES
\kənd͡ʒˈɛnɪtə͡l ˈabsəns ɒv səɹˈiːbɹə͡l hˈɛmɪsfˌi͡əz], \kəndʒˈɛnɪtəl ˈabsəns ɒv səɹˈiːbɹəl hˈɛmɪsfˌiəz], \k_ə_n_dʒ_ˈɛ_n_ɪ_t_əl ˈa_b_s_ə_n_s ɒ_v s_ə_ɹ_ˈiː_b_ɹ_əl h_ˈɛ_m_ɪ_s_f_ˌiə_z]\
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A congenital condition where the greater portions of the cerebral hemispheres and CORPUS STRIATUM are replaced by CSF and glial tissue. The meninges and the skull are well formed, which is consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusions of the internal carotid arteries in utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. (Menkes, Textbook of Child Neurology, 5th ed, p307)
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Dopamine Acetyltransferase
- An enzyme that catalyzes the of groups from acetyl-CoA to arylamines. They have wide specificity for aromatic amines, particularly serotonin, and can also catalyze acetyl transfer between arylamines without CoA. EC 2.3.1.5.