CIRCUMSCRIBED LOBAR ATROPHY OF THE BRAIN
\sˈɜːkəmskɹˌa͡ɪbd lˈə͡ʊbɑːɹ ˈatɹəfi ɒvðə bɹˈe͡ɪn], \sˈɜːkəmskɹˌaɪbd lˈəʊbɑːɹ ˈatɹəfi ɒvðə bɹˈeɪn], \s_ˈɜː_k_ə_m_s_k_ɹ_ˌaɪ_b_d l_ˈəʊ_b_ɑː_ɹ ˈa_t_ɹ_ə_f_i_ ɒ_v_ð_ə b_ɹ_ˈeɪ_n]\
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A rare form of DEMENTIA that is sometimes familial. Clinical features include APHASIA; APRAXIA; CONFUSION; ANOMIA; memory loss; and personality deterioration. This pattern is consistent with the pathologic findings of circumscribed atrophy of the poles of the FRONTAL LOBE and TEMPORAL LOBE. Neuronal loss is maximal in the HIPPOCAMPUS, entorhinal cortex, and AMYGDALA. Some ballooned cortical neurons contain argentophylic (Pick) bodies. (From Brain Pathol 1998 Apr;8 (2):339-54; Adams et al., Principles of Neurology, 6th ed, pp1057-9)
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Procollagen Proline Dioxygenase
- mixed-function oxygenase that catalyzes hydroxylation prolyl-glycyl-containing-peptide, usually in protocollagen, hydroxyprolylglycyl-peptide. The enzyme utilizes molecular oxygen with a concomitant oxidative decarboxylation of 2-oxoglutarate to succinate. EC 1.14.11.2.