JAKOB CREUTZFELDT SYNDROME
\d͡ʒˈakɒb krjˈuːtsfɛlt sˈɪndɹə͡ʊm], \dʒˈakɒb krjˈuːtsfɛlt sˈɪndɹəʊm], \dʒ_ˈa_k_ɒ_b k_r_j_ˈuː_t_s_f_ɛ_l_t s_ˈɪ_n_d_ɹ_əʊ_m]\
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A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339 (27))
By DataStellar Co., Ltd
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