PROGRESSIVE CHOREA, HEREDITARY, CHRONIC (HUNTINGTON)
\pɹəɡɹˈɛsɪv kɔːɹˈi͡ə], \pɹəɡɹˈɛsɪv kɔːɹˈiə], \p_ɹ_ə_ɡ_ɹ_ˈɛ_s_ɪ_v k_ɔː_ɹ_ˈiə]\
Definitions of PROGRESSIVE CHOREA, HEREDITARY, CHRONIC (HUNTINGTON)
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A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse CHOREA involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
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Word of the day
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.
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