PROGRESSIVE AUTONOMIC FAILURE
\pɹəɡɹˈɛsɪv ɔːtənˈɒmɪk fˈe͡ɪli͡ə], \pɹəɡɹˈɛsɪv ɔːtənˈɒmɪk fˈeɪliə], \p_ɹ_ə_ɡ_ɹ_ˈɛ_s_ɪ_v ɔː_t_ə_n_ˈɒ_m_ɪ_k f_ˈeɪ_l_iə]\
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A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord, which differentiates this condition from other forms of idiopathic orthostatic hypotension (HYPOTENSION, ORTHOSTATIC). This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with orthostasis and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536)
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Word of the day
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.