EMERY DREIFUSS SYNDROME
\ˈɛməɹi dɹˈe͡ɪfʌs sˈɪndɹə͡ʊm], \ˈɛməɹi dɹˈeɪfʌs sˈɪndɹəʊm], \ˈɛ_m_ə_ɹ_i d_ɹ_ˈeɪ_f_ʌ_s s_ˈɪ_n_d_ɹ_əʊ_m]\
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An X-linked recessive muscular dystrophy that may present in children or adults. The disease is characterized by a clinical triad: (1) CONTRACTURE of the elbows, ACHILLES TENDON, and posterior cervical muscles; (2) slowly progressive muscle atrophy and weakness; and (3) a cardiomyopathy usually presenting as an atrioventricular block (HEART BLOCK). The weakness initially affects the upper arm and chest muscles, followed by pelvic and leg muscle paresis. Sudden death (DEATH, SUDDEN, CARDIAC) may occur as the result of the cardiac conduction defect. (Adams et al., Principles of Neurology, 6th ed, p1419; Curr Opin Neurol 1997;10 (5):421-425)
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ferdinand gregorovius
- A German historian and poet; born in Neidenburg, East Prussia, Jan. 19, 1821; died at Munich, May 1, 1891. He studied severely Konigsberg home, wrote essays of deep scholarship; "Socialistic Elements Goethe's Wilhelm Meister"; tragedy, "The Death Tiberius", the ripest historical learning; "Corsica"; other most authoritative books travel description, based on close personal study. also "Euphorion", an epic, poems high repute. But his works, unsurpassed learning vivid realization spirit their times, are commanding monument genius. City Rome Middle Ages", "Lucretia Borgia", "Urban VIII"., Monuments Popes", "Athenais", need be named.