LOU GEHRIG DISEASE
\lˈuː d͡ʒˈe͡ɪɹɪɡ dɪzˈiːz], \lˈuː dʒˈeɪɹɪɡ dɪzˈiːz], \l_ˈuː dʒ_ˈeɪ_ɹ_ɪ_ɡ d_ɪ_z_ˈiː_z]\
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A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)
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